Neurology Minutiae

Monday, March 16, 2009

UPDRS

Hoehn and Yahr Staging of Parkinson's Disease

1. Stage One
1. Signs and symptoms on one side only
2. Symptoms mild
3. Symptoms inconvenient but not disabling
4. Usually presents with tremor of one limb
5. Friends have noticed changes in posture, locomotion and facial expression

2. Stage Two
1. Symptoms are bilateral
2. Minimal disability
3. Posture and gait affected

3. Stage Three
1. Significant slowing of body movements
2. Early impairment of equilibrium on walking or standing
3. Generalized dysfunction that is moderately severe

4. Stage Four
1. Severe symptoms
2. Can still walk to a limited extent
3. Rigidity and bradykinesia
4. No longer able to live alone
5. Tremor may be less than earlier stages

5. Stage Five
1. Cachectic stage
2. Invalidism complete
3. Cannot stand or walk
4. Requires constant nursing care

This rating system has been largely supplanted by the Unified Parkinson's Disease Rating Scale, which is much more complicated.

Unified Parkinson's Disease Rating Scale (UPDRS)

The UPDRS is a rating tool to follow the longitudinal course of Parkinson's Disease. It is made up of the 1) Mentation, Behavior, and Mood, 2) ADL and 3) Motor sections. These are evaluated by interview. Some sections require multiple grades assigned to each extremity. A total of 199 points are possible. 199 represents the worst (total) disability), 0--no disability.

I. Mentation, Behavior, Mood

Intellectual Impairment
0-none
1-mild (consistent forgetfulness with partial recollection of events with no other difficulties)
2-moderate memory loss with disorientation and moderate difficulty handling complex problems
3-severe memory loss with disorientation to time and often place, severe impairment with problems
4-severe memory loss with orientation only to person, unable to make judgments or solve problems

Thought Disorder
0-none
1-vivid dreaming
2-"benign" hallucination with insight retained
3-occasional to frequent hallucination or delusions without insight, could interfere with daily activities
4-persistent hallucination, delusions, or florid psychosis.

Depression
0-not present
1-periods of sadness or guilt greater than normal, never sustained for more than a few days or a week
2-sustained depression for >1 week
3-vegetative symptoms (insomnia, anorexia, abulia, weight loss)
4-vegetative symptoms with suicidality

Motivation/Initiative
0-normal
1-less of assertive, more passive
2-loss of initiative or disinterest in elective activities
3-loss of initiative or disinterest in day to say (routine) activities
4-withdrawn, complete loss of motivation

II. Activities of Daily Living

Speech
0-normal
1-mildly affected, no difficulty being understood
2-moderately affected, may be asked to repeat
3-severely affected, frequently asked to repeat
4-unintelligible most of time

Salivation
0-normal
1-slight but noticeable increase, may have nighttime drooling
2-moderately excessive saliva, hay minimal drooling
3-marked drooling

Swallowing
0-normal
1-rare choking
2-occasional choking
3-requires soft food
4-requires NG tube or G-tube

Handwriting
0-normal
1-slightly small or slow
2-all words small but legible
3-severely affected, not all words legible
4-majority illegible

Cutting Food/Handing Utensils
0-normal
1-somewhat slow and clumsy but no help needed
2-can cut most foods, some help needed
3-food must be cut, but can feed self
4-needs to be fed

Dressing
0-normal
1-somewhat slow, no help needed
2-occasional help with buttons or arms in sleeves
3-considerable help required but can do something alone
4-helpless

Hygiene
0-normal
1-somewhat slow but no help needed
2-needs help with shower or bath or very slow in hygienic care
3-requires assistance for washing, brushing teeth, going to bathroom
4-helpless

Turning in Bed/ Adjusting Bed Clothes
0-normal
1-somewhat slow no help needed
2-can turn alone or adjust sheets but with great difficulty
3-san initiate but not turn or adjust alone
4-helpless

Falling-Unrelated to Freezing
0-none
1-rare falls
2-occasional, less than one per day
3-average of once per day
4->1 per day

Freezing When Walking
0-normal
1-rare, may have start hesitation
2-occasional falls from freezing
3-frequent freezing, occasional falls
4-frequent falls from freezing

Walking
0-normal
1-mild difficulty, day drag legs or decrease arm swing
2-moderate difficultly requires no assist
3-severe disturbance requires assistance
4-cannot walk at all even with assist

Tremor
0-absent
1-slight and infrequent, not bothersome to patient
2-moderate, bothersome to patient
3-severe, interfere with many activities
4-marked, interferes with many activities

Sensory Complaints Related to Parkinsonism
0-none
1-occasionally has numbness, tingling, and mild aching
2-frequent, but not distressing
3-frequent painful sensation
4-excruciating pain

III. Motor Exam

Speech
0-normal
1-slight loss of expression, diction,volume
2-monotone, slurred but understandable, mod. impaired
3-marked impairment, difficult to understand
4-unintelligible

Facial Expression
0-Normal
1-slight hypomymia, could be poker face
2-slight but definite abnormal diminution in expression
3-mod. hypomimia, lips parted some of time
4-masked or fixed face, lips parted 1/4 of inch or more with complete loss of expression

*Tremor at Rest
Face
0-absent
1-slight and infrequent
2-mild and present most of time
3-moderate and present most of time
4-marked and present most of time

Right Upper Extremity (RUE)
0-absent
1-slight and infrequent
2-mild and present most of time
3-moderate and present most of time
4-marked and present most of time

LUE
0-absent
1-slight and infrequent
2-mild and present most of time
3-moderate and present most of time
4-marked and present most of time

RLE
0-absent
1-slight and infrequent
2-mild and present most of time
3-moderate and present most of time
4-marked and present most of time

LLE
0-absent
1-slight and infrequent
2-mild and present most of time
3-moderate and present most of time
4-marked and present most of time

*Action or Postural Tremor

RUE
0-absent
1-slight, present with action
2-moderate, present with action
3-moderate present with action and posture holding
4-marked, interferes with feeding

LUE
0-absent
1-slight, present with action
2-moderate, present with action
3-moderate present with action and posture holding
4-marked, interferes with feeding

*Rigidity

Neck
0-absent
1-slight or only with activation
2-mild/moderate
3-marked, full range of motion
4-severe

RUE
0-absent
1-slight or only with activation
2-mild/moderate
3-marked, full range of motion
4-severe

LUE
0-absent
1-slight or only with activation
2-mild/moderate
3-marked, full range of motion
4-severe

RLE
0-absent
1-slight or only with activation
2-mild/moderate
3-marked, full range of motion
4-severe

LLE
0-absent
1-slight or only with activation
2-mild/moderate
3-marked, full range of motion
4-severe

*Finger taps

Right
0-normal
1-mild slowing, and/or reduction in amp.
2-moderate impaired. Definite and early fatiguing, may have occasional arrests
3-severely impaired. Frequent hesitations and arrests.
4-can barely perform

Left
0-normal
1-mild slowing, and/or reduction in amp.
2-moderate impaired. Definite and early fatiguing, may have occasional arrests
3-severely impaired. Frequent hesitations and arrests.
4-can barely perform

*Hand Movements (open and close hands in rapid succession)

Right
0-normal
1-mild slowing, and/or reduction in amp.
2-moderate impaired. Definite and early fatiguing, may have occasional arrests
3-severely impaired. Frequent hesitations and arrests.
4-can barely perform

Left
0-normal
1-mild slowing, and/or reduction in amp.
2-moderate impaired. Definite and early fatiguing, may have occasional arrests
3-severely impaired. Frequent hesitations and arrests.
4-can barely perform

*Rapid Alternating Movements (pronate and supinate hands)

Right
0-normal
1-mild slowing, and/or reduction in amp.
2-moderate impaired. Definite and early fatiguing, may have occasional arrests
3-severely impaired. Frequent hesitations and arrests.
4-can barely perform

Left
0-normal
1-mild slowing, and/or reduction in amp.
2-moderate impaired. Definite and early fatiguing, may have occasional arrests
3-severely impaired. Frequent hesitations and arrests.
4-can barely perform

*Leg Agility (tap heel on ground, amp should be 3 inches)

Right
0-normal
1-mild slowing, and/or reduction in amp.
2-moderate impaired. Definite and early fatiguing, may have occasional arrests
3-severely impaired. Frequent hesitations and arrests.
4-can barely perform

Left
0-normal
1-mild slowing, and/or reduction in amp.
2-moderate impaired. Definite and early fatiguing, may have occasional arrests
3-severely impaired. Frequent hesitations and arrests.
4-can barely perform

*Arising From Chair (pt. arises with arms folded across chest)
0-normal
1-slow, may need more than one attempt
2-pushes self up from arms or seat
3-tends to fall back, may need multiple tries but can arise without assistance
4-unable to arise without help

*Posture
0-normal erect
1-slightly stooped, could be normal for older person
2-definitely abnormal, mod. stooped, may lean to one side
3-severely stooped with kyphosis
4-marked flexion with extreme abnormality of posture

*Gait
0-normal
1-walks slowly, may shuffle with short steps, no festination or propulsion
2-walks with difficulty, little or no assistance, some festination, short steps or propulsion
3-severe disturbance, frequent assistance
4-cannot walk

*Postural Stability (retropulsion test)
0-normal
1-recovers unaided
2-would fall if not caught
3-falls spontaneously
4-unable to stand

*Body Bradykinesia/ Hypokinesia
0-none
1-minimal slowness, could be normal, deliberate character
2-mild slowness and poverty of movement, definitely abnormal, or dec. amp. of movement
3-moderate slowness, poverty, or small amplitude
4-marked slowness, poverty, or amplitude

Schwab and England Activities of Daily Living

Gillingham FJ, Donaldson MC, eds., Third Symp. of Parkinson's Disease, Edinburgh, Scotland, E&S Livingstone, 1969, pp.152-7.

Rating can be assigned by rater or by patient.

* 100%-Completely independent. Able to do all chores w/o slowness, difficulty, or impairment.

* 90%-Completely independent. Able to do all chores with some slowness, difficulty, or impairment. May take twice as long.

* 80%-Independent in most chores. Takes twice as long. Conscious of difficulty and slowing

* 70%-Not completely independent. More difficulty with chores. 3 to 4X along on chores for some. May take large part of day for chores.

* 60%-Some dependency. Can do most chores, but very slowly and with much effort. Errors, some impossible

* 50%-More dependant. Help with 1/2 of chores. Difficulty with everything

* 40%-Very dependant. Can assist with all chores but few alone

* 30%-With effort, now and then does a few chores alone of begins alone. Much help needed

* 20%-Nothing alone. Can do some slight help with some chores. Severe invalid

* 10%-Totally dependant, helpless

* 0%-Vegetative functions such as swallowing, bladder and bowel function are not functioning. Bedridden.

Disclaimer: The information and reference materials contained herein is intended solely for the information of the reader. It should not be used for treatment purposes, but rather for discussion with the patient's own physician.

A Good Credit Score is 700 or Above. See yours in just 2 easy steps!

ALS Functional Rating Scale

http://www.alsconnection.com/ALSFRS.asp

ALSFRS
Instructions for completing the ALSFRS-R (ALS Functional Rating Scale)
A. Comparisons are made with the patient's status prior to the onset of the disease, not with status at the last visit.B. Patient's response (on a 5 point scale) is recorded in relation to the question "How are you doing at (...)? for each of the 12 functions listed in the ALSFRS-R
SPEECH
4. Normal speech processes
3. Detectable speech disturbance
2. intelligible with repearing
1. speech combined with non-vocal communication
0. loss of useful speech
SALIVATION
4. Normal
3. slight but definite excess of saliva in mouth, may have nighttime drooling
2. moderately excessive saliva, may have minimal drooling
1. marked excess of saliva with some drooling
0. marked drooling, requires constant tissue
SWALLOWING
4. Normal eating habits
3. early eating problems, occasional choking
2. dietary consistency changes
1. needs supplemental tube feedings
0. NPO (exclusively parental or enteral feedings)
HANDWRITING
4. Normal
3. slow or sloppy, all workds legible
2. not all words legible
1. able to grip pen, unable to write
0. unable to grip pen
CUTTING FOOD AND HANDLING UTENSILS(patients without gastrostomy)
4. Normal
3. somewhat slow and clumsy, needs no help
2. can cut most foods, slow of clumsy, some help needed
1. foods cut by someone else, can still feed slowly
0. needs to be fed
CUTTING FOOD AND HANDLING UTENSILS(patients with gastrostomy)
4. Normal
3. clumsy, able to perform all manipulations
2. some help needed with clsures and fasteners
1. provides minimal assistance to caregiver
0. unable to perform any aspect of task
DRESSING AND HYGEINE
4. Normal
3. independent self care with effort of decreased effieicency
2. intermittent assitance or substitute methods
1. needs attendant for self care
0. total dependence
TURNING IN BED AND ADJUSTING BEDCLOTHES
4. Normal
3. somewhat slow or clumbsy, needs no help
2. can turn alone or adjust sheets with great difficulty
1. can initiate, cannot turn or adjust sheets
0. helpless
WALKING
4. Normal
3. early ambulation difficulties
2. walks with assistance
1. non-ambulatory functional movement only
0. no purposeful leg movement
CLIMBING STAIRS
4. Normal
3. slow
2. mild unsteadiness or fatigue
1. needs assistance
0. cannot do
DYSPENA
4. None
3. occurs when walking
2. occurs with one more more:eating, bathing, dressing
1. occurs at rest, either sitting or lying
0. significant difficulty, considering mechanical support
ORTHOPNEA
4. None
3. some difficulty sleeeping, d/t shortness of breath, does not routinely use >2 pillows
2. needs extra pillows to sleep (>2)
1. can only sleep sitting up
0. unable to sleep
RESPIRATORY INSUFFICIENCY
4. None
3. intermittent use of BiPAP
2. continuous use of BiPAP at night
1. continuous use of BiPAP day and night
0. invasive mechanincal ventilation by intubation/trach

Sunday, March 15, 2009

Childhood Myasthenia Gravis Pearls

1. Half of patients may be seronegative, but of those who have disease the treatment in many cases is similar to treatment of adult disease
2. Steroid, PE and IVIG all have been used safely during pregnancy. CI's should not be overused due to possibility of causing uterine contractions. Regional anesthesia or C section is preferred. Avoid Magnesium sulfate.
3. The slow channel congenital myasthenic syndrome (SCCMS) appears in later childhood or adolescence. It is associated with relatives with various adult onset MG subtypes. It is inherited as autosomal dominant. Neck flexor weakness, progressive myopathy, and failure to respond to CI's are common, as are skeletal deformities.
4. Pupillary hyporeflexia is relatively specific for congenital ach deficiency. Progressive myopathy occurs in it and SCCMS, as does non response to CI's. Congenital Ach deficiency also is associated with basal lamina on muscle surface.
5. Dok 7 mutation is associated with limb girdle myasthenia.
6. Fast channel syndrome occurs in infancy and early childhood and presents similarly to other conditions. it responds well to CI's and 3,4 DAP.

Features of anti MuSK positive ab in MG (myasthenia gravis)

The presentation may be atypical with severe bulbar, axial, and respiratory weakness, with relative sparing of extraocular muscles. Patients may get worse paradoxically with Mestinon, may have an atypical or myopathic EMG, and respond, sometimes dramatically to aggressive immunomodulation eg. plasmapheresis, MMF steroids and Rituxan

Dosing of anticholinesterase drugs

from Continuum 2009

Mestinon (pyridostigmine bromide).
Oral dose 30-50 mg q 4-6 hours
Intramuscular dose 2.0 mg q 4-6 hours
Intravenous dose 0.7 mg q 4-6 hours
pediatric oral dose 1.0 mg/kg to 7.0 mg/kg q 4-6 hours

Neostigmine (prostigmine)
oral dose 15 mg q 4-6 hours
intramuscular dose 1.5 mg (methylsulfate) q 4-6 hours
Intravenous dose 0.5 mg (methylsulfate) q 4-6 hours
pediatric 0.3 mg/kg in divided doses 2-3 times per day

Ambenonium chloride (mytelase)
for patients allergic to or developing a rash to bromides
oral dose 7.5 mg in divided doses 3-4 times daily
pediatric oral dose .15 mg/kg up to a maximum of 1.5 mg/kg/d in divided doses

Contraindicated medication list in Myasthenia gravis

from Continuum 2009

1. Absolute contraindication-- curare, d-penicillamine, botulinum toxin, interferon alpha
2. Contraindicated
a. Antibiotics-- aminoglycosides (gentamycin, kanamycin, neomycin, streptomycin, tobramycine); macrolides (erythromycin, azithromycin (Z-pack), telithromycin, Biaxin)
Fluoroquinolones ( ciprofloxacin, norfloxacin, levofloxacin);
b. quinine, quinidine, procainamide,
c. magnesium salts, iv magnesium replacement.
3. Caution- may exacerbate weakness in some myasthenics
a. Calcium channel blockers
b. Beta blockers
c. Lithium
d. Statins
e. Iodinated contrast agents

article on public domain
http://www.ispub.com/journal/the-internet-journal-of-neurology/volume-10-number-2/drugs-which-may-exacerbate-or-induce-myasthenia-gravis-a-clinician-s-guide.html

link to article by Pascuzzi discussing contraindicated drugs in more detail
http://myasthenia.org/LinkClick.aspx?fileticket=JuFvZPPq2vg%3d

Pearls on antibody testing in MG

1. AchR antibody is positive in 80-85 % of patients with MG, but only 55 % of pure ocular disease
2. Less than one percent of patients have pure blocking antibodies (others may have associated with binding antibodies) and so blocking antibodies are of little clinical use.
3. Modulating antibodies cross link the receptor and modulate their rate of degradation. It is most helpful as a test when the level of binding antibody is negative which is 3-4 %.
4. High levels of modulating antibodies, like anti striated muscle antibodies, occur in association with lymphoma, although can occur sporadically too especially in old.
5. MuSK antibody, important in clustering of receptors in NMJ, are positive in 40 % of AchR negative patients, only occassionally in those with pure ocular disease.

Differential diagnosis of head drop

1. ALS
2. GBS/CIDP
3. MG
4. IBM/DM/PM
5. PD/Parkinsonism
6. Isolated neck extensor myopathy
7. Congenital muscular dystrophy (mutations in LMNA or SEPN1 genes)
8. Congenital myopathy (nemaline)
9. Hypothyroidism
10. Syringomyelia
11. Post mantle irradiation

Differential diagnosis of dysarthria and dysphagia

from Continuum 2009
1. ALS
2. MG
3. Stroke
4. Syringomyelia
5. Kennedy syndrome
6. GBS/CIDP
7. Myopathy/botulism

Pearls
1. Dysarthia of MG is nasal, with slurred , nasal but not spastic speech, weak tongue movements but not arduous movements of tongue. In ALS, speech is slow and effortful, has a spastic quality as well, slow and effortful speech with a strangled quality. In ALS initial weakness may be described as a tickle in throat or inability to clear mucus , which is not typical of MG. MG patients may have nasal regurgitation, trouble with food not going down or getting stuck. Choking or weight loss also are typical of ALS.

Differential diagnosis of tongue fasciculations

Continuum p21 2009

1. Lower motor neuron disease (ALS, Kennedy disease, SMA, poliomyelitis
2. Muscle specific receptor tyrosine kinase, MG
3. Brainstem lesion
4. Base of skull tumor
5. Radiation in area of skull base
6. Unilateral hypoglossal neuropathy
7. organophosphates

Pearl tongue weakness is as useful as tongue fasciculations. Test by asking patient to move tongue against cheek on each side and hold against resistance. If the disease is UMN only, this may be only sign seen on tongue. Advanced ALS patients may be unable to move their tongues at all.

Tuesday, March 10, 2009

Ptosis as presenting sign of levator palpebrae myositis

Neuroimages. Neurology 2008; 71:1202

A 45 year old surgeon presented with 2 days of eye pain and ptosis, isolated. The key study was an enhanced MRI orbits, with fat suppression sequences, that showed enhancement of the right levator palpebrae. Corticosteroids are usually effective but were not required in this patient who resolved spontaneously.

British Medical Journal (BMJ) lies, goes on attack

Ordinarily, we do not mix medicine and politics on this site. However, we do believe in academic freedom and honesty, and fear that an attack on any is an attack on the rights of all that will ultimately end badly. While we do hold views on truth in the Middle East conflicts, these thoughts are not germane to this article. Rather, we have a major issue with a medical journal leaving science, entering politics, where it does not belong, committing gross errors of fact, and then attacking the organization that pointed out the errors. While arrogance is not in short supply at the BMJ, a commitment to accuracy and truth is deficient in this case. We feel we have the obligation to report the BMJ mistakes so that our readers can keep a cool head when digesting what they read.

Honestreporting.com, a watchdog news agency that checks media facts, reports that the BMJ devotes five articles (1, 2, 3, 4, 5) in a recent edition to reviewing the "perils of criticizing Israel." "Chief amongst these" is Karl Sabbagh's analysis of hundreds of e-mails sent to the BMJ in response to an article published way back in 2004. According to Sabbagh, most of the hostile emails resulted "from a request from HonestReporting, a website operated from the United States and Israel." Also writing on this topic in the BMJ, Jonathan Freedland even admits that Derek Summerfield (the author of the 2004 piece)made a "mistake to open his piece with a clear error, one that inevitably made his essay appear tendentious." So why is the BMJ so defensive towards those who pointed out this admitted error?

HR wonders whether the BMJ's shot at a shadowy and highly effective "Israel lobby" is designed not to inform but to make crticism of itself more difficult. "Needless to say," HR opines, "if an 'Israel lobby' was so influential over the media, there would be no need for HonestReporting to exist."

In the 2004 article, the BMJ, under the byline of Dr (?) Summerfield, stated that "The Israeli army, with utter impunity, haskilled more unarmed Palestinian civilians since September 2000than the number of people who died on September 11, 2001." Leaving aside the grotesqueness of the comparison, that is based on assumptions that are, to put a polite face on it, are incorrect, the numbers cited by the BMJ are themselves wrong. As HR noted, "The only actual similarity between the two is the death count ― approximately 3,000. Summerfield labels all Palestinian casualties 'unarmed civilians' ― denying the fact that (1) the clear majority of Palestinians who have died since September 2000 were terrorists and armed combatants (according to the Institute for Counter-Terrorism), and (2) no Palestinian civilian has been deliberately killed 'with impunity' ― in stark contrast to 9/11. "

As a physician, I have personally found arrogance much more dangerous in the care of patients than stupidity. Doctors who don't know something, can, after all, ask for help. However, the arrogant are left on an island with no idea how to undo harm that they have caused. Moreover, the best response to having made a mistake is to ADMIT the mistake and move on. The original article by Summerfield was not true, because more civilians died in the 9/11 attacks than in Palestinian territories during the cited period, by far, and the campaign to get the BMJ to retract the error was neither unprecedented nor inappropriate as alleged. If the BMJ wishes to become a political magazine instead of a medical journal, it will have to engage the ideas of nonphysicians who are interested in politics. Backtracking, writing even more stubborn and one sided articles, and accusing the watchdogs makes the BMJ look even more foolish than wrong.

The BMJ does have a storied history and reputation. Its a pity that its being sullied by rank journalists.

A Good Credit Score is 700 or Above. See yours in just 2 easy steps!

Sunday, March 08, 2009

Stiff person syndrome with ampiphysin antibodies: distinctive feature of a rare disease

Murinson BB, Guarnaccia JB. Neurology 2008; 71: 1955-58.

Principal form of SPS has stiffness of the spine and legs with spasms worse with emotional stress and triggers, and associated with anti GAD antibodies. Variants include less strong association with anti GAD AB, and limited stiff limb syndrome, and a progressive variant with encephalomyelitis, rigidity and myoclonus (PERM).

A distinct form with ampiphysin antibodies is a small subset (11/126 cases) and has the following distinctive features: association with breast cancer (10/11), female exclusively, mean age close to 58, association with other paraneoplastic antibodies and other neurologic disorders including sensory neuronopathy, encephalopathy, and myelopathy. Treatment may involve steroids, plasmapx or cancer treatment rather than IVIG as in anti GAD ab associated disease.

Clinically, all had stiffness and rigidity, half had pain, NONE had diabetes, EMG was positive for continuous motor activity or consistent with SPS. Nine were responsive to very high dose benzodiazepines (> 50 mg.day diazepam), none responsed to IVIG. Arm or neck involvement was specifically cited by referring physician in 80 % of the cases. Some patients responded completely to excision of the tumor.

Lab testing with immunocytochemisty is not sufficient, ELISA or RIA is needed and special labs are needed to test for this antibody.

Saturday, March 07, 2009

"staggers" or trematol poisoning

cf Loren Rolak, Neurology Secrets p. 410.

This disease suffered by Mary Lincoln, Abe's mother, was due to a toxin the white snakeroot plant which grows in dense woods and occurred when a woods was insufficiently cleared. It has not been described in over 60 years. Typically, cows would eat the snakeroot, and humans would drink the milk "milk sickness."

Presentation in Mrs. Lincoln included fatigue and stiffness, then whole body tremors, upper more than lower extremities, present at rest and resembling a shiver, leading over days to epigastric pain, nausea and vomiting. Eventually, lethargy, large pupils and hiccups occurred. She developed lethargy and large red tongue and eventually died.

Thursday, March 05, 2009

Predictors of recovery after postanoxic status epilepticus

Rosetti AO et al. Predictors of awakening from postanoxic status epilepticus after therapeutic hypothermia. Neurology 72; 744-749.

The authors describe 6 patients who woke and recovered. All recovering patients had preserved brainstem reflexes, reactive EEG background during PSE. Half had myoclonic and half NCSE. Age range 53-68.

Thursday, February 26, 2009

Nonviolent elaborate behaviors in REM sleep behavior disorder

Neurology 2009; 72:551-557
Authors hypothesized that nonviolent behaviors may be underestimated. Behaviors are nonstereotyped and complex. They include talking, laughing, shouting, swearing, yelling, crying, gesturing, grabbing, arm flailing, slapping punching, kicking, jerking, strangulating, thrashing, sitting up, leaping from bed, crawling and running.

Unpleasant, action filled aspects include being pursued or attacked, authors add chewing, feeding drinking, urinating defecating or performing a sexual act (masturbating), coitus like thrusting, thumbs up, flying and others. Authors suggest a release of learned, engraved programs.

Saturday, February 21, 2009

lupus associated vasculopathy with MMN and livedo reticularis

NEJM Case records 2009 Case 5 2009 A 47 year old woman with a rash and numbness and pain in the legs. 360:711-720

Also see post on livedo reticularis and neurologic disease: http://strokenotes.blogspot.com/2007/02/livedo-reticularis-and-neurologic.html

Case: 47 year old woman developed slowly progressive numbness then pain in LE's, swelling, mottled discoloration, swelling and blanching nodules. Rheum screen was normal. Biopsy of TA and nodule showed lymphistiocytic infiltrate but not vasculitis. family history positive for Sjogren's ( mother, with AI hepatitis) and "vasculitis" (sister). She atrophie blanche and cytopenias as well.

Teaching Points
1. MMN--with the other findings point to SLE, Sjogren's, APL syndrome, cutaneous PAN, or livedoid vasculopathy. Second tier ddx includes cryoglobulinemia, sarcoid, DM, amyloid, neoplasms and infections.

2. 50 % SLE patients have rash before meeting criteria for SLE. LRET and nodules is enough to count. Small vessel vasculitis characterized by palpable purpura is commonest, but medium size vasculitis with livedo reticularis also occurs. Patients with SLE and medium sized vasculitis have more MMN, visceral vasculitis, and ischemic cutaneous lesions, but less malar rash and discoid lesions than patients without medium sized vasculitis.

3. ANA negative SLE is rare since introduction of testing with HEp-2 cell substrate (10 %) and this patient initially had a FALSE negative ANA due to wrong method for test being performed by the commercial lab. Occassional patients who are ANA negative are anti Ro *SSA) positive or have secondary APL syndrome. Repeat testing showed positive 1:1280 ANA.

4. APL syndrome may be associated with thrombocytopenia and leuokopenia and be primary or secondary. Patient was initially negative, then positive.

5. Cutaneous PAN is challenging since there are no antibodies that are distinctive. Typically one sees elevation of acute phase reactants and anemia which is not the presentation here.

6. The patient's biopsy showed livedoid vasculopathy, she was treated with ASA and plaquenil with warfarin in reserve and she did well.

Wednesday, January 28, 2009

Styloid syncope

Neuroimages p 1649 Neurology71 2008
Patient had craniocervical pain and 30 years of syncope preceded by neck flexion and brief vertigo. 3D CT showed hypertrophied styloid process.

Eagle syndrome is characterized by a long SP with craniocervical or throat pain. Elongate SP occurs in 4 % of population. Syncope is due to compression of carotid artery. It should be considered in young patients with chronic throat pain and syncope provoked by neck flexion. It is diagnosed with 3d Ct and cured with styloidectomy.

foreign language ictal speech automatisms

Neurology 2008; 71: 1579-1585
Most cases reflect right TLE. However, FLISA occur with spread to temporal neocortex of frontal operculum. Authors suggest a male preponderance although this blogger's only personal observation is a woman.

Tuesday, January 20, 2009

Brain calcinosis syndrome

ref Baba et al. Heredofamilial brain calcinosis syndrome Mayo Clin Proc 2005;80:641-651
(a little dry for this blog)

List of causes:

80+% clinically benign
sporadic or familial I With abnormal calcium or magnesium metabolism
Hypoparathyroidism (idiopathic, postsurgical, external radiation, hypomagnesemia)
Pseudohypoparathyroidism (type II, idiopathic)
Hyperparathyroidism

II Without Ca/Mg abnormalities
Down's syndrome
Mitochondrial neuromyopathy (Kearns-Sayre syndrome, Pearson s.)
SLE
ALL
IgG kappa M proteinemia
Revesz syndrome
infectious (toxopl, mumps, EBV, CMV, HIV)
postanoxic
angiomatous malformations of the vein of Galen
Toxic (lead, CO)
Therapy induced ( XRT, anticancer drugs, mineralizing microangiopathy)
Without systemic involvement
Diffuse NFT with calcification
hyperkinetic mutism
idiopathic (Fahr's dis, striatopalidodentate calcinosis, bg calcification)
Aging

Genetic syndromes with chromosome identified (see reference for actual gene loci)

Familial isolated hypoparathyroidism
Autoimmune polyglandular syndrome
Pseudohypoparathyroidism types Ia and Ib
Aicardi syndrome (in first year of life, may see calcium in thalamus, cerebellum also)
Dihydropterine reductase syndrome (=PKU type 2 atypical form)-- may be arrested by folic
Cockayne syndrome
Krabbe
MELAS
Others (see text)

Tuesday, January 13, 2009

localizing signs with seizures

Clinical lateralizing signs are the phenomena which can unequivocally refer to the hemispheric onset of epileptic seizures. They can improve the localization of epileptogenic zone during presurgical evaluation, moreover, their presence can predict a success of surgical treatment. Primary sensory phenomena such as visual aura in one half of the field of vision or unilateral ictal somatosensory sensation always appear on the contralateral to the focus. Periictal unilateral headache, although it is an infrequent symptom, is usually an ipsilateral sign. Primary motor phenomena like epileptic clonic, tonic movements, the version of head ubiquitously appear contralateral to the epileptogenic zone. Very useful lateralization sign is the ictal hand-dystonia which lateralizes to the contralateral hemisphere in nearly 100%. The last clonus of the secondarily generalized tonic-clonic seizure lateralizes to the ipsilateral hemisphere in 85%. The fast component of ictal nystagmus appears in nearly 100% on the contralateral side of the epileptic focus. Vegetative symptoms during seizures arising from temporal lobe such as spitting, nausea, vomiting, urinary urge are typical for seizures originating from non-dominant (right) hemisphere. Ictal pallor and cold shivers are dominant hemispheric lateralization signs. Postictal unilateral nose wiping refers to the ipsilateral hemispheric focus compared to the wiping hand. Ictal or postictal aphasia refers to seizure arising from dominant hemisphere. Intelligable speech during complex partial seizures appears in non-dominant seizures. Automatism with preserved consciousness refers to the seizures of non-dominant temporal lobe.

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Wednesday, December 10, 2008

Features of microscopic polyangitis

Cited text from Nadeau's

Clinical Neurology chapter from 2000:
This recently recognized variant of PAN52 is defined by inflammation that is largely confined to smaller vessels than in PAN - arterioles, capillaries and postcapillary venules. Angiograms are correspondingly normal. Major organ infarction is rare, glomerulonephritis is universal, and there is a high frequency of pulmonary hemorrhage. MPA is considerably more common than classic PAN and it is a considerably more common cause of a pulmonary-renal syndrome than Goodpasture's syndrome. A forme-fruste of MPA is characterized exclusively by glomerulonephritis. Evidence of hepatitis B infection is usually absent. In contrast to classic PAN as well as such small vessel vasculitides as cryoglobulinemic vasculitis and Henoch-Schönlein purpura, there is little or no evidence of immune deposits. Fifty to 80% of patients with MPA have circulating ANCA - usually myeloperoxidase (perinuclear or p-ANCA)(see WG), a rare phenomenon in classic PAN. Some have antibodies to proteinase-3 (central or c-ANCA) as in WG. Clinical distinction from WG may be difficult. Polyneuropathy is present in only 10-20% of patients, in contrast to the 50% or greater frequency in classic PAN. Patients with MPA have a high early case-fatality rate due to pulmonary and renal failure and they are considerably more prone to relapse after treatment.

Friday, December 05, 2008

Causes of secondary headache to consider

head or neck trauma
dissection
CVT
Low pressure headache
neoplasm
neuroinfectious disease
medication overuse syndrome
systemic infection
acute glaucoma
other disorder of face sinuses
sleep apnea
fasting
somatization
occipital neuralgia

taken from NEJM 2008 359:21:2274

Chronic headaches notes

Case records of MGH nov 20 2008
chronic headaches, negative studies except dural enahncements. Pachymeningitis unlikely to be doe to Wegener's (which is ordinarily the most common cause) due to lack of pulmonary extran CNS signs and because of negative ANCA.

Sarcoid, Churg Strauss considered unlikely (former, no pulmonary findings, latter, normak eosiniphil count). RA has rare meningeal inflammation but this patient lacked arthritis even though RF positive. Sjogren's with dry mouth and eye irritation was considered, but Ro & La antibodies were negative as was a lip biopsy. Sjogren's does not explain pachymeningitis, DI, HA's or jaw claudication.

GCA is consistent with unrelenting headache, myalgia and weakness, PMR< pain in jaw with chewing (is specific but occurs only in 1/3 of patients with disease).

GCA was diagnosed by TA biopsy. Steroids were started with concomitant Calcium, Vitamin D, orla biphosphonate, and antiplatelet therapy, and eventually CPAP for sleep apnea associated with weight gain due to prednisone. They also gave trimethoprim to prevent PCP and PPI's.

Tuesday, November 11, 2008

PSP current nomenclature and types

"The postmortem room has become the temple of truth" (Donald Calne, re PSP) (Neurology 2008)

Current nomenclature "Richardson disease" corresponds with initial descriptions. There is a gradual onset of postural instability and falls within the first two years, with vertical supranuclear gaze palsy, a frontal dysexecutive syndrome, rigidity and bradykinesia that is not responsive to leveodopa, and a life expectancy of six years.

A second milder group at pm (post-mortem) have PSP tau pathology that is more restricted and less severe are called PSP-P (PSP-parkinsonism). They have assymmetric bradykinesia of the limbs, an initial response to levodopa, tremor and limb dystonia without early falls, eye movement problems, or cognitive dysfunction. Most patients with "atypical PSP" are in this category.

The third rarer category is pure akinesia with gait freezing (PAGF). There is gradual onset of unsteady or slow gait and hypophonia progressing to gair freezing and start hesitation, without limb rigidity or tremor. There is no response to levodopa and there is no dementia or opthalmoplegia in the first five years. In types 2 and 3 the median duration of the disease is around ten years.

Other patients with similar tau-PSP pathology present with corticobasal ganglionic degeneration, progressive nonfluent aphasia, or apraxia of speech.

Practice pearl: pay attention to SPEED of vertical saccades

References up to date: Williams DR, de Silva R, Pavour DC et al. Characteristics of two distinct clinical phenotypes in pathologically proven progressive supranuclear palsy: Richardson' syndrome and PSP - parkinsonism. Brain 2005; 128:1247-1258. Williams DR, Holton JL, Strand C. et al. Pathological tau burden and distribution distinguishes progressive supranuclear palsy-parkinsonism from Richardson's syndrome. Brain 2007; 130: 1566-1576. Mizusawa H, Mochizuki A , Ohkoshi N, et al. Progressive supranuclear palsy presenting with pure akinesia. Adv Neurol 1993; 60: 618-621. Josephs KA, Duffy JR, Strand EA et al. Clinicopathological and imaging correlates of progressive aphasia and apraxia of speech. Brain 2006; 129: April 13. Tsuboi Y, Josephs KA, Boeve BF et al. Increased tau burden in the cortices of progressive supranuclear palsy presenting with corticobasal syndrome. Mov Disord 2005; 20: 982-988.
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Tuesday, November 04, 2008

Horners syndrome pearls

Note-skipping standard textbook items, this is a(my) blog and I am ONLY including minutiae that I want to remember later and am purposely omitting much common information about horner's that is already well known to me. This post is based on H Wilhelm, "The Pupil" Curr Opin Neurol 21:36-42 2008.

1. Many cases of Horners after evaluation remain idiopathic.
2. Iatrogenic Horners after subclavian/jugular venous puncture is fairly common and well reported
3. Carotid dissection is associated with 25 or 37 % Horners s., and the key clinical pearl is the association of PAIN and acuity. 15 % have a bad outcome so beware of this diagnosis.
4. Beware of VI paresis and Horners which usually localizes to cavernous sinus and indicates a mass lesion there.
5. Previously unknown mediastinal tumors are RARE. Only two percent of bronchogenic tumors are Pancoast tumors . Other tumors that RARELY present are thyroid (usually benign goiter but could be CA) and nasopharyngeal CA.
6. In children neuroblastoma is fairly common and needs to be ruled out. rhabdomyosarcomas and gangliogliomas also are reported in this population.
7. Cocaine testing in texts is difficult to the trouble getting cocaine drops. Apraclonidine can be an effective substitute. Like cocaine, the pupil is only denervated in the third neuron in the arc and only such third neuron lesions dilate with apraclonidine. It does reverse anisocoria in such cases. The jury is truly not back in for apraclonidine. More studies are indicated.
8. To differentiate meiosis in Horners from physiologic anisocoria, check time to dilatation in darkness. Infrared video can be used if available. Normal pupils start to filate by .5-1 s and reach maximal dilatation by 5 s, whereas Horner pupils reach maximal dilatation by 10 seconds. Its only sensitive if repeated about four times; if only done once, it will most likely be false negative, if done four times, is 83 % sensitive.

More facts about Horner's
9. Reported after chest tube insertion
10. reported after cervical block/epidural
11. Klumpke's paralysis, goiter,MS sympathectomy, chiari, lateral medullary infarct,acute otitis, mandibular abscess, neuroblastoma,and cervical rib are other causes
12. Signs may include loss of ciliospinal reflex, heterochromia (iris)
13. In animals leashes if too tight can cause Horner's syndrome
14. Drugs are overlooked cause esp drugs that affect DA levels
14. Sweating pattern analyzed by Morris lee and Lim:
The distribution of sweating on the face was studied in 31 patients with Horner’s syndrome. In patients whose lesion was known to be distal to the bifurcation of the common carotid artery impairment of sweating was confined to the medial aspect of the forehead and side of the nose. In more proximal lesions loss of sweating involved the whole of one side of the face. Facial sweating was normal in 6 patients with avulsion injuries of the brachial plexus and in 2 patients with a lateral meduliary syndrome. These findings suggest that the pattern of sweating in Horner's syndrome may be useful in some patients in localizing the site of the lesion. (Brain, 1984)

Oculomotor syndromes PEARLS

cf Tilikete C, Pelisson D. Oculomotor syndromes of the brainstem and cerebellum. Curr Opin Neurol 21:22-28 2008

Head shaking nystagmus (hsn)- usually is a peripheral vestibular lesion. However it can occur in Wallenberg syndrome.

In central positional nystagmus, Dix Hallpike testing induces downbeat nystagmus(DBN). DBN also occurs in floccular disturbance and the gravity dependent component can be suppressed with 3,4 aminopyridine. Other oddball causes of DBN include West Nile virus encephalomyelitis, intrathecal morphine, a particular genetic syndrome of cerebellar ataxia, or another syndrome combining DBN with motor neuronopathy and cerebellar ataxia.

Saturday, November 01, 2008

Abnormalities (hemorrhages etc.) conjunctiva-causes

1.Subconjunctival hemorrhages-- common in trauma, rare in SAH and severe HTN
2. Leptospirosis-- injection of conjunctiva plus meningitis and myopathy
3. Filarial migratory phase of loa-loa-- injection is seen
4. telangiectasia in conjunctiva seen in sickle cell disease and ataxia telangiectasia
5. Retroorbital tumors can produce injection
6. Renal failure can produce severe conjunctival injection

Hypertelorism-- list of causes

1. normal
2. congenital absent callosum
3. Aicardi's syndrome
4. Schapiro's syndrome ( hypothermia and other congenital defects)
5. Septo-optic dysplasia

Friday, October 31, 2008

More pearls of fundoscopic exam temporal pallor and atrophy

Pallor is noted in any process affecting maculopapillary bundle. The disk is alabaster white and bound by a gray white cup. Green light of the opthalmoscope shows the fibers as they enter the disk.

Optic atrophy has seven major features: 1) pale aspirin-white disc. 2) sharp margins 3) loss of lamina cribosa 4) increased cup to disc ratio 5) decreased arterioles off disc (less than 14) 6) small arteries and 7) gray pale retina.

Swelling has seven features 1) Loss of disk margin, superior then inferior then temporal Always blurred nasal margins 2) Fat veins without pulsations 3) Erythema off the disk 4) loss of lamina cribosa, whole disc pushed forward 5) Slit hemorrhages off disc margin 6) engorged veins appear and disappear near macula 7) Folds in retina spread towards the macula. Last normal acuity.

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Neuroopthalmology testing-- bedside pearls

Riddoch's phenomenon-- patient won't see an object in a damaged field unless it is moved.

shading of a visual field-- if larger objects are seen in a damaged field where smaller objects are missed, , suggests defect is partly caused by edema or pressure phenomenon.

Bjerrum screen (tangent screen) enlarges central meridian to 30 degrees and is most helpful for measuring central scotomata and the blind spot.

Graying of vision (finger does not appear flesh colored) or red desaturation (eg. red pin ) is appreciated before a quantifiable field defect.

Notes bitemporal upper quadrantic defect suggests chiasmal compression from above, but bitemporal lower field defect suggests compression from below.

Arcuate defect- optic nerve lesion prechiasmatic.

Bitemporal scotomata-- early bitemporal field defect or consider bilateral optic nerve lesions eg in kids.

Fundoscopic exam
Venous pulsations are seen only in the middle and not the margins of the disk unless there is a large pulse pressure such as in aortic insufficiency. severe hyperthyroidism or arteriovenous fistula.

Miscellaneous findings include commotio retina, an intense light streak seen with acute head injury of Kohlmeyer-Degos disease (arteritis with atrophic skin lesions).

Hemorrhages from papilledema occur off the disk margin (slit hemorrhages) whereas those from venous occlusion occur in the central retina and macula.

Torsten's syndrome is a hemorrhage that moves with head position following a burst aneurysm, also called preretinal or subhyaloid hemorrhage. Often can identify side of hemorrhage based on.

Lupus patients may have "grains of rice" or cytoid bodies in peripheral retina.

Renal patients may have a macular star (edema outlining the nerve sheath layer).

Hollenhorst plaque or branch point occlusion of cholesterol emboli are larger than occluded vessel birefringent and yellow.

Platelet fibrin emboli from HIT are white and multiple.

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Schwartzmann 2 second mental status exam

The tests below are not thorough or well vetted, but they can be done easily at grand rounds, appear novel to the students, and are explained in Robert Schwartzman's book on the neurological examination.

He tests judgment ("What would you do if you found a letter addressed to someone else?); visual praxis (copy a hand posture after seeing it for two seconds); language/memory/frontal four part oral command, and frontal face hand test (touches patient's hand and own face and asks where am I touching you).

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Monday, October 27, 2008

Transient visual loss

see also the following behavioral neurology post http://behavioralneurologynotes.blogspot.com/2008/05/hallucinations-and-related-conditions.html

Consider: noninschemic
1. opthalmic surface disorders

a. tear film abnormalities-- visual blurring occurs many times a week often at the end of the day and under one environmental circumstance. Blinking and lubricating eye helps. Patients with blepharitis may be worse in the morning due to accumulated meibomian gland secretion overnight. With dry eyes causing epithelial breakdown, there may be foreign body sensation, pain or redness with a positive Schirmer's test. Patients with rosacea frequently have opthalmic involvement. Also PD.

2. Other ocular causes-- narrow angle glaucoma causes transient blurring with halos. Recurrent spontaneous anterior chamber hemorrhages is such patients needs to be differentiated from those occurring in juvenile xanthogranuloma, malpositioned lens with iris contact, and iris neovascularization.

Patients with corneal dysfunction have episodes of blurred vision that last for hours especially in the mornings, must examine during an episode. Episodes in patients with diabetes due to hyper or hypoglycemia occur.

Ischemic Causes
Causes-- 60 % are due to carotid stenosis/occlusion. Also called TMB, amaurosis fugax, or retinal tia. In usual cases , emboli from carotid are common but low flow also occurs when collaterals from the Circle of Willis and eca are compromised. In these cases symptoms may be inducible by change in position or eating, or when distal flow is inadequate such as chronic ocular ischemia or bright light amaurosis. The latter occurs with TMB after sunlight or looking at a white wall, in the setting of carotid occlusion and is a well known phenomenon.
Risk of stroke per annum is 2 % with 1 % risk of permanent visual loss, v. 5-8 % risk of stroke with hemispheric tia's.

Symptoms are abrupt onset, painless, lasts 1-5 minutes, darkening or fogging (not blurring) of visual field, altitudinal pattern (shade closing) of vision loss, and return of vision over minutes.

Vision loss may be altitudinal, peripheral, central or even vertical. A nasal field defect may suggest emboli due to lodging in temporal retinal circulation. Rarely, scintillating scotomas occur. Pain which is rare may suggest temporal arteritis.

Neuroopthalmic signs-- the most common are retinal emboli, including cholesterol (Hollenhorst) plaques which are refractile, metallic gold appearance and indicate carotid disease and platelet fibrin emboli which are creamy white grey longitudinal intravascular opacifications that fill the lumen, and indicate either carotid thrombosis or thrombosis with recent MI. Chronic severe carotid disease leads to chronic ocular ischemia including episcleral and conjunctival injection, corneal edema, and neovascular glaucoma. Venous stasis causes microaneurysms and blot hemorrhages. Unlike diabetes they are unilateral (ipsilateral to stenosis) and mid periphery instead of posterior pole. Rarely retinal calcific emboli occur in patients with cardiac valvular disease. They are grey white and ovoid and infarct the retina.

In papilledema, episodes of grey black and white vision lasting seconds may occur. Its fleeting and associated with changes in position. This also occurs in pseudopappilledema drusen or colobomas. Gaze evoked amaurosis suggests intraorbital lesion such as glioma, nerve sheath meningioma, or intraconal mass such as cavernous hemangioma. Vision deteriorates seconds after eccentric gaze and returns to normal with straight ahead gaze.

Vision loss lasting seconds
papilledema, IIH, optic disc drusen, optic nerve sheath meningioma (need MRI with contrast),
gaze evoked (orbital tumor suspect). Get T1 image in 3 planes to differentiate MRV clot from physiologic narrowing of vessel

Vision loss can occur due to ACA aneurysm due to leakage of blood into opthalmic sheath

Sunday, October 26, 2008

Patterns of functional visual loss

taken from Liu Volpe and Galetta text p. 388
TOTAL BLINDNESS
1. General observation of functionally blind-- may move into and bump objects without falling and hurting themselves; may not look at examiner speaking to them as a truly blind person might do; may wear sunglasses or mimic Stevie Wonder or other famously blind people.

2. Pupils-- should be abnormal except in cortically blind. A patient with total blindness and intact pupillary reflexes is likely to be functional. A complaint of photophobia with orbicularis oculi contraction to bright light is incompatible with an ocular cause of blindness.

3. OKN's- response is involuntary, but can be blocked by looking away from or beyond stimulus, or by excessive convergence.

4. Proprioceptive tests are performed by truly blind but not by functional. For example, looking at one's hand, bringing the tips of the fingers together from a distance, or signing one's name are better done by truly blind.

5. Mirror test-- a large mirror in front of a blind person, rocked, will produce an involuntary response if the patient is sighted and has better than hand motion vision..

6. Surprise-- atypical behaviors such as making faces, writing shocking words.

7. Evoked potentials-- a normal test can help, but an abnormal test can be ambiguous. My have in evoked fields, have characteristic hemifield loss from patient looking down.

UNILATERAL VISION LOSS
1. RAPD
2. Stereopsis-- requires binocular vision-- Titmus test
3. Placement of green lens should prevent visualization of Ishihara color plates

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Monday, October 13, 2008

IRIS Immune reconstitution syndrome in HIV

NEUROLOGY 2009;72:835-841

Patients with low CD4 counts initiating cART therapy who then bump their CD4 counts and lower viral counts are at risk. Patients may or may not have concurrent infection. Authors describe 7 patients, with Neuro IRIS.

Presentations include trouble walking, seizure, encephalopathy, and occurred in early phase-- first 2-3 weeks after initiation of therapy (early, due to memory cells) or delayed, after 4-6 weeks phase (due to proliferation of new T lymphocytes)/ Latter tended to be a more prolonged course. Biopsy of one patient showed lots of CD8 and CD40 cells stained positive. CSF showed pleocytosis, and MRI showed diffuse white matter changes with enhancement. Lamivudine was in all cases one of the therapies used. The overall risk was 0.9 % among patients starting cART, but 1.5% when stratified with those having low CD4 count to start (<200). This is much lower than the number of non neuro IRIS cases that have been considered to be as high as 15 % although these numbers may be high.

Pearls for Multiple System Atrophy

Neurologist July 2008 14L224-237 authors Bhidayasiri R, Ling H.

MSA encompasses sporadic OPCA (MSA-C) and SND (OPC-P) with predominant Parkinsonism and both types including dysautonomia. Tremor is common but not classic pill rolling tremor. Impotence in men and urge incontinence in women are nearly universal, postural hypotension occurs in 2/3 and syncopal episodes in 15 %. Stridor and respiratory insufficiency may be presenting signs. 29 % respond to levodopa AT SOME POINT.

Red flags suggestive of MSA include early severe autonomic dysfunction, spontaneous or L dopa induced orofacial dyskinesias, which may even resemble risus sardonica in tetanus. Pisa syndrome is a form of axial dystonia, and camptocormia is forward flexion of the trunk. These are not specific and may be seen in PD. Disproportionate anterocollis may occur at any point of the disease, although rare, and botox for this can worsen dysphagia; this is a red flag. So is minipolymyoclonus, which is postural or stimulus sensitive small amplitude nonrhythmic movements of a few fingers or the whole hand. Dysarthria may be diagnostic, with a quivering, croaky strained element "reminiscent of myoclonic speech." (cit Arch Neurol 1996 53:545-548). Nighttime stridor is helpful diagnostically but poor prognostically and may present as laryngeal paresis. Sleep apnea, the "cold hand sign" and emotional incontinence comprise the other red flags.

groupings of "red flags: show six categories early instability, rapid progression, abnormal postures, bulbar dysfunction, respiratory dysfunction, emotional incontinence. Presence of two or more categories had high sensitivity (84 % ) and specificity (98%). see Hughes et al. JNNP 1992; 55:181-184.

Exclusion criteria also are important, including age under 30, positive family history of MSA (PD is "OK"), frank dementia at onset, and eye movement abnormalities suggestive of PSP or CBGD.

The Quinn criteria for possible, probable and definite MSA are reviewed. International Consensus Conference Criteria of the AAN are considered better. See NEJM 2004; 351:912-924 (Case Records of the MGH).

MRI signs include atrophy of the cerebellar vermis, ponsmiddle cerebellar peduncles and lower brainstem. Signal change in the pons may resemble a "hot cross bun" (Catholics may understand this). In MSA-P, patients may have putamenal atrophy, with slit like void signal (black reflecting gliosis) which is sensitive but not specific, but is sensitive and specific if coupled with hyperintense T2 signal in the putamen. For a PD/MSA algorithm, see Arch Neurol 2002; 59:835-842). Onuf's nucleus degenerates which accounts for bladder problems. Pathologic confirmation is made by finding of alpah synuclein positive GCI's composed of misfolded alpah synuclein.

Pearls for treatment include, use of levodopa in many, the benefits of paxil 90 in one trial for motor function, botox of submandibular glands for drooling, and into adductor muscles for stridor, with concerns as above for other uses of botox. PT/OT/ST/ gait training and assistive devices are useful. Treatment of dysautonomic can include, if needed, avoiding large meal, alcohol, straining, certain meds; elastic stockings, bed tilt up at night. Drugs used for orthostasis include florinef, midodrine, desmopressin, octeotride, and eryuthorpoeitin. NGB can be treated with intermittent catheterisation, anticholinergics if detrusor hyperreflexia exists, suprapubic vibration devices, alpha adrenergic receptor antagonists, but NOT surgery. Beware of sildafenil for impotence due to hypotension. Macrogol 3350 for chronic constipation is safe and effective, increasing water content of stools. CPAP, may be needed, tracheostomy may be needed but also may be fatal due to hypercapnia. CPAP mat be contra-indicated if the epiglottis is floppy (Neurology 2011; 76:1841)

Thursday, October 09, 2008

periictal water drinking localizes as does kissing epilepsy

Peri-ictal water drinking lateralizes seizure onset to the nondominant temporal lobe

E. Trinka, MD, G. Walser, MD, I. Unterberger, MD, G. Luef, MD, T. Benke, MD, L. Bartha, PhD, M. Ortler, MD and G. Bauer, MD

From the Universitätskliniken für Neurologie, Innsbruck (Drs. Trinka, Walser, Unterberger, Luef, Benke, Bartha, and Bauer), and Universitätsklinik für Neurochirurgie (Dr. Ortler), Innsbruck, Austria.

Address correspondence and reprint requests to Eugen Trinka, MD, Universitätsklinik für Neurologie, Innsbruck, Anichstrasse 35, A-6020 Innsbruck, Austria; e-mail: eugen.trinka@uklibk.ac.at

The authors describe seven patients with medically refractorytemporal lobe epilepsy whose seizures were associated with peri-ictalwater drinking behavior. Presurgical evaluation, including video-EEGmonitoring, MRI, SPECT, and neuropsychological testing, revealeda seizure onset in the nondominant temporal lobe. All patientshad an excellent outcome after epilepsy surgery. Peri-ictalwater drinking may represent a lateralizing sign indicatingseizure onset in the nondominant temporal lobe.

^{AAN 2010 PO5:210}
^{Ictal kissing is a release phenomenon in nondominant temporal lobe epilepsy. 3 patients with ictal kissing were all righthanded women with longstanding right TLE. Its occurrence to environmental cues suggests its a release phenomenon rather than an ictal event}

ictal eye closure = pseudoseizures

Ictal eye closure is a reliable indicator for psychogenic nonepileptic seizures

Steve S. Chung, MD, Paula Gerber, MD and Kristin A. Kirlin, PhD

From the Departments of Neurology (S.S.C., P.G.) and Clinical Neuropsychology (K.A.K.), Barrow Neurological Institute, St. Joseph's Hospital and Medical Center, Phoenix, AZ.

Address correspondence and reprint requests to Dr. Steve S. Chung, Department of Neurology, 500 West Thomas Road, Suite 300, Phoenix, AZ 85013; e-mail: sschung@chw.edu

Using data from video-EEG monitoring, the authors studied whetherictal eye closure was a reliable indicator of psychogenic nonepilepticseizures (PNES). Among the 52 patients with PNES, 50 consistentlyclosed their eyes, while 152 of the 156 patients with epilepticseizures (ES) opened their eyes during seizures. These findingssuggest that ictal eye closure is a highly reliable indicatorfor PNES, while ictal eye opening is an indicator of ES.

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Monday, October 06, 2008

Photic seizures

"Pokemon incident" showed alternating red and blue light each for 12 hz caused seizures in japanese kids. 1 seizure per 10000 kids watching (as opposed to 1-3 % photosensitive) but more have sensitivity to other stimuli. Most such kids had no knowledge they had prior seizures. More females by percentage but more males by number, more young, usually generalized but sometimes focal. Brightness, esp over 100 lux; flash rate over 5-30, must go on for more than a half second and fill at least half the field, colors red flashing are the worst (advise blue sunglasses); especially moving vertical bars (stripes), and viewer factors such as covering an eye, tired, meds, exposure time. More TV seizures in Europe because there is higher flicker rate in Europe.

Posterior reversible leukoencephalopathy s (PRES) PEARLS

First described at Tufts University Department of Neurology, 1996, by Judy Hinchey, Claudia Chaves et al.

1. Associations:
cytotoxic drugs (steroids, mtx, tacrolimus, cyclosporine, imuran)
hypertension
glomerulonephritis
porphyria
pheochromocytoma
malignant hypertension
eclampsia
NMO (neuromyelitis optica or NMO spectrum disorder)
autoimmune disease
sepsis
TTP
multiple organ dysfunction especially renal
2. MRI pearls
--spares the calcarine cortex whereas stroke affects midline calcarine cortex
--may also affect frontal lobes, basal ganglia, brainstem, cerebellum
--no relationship between clinical severity, association, and location of lesions

3. treatment
remove drug
mag sulfate
control blood pressure

4 Review article Mayo Clin Proceedings May 2010 85:5: 427-432
120 cases in 113 patients over several years
-- may self resolve, but substantial Mortality and morbidity due to potential complications including massive infarction, hemorrhage, status epilepticus.
--hypertension present in 74 percent, but only 46 % of those had a history of chronic hypertension
--renal failure present in 57 %, but only half of those had chronic renal insufficiency
--clinical presentations included seizures in 74 % (partial or generalized, only rarely with history of seizures), 18 % with status epilepticus, 28 % with encephalopathy, 26 % with headaches, and 20 % with visual disturbances
-- Autoimmune disease was present in 45 %, of whom three fourths were women.These included TTP (27 %), SLE (18 %), hypothyroid (10 %), scleroderma (6%), Crohn's disease (6 %), ulcerative colitis or sclerosing cholangitis (4 %), RA (4%), DM ( 4%), and 1 patient each or 2 % for Graves' disease, Hashimoto's thyroiditis, APL syndrome, anti glomerular basement disease, autoimmune hepatitis, polyarteritis nodosa, thromboangitis obliterans, polyglandular autoimmune syndrome, Sjogren's, gfrnulomatous interstitial nephritis, NMO
-- Of patients with autoimmune disease, 65 % had cerebellar involvement, and majority of septic patients had involvement of the cerebrum.
-- transplant and chemo and pediatric populations not represented in above article

Saturday, September 27, 2008

Clinical Care of ALS patients

based on Radunovic Am Mitsumoto H, Leight PN Review article in Lancet Neurology October 2007. Based on the idea that basic medical management is underutilized. Major points:
1. Diagnosis (correct diagnosis) necessitates evaluation initially including brain and spine imaging (most of the time), CXR, lab tests, EMG. Use El Esocorial criteria http://www.wfnals.org/guidelines/1998elescorial/elescorial1998criteria.htm. Prior links to other important ALS information including EMG criteria, mimics and others. Dx is hardest with only UMN or only LMN signs. Mean survival is 30 months from diagnosis. Long survivors were not necessarily the ones taking riluzole. The ALS Functional Rating Scale http://www.outcomes-umassmed.org/als/alsscale.cfm and vital capacity are the most useful clinical measures.

Authors emphasize team approach and palliative care. Some of the treatments especially medicines are well known and obvious and are not reblogged. Some "tricks" are listed below.

excess saliva-- non medicine treatments include home suction, dark grape juice, sugar free citrus lozenges, nebulizers, botox, parotid radiation, steam inhalers. Medicines include elavil glycopyrrholate, hyoscine.

Excess broncial secretions-- propanolol, metoprolol, carbocysteine?, assisted cough insufflator/exsufflator. rehydration, pineapple or papaya juice, butter, decreased intake dairy products, alcohol and caffeine.

excess yawning-- baclofen

laryngospasm-- baclofen

emotional lability-- TCA's, Sinemet, dextromethrophan and quinidine

Noninvasive ventilation at onset of respiratory insufficiency among patients with no/moderate bulbar dysfunction improves survival. It also improves quality of life. Initiate when FVC is less than 40 % that of predicted and it doubles survival. Authors state measure of sniff nasal pressure is better than FVC and that measure of 32 % (= 25 cm H2O) or less predicts respiratory failure whereas FVC of 40-50 % does not do so as well . SNP less than 40 cm H20 predict survival. Other measures such as inspir/expir mouth pressures, PSM, sniff transdiaphragmatic pressureand diaph EMG provide more info. Begin noninvasive ventilation when patient has nocturnal hypoventilation including dyspnea and orthopnea. , low SNP, nocturnal desaturation (< 90 % for more than 5% of sleep) or AM hypercapnia more than 6.5 kPa.

Discuss tracheotomy with patient in advance. It prolongs life but is "beyond means" of many patients, who might not want it. Lorazepan, morphine are OK but not oxygen which (unless patient is hypoxic) worsens mouth dryness and hypercapnia.

Nutrition can be assessed with dietary history, BMI, and weight. Enteric feeding can be considered with more than ten percent weight loss. Swallow studies, assessment of choking, food texture, drooling, meal duration , fatigue, video studies to assess silent aspiration. Use thickeners, lip seal, tongue exercises, chin tuck flexing neck forward when swallowing) with eating smaller more frequent meals with enough calories.

Authors emphasize role of hospice, grief couselling for families.

Thursday, September 18, 2008

new seizure drugs coming up

Drugs. 2008;68(14):1925-39.

Monday, July 28, 2008

Misc. Signs of drug use/withdrawal

1. tachycardia
2. hyperthermia
3. purulent drainage from nares with erosions (think cocaine)
4. pop marks on skin
5. hear murmur/ other signs of SBE (see Harrison's textbook for list)

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Differential of delirium with quadriparesis/paraparesis

1. central pontine myelinolysis
2. progressive multifocal leukoencephalopathy
3. acute disseminated encephalomyelitis
4. Cobalamin deficiency (have not seen like this)
5. cryptococcal meningitis
6. critical illness neuromyopathy
7. new onset myasthenia gravis, unsuspected, postoperative

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Wednesday, July 16, 2008

Porphyria pearls

taken from NEJM CPC 2008; 358;2813-2825

case (abbreviated severely). 57 year old woman was admitted with abdominal pain and weakness. She had just had bariatric surgery for obesity. MRI showed changes of posterior reversible leuokoencephalopathy syndrome (turns out it occurs in porphyria). She had had intermittent dark urine and tachycardia.

Patient developed numbness, weakness and tingling in lower extremities, fell while walking and had an episode of confusion and urinary incontinence. She quickly (within days) could not stand or sit independently, became incontinent of stool with numbness and tingling along her body. She had 4/5 strength throughout, with fatigueing and absent reflexes. Sensation was normal. CSF showed normal glucose, protein and no cells. EMG showed an acute motor neuropathy. Strength deteriorated to 0-3/5, worse in proximal than distal legs. There was no response to IVIG.

Pearls-- factors for porphyria in this case were recurrent severe abdominal pain, dark or reddish urine, arterial hypertension, tachycardia and constipation. The diagnostic test in 5-ALA and PBG in urine or serum. A rapid test is the PBG in urine (Hoesch or Watson-Schwartz reaction). Key is diagnose porphyria first then type it. (False positives in 5-ALA are lead posioning and hereditary tyrosinemia type I).

Most types are inherited but the most common type, porphyria cutanea tarda is acquired, associated with liver disease and iron overload. Of 4 types, any may present with neurovisceral presentation esp colicky abdominal pain, and hereditary coproporphyria and variegate porphyria can also present with cutaneous features. Attacks are likely precipitated by adverse effects of excess ALA which is structurally similar to GABA. Starvation, poor CH2O/ energy intake, drugs, alcohol, smoking, infections and stress can ppt. In this case the negative energy balance with surgery caused up regulation of hepatic ALA synthase 1, due to loss of CH2O repression of rate controlling enzyme for heme synthesis in the liver. Other "bad " drugs in this case were phenytoin, tramadol and bactrim. Sulfonamides and barbiturates are also "bad." So is progesterone (which is why postpubertal women are more susceptible). The treatment is i-v heme. It can prevent reversible axonal death. Prognosis is slow and incomplete recovery. Noted also in this case wwre blisters in sun exposed parts of the body. She had variegate porphyria.

Monday, July 14, 2008

MoCa test for cognitive impairment

The MoCA test is an alternative to the MMSE which has had its share of problems. Link for information http://www.mocatest.org/

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Friday, July 11, 2008

Insulinoma mimicking seizure

Bazil CW, Pack A Insulinoma presenting as a seizure disorder. Neurology 2001; 56:817-818.

This rare disease mimicking a common disease needs to be added to the knowledge of doctors diagnosing individuals with various "spells." A 45 year old woman had troubel with panic attacks, inability to focus, tongue/limb tingling, perception of yellow spots and cold sweat. She later developed episodic unresponsiveness and jerking. Waxing and waning irda and polymorphic delta was seen on eeg, without interictal discharges, and the patient was felt to have pseudoseizures. A low glucose of 55 was found, islet cell tumors of the pancrease were found and removed and the patient took diazoxide with improvement.

Insulinoma usually includes nonspecific symptoms such as dizziness, apathy, headache and confusion. It is rare. affecting at most 5 per million.

Neuropsychiatric Profiles of Patients with Insulinomas; Ding Y, Wang S, Liu J, Yang Y, Liu Z, Li J, Zhang B, Chen Y, Ding M; European Neurology 63 (1), 48-51 (Dec 2009)
A review of 42 patients with insulinoma was undertaken, showing that many went undiagnosed for up to five years with neurologic and neuropsychiatric symptoms. 12 were misdiagnosed as epilepsy with eeg findings. Others received psychiatric diagnoses. Clinical presentation was usually confusion, convulsions and visual disturbance.

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Tuesday, June 17, 2008

manage carotid body tumor

fromClinical Neurology News:

Resection Remains Best Treatment for Carotid Body Tumors
PATRICE WENDLING (Chicago Bureau)
Article Outline
• Skip Angiography, and Other Surgical Pearls
• Copyright
CHICAGO — Surgical resection remains the treatment of choice for carotid body tumors, as presented in a review of 88 patients at one center.
Radiation therapy and chemotherapy are unsuitable alternatives because these rare tumors are too slow growing, and radiation exposes the carotid arteries to radiation arteritis, accelerated atherosclerosis, and even necrosis, Dr. Thomas A. Whitehill said at a vascular surgery symposium sponsored by Northwestern University.
Preoperative percutaneous tumor embolization has been tried with mixed results, but can be an important adjunct when treating select patients with large tumors (greater than 6 cm). There has been one report of a successful use of covered stents to facilitate resection (J. Vasc. Surg. 2003;38:389–91).
The malignancy rate for carotid body tumors is hard to define because there are no reliable histologic markers, but is thought to range from 2% to 5%, he said. Even if benign on histologic exam, all tumors, once discovered, should be surgically removed because they will ultimately wrap around the internal and external carotid arteries, erode into the base of the skull, and entrap neighboring cranial nerves. Increasing size also can interfere with speech, swallowing, and respiration, said Dr. Whitehill of the vascular surgery division of University of Colorado Health Science Center, Denver.
From 1993 to 2007, Dr. Whitehill and colleagues surgically resected 88 Shamblin classification II or III carotid body tumors, with an average diameter of 10.4 cm (range 5–16 cm). The patients ranged in age from 30 to 40 years.
Surgery time ranged from 4 to 14 hours, with an average blood loss of 375 mL (range 50–1800 mL). An internal carotid artery (ICA) resection bypass was performed in three patients, and ICA ligation in none.
Complications were relatively low, Dr. Whitehill said, and included cranial nerve IX neuropraxia (4%) or injury (1%), cranial nerve XII neuropraxia (30%), and superior laryngeal nerve injury (10%). There were no strokes or deaths.
Surgical advances and the widespread use of CT and MRI have decreased the overall risk of postoperative stroke over the past 25 years from about 30% to less than 2%, although the incidence of cranial nerve injury remains high at 15%–35%, he said.
Skip Angiography, and Other Surgical Pearls
Dr. Thomas A. Whitehill offered tips for carotid body tumors.
▸ Skip the angiography suite when making the diagnosis, and focus on CT imaging, preferably axial cuts rather than reconstructions. MRI may be slightly better at evaluating distant, metastatic deposits at the skull base.
▸ A nerve stimulator may be useful for preoperative identification of the cranial nerve.
▸ Do preoperative vocal cord and speech evaluations.
▸ Consider serial embolization in patients who are too old or have too many comorbidities to tolerate surgery.
▸ On a side CT view, draw a line between the mastoid tip and the angle of the mandible to get an idea of how high an exposure is needed and to help with preoperative planning.
▸ Utilize nasotracheal intubation in most patients, as it provides greater mobility with the mandible when resecting large tumors.
▸ In high access cases, mobilize the parotid gland anteriorly, up to the level of the facial nerve.
▸ Gain vascular control, if possible, and mobilize the tumor circumferentially to assess the extent of disease.
▸ Resect the tumor from proximal to distal.
▸ Fine mosquito clamp dissection and 3–0 or 4–0 silk ligation can give the best hemostasis.
▸ Send all suspicious lymph nodes for frozen permanent sections.
▸ Rather than using maxillomandibular arch bar fixation to obtain mandibular subluxation, consider interdental cross-wiring between the maxilla and mandible using bicuspids in dentate patients and Steinmann pins in patients with no teeth.
▸ For very distal tumors, cutting the digastric muscle will get you within 2 cm of the skull base.
▸ For large tumors, ligating the external carotid artery near its takeoff provides greater mobility.
▸ Avoid ligation of the internal carotid artery.
▸ If a tumor is 6 cm or more in diameter, consider preoperative embolization.
▸ Pushing the tumor completely through the bifurcation or pulling it anteriorly through the bifurcation may improve exposure angles and ease dissection.
▸ Take your time after the tumor is cleared of the two carotid arteries. The posterior surface and medial side of the tumor still must be separated from the deeper parapharyngeal tissues. Haste at this stage can result in the superior or inferior laryngeal nerves being transected or medial pharyngeal injuries, causing substantial swelling and neck pain in patients.

Tuesday, June 03, 2008

Typing PSP

"The postmortem room has become the temple of truth" (Donald Calne, re PSP) (Neurology 2008)Current nomenclature"Richardson disease" corresponds with initial descriptions. There is a gradual onset of postural instability and falls within the first two years, with vertical supranuclear gaze palsy, a frontal dysexecutive syndrome, rigidity and bradykinesia that is not responsive to leveodopa, and a life expectancy of six years.A second milder group at pm (post-mortem) have PSP tau pathology that is more restricted and less severe are called PSP-P (PSP-parkinsonism). They have assymmetric bradykinesia of the limbs, an initial response to levodopa, tremor and limb dystonia without early falls, eye movement problems, or cognitive dysfunction. Most patients with "atypical PSP" are in this category. The third rarer category is pure akinesia with gait freezing (PAGF). There is gradual onset of unsteady or slow gait and hypophonia progressing to gair freezing and start hesitation, without limb rigidity or tremor. There is no response to levodopa and there is no dementia or opthalmoplegia in the first five years. In types 2 and 3 the median duration of the disease is around ten years. Other patients with similar tau-PSP pathology present with corticobasal ganglionic degeneration, progressive nonfluent aphasia, or apraxia of speech. References up to date:Williams DR, de Silva R, Pavour DC et al. Characteristics of two distinct clinical phenotypes in pathologically proven progressive supranuclear palsy: Richardson' syndrome and PSP - parkinsonism. Brain 2005; 128:1247-1258.Williams DR, Holton JL, Strand C. et al. Pathological tau burden and distribution distinguishes progressive supranuclear palsy-parkinsonism from Richardson's syndrome. Brain 2007; 130: 1566-1576.Mizusawa H, Mochizuki A , Ohkoshi N, et al. Progressive supranuclear palsy presenting with pure akinesia. Adv Neurol 1993; 60: 618-621.Josephs KA, Duffy JR, Strand EA et al. Clinicopathological and imaging correlates of progressive aphasia and apraxia of speech. Brain 2006; 129: April 13.Tsuboi Y, Josephs KA, Boeve BF et al. Increased tau burden in the cortices of progressive supranuclear palsy presenting with corticobasal syndrome. Mov Disord 2005; 20: 982-988.

Sunday, June 01, 2008

Limbic encephalitis : An expanding concept: teratomas and "things"

Graus F, Saiz A. Neurology 2008; 70: 500-501 (editorial) . Also see http://dementianotes.blogspot.com/2008/06/vgkc-autoantibodies-mimicking-cjd.html re VGKC antibodies in particular.

A syndrome of encephalitis in 12 women with teratomas of the ovary follows a predictable clinical pattern. A prodromal fever, is followed in a few days by a prominent psychiatric syndrome or short term memory loss, then, seizures, then progressive unresponsiveness (catatonia like stage) , dyskinesias, autonomic instability, and central hypoventilation. ICU support for weeks or longer may be necessary, and patients recover generally after tumor removal or immunotherapy (pheresis or IVIG). MRI is usually negative (9/12). All patients had CSF and serum antibodies against components of NMDA receptor (NMDAR) especially 2 subunits NR1/NR2). These particular patients express NR1, which is ubiquitously expressed in brain, but in these patients distributes with NR2 (basal forebrain) and disappears with treatment. This contrasts with NR2B which is seen in Rasmussen's excephalitis, refractory epilepsy, and stroke. In neuropsych lupus, this epitype leads to apoptosis.

Iizuka et al. now describe this epitype in Japanese juvenile non-herpetic encephalitis (with NMDAR). They emphasize the abnormal movements, especially orolingual after initial psychiatric syndrome and catatonic like state. These patients can reverse even after long periods of ventilator dependence.

Update pearls for NMDAR from Neurology 2014; 82: 550-551 (editorial)

1. Tests for: CSF with HEK cells expressing GluN1/2 subunuts of the NMDAR cell based assay. or serum. with serum there is a 15 % false negative/positive result

2. Adult men more often presented with seizures (18 %) whereas women more oftenpresent with behavior/psych symptoms

3. Among women, 41 % had tumors, all ovarian teratomas. Among men only one had a tumor likely unrelated schwannoma.

Friday, May 23, 2008

Rosai-Dorfman disease presenting with widespread intracranial and spinal involvement

Kidd DP, Revesa T, Miller NR. Neurology 2006; 67:1551-1555.
Disease is also known as sinus histiocytosis with masssive lymphadenopathy. It has a much more benign prognosis than histiocytosis X. A registry published in 1990 of 238 patients showed that of 238 patients, 49 had a lasting remission, 165 had stable disease not requiring treatment, and 21 had died 4 of the disease. It presents in the third to fifth decade with cervical lymphadenopathy often preceded by a viral infection, with elevated ESR and polyclonal gammopathy. Steroids and/or XRT is used for treatment. Authors present two neurologic cases. One mimicked a sellar meningioma with loss of vision, hearing and smell. A gallium scan and chest CT showed chest lesions. The other presented with four months of progressive visual loss. Only 4 % have neurologic involvement (8 patients) five with spinal dural based lesions, three with intracranial epidural lesions.

My question: cases did not describe cervical adenopathy so why was diagnosis made?

Wednesday, May 14, 2008

Unusual sources of lead leading to intoxication

Lead was an ingredient of antibacterial ointments until the 20th century and are still sold in rural Switzerland. Traditional remedies including Asian plant products may be contaminated. Beauty ointments may be a culprit including lip balms (Fluri et al. Neurology 69:929 2007).

Serum, not urine lead levels should be checked and porphoryns may be elevated. MRI may show hyperintensity in the basal ganglia. Encephalopathy and motor neuropathy both can occur in adult intoxications.

Bacterial and fungal meningitis in patients with cancer

Safdieh JE, Mead PA, Sepkowitz KA et al. Neurology 2008; 943-947. High points-- only five percent of cases had the triad of fever, nuchal rigidity and mental status changes. VP shunt patients usually presented with mental status changes. Most patients had had prior neurosurgery. organisms included more gram positive infections and a smattering of diverse other organisms. CSF response was often muted.

Monday, May 12, 2008

Differential diagnosis of cervical radiculopathy

1) Peripheral nerve entrapment (eg CTS)- see positive Phalen's and Tinel's sign, typical distribution, abnormal nerve conduction studies v. normal in cervical radiculopathy

2)Rotator cuff abnormal-- pain in shoulder or arm, rarely below elbow, worse with shoulder movements, normal sensation and reflexes.

3) Acute brachial plexitis-- (Parsonage Turner)-- see pain in neck shoulder and arm followed within days to weeks by weakness especially in C5-6 region as pain recedes (whereas in radiculopathy pain and weakness coincide).

4) Thoracic outlet syndrome-- Pain in shoulder and arm, intermittent paresthesia, C8-T1 symptoms (rare in radiculopathy), reproduction with provocative tests including Roo's test (rapid flexion and extension of fingers with arms abducted at 90 degrees and externally rotated 90 degrees), normal neuro exam, decreased radial pulse with vascular compression (rare) and usually normal NCS.

5) Herpes zoster-- neuropathic pain in dermatomal distribution followed by vesicular rash.

6) Pancoast syndrome-- Pain in shoulder/arm due to plexus compression, paresthesia is in C8-T1 distribution (intrinsic hand muscles), ipsilateral ptosis, miosis, and anhidrosis.

7) Sympathetic mediated syndromes-- pain and burning in arm with swelling, hyperesthesisa, allodynia, and vasomotor changes (temperature and color) with normal neurologic examination.

8) Referred somatic pain from neck-- such as discs, joints, usually segmental C5-6 felt in posterior neck and supraspinatus fossa, normal neuro exam.

9) SUNCT/SUNA syndromes with pain in cervical region

Cervical radiculopathy

Carette S, Phil M, Fehlings MG. Clinical practice. NEJM 2005; 353:392-399.

The etiology in 70-75% is encroachment of the foramen due to cervical spondylosis or other cause of degeneration, whereas HNP is much less frequent (20-25 %) and tumors and other causes even less frequent. Pain occurs only if the dorsal root ganglion (DRG) is compressed. Hypoxia of the nerve root and DRG exacerbates the pain. Inflammatory mediators including MMP's, PGE2, IL6 and NO. Nonsurgical management resolution correlates with imaging improvement.

Neck and arm pain predominate. Sensory symptoms (burning, tingling) follow a dermatomal distribution, but pain follows a myotomal pattern. C7 pain for example includes radicular pain to forearm, and N/T to the third digit. Pain is relieved by looking to opposite contralateral side and holding hand on top of the hand and exacerbated by turning the head towards the pain. Red flags including systemic signs of illness (fever, chills, weight loss), diffuse hand numbness attributed to CTS, clumsiness, urinary urgency or frequency not retention or incontinence. Provocative tests for radiculopathy are mostly unreliable. C7 is most commonly affected followed by C6.

Signs of C5 involvement include pain in the medial scapular border radiating to the elbow, weakness of the deltoid, supraspinatus and infraspinatus, sensory loss in the lateral arm, and loss of the supinator reflex.Signs of C6 involvement include pain in lateral forearm, thumb and index finger, weak biceps, b-r, and wrist extensors, thumb and index finger sensory loss and loss of biceps reflex.

Signs of C7 involvement ae pain in medial scapula, posterior arm, dorsum of forearm, third finger, weak triceps, wrist flexors, finger extesnors, sensory loss in posterior forear and third finger, and loss of triceps reflex.

Signs of C8 involvementare pain in shoulder, ular side of forearm, fifth finger, weak thumb flexors, abductors, and intrinsic hand muscles,and sensory loss of the fifth finger.

Treatment is not proved in large trials. Analgesics including opiates and NSIAA's are first line sometimes with prednisone. Epidural injections result in relief that is longstanding in many patients but complications include spinal cord and brainstem infarction. Hard/soft cervical collar for short term or cervical pillow at night are used. Cervical traction is unproved. Exercise therapy including active AROM, aerobic conditioning with isometric and progressive resistive exercises are usually recommended after initial period.

Indications for surgery include cervical root compression on imaging, concordant pain or dysfunction, persisting pain, or functional motor deficit, or compression of the cord. Anterior decompression with strut reconstruction is common.

Clinical feature and prognostic factors in adults with bacterial meningitis

van de Beek B, de Gans J, Spanjaard L et al. NEJM 2004; 351: 1849-59. Dutch study analysed 696 cases of community acquired acute bacterial meningitis seen 1998-2002. The classic triad of neck stiffness, fever, and change of mental status was seen in only 44 %, but 95 % had two of the four symptoms (the fourth symptom being headache). Mortality was 21 % and much higher with pc meningitis than mc meningitis (30 v. 7 %). Risk factors (negative risk) were advanced age, present otitis or sinusitis, absent rash, low GCS on admission, tachycardia, positive blood culture, elevated sed rate, thrombocytopenia, and low CSF WBC. Worst risk thus was with systemic compromise, low level of consciousness and infection with SC.

In more detail, HA occurred in 83 %, fever in 77 %, change in mental status in 69 % (GCS <14). Rash was present in 26 % and usually indicated mc infection (98 % of time) . 89 % of rashes were petechial. Only 3 % of those with information had papilledema.

Discussion of steroids: The european dexamethasone study showed adjunctive treatment with corticosteroids was beneficial although a posthoc analysis showed it to be more likely due to reduction in systemic than neurologic complications.

HABC Hypomyelination of basal ganglia nad cerebellum

van der Kmaap MS, Linnannkivi T, Paetau A et al. Hypomyelination with atrophy of the basal ganglia and cerebellum: followup and pathology. Neurology 2007; 69: 166-171.

A report of 11 new patients is made. Typically a pediatric population presented with normal or delayed psychomotor development, with increasing extrapyramidal movement disorders, spasticity and ataxia. The putamen was small or absent, the caudate often atrophic with normal thalamus and globus pallidus. The cerebellar granular layer was typically affected. All known patients were sporadic. This is a recently described syndrome (2002) with less than 20 reported cases.

Tuesday, March 25, 2008

Impulse control and PD Quick hits AAN 2008

1. Patients with PD showed altered decision making under ambiguity but had preserved decision making under risk conditions with explicit rules for gains and loss.
2. Acamprosate was used to treat impulse control disorders in 2 patients with PD in whom discontinuation of dopaminergic drugs did not help. It worked
3. Topiramate was used in 8 Spanish patients to reduce ICD and it appeared to work.
4. The ICD spectrum included pathological gambling, hypersexuality, shopping, computer use and binge craving of sweets, which should be specifically sought in the history taking.

AAN 2010
Hoarding may be a component of the syndrome

Neuroopthalmic findings that reliably differentiate PSP and PD

P05.042 AAN book 2008 Brad Katz et al. Salt Lake City Utah

In addition to standard criteria , the following differentiated the 2 disorders: slowed horizontal saccades, square wave jerks, eyelid opening apraxia, abnormal vertical OKN's, lid retraction stare, blink rate and light sensitivity were all implicated. A cutoff score of 2 or more of the the following three features: greater than 5 swj's, abnormal OKN's downward, and lid retraction stare identified all of 28 patients correctly (including placebos).

Hardwick A, Rucker JC, Cohen ML, et al. Evolution of oculomotor and clinical findings in autopsy proven Richardson syndrome. Neurology 73; 2122-2123 2009.

Authors note the SPEED of vertical saccades (more than amplitude) remain the cornerstone for differentiating PSP from other disorders. Clinical presentation was young, absent falls, complaints of horizontal diplopia due to vergence abnormalities, cognition c/w FTD. At autopsy atrophy was restricted to the superior colliculi with rare NFTs.

Monday, March 16, 2009

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Monday, October 13, 2008

Thursday, October 09, 2008

Peri-ictal water drinking lateralizes seizure onset to the nondominant temporal lobe

Ictal eye closure is a reliable indicator for psychogenic nonepileptic seizures

Monday, October 06, 2008

Saturday, September 27, 2008

Thursday, September 18, 2008

Monday, July 28, 2008

Wednesday, July 16, 2008

Monday, July 14, 2008

Friday, July 11, 2008

Tuesday, June 17, 2008

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Does this depict frontal lobes?

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