Hardscience of concussions

rapid acceleration and deceleration of brain can be estimated as follows
a=(v2-v0)/2sg, easily calculates deceleration based on initial speed v0, final speed v2, and distance travelled s. The result obtained is in terms of g, which is equivalent to 10.73 yards per second (Varney and Roberts 1999). Since the final speed is zero after player stops, formula becomes a= -v0squared/2sg. Then, calculate force applies by F=mg. The number 200g force has been proposed as the amount to cause permanent damage but is not really known.

Brain injury in organized sports

Boxing
Mendez MF. The neuropsychiatric aspects of boxing. Int J Neuropsychiatry Med 25: 249-262, 1995.

Stewart WF, Gordon B, Selnes O. et al. Prospective study of central nervous system function in amateur boxers in the United States. Am. J Epidem 139;573-588, 1994. Bouts fought before 1984 when safety measures were instituted were correlated with neuropsychological impairments.

Football
Abnormalities in sustained attention and visuomotor speed with absent normal practice effects. Preseason baseline assessment by SLAM model is becoming gold standard for concussion assessment and management.
Sideline assessment use SAC,a 5-10 minite measure (McCrea et al, 1996).

Mild traumatic brain injury important articles

JT Barth et al, 1989, 2002 4 yr prospective study of MTBI in college athletics in Sports Laboratory Assessment model (SLAM) with 2300 football players at 10 universities with pre and post neuropsychological assessments. It used a matched control group plus patients as their own control group. After very mild injuries athletes showed a 5-10 day recovery curve for mild cognitive deficits.

Consensus Conference: Rehabilitation of persons with traumatic brain injury . NIH Consensus Development Panel on Rehabilitation of Persons with TBI. JAMA 282:974-983, 1999. Neuropsychological deficits include problems with memory, attention, concentration, executive skills.

headache classification

http://ihs-classification.org/en/02_klassifikation/

Adult onset ataxia telangiectasia (variant AT)

Verhagen MMM, Abdo WF, Willemsen MAAP.  Clinical spectrum of ataxia-telangiectasia in adulthood. Neurology 2009; 73:  430-437 and accompanying editorial.

This important article describes another way adult neurologists cannot completely hide from their brief 3 month training in pediatric neurological diseases that they underwent in training. The summary below focuses on clinical aspects but the article contains genetics information for those interested.

AT is typically an AR childhood disease that one can read about in a 1980 edition of Adams and Victor.  Variant AT occurs in older adults as a forme fruste perhaps of the pediatrics form. It is described in about 13 patients.  Patients usually had young onset movement disorders, either choreoathetosis or resting tremor.  Distal muscle weakness occurred in one patient.  By age 27 patients began to experience progressive cerebellar atrophy with eventual development of dysarthria in all, continued movement disorders, nystagmus, dystonias and oculomotor apraxias.  EMG/NCS showed either anterior horn cell involvement or sensorimotor polyneuropathy.  Cerebellar atrophy on imaging affected vermis primarily. 

Minimal  telangiectasias of either eye or skin occurred in variant form.  Lung function was normal.  Four of 13 developed malignancies including ALL, ectopic pituitary tumor, and breast cancer.  Alpha feto protein levels were markedly elevated in all patients  to ten to fifty times normal, although this is still less than in classic AT. Ig deficiencies occurred in only one patient and were of minor degree. 

Adult patients with classic AT also exist and present with cerebellar ataxia, ocular telangiectasia, immunodeficiency, rearrangement of chromosomes 7 and 14, X ray hypersensitivity and elevated alpha feto protein levels.  Patients were all wheelchair bound, had growth retardation, endocrinopathy including type 2 DM, diminished secondary sexual characteristics, restrictive lung disease, and more cancer especially lymphoproliferative diseases.  Defect is in DNA repair. 

Alpha fetoprotein appears to be good screening tool.

MGUS: significant beyond hematology

Vanderschueren SW, Mylle M, Dierickx D et al. Monoclonal gammopathy of undetermined significance: significant beyong hematology. Mayo Clin Proc. 2009; 84:842-847.

MGUS is found in 3% of patients older than 50, 5% older than 70, in 7 % of patients seeking medical evaluation, and converts to myeloma at rate of one percent per year. Authors present five OTHER associations of MGUS that are commonly underrecognized.
1. Acquired C1 inhibitor deficiency. should be suspected in patients with repetitive often stereotypic episodes of angioedema, with no urticaria or pruritus or family history, & in pts with recurrent acute abdomen with normal CRP and low C4 levels. Hereditary form occurs in first two decades usually, acquired forms in elderly patients with MGUS in up to 40 % of cases (IgG, IgM or IgA). In acquired forms C1q levels are low due to consumption. Acute prophylaxis can be done with antifibrinolytics eg. transexamic acid or danazol, and acutely FFP has variable success.
2. Systemic capillary leak syndrome should be suspected in patients with repetitive hypovolemic shock, capillary leak and hemoconcentration (in case report, HB went from 15 to 23 in one day). Attacks characterized by prodrome malaise, fatigue, OH, polydipsia, palpebral edema, last 1 to several days and have high fluid requirements during an attack. MGUS usually IgG is typically present. Intestinal edema, ascites, muscular edema, pleural and pericardia effusions occur, may have compartment syndrome. Terbutaline, theophylline, and IVIG are used to treat.
3. Acquired von Willebrand syndrome-- uncommon condition occurs in elderly patietns with no history or family history of bleeding, with MGUS in 50-60 %. Desmopressin works transiently. High dose IVIG has been used. Suspect with mucocutaneous or postop bleeding.
4. Schnitzler syndrome-- heralded by repetitive fevers and chronic, initially nonpruritic urticaria. CRP and ESR are typically very high. MGUS is usually M type. Anakinra, an IL1 antagonist shows promise.
5. Scleromyxedema-- characterized by typical skin eruption and visceral or repetitive neurologic involvement. Case reported of 39 yo man with episodic confusion preceded by flu like illness proceeding to status epilepticus and prolonged postictal coma. Over weeks, appeared erythematous plaques on face and skin creases over glabella , confirmed with biopsy to be scleromyxedema. Tx was plasmapx, IVIG, dexamethasone, autologous stem cell, thalidomide. Its caused by hyaluronic acid deposition in superficial dermis and production of MGUS usually IgA. Also called papular mucinosis and generalized lichen myxedematosus. Occurs in patients usually 30-50 , may include MI as well. Also called dermatoneural syndrome.

Neuroopthalmology of chiasm: Pearls

1. a prefixed chiasm can result in deficit resembling congruous visual field loss
2. A postfixed chiasm can resemble bilateral optic neuropathy
3. Fixation blindness: attempts to focus on a near target results in overlap of 2 nonseeing temporal fields, and everything beyond fixation appears to disappear.
4. See saw nystagmus seen with masses or trauma cause one eye and other alternately elevating, intorting, and then depressing and extorting, in a pendular fashion
5. Gliomas of chiasm are similar to optic nerves, presenting with decreased vision, optic atrophy and strabismus
6. In pituitary apoplexy, pain is frequently retroorbital, and field defect is classically bitemporal superior quadrantanopsias, due to upward expansion of the tumor, 70 % vomit, LP is abnormal, MRI is diagnostic test of choice, and substantial medicolegal risk resides therein. Men have nmore than women
7. Aneurysms causing chiasmal compression include supraclinoid ICA, the ICA-opthalm a junction, and occassionally cavernous or ACOM arteries. Nonruptured aneurysms causing compression require treatment usually with clipping rather than coiling (Continuum 2009).

Compressive optic neuropathies- dDx

taken from Continuum, Walsh and Hoyt, and Frohman

primary tumors
glioma
ganglioglioma
capillary/cavernous hemangioma
melanocytoma

secondary tumors
metastatic carcinoma
nasopharyngeal carcinoma
leukemia
lymphoma

infectious/inflammatory
sarcoidosis
lupus
APL syndrome
paraneoplastic syndrome
Wegener's granulomatosis
GCA
PAN
IBD
Behcet's
Sjogren's
postvaccination
perioptic neuritis
Infectious (TB, syphilis, lyme)
Viral (HSV, HIV, CMV, EBV, Cox A, Hep A, Hep B, MMR, rubeola)
fungi (aspergillus, mucor, crypto, candida, histoplasmosis
parasites ( toxo, cysticercosis, toxicariasis)

Sarcoid and lupus related eye diseases

sarcoid:

-- Anterior uveitis (most common)
--optic neuritis like
--Perineuritis
--retrobulbar optic neuropathy
-- granulomatous infiltration of the posterior orbital, intracanalicular, and intracranial optic nn.

All have disc edema (eventually)
May have slowly progressive or acute vision loss
diagnosis may be helped by findings of conjunctival nodules, uveitis, lacrimal gland enlargement, and vitreous opacities.

lupus
--PION more common, but AION occurs
-- can see IIH like presentation (except not really "idiopathic"

Intranasal contact point headache

Rozen TD. In...: missing the point on brain MRI. Neuroimages. Neurology 2009; 1107.

Nasal septal deviation with contact point on the lateral naasal wall can trigger episodic or daily headaches. Its easy to visualize on MRI but rarely reported. Authors show an image of an MRI of 2 cases.

REM Behavior Disorder (RBD) Random Pearls

1. Differential diagnosis might include nocturnal seizures, which would occur earlier in night during NREM sleep; may or may not be able to diagnose without video/EEG
2. Differential diagnosis would include OSA which causes disruptions in sleep architecture and increased nighttime arousals so sleep study for that problem would be useful (pseudoRBD)
3. Drugs that cause/exacerbate include SSRI's, TCA's, MAO A inh, ETOH withdrawal,
4. Actions can be purposeful violence as patients are actually enacting dreams which are violent
5. Dreams of being chased are common (50 v. 8 % in RBD v. non RBD PD patients)
6. Associated with synucleinopathies ie. PD, CBD, LBD (52 % risk at 12 years)
7. Environmental precautions are medicolegally important including separate beds, locking windows, etc.
8. Effectively treated in 90 % with clonazepam, with most of the rest treated with carbamazepine.
9. Patients can have nonviolent behaviors as well as violent ones including included masturbating-like behavior and coitus-like pelvic thrusting, mimicking eating and drinking, urinating and defecating, displaying pleasant behaviors (laughing, singing, dancing, whistling, smoking a fictive cigarette, clapping and gesturing "thumbs up"), greeting, flying, building a stair, dealing textiles, inspecting the army, searching a treasure, and giving lessons. Speeches were mumbled or contained logical sentences with normal prosody. In PD with RBD (n = 60), 18% of patients displayed nonviolent behaviors. In this series (but not in incidental cases), all RBD patients with nonviolent behaviors also showed violent behaviors. NEUROLOGY 2009;72:551-557

Orthostatic tremor; writing tremor

Pearls Orthostatic tremor

--low amplitude high frequency tremor of legs

-- can'tstand but can walk

-- can auscultate legs

-- have to suspect in history

-- Original described by Ken Heilman in Arch Neurol

Writing tremor "hand shakes only when I write"

-- no tremor otherwise; task specific movement disorder

-- akin to dystonia

-- other tasks could be brushing teeth or shaving

ET v PD

Obvious-- age onset, duration, family history and response to ETOH

Subtle-- Body part affected                handwriting            associated signs     natural history

 

PD            hand, foot, chin lips            micrographic           +                        progressive

ET             hand, head, voice               tremor not micro      absent                insidious

 

Pearls 

1.  best position for postural tremor is fingers touching wings (elbows) up

2.  saying "ahhhh" shows rhythmical oscillation which is typical

 

 

Coat hanger sign of orthostatic hypotension and other MSA pearls/ signs

1.  Patients complain of characteristic muscular pain across neck or shoulder of coat hangar, when standing. 

2.  Squeaky voice distinct from PD (needs oiling)

3.  Erectile dysfunction is present in nearly all men with MSA

4.  Check BP anyway can't rely on typical OH symptoms

5.  Inspiratory  stridor due to vocal cord paralysis or dystonia, may have OSA as well

6.  Cold dusky mottled hands

7.  Anterocollis think MSA, retrocollis think PSP

8.  Applause sign-- ask patients to clap quickly three times, if they continue that is frontal (PSP, PS not PD)

9.  Other frontal signs-- concrete proverbs highly characteristic of PSP, decreased verbal fluency

10. Palilalia / echolalia (seen in PSP) (equivalent of applause sign)

11. Absent vertical opthalmopledia, see slow vertical limited saccades occur first-- compare speed of horizontal and vertical saccades, and no fast phase nystagmus on OKN on vertical.  Beware, pursuit may be OK so must  test saccades.

12.  Unilateral apraxia (note COMPLAINS of apraxia)

13.  Unilateral jerky dystonia = CBD.  (Only other movement disorder that starts unilaterally is PD)

7.

red flags for psychogenic movement disorders

credit AAN webcast Dr Sethi

 

wearing dark glasses indoors

triad of dark glasses, inappropriate underwear and teddy bears

failure to make or maintain eye contact

spells in front of audience

history given by significant other

extreme sensitivity to sounds, smells, bright lights, unusual precipitating factors

psychogenic dystonia

Helps if they have psychogenic tremor too.  See Fahn and Williams 1988 ADV NEUROL and Lang A Can J Neurol Sci 1995.

 

Features include abrupt onset, variability, bizarre extraneous movements, slow voluntary movements, resistance to passive movements, recurrence and remission, suggestibility, less distractibility.  Again, like parkinsonism, response to medication such as levodopa does not exclude psychogenic tremor.  It may be painful, exquisitely so.  Toes often are down and turned in, typical.  Big toe is usually down and in, if up it may be a striatal toe.

Psychogenic Parkinson's clues

Abrupt onset (71 % in Lang)

Maximal severity quickly

Spread of tremor with restraint of affected body part

Tremor is complex and distractible

Rigidity decreases with distraction (opposite of PD Froment's maneuver)

psychogenic movement disorders features

from Jankovic (5)

 

abrupt onset 79

distractability 72

variable amplitude and frequency 62

intermittent occurrence 35

inconsistent movements 30

variable direction 17

irregular pattern 12

suppressibility  12

incongruous movements  11

la belle indifference  11

suggestibility  sensory split 10

entrainment  9

active resistance to passive movement  8

deliberate slowing  7

nonpatterned  7

position induced  6

 

 

 

Drugs that affect the seizure threshold

Antiasthmatics-- aminophylline, theophylline

antibiotics-- inh, metronidazole, penicillin, lindane

antidepressants: buproprion, SSRI, TCA

general anesthesia-- enflurane, ketamine

hormones-- estrogen, esp without progesterone

immunosuppressants-- chlorambucil, cyclosporine

local anesthetics- lidocaine, bupivicaine, procaine

narcotics-- fentanyl, meperidine, pentazocine, propoxyphene

psychostimulants- amphetamines, cocaine, methylphenidate

neuroleptics-- clozapine, phenothiazines, butyrophenones

others-- anticholinergic, anticholinesterase, antihistamine, baclofen, heavy metals, lithium, mefenamic acid, oral hypoglycemics, oxytocin

Seizure risk associated with neuroactive drugs: Data from the WHO adverse drug reactions database; Kumlien E, Lundberg PO; Seizure (Dec 2009)


PURPOSE: To explore the association between the use of neuroactive drugs and reports of epileptic seizures. MATERIAL: Using the WHO adverse drug reactions (ADR) database, VigiBase, we surveyed reports of suspected seizures from 1968 until February 2006. Case reports of ADRs, that were classified as convulsions were collected and compared to the total number of ADRs reported. RESULTS: The total number of ADRs was 7,375,325. The number of convulsive events was 71,471. The ratio of convulsive ADRs to the total number of ADRs reported for each drug was evaluated and expressed as a percentage. The 10 drugs most frequently associated with convulsive ADRs were maprotilene (14.42%), escitaloprame (9.78%), buproprione (9.49%), clozapine (9.0%), chlorprothiexene (8.89%), amoxapine (8.74%), donepezil (8.40%), rivastigmine (6.41%), quetiapine (5.90%) and trimipramine (5.69%). CONCLUSIONS: Based on the reports in VigiBase, ADR reports relating to antidepressants, antipsychotic and cholinomimetic drugs included seizures more often than other neuroactive drugs.

Epilepsy random pearls

1. Valproate works for tilt positive treatment (Eugene Ramsey)

2. Potential analogy of SUDEP and SIDS (failure of brainstem to respond to hypopnea (Mary Donner)

3. In VA study 80 % of patients had undiagnosed serious dyslipidemia, 70 % htn (Ramsey)

4. Asking patients if they turn hues esp. to shade of gray, almost certainly diagnoses NCS

5. Lamotrigine works extremely well for classic migraine (comorbid with epilepsy), less well for common migraine

6. Epilepsy patients describe panic attacks eloquently, but they may be unrecognized seizures (feeling in stomach, etc.)

7. Drug AE's are age related .

8. Carbamazepine causes weight gain, usually not recognized and may worsen tremor(Ramsey)

9. Topirimate helps BP, tremor, glucose control, weight

10. Ethosuximide worsens headache

11. PPI's raise gastric pH, making phenytoin absorption not happening

12. Stopping inducer, PHT or CBZ dramatically decreases clearance of LTG and TPM resulting in blood levels shooting up

13. Simvastatin levels go down 80 % with CBZ. Only statin not affected is ____/ Also beware of Calcium channel blockers (including nimodipine for SAH), HAART, drugs for ED

SMA atrophy

most common fatal genetic disease in newborns

Aut recessive carrier frequency 1:35 occurrence 1:6000

Caused by deletion mutation in SMN 1

Human diseases with no mouse models

Down's syndrome-- mice lack chromosome 21

Parkinson's disease-- mice endogenous A53T

SMA no SMN2 gene

Treatment of Parkinson's disease: gyrokinetics

gyrokinetics.com

lee silverman voice technique

advanced pt techniques

vital stim

tongue biting and seizures pearls

Lateral tongue biting is poorly sensitive but highly specific (99%) for a generalized seizure.
Lateral tongue biting usually indicates true epileptic seizures as opposed to bites to the tip of the tongue which are typically nonepileptiform events.
Lateral tongue biting has a predictive value of 71% for ipsilateral seizure focus.

from AAN website

Differential diagnosis of thunderclap headache

Subarachnoid hemorrhage
unruptured aneurysm
cerebral sinus thrombosis
cervical artery dissection
hypertensive crisis
spontaneous intracranial hypotension
acute ischemic stroke
acute cerebral hematoma
pituitary apoplexy
intracranial infection
"benign" thunderclap (explode category)

Treatable autoimmune encephalitis

Bataller L et al. Autoimmune limbic encephalitis in 39 patients: immunophenotypes and outcomes. JNNP 2007: 78: 381-385.

17 patients were studied at University of Pennsylvania, as well as 22 "outside cases" with sera sent in. . The most important points were that

1. Most of the patients were accurately diagnosed clinically before antibodies were returned.

2. 9/17 had antibodies to known neuronal antigens (paraneoplastic or VGKC's) and 5/17 to novel cell membrane antigens (hippocampus/cerebellum). For the whole group, including the sent in cases, the numbers were 19/39 had antibodies to known antigens, 17/39 to nCMA's.

3. A significant treatment response effect was seen in those with VGKCA's and nCMA's.

4. Those with known intraneuronal antigens (anti Hu, anti Ma) are associated with cancer and may be associated with a worse prognosis than the antibodies to VGKCA's and nCMA's

5. Ovarian teratoma and thymoma are paraneoplastic treatable and benign conditions once the tumor is excised

6. No MRI or CSF characteristics help in diagnostic process.

In another article, some cases of ovarian teratoma associated paraneoplastic encephalitis are related to NMDA receptor antibodies (NMDAR) (Dalmau J, et al. Ann Neurol 2007; 61: 25-36. ) Posner's group described 12/12 with teratomas of ovary who had NMDAR with antibodies to the NR2 subunit, which binds glutamate in the forebrain and hippocampus, AND, a VIRUS LIKE PRODROME.

In yet another article, Mathew et al. (Neurology 2007; 68: 900-905) described orchiectomy for Ma2 related encephalitis as successful in showing otherwise undocumented tumors in 6/25 patients who underwent the procedure (the other 19 had known germ cell tumors). The criteria for orchiectomy were 1) present Ma2 antibodies with clinical/MRI evidence of encephalitis 2) life threatening or progressive neurologic deficits 3) age< 50 4) absent evidence for other tumors 5) new testicular enlargement, microcalcifications on testicular ultrasound, cryptorchidism or other evidence of testicular disease. All testicular specimens showed germ cell tumors. Tumor markers, body CT, PET, and other tests were negative in those undergoing the procedure.

Other antibodies reported include Hu, Ri, Ma1, Ma2, CAR, CRMP 3, amphiphysin, and others.

Thunderclap headache and benign CNS angiopathy

Chen SP et al. Reccurrent primary thunderclap headache and benign CNS angiopathy: spectra of the same disorder? Neurology 2006; 67:2164-2169.

56 patients with recurrent thunderclap headache were recruited. Segmental vasoconstriction was found in 22 (39 %). HA recurred .7 times per day for fourteen days. Each attack lasted 3 hours, and 84 % of patients reported at least one trigger. Nimodipine aborted attacks in 83 % and stopped headaches within 3 months. 4 patients (7%) developed stroke. Authors note that this entity is identical to benign CNS angiopathy. Exertion and defecation are greater triggers in vasoconstriction group.  Other triggers are emotional situations,laughing, bathing, bending down.

Blogger note: this is identical to Call Fleming syndrome frequently discussed in USA. See http://strokenotes.blogspot.com/search?q=call for more minutiae about it.

Review article
Ducros A. Reversible cerebral vasoconstriction syndrome.  Lancet Neurology 2012;11:906-917

additional pearls

1.  Pain peaks in 1-2 weaks, angiogram findings in about 3 weeks.

2.  Many synonyms exist (see below)

3.  Thunderclap headache usually peaks within 1 minute, unilateral or bilateral and mimics aneurysm rupture; each episode may last 3 hours but can vary with up to 3 day duration, and 4 attacks over the 3 month period with moderate headache in between

4.  Ten percent have focal findings; ten percent may have seizures including inaugural, and they rarely recur.  Marching numbness can mimic migraine, focality, a stroke.

5. Beware of neck pain (dissection).  Other differentials are CVT (check d Dimer and MRV), transfusion or products (red cell transfusion, alpha interferon, IVIG), phenytoin intoxication, CEA, low pressure headache.

6.  May screen for pheochromocytoma or glomus tumor or carcinoid. Do a drug screen in some patients.  Not only is cocaine in differential, cannabis is a trigger as well as LSD.  Other triggers include SSRI's, triptans, ergot derivatives, nicotine patches, ginseng and binge drinking.  Inquire about scorpion poison and star fruit.

7.  Convexity SAH may be subtle, and missed on CT, strokes occur in watershed areas (including cerebellar watershed areas); brain edema can mimic PRES.

8.  Catheter angiography can trigger TIA. 

9. Postpartum cases occurs in first week after delivery.  Inquire about exposure to vasoconstrictors during anesthesia, depression. 

10.  Synonyms and old names:  isolated benign CNS vasculitis, acute benign cerebral angiopathy, reversible cerebral segmental vasoconstriction, Call Fleming syndrome, CNS pseudovasculitis, benign angiopathy of CNS, postpartum angiopathy, migraine angiopathy, migraine angiitis, migraine vasospasm, primary thunderclap headache, cerebral vasculopathy, vasospasm infatal migrainous infarction

11.  Factors that independently make RCVS worse:  those who receive glucocorticoids, serotonergic antidepressants, patients with ischemic stroke (Singhal AB, Topcuoglu MA. Glucocorticoid associated worsening in reversible cerebral vasoconstriction syndrome.  Neurology 2017; 88:228-236.

Transient epileptic amnesia syndrome

Butler CR, Ann Neurol 2007; 61:587-598

Another hard to diagnose syndrome. 50 patients were recruited with recurrent amnesia, other cognitive functions intact, and compelling evidence of epilepsy. 24 controls also were studied. Clinical pearls to diagnosing this include: average age 62, frequent spells (eg one a month for a year), duration 30-60 minutes, occurrence upon awakening, prompt cessation with antiepileptic drug treatment. Neurocognitively patients complained of forgetting, but did well on standard (? what) neuropsych, but with accelerated forgetting of verbal and visual material over 3 weeks and some loss of autobiographical memory. Its diagnosis is usually missed.

Ictal asystole: clinical characteristics & SUDEP

Neurology 2007; 69: 434-441.


Occurred in .27 % of patients undergoing VEEG. There was a sudden loss of atonia 42 seconds into the seizure with characteristic EEG findings of hypoperfusion. It occurred in TLE and extratemporal epilepsy. Clinical predisposing characteristics were not identified. Patients should be sent for pacemakers to avoid SUDEP.

Other predisposing factors for SUDEP are long QT syndrome, Brugada syndrome, and catecholaminergic ventricular arrythmia. Absence of treatment or insufficient treatment are neurologic risk factors. 

Carbamazepine, which can decrease heart rate variability, has been proposed as a risk factor for SUDEP.  SUDEP in most cases is triggered by a GTCS.  Four cases that were monitored during SUDEP showed EEG flattening before cardiorespiratory changes.  Another showed seizure triggered ventricular fibrillation.  Sleeping prone may be a risk factor,

Nice review article:
Tomson T, Nashef L, Ryvlin P.  Sudden unexpected death in epilepsy:  current knowledge and future directions.  Lancet Neurology 2008; 7:1021-1031.

More pearls  Kloster R. et al. JNNP 1999; 67:439-444

risk factors
male  predominance
Laying prone (17/24) usually during sleep.
At least one AED is subtherapeutic
Pulmonary edema is present in 26/42
Seizure just before death in 28/39

Alcohol levels were usually zero.

NeuroBehcet's pearls

multiple sources but NEJM CPC from May 28, 2009 was one.

1. Known as Silk Route disease due to occurrence along trading paths in Asia
2. Criteria include (major) oral ulcers occurring at least three times per year, genital ulcers or scars, ocular involvement (uveitis), skin (erythema nodosa, folliculitis, acne), positive skin pathergy test. Minor criteria are arthritis, DVT, superficial thrombophlebitis, epididymitis, positive family history, GI, CNS, or vascular involvement.
3. Neurologic involvement occurs in 5-10 %, with vascular involvement (venous sinus thrombosis or arterial thrombosis), or parenchymal infiltration. Brain stem and basal ganglia are common areas of predilection. The disease can be relapsing-remitting, chronic progressive, or secondary progressive.
4. Abnormal labs include high sed rate, CRP, complement, IL6,8 , TNF, and distinctive CSF neutrophilic pleocytosis.
5. Best treatment is not know, but in CPC authors suggested high dose pulsed steroid and cyclophosphamide, and maintenance immunoosuppression with azathioprine.

GAD 65 autoimmunity in PSP mimics

Article with reply by Mayo group in Neurology 1009 June. Original case was a 54 year old woman with supranuclear gaze plasy and parkinsonism mimicking PSP. There were stiff man like symptoms and strong GAD65 positivity. PSP was excluded in part by prominent early upgaze palsy, and startle induced spasms typical of SMS, and video ENG findings of a fatiguing pattern of saccade initiation with repetive saccades. The Mayo report (Pittock SJ, Mayo Clin Proc 2006) presented with brainstem, eps and spinal cord syndromes and were initially misdiagnosed as PSP or MSA. Pittock's group also reported OCB in CSF and improvement with prompt initiation of immunotherapy.

Superior division oculomotor paresis

Clin note in Neurology June 2009.

KEY POINTS
Clinical Patient has diplopia in vertical gaze, right ptosis, and right retroorbital pain. Exam showed ptosis, hypotropia, and elevation paresis in the right eye (looking in and up). Angio showed cavernous right ICA aneurysm that was coiled. The IIIn divides into superior and inferior divisions near SOF or anterior cavernous sinus. Clinical presentation is ptosis and superior rectus palsy.

BNP levels in neurologic disease

2 uses
1. Document cadiac valvulopathy among patients exposed to pergolide
2. Document CHF in stroke patients ie possible need for anticoagulation and cardiac evaluation

SWEDDs non Parkinson's disease

4-15 % of patients entered into trials for PD had normal presynaptic nigrostriatal dopaminergic imaging and were termed "subjects with scans without evidence of dopaminergic deficits." (SWEDD's). Withdrawal of dopamine was nondeleterious and rescanning 4 years later continued to show no deficit. Case was presented of a subject with jerky assymmetric rest and action tremor, arm dystonia, hypomimia, increased limb tone, and slow repetitive finger movements. 3/10 had abnormal thumb extension (Froment's sign in this case), none had micrographia, and none had loss of sense of smell. True bradykinesia, as defined by progressive decrement of speed and amplitude of repetitive movement (decremental bradykinesia) was not present and should be contrasted to just slowing or hesitation of voluntary movement. The action tremor occurred as the arms were held up (postural tremor).

Blogger note: differential diagnosis could include corticobasal ganglionic degeneration, difference being more rapid progression in that condition, particularly if functional imaging is not done or not available.

Small, spastic furrowed tongue of Allgrove's syndrome

Houlden H. Neurology (Neuroimages) 2009; 72:1366.

35 yo with slowly progressive achalasia, alacrima, motor neuropathy, bulbar palsy, and a small furrowed tongue. Sister had same plus adrenal deficiency (triple A syndrome). DNA analysis of ALADIN gene confirmed.

Chronic progressive myeloneuropathies

Acquired
Nutritional deficiency-- B12, folate, vitamin E, copper, nitrous oxide deficiency with impaired
B12 metabolism, AIDS myelopathy with impaired B12 metabolism
Infectious-- HTLV I, AIDS, syphilis
Inflammatory- Sjogren's, sarcoid
Geographic - toxic-- cassova toxicity, lathyrism, fluorosis, subacute optico myeloneuropathy,
tropical myeloneuropathies
Toxic-- chemo ( cisplatin, cytarabine, intrathecal therapy, organophosphate
Genetic
With metabolic abnormality: adrenomyeloneuropathy, Krabbe disease, MLD, CTX, familial
vitamin E deficiency, abetalipoproteinemia, cobalamin and foalte metabolism defects,
respiratory chain defects, APBD
Without metabolic abnormalities: HSP, SCA


Evaluation:
vitamin B12, folate, vitamin E, calcium, glucose, TSH, alkphos, lactate, pyruvate, paraneoplastic screen, arylsulfatase A, beta galactosidase, peroxisomal screen ( VLCFA, pristanic acid, phytanic acid), ACE level, ceruloplasmin, copper, ANA, cyclic citrullinated peptide antibodies, SPEP, SIEP, lyme screen, VDRL, HIV, HTLV I/II, NMO IgG, SSA, antibodies to Gad 65, AST, ALT
CSF--cells, diff, MS panel, lyme titers, JC virus pcr, and paraneoplastic screen.

APBD Adult polyglucosan body disease

Neurology 2009; 72: 1609-1613 (CPC)
Jewish diseases with leukoencephalopathy:
APBD--Ashkenazi
megelencephalic encephalopathy with subcortical cysts-- Libyan
mitochondrial neurogastrointestinal encephalomyelopathy-- Ashkenazi and Libyan
cerebrotendinous xanthomatosis-- Moroccan
metachromatic leukodystrophy-- Yemenite
Canavan disease
mucolipidosis type IV

APBD-- uniquely occurs in 50 yo with CNS and PNS involvement
AR
diagnosis with skin.fibroblasts measurement of glycogen branching enzyme activity
Genetic spectrum well beyond Ashkenazi Jews. Allelic to type 4 glycogen storage disease. Unlike glycogen storage, liver is affected in kids.

Clinical
progressive late onset spastic paraparesis, progressive, neurogenic bladder, sensorimotor neuropathy with painful feet. Cognitive involvement even dementia is common.
CSF normal
MRI progressive involvement of hemisphere and PVWM, post limb of ic, brainstem tracts, sparing of corpus callosum and u fibers. Lesions are hyperintense on T2 and not enhancing. Atrophy of spinal cord is early and invariable. Sural nerve biopsy or axillary apocrine biopsy are diagnostic of polyglucosan bodies.

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.

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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

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.

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, has killed more unarmed Palestinian civilians since September 2000 than 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.

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

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.

"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.

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.

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.

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.

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.

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)

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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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.

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.

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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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.

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

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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