Wednesday, June 01, 2016

Froin's syndrome


CSF showing high protein, xanthochromia and hypercoagulation of CSF is pathognomonic and it can occur with blockage of CSF by a spinal cord mass or meningeal irritation from meningitis.

Tuesday, February 23, 2016

Orthostatic tremor - pearls

Hassan A, Ahlskog AE, Matsumotos JY.  Orthostatic tremor: clinical, electrophysiologic and treatment findings in 184 patients.  Neurology 2016; 86: 458-464.

The article is a Mayo series of 184 patients seen over 37 years. 

Definition: lower body tremor activated on standing, absent when seated or lying, improved by walking or leaning.  Patients report leg shaking, unsteadiness and imbalance.  Electrophysiologic findings are unique:  a 13-18 hz tremor of lower limbs or trunk. 

Demographics:  64 percent were female, mean age 59 years, (range 13-88) .  One hundred percent reported symptoms only when standing and absent while seated.  Descriptions included "imbalance, unsteadiness, weakness, 'funny feeling,' 'jelly legs,' leg tightness, pain, tremor, shakiness, or quivering.  Sixty percent of cases included the arms.  24 percent had falls.  28 percent had other types of tremors included ET, which could be associated with a response to alcohol.  Other associated tremors included head tremor  (1), handwriting tremor (2), functional (1), jaw tremor (1).  Forty percent had other neurologic diseases including Parkinson's and many others, degenerative or otherwise.  Nine patients had a family history of orthostatic tremor. 

Medication responsiveness occurred in 139 patients.  Sixty seven percent of medications prescribed did not demonstrate benefit.  The most efficacious was benzodiazepines, especially clonazepam, with 48 percent of patients so treated showing moderate or marked benefit.  Thirty three percent of patients given gabapentin showed mild or moderate benefit, with lower responses to valproate, primidone, levodopa (only 1/33 responding), and no benefit from anxiolytics or antidepressants.  The responsiveness to clonazepam diminished over time , and of those only a few, 16 got benefit from another drug, including gabapentin, valproic acid, propranolol, pramipexole, bromazepam, primidone, carbi-levodopa, and pregabalin.  Three patients underwent DBS and improved.

There was a high personal anf family history of PD (8.9 and 10.7 percent). 

EMG is easy and pathognomonic.  MRI's often show various types of white matter disease and occasional meningiomas.

Sunday, January 03, 2016

Pellagra and spinal myoclonus

Park K, Oeda T, Sawada H. A case of alcoholic pellegra encephalopathy presenting with spinal myoclonus.  Neurology Clinical Practice 5; 472-3.
 
The authors present a case of alcoholic pellagra  with confusion and myoclonus responding dramatically to administration of niacin1500 mg per day starting 16 days after admission.  Essential points include:
 
1. Pellagra is rare in US but not in alcoholics
2. Dermatitis may be subtle and not appreciated
3.  Thiamine and niacin levels may be normal
4.  Thiamine may cause worsening due to increased demand for niacin
5.  Myoclonus in context is important to diagnosis, often stimulus sensitive
6.  Severe sensory ataxia, incontinence and dysautonomia also occur and improve with treatment
 
the 4 D's of pellagra, again, are , diarrhea, dementia, dermatitis and death

Scurvy and Neurologic disease

Meisal K, Daggubati S,Josephson SA. .  Scurvy in the 21st century?  Vitamin C deficiency presenting to the neurologist.  Neurol Clin Prac 2015; 5:491-493.
 
Authors present a series of cases with vitamin C deficiency and review some of the neuro manifestations and non neuro manifestations, ; the former are not widely known. 
 
Patients with deficiency were caused by various other causes,including autism, poor status without access to produce, usually rural, were not alcohol users, had measurable low vitamin C levels.  Gingival hyperplasia, rash and bleeding were non neurologic manifestations. People bruised,especially on their thighs, Some had other nutritional diseases..  Pain, achiness and weight loss are expected.
 
Neuro manifestations included positional tremor, neuralgias100 %), focal weakness (50 %)  including footdrop and scapular winging, normal MRI's, long tract signs including hyperreflexia and plantar extensors, fatigue, trouble concentrating, headache, anxiety, and imbalance.
 
Patients recovered dramatically with treatment.

Malignant subtypes of Parkinsons

JAMA Neurology august 2015
Importance  There is increasing evidence that Parkinson disease (PD) is heterogeneous in its clinical presentation and prognosis. Defining subtypes of PD is needed to better understand underlying mechanisms, predict disease course, and eventually design more efficient personalized management strategies.
Objectives  To identify clinical subtypes of PD, compare the prognosis and progression rate between PD phenotypes, and compare the ability to predict prognosis in our subtypes and those from previously published clustering solutions.
Design, Setting, and Participants  Prospective cohort study. The cohorts were from 2 movement disorders clinics in Montreal, Quebec, Canada (patients were enrolled during the period from 2005 to 2013). A total of 113 patients with idiopathic PD were enrolled. A comprehensive spectrum of motor and nonmotor features (motor severity, motor complications, motor subtypes, quantitative motor tests, autonomic and psychiatric manifestations, olfaction, color vision, sleep parameters, and neurocognitive testing) were assessed at baseline. After a mean follow-up time of 4.5 years, 76 patients were reassessed. In addition to reanalysis of baseline variables, a global composite outcome was created by merging standardized scores for motor symptoms, motor signs, cognitive function, and other nonmotor manifestations.
Main Outcomes and Measures  Changes in the quintiles of the global composite outcome and its components were compared between different subtypes.
Results  The best cluster solution found was based on orthostatic hypotension, mild cognitive impairment, rapid eye movement sleep behavior disorder (RBD), depression, anxiety, and Unified Parkinson’s Disease Rating Scale Part II and Part III scores at baseline. Three subtypes were defined as mainly motor/slow progression, diffuse/malignant, and intermediate. Despite similar age and disease duration, patients with the diffuse/malignant phenotype were more likely to have mild cognitive impairment, orthostatic hypotension, and RBD at baseline, and at prospective follow-up, they showed a more rapid progression in cognition (odds ratio [OR], 8.7 [95% CI, 4.0-18.7]; P < .001), other nonmotor symptoms (OR, 10.0 [95% CI, 4.3-23.2]; P < .001), motor signs (OR, 4.1 [95% CI, 1.8-9.1]; P = .001), motor symptoms (OR, 2.9 [95% CI, 1.3-6.2]; P < .01), and the global composite outcome (OR, 8.0 [95% CI, 3.7-17.7]; P < .001).
Conclusions and Relevance  It is recommended to screen patients with PD for mild cognitive impairment, orthostatic hypotension, and RBD even at baseline visits. These nonmotor features identify a diffuse/malignant subgroup of patients with PD for whom the most rapid progression rate could be expected.

Friday, August 21, 2015

Drug choices for juvenile myoclonic epilepsy

valproic acid
topiramate
lamotrigine
levetiracetam
zonisamide


note these are the "broad spectrum drugs"
also note: valproic acid and topiramate are teratogenic

AED's and psychiatric function

Psychiatric function worse:


levetiracetam
topiramate
zonisamide
tiagabine
phenobarbital
periampanel


psychiatric function better


carbamazepine
valproic acid
lamotrigine
pregabalin

AED's compared head to head to standard therapy eg. carbamazepine

note this is a test done by EU>> FDA


favorably compare: 


oxcarbazepine
eslicarbazepine
lamotrigine
gabapentin
topiramate
levetiracetam
zonisamide


unfavorably compare (are inferior)


pregabalin
vigabatrin


test not done


tiagabine
lacosamide
ezogabine
perampanel

Enzyme inducers-- pearls

enzyme inducers adverse effects (partial) include


1.  decrease efficacy of oral contraception
2.  osteomalacia
3.  halve dose of many drugs, rendering them ineffective; this includes chemotherapeutic agents for children having CLL who have greater mortality on these drugs
4.  increase cholesterol
5.  Decrease testicular size

Narrow and broad spectrum antiepileptic drugs

Narrow spectrum


carbamazepine
oxcarbazepine
tiagabine
gabapentin
pregabalin


Broad spectrum


valproic acid
topiramate
lamotrigine
levetiracetam
zonisamide
parampanel

New Epilepsy classification

I  Genetic
II Focal
    a. Aware
    b  Unaware
III Mixed
IV Unknown
V Secondary generalized

Saturday, April 18, 2015

IV valproate inferior for acute migraine

Friedman BW, Garber L, Yoon A, et al.  Randomized trial of iv valproate vs.metoclopramide v. ketorolac for acute migraine.  Neurology 2014; 82:976-983.
 
Authors randomized 330 patients in ER to get 1000 mg, 10 mg, or 30 mg of respective drugs above over an iv drip over 15 minutes in a double blind trial.  On the primary measure of pain relief, valproate lost big to the other two drugs.  On secondary measures of needing a rescue medication, iv valproate also lost.
 
Comment-- great to have this knowledge but the two winning drugs each had relatively low sustained headache relief, 4 v. 11 v. 16 % with respective drugs above.  Also metoclopramide made people feel restless.

Wednesday, April 01, 2015

GPDS and NCSE

Foreman BM, Chassen J, Abou Khaled K, et al. Generalized periodic discharges in the critically ill:  a case control study of 200 patients.  Neurology 2012; 79:1951-1960
 
and editorial Jette N, Mosely BD. Generalized periodic discharges : More light shed on the old GPEDs Neurology 2012; 79: 1940-1.
 
Authors found GPD's in 4.5 % of 3000 patients undergoing cEEG.  These 200 patients  had brain injury (44%), acute systemic illness (38%), cardiac arrest (15%) and epilepsy (3%). 
 
27 % of GPD's had NCSE v. 8 % of controls.  However, GPD's were not associated with convulsive seizures. 
 
Authors/editorial notes that the distinction and semiology of GPD's v. triphasic waves is "challenging" even for board certified epileptologists. 
 
Take home messages, per the editorial are:
1) Patients with GPD's on routine EEG should undergo cEEG
2)  NCSE should be promptly treated when diagnosed to prevent mortality
3) Standard terminology and interrater reliability should be assessed within institutional readers.

Wednesday, March 25, 2015

code for tpa

If you physically administer tpa; code 37195
If you are present evaluating a acute stroke, bill as highest level code (if you meet all the 'bullet points').
Add a statment "patient is critical and unstable'; document time spent; if it is 30-74 min; add 99291

Idiopathic hypertrophic pachymeningitis

 
  Dumont AS, Clark AWm Sevick RJ, Myles ST. Idiopathic hypertrophic pachymeningitis:  A report of two cases and review of the literature.
 
Background-- Authors note entity was described by Charcot and Joffroy, and that there are three forms:  spinal, intracranial and craniospinal (latter is rarer). 
 
Past cases were often attributed to specific etiologies but most recent cases are idiopathic after investigation.  Authors case 1 underwent 2 surgeries for biopsy/decompression , had persistent pain and numbness, but was non progressive for 15 years after one early relapse.  The second one received steroids after biopsy with resolution of symptoms and MRI changes.  Authors argue based on above that the condition is not autoimmune. 
 
Literature review suggested a worse prognosis for patients with "inflammatory signs" (fever, high sed rate, CRP, or elevated WBC). 
 
Associations include infections: syphilis, TB, HTLV-1, fungi; may be presenting sign of adjacent ear or sinus infection.  Autoimmune associations include RA, orbital pseudotumor, multifocal fibrosclerosis, MCTD, Wegener's granulomatosis.    The 23 reports run the gamut from marked to slight improvement to deterioration and late recurrence or surgical death. 
 
For interest, Charcot's clinical descriptors divided the spinal form into distinct stages: first intermittent radicular pain that later became continuous; then muscle weakness and atrophy; then spastic paralysis and loss of sphincter control.  Radicular signs can be confined to the upper extremities and evolve over weeks to months or even a year. 
 
The cranial form frequently presents with a headache, cranial neuropathies and ataxia. 
 
Authors emphasize the need for pathologic confirmation.