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

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.

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.

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.

Encephalitis serological evaluation

Hsve
Cmv
Hiv 1 and 2
Vzv
Ebv
Toxo
Lyme
Mycoplasma pn
Leptospirosis
Legionella
Brucellosis
Chlamydia
Syphilis
Aspergillosis