Sunday, May 16, 2010

Q & A AAN 2010

COMMENTS
1.  possible association of seminoma and demyelination disorder
2.  dural av fistula lesions identified by stepwise progression and involvement of conus, angiography can miss it
3.  Rabies case of Weinshenker-- rabies is increasing in bat population, catch the bat, give everyone a shot even if they don't have a bite, only have a week or so to get a shot
4.  NMO CSF may be positive with negative serum studies, but is rare.  Antibody arises in blood and leaks into CSF, does not get produced in CSF.  Could be a question of CSF is cleaner with less noise of other antibodies in blood interfering with test.
5.  Persistent black holes at onset is a good sign of non-ADEM; rarely if ever seen all enhancing lesions with ADEM more common to see none of lesions enhance.

Superficial siderosis

check 2009 article Neurology

check cerebellar folia
do myelogram look for extradural defect
consider fixing it

ADEM and atypical demyelinating disease pearls

1. Occurs more in childhood than adulthood
2.  Occurs post infection, infection may include VZV, EBV, HSV 6, measles, influenza
3.  Acutely, all lesions enhance rather than being of different ages (MAY occur)
4.  Pathologically perivenous inflammation with very little tissue or axonal destruction
5.  Hurst's hemorrhaghic leuokoencephalitis is sometimes considered as part of spectrum of ADEM, with severe course, may be fatal, hemorrhage may be petechial, with pathological and MRI diagnosis and severe demyelination
6.  Marburg's MS -- severe and unrelenting MS even within one year. Otto Marburg, 1906
7.  Tumefactive MS--may be monophasic course or develop into MS, typical or otherwise
8.  Balo's concentric sclerosis with concentric rings of demyelination alternating with remyelination, described 1927, variable course, more common in Southeast Asia, high level if inducible nitrous oxide synthase similar to hypoxia.
9.  Isolated optic neuritis without MS--half may not progress to MS without associated MS lesions
10  CRION chronic relapsing inflammatory optic neuritis disease is restricted to optic nerves
11.  NMO spectrum disease with isolated and recurrent optic neuritis

For above, treatment algorithm is five days of solumedrol, then plasma exchange (at Mayo Clinic) then cytoxan.

Saturday, May 15, 2010

Weinshenker on Acute Myelopathies from AAN 2010 pearls

1.  Most myelopathies are undetermined cause at initial diagnosis, then infectious, then CVA, then systemic disease eg. lupus

2. NMO may have a central cord syndrome

3.  Initial functinal score and a central lesion on MRI are predictors at outcome, as is systemic disease or NMO at outcome.

4.  Paraneoplastic case with CRMP 5 in a 42 year old man with positive vep, cigar shaped faintly enhanicng lesions improved with removal of papillary thyroid cancer.  One radiographic sign not well known is owl eye sign with 2 "eyes"  suggests cancer or paraneoplastic.

5.  Cord compression can produce abnormal signal mimicking transverse myelitis clue check axials, and clinically symptoms did not progress over 3 weeks. Signet ring pattern of enhancing signal is c/w compression

6.  Case zoster leading to myelitis indistinguishable from NMO by MRI abnormalities.  Infections that cause acute myelopathy include:  Schistosomiasis (esp in Mideasterners), rabies virus, TB, lyme, syphilis, HSV, VZV, West Nile Virus, dengue, polio, coxsackie and Echovirus, actinomyces, blastomyces, >50 % none found, MAY HAVE OCB's

7. 71 yo woman with recurrent TM after 6 months, then paratonic spasms, TPO antibodies, letm, was NMO

8.  ADEM can be NMO positive and turn out to be NMO


Summary- conclusions  Algorithm: 1) is it compressive (subtle types included such as lipomatosis, spondylosis)  2)  is it really a myelopathy (parasagittal meningioma, CIDP)  3)  is it an acute presentation of a metabolic disorder (eg. B12 deficient patient exposed to nitrous oxide)  4) Is image quality and timing adequate?  (too early, too late)   5)  Is it functional?

New MRI Montalban criteria for diagnosis of multiple sclerosis

Neurology 2010; 74: 427-434.  called MAGNIMS proposal

* An MRI at any time showing dissemination in space (DIS) and showing 1 or more asymptomatic lesions enhancing and nonenhancing thus meeting criteria for dissemination in time (DIT) is sufficient to diagnose MS

*  An MRI showing DIS but without enhancing lesions, or with all lesions enhancing (thus no DIT), would require a new MRI to demonstrate additional lesions

*  An MRI at any time showing lesions but not DIT or DIS requires new MRI's

One DIS criterion: need one or more asymptomatic lesions in 2 of 4 locations considered characteristic for MS: juxtacortical (JC), periventricular (PV), infratentorial (IT), and spinal cord (SC).

Two DIT criteria:  1)  presence of one or more enhancing and nonenhancing lesions irrespective of the time of the scan and 2) presence of a new T2 and/or Gd+ lesion compared to a previous scan, irrespective of the time of the scan

The above apply only to those with CIS, ie symptomatic patients. 

fatigue components

Nocturnal jerks and phasic spasms
Nocturia multiple NGB
Depression
Deconditioning
increased energy requirements to move-- due to spasticity, balance
lots of drugs that contribute to fatigue
anemia
low vitamin levels, b12, D
temperature effects especially perimenstrual
effects of interferons. Try Naprelan, the long acting naprosyn, treximet,or pentoxifylline to prevent AE's before injections.

pearls symptoms management elliott froman

1. nocturia due to low compliance bladder-- consider DDAVP, urology eval to look at pelvic floor
2. always get urology exam to look at pelvic floor after you have gotten PVR
3. Phasic spasms are treated differently than tonic spasms or restless legs, try levitiracetam
4. check ferritin, not just in restless legs patients along with vitamin D, thyroid
5. fampridine flattens decay curve of temperature effects, fatigue
6. Vyvanse given for fatigue and cognitive slowing-- works.  hypertension uncommon but works
7.  4 AP the more truncal and postural instability, the likelier to respond to 4 AP
8.  Hold 4 AP if you have a fever, uti, sepsis due to risk of seizure
9. aceto L carnitine double effect of amantadine on fatigue, 2 nice studies, 1-2 grams bid buy bronson's vitamins online cheap, may even be able to go off provigil or amphetamines
10. tizanidine occassionally causes formed visual hallucinations not always require stopping drug, eg. "Indian shaman visiting her, like having a doctor at home"
11.  aerosolized ethyl chloride anesthetizes skin is best for injection pain.
12.  Neuropathic sensations after sensory myelitis, MS "hug" (aka anaconda sign), sharp burning rhythmic oscillating pain after defecation and sexual intercourse ("real neurologists do "heads and tails").  tried levitiracetam, it helped, think of phasic spasticity and saw dramatic benefit.  Thoracic squeezing continued clonazepam helped a little.  Used belladonna and opiate suprettes (b & o supp) completetely resolved defecation spasms.
13.  trigeminal neuralgia-- refractory-- suboccipital decompression first, then rhizotomy or gamma knife.  What are you decommpressing? Decompress but also scrape and cut trigeminal root. Decompression works better when done as first procedure.
14.  Causalgia on end of hand-- use topical compounded agents rather than systemic agents if topical and superficial-- compound almost any drug  GBN, mexilitene/clonidine/lidocaine
15.  Gait mechanics--swing limb advance is key causes toe drag, falling.   Medicare now pays for many walk aid and similar devices (fes- functional electrical stimulators).  prevent falls, decrease energy utilization
16.  poor hip thrust due to weak iliopsoas, clonus, tonic and phasic spasticity, tight gastroc,
17. genu encurvatrum (hyperextension of knee during standing) cure with AFO.  Dorsiflexion causes flexion of joint above.
18.  Edema-- everyone with weakness has it.  huge factor needs to be addressed buy at discountsurgicalstockings.com cheaply get 8-12 mm Hg "like a bilge pump" start at 15-20.  ten dollars a pair. leg is lighter and movement is aided not carrying a gallon of water in each leg.
19.  AFO pearls-- single piece (no articulation) is good for flail or dead foot.  Hinged or articulated brace are preferred more like normal movement only works if patient has some control.  FES is taking over for many. (Bioness or walkaid)
20.  Walkers-- use all wheel or all rolling walkers not ones with "skis" likes U step walker because it has adjustable tension on wheel so patient cannot festinate and has laser light can pattern gait cycle
21.  Pelvic obiquity hurts-- hip, gluteal girdle, takes energy, puts pressure on stance leg, AFO cures, also cures knee hyperextension.  If single piece need to tell orthotist how many degrees of  dorsiflexion (easier with articulated brace) start 5-7 degrees dorsiflexion with single piece
22.  Some people with ballet foot needs to start with botox before AFO for it to work
23.  Do 6 minute walk as well as 25 foot walk to test
24.  Bone loss in men and women--at demarcation of EDSS of 4 and beyond.  Get dexa scans every few years check femur hip and spine
25.  Vitamin D goal is 60-80 not lower level
26.  Dysautonomia in limbs (pain, cold, purple, edema) responds to ciolostazol (pletal) 50 bid or 100 bid affects platelets and relaxes smooth muscle.  May work in Asa resistant strokes too.  Viagra may also work for this, help acrocyanosis
27.  baclofen less sedating than tizanidine,need to monitor lft's.  Zanaflex is less sedating than tizanidine.  May use at night.
28.  hypertensive crisis may occur if stop tizanidine suddenly its an alpha two agonist big rebound effect
29.  Dantrolene last choice can be hepatotoxic need to check, different mechanism, not gaba B or alpha 2 but works on sarcoplasmic reticulum it works sometimes.
30.  Benzodiazepines are drug of choice for phasic spasticity usually clonazepam, some valium, and others GBN, levitiracetam
31.  Botox for focal spasticity especially tight heel cords before bracing
32.  Intrathecal baclofen can be dramatic-- use in patients you  really know well and can trust to do adjustments for nine months realistic expectations are key.
33.  Constipation--pay attention to fluid intake, physical activity, pelvic spasticity (benefit from antispasticity drugs or enemas), drugs  that constipate, bulking agents, softeners, mild osmotics esp magnesium, avoid harsh laxatives, dulcolax or glycerin suppositories.  Enemese plus has both glycerol, docusate (softening) and benzicaine for pain.
34.  Bladder dysfunction ubiquitous even in pediatrics 100 %. Bladder stores to do need 2 things need.  To store need competent sphincter annd relaxed detrusor, and to void need reflexive detrusor and ability to relax sphhincter.  Detrusor hyperreflexia with closed sphincter is commonest type of neurogenic bladder. Symptoms are frequency, urgency, urge leaking, and nocturia.   Dyssynergia of detrusor 2 types.  Both DSD have tight sphincter.  One has detrusor hyperreflexia, urgency, frequency and nocturia, other detrusor areflexia.  This occurs with longstanding disease and leads to high post void residuals.Take history and get a post void residual and if its high send to urologist.If PVR is less than 100 treat, if its greater > 150 send to urologist.  Couuld try relaxation, double void, triple void, vibrator, drugs like Cardura BEFORE intermittent catheterisation.  Patch has 70 % reduction of side effects.  Gel nique new rub on form of oxybutynin. Imipramine for enuresis.  Alpha one agonism ( helps tighten sphincter). If can't get urine out use alpha blocker, such as flomax.  These are sphincter drugs not bladder drugs.  DDAVP most effective drug for nocturia.  Only .1 to .4 at night to reduce urine and decrease voiding.  Don't use in daytime.  If have headache and confused, check sodium (hyponatremia).  Catheters hurt urethra.  Chronic-- consider suprapubic.  Bricker procedure or ileoostomy effective.  (diversion procedures)
35.  intimacyinstitute.com and tootimid.com are 2 online sites to discuss sex
36. Eros clitoral device helps sensation, lubrication, threshold to orgasm its 400 dollars or so. Eroscillator is wall powered three speeds. Wall powered is better need high intensity to get thresholds.  Libigel is hormone therapy for women not yet approved.

Sunday, May 09, 2010

differential diagnosis of longitudinally extensive spinal cord lesions

sarcoid
neuromyelitis optica
lupus
Sjogren's
multiple sclerosis
glioma (don't biopsy these patients deteriorate over weeks to months not days)

Pearls

evaluation includes

CSF
ESR
HIV status
CXR
MRI with contrast
B12
copper
Gallium scan for sarcoid
? Ace level
noncontrast CT chest to screen for  neurosarcoid (even in whites)

differential diagnosis of ring enhancing lesions on MRI

h/t Benjamin Greenberg AAN 2010

metastasis
abscess
glioma
lymphoma
infarction
contusion
demyelination
resolving hematoma
radiation necrosis