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

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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