Wednesday, June 04, 2014

ADEM pearls



old paper, useful insights
Menge T, Hemmer B, Nessler S et al  Acute disseminated encephalomyelitis: an update. Arch Neurol 2005; 62: 1673-1680
 
1.  Consider a temporal relationship to a vaccine or infection.  If vaccine, especially MMR, also polio and European tick borne encephalitis vaccination. Many organisms are related, but temporal relationship is almost always within 3 months.  Average latency however is 4-13 days.

2.  No sex preponderance in ADEM.  Also, although it occurs most in children, adults of any age can ge the disease.

3.  Measles vaccine associated ADEM is 10-20 / 100,000 whereas ADEM after measles encephalitis is 100 per 100,000.

4.  Upper respiratory infections (URI's) with fever occur in 50-75% of cases.


5.  Children present with fever and headaches, adults with motor and sensory deficits.

6.  Bilateral optic neuritis appears to be associated with chicken pox and has a less polysymptomatic course.

7.  12.5 % of kids , and 37-58% of adults may have OCB's, these often are transient. 

8.  apl AB syndrome may mimic ADEM in kids

9.  Flareups while tapering medication eg. steroids should be regarded as flare ups of the initial monophasic courese (multiphasic disseminated encephalomyelitis or MDEM) not as MS which is the chief dDx of ADEM

10. Authors propose pulse iv steroids for 3-5 days, followed by prolonged oral prednisone taper over 3-6 weeks. Second line is plasma exchange, third line is immunosupression, cyclophosphamide or mitoxantrone. 

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