What is Acute Disseminated
Encephalomyelitis?
Acute disseminated encephalomyelitis (ADEM)
is characterized by a brief but intense
attack of inflammation in the brain and
spinal cord that damages myelin – the
protective covering of nerve fibers. It
often follows viral infection, or less
often, vaccination for measles, mumps, or
rubella. The symptoms of ADEM come on
quickly, beginning with encephalitis-like
symptoms such as fever, fatigue, headache,
nausea and vomiting, and in severe cases,
seizures and coma. It may also damage white
matter (brain tissue that takes its name
from the white color of myelin), leading to
neurological symptoms such as visual loss
(due to inflammation of the optic nerve) in
one or both eyes, weakness even to the point
of paralysis, and difficulty coordinating
voluntary muscle movements (such as those
used in walking). ADEM is sometimes
misdiagnosed as a severe first attack of
multiple sclerosis (MS), since some of the
symptoms of the two disorders, particularly
those caused by white matter injury, may be
similar. However, ADEM usually has symptoms
of encephalitis (such as fever or coma), as
well as symptoms of myelin damage (visual
loss, paralysis), as opposed to MS, which
doesn’t have encephalitis symptoms. In
addition, ADEM usually consists of a single
episode or attack, while MS features many
attacks over the course of time. Doctors
will often use imaging techniques, such as
MRI (magnetic resonance imaging), to search
for old and new lesions (areas of damage) on
the brain. Old “inactive” brain lesions on
MRI suggest that the condition may be MS
rather than ADEM, since MS often causes
brain lesions before symptoms become
obvious. In rare situations, brain biopsy
may show findings that allow differentiation
between ADEM and severe, acute forms of MS.
Children are more likely than adults to have
ADEM.
MRI showing inflammation
Is there any treatment?
Treatment for ADEM is targeted at
suppressing inflammation in the brain
using anti-inflammatory drugs. Most
individuals respond to intravenous
corticosteroids such as
methylprednisolone. When
corticosteroids fail to work,
plasmapheresis or intravenous
immunoglobulin therapy has been shown to
produce improvement. Additional
treatment is symptomatic and
supportive. Current treatments are
provided in our E-Book.
What is the prognosis?
Corticosteroid therapy can shorten the
duration of neurological symptoms and
halt further progression of the disease
in the short term, but the long term
prognosis for individuals with ADEM
varies. For most, recovery begins
within days, and half will recover
completely. Others may have mild to
moderate lifelong impairment. Severe
cases of ADEM can be fatal. Some
individuals who initially diagnosed as
having ADEM will later be reclassified
as MS, but there is currently no method
to determine whom those individuals will
be.