Acute Disseminated Encephalomyelitis (ADEM)
Acute Disseminated Encephalomyelitis
is a rare autoimmune disease of the brain that can
attack the brain and spinal cord, leaving inflammation
and multiple lesions within the brain and spinal cord.
It's usually triggered by a viral infection or vaccination,
most often in children between the ages of 5 and 8,
and can leave long-term problems with motor skills
and cognitive abilities.
Some of the viral infections that have been thought
to trigger ADEM may include the Influenza Virus, Measles,
Mumps, Rubella, the Epstein-Barr Virus, Herpes Simplex
Virus, Hepatitis A, and the Coxsackie Virus. The vaccinations
that have been thought to trigger ADEM include vaccinations
for Rabies, Hepatitis B, Pertussis, Diphtheria, Measles,
Mumps, Rubella, Pneumococcus, Varicella, Influenza,
and Polio vaccines.
ADEM usually presents pretty quick after an infection
or vaccine, typically within 1-3 weeks. The symptoms
might be mild at first, maybe a fever, headache and
drowsiness, but within a couple hours to a couple
of days the symptoms will get worse. Seizures and
coma can be the result.
It is very important that aggressive treatment is
given quickly, to lessen any long-term damage and
disability. Most patients can fully recover from ADEM
with minor disabilities and never have another episode
(remission). Few have a return of the symptoms (relapse).
If a patient experiences additional ADEM episodes,
it is called Recurrent Disseminated Encephalomyelitis
or Multiphasic Disseminated Encephalomyelitis (MDEM).
Symptoms:
Drowsiness
Fever
Headaches
Seizures
Coma
Diagnosis:
Diagnosis is usually made using MRI to look for brain
lesions and inflammation. Typically, the lesions will
be newer lesions. If older lesions are there, the
doctors may consider an alternative diagnosis of Multiple
Sclerosis instead. Many cases of ADEM are misdiagnosed
as MS since the symptoms and signs are very similar.
Blood tests may also be used to help with diagnosis,
and may show:
Elevated Total Protein (TP)
Elevated White Blood Cell Count (WBC)
Treatment:
The goal of treatment is to quickly reduce the inflammation,
with the first treatment usually including high doses
of Corticosteroids followed by lower doses of oral
Prednisolone.
If patients do not respond to initial treatment,
or health concerns prevent the use of Corticosteroids,
the next option for treatment is usually immunosuppressive
therapies such as Plasmapheresis, high doses of intravenous
Immunoglobulin (IVIg) therapy, Mitoxantrone and Cyclophosphamide.
Prognosis:
Most patients respond well to therapies and will
recover within 1-6 months with residual motor deficits
seen in some, ranging from mild clumsiness to muscle
weakness and a weakened coordination.
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