ADEM- Acute Disseminated

Encephalomyelitis
• initial inflammatory, demyelinating event

• with multifocal neurologic deficits

• typically accompanied by encephalopathy

• occur at any age

• mean age between 5 and 8 yr with a slight male predominance

PATHOGENESIS
• Molecular mimicry induced by infectious exposure or vaccine may

trigger production of CNS autoantigens.

• transient febrile illness in the month prior to ADEM onset.

• Preceding infections associated with ADEM include influenza,

Epstein-Barr virus, cytomegalovirus, varicella, enterovirus, measles,

mumps, rubella, herpes simplex, and Mycoplasma pneumoniae.

CLINICAL MANIFESTATIONS
• Initial symptoms of ADEM may include lethargy, fever, headache,
vomiting, meningeal signs, and seizures, including status epilepticus.

• Encephalopathy is a hallmark of ADEM, ranging from ongoing confusion to

persistent irritability to coma.

• Focal neurologic deficit can be difficult to ascertain in the obtunded

• Common neurologic signs in ADEM include visual loss, cranial

neuropathies, ataxia, motor and sensory deficits, plus bladder/bowel
dysfunction with concurrent spinal cord demyelinations
DIAGNOSIS / LAB FINDIGS

• CSF studies often exhibit pleocytosis with lymphocytic or monocytic

predominance.

• CSF protein can be elevated, especially on repeat studies.

• Up to 10% of patients with ADEM have oligoclonal bands in the CSF

and/or elevated CSF immune globulin production.

• Patients with ADEM may occasionally demonstrate antibodies against

myelin oligodendrocyte glycoprotein, or anti–N-methyl-d-aspartate
receptor antibodies.
DIAGNOSIS / NEUROIMAGING
• Head CT may be normal or show hypodense regions.

• MRI Brain :typically exhibits large, multifocal and sometimes

confluent or large edematous mass-like tumefactive T2 lesions
with variable enhancement within white and often gray matter
of the cerebral hemispheres, cerebellum, and brainstem.

• Deep gray-matter structures (thalami, basal ganglia) are often

involved, although this may not be specific to ADEM.
Axial T2-weighted fFLAIR
• Serial MRI imaging 3-12 mo following ADEM shows
MRI of the brain in a child with acute
improvement and often complete resolution of T2 disseminated encephalomyelitis.
abnormalities, although residual gliosis may remain. High signal (white) lesions in the T2-
weighted image reflect areas of
demyelination and edema in deep
subcortical and periventricular white
matter as well as the basal ganglia and
thalamus on the left side
DIFFERENTIAL DIAGNOSIS

• new or enlarging T2 lesions should prompt re-evaluation for other

etiologies such as
• MS,

• leukodystrophies,

• tumor, vasculitis, or

• mitochondrial, metabolic, or rheumatologic disorders

TREATMENT
• High-dose intravenous steroids are commonly employed
• Methylprednisolone 20-30 mg/kg per day for 5 days with a maximum
dose of 1,000 mg per day).
• Oral prednisone taper over 1 mo may prevent relapse.
• Other treatment options include
• Intravenous immune globulin IVIG (usually 2 g/kg administered over 2-5
days) or
• Plasmapheresis (typically 5-7 exchanges administered every other day).
• In severe cases of suspected ADEM, rituximab or cyclophosphamide have
been used
PROGNOSIS

• Many children experience full recovery after ADEM but

• some are left with residual motor and/or cognitive deficits.

• ADEM is usually a monophasic illness

• but demyelinating symptoms can fluctuate for several months.

• Repeated bouts of demyelination more than 3 mo after ADEM later

raise the question of MS