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(ADEM)
Dr/Reyad Alfaky
Demyelinating Diseases
• Definition: Degeneration of previously normal myelin
Demyelinating Diseases
A. Central nervous syetem:
1. Acute disseminated encephalomyelitis (ADEM)
2. Multiple sclerosis(MS)
3. Neuromyelitis optica(NMO)
B. Peripheral nervous syetem:
1. Acute inflammatory demyelinating polyneuropathy (AIDP)
Definition
is a demyelinating disease of the central nervous system
that typically presents as
1. monophasic disorder
2. encephalopathy
– Usually monophasic
– Demyelinating disorder
Postvaccinial
– including :
• Enterovirus infections.
PATHOGENESIS
• Less than 5 percent of ADEM cases follow immunization.
• Japanese B encephalitis
1. Fever,
2. Headache,
3. Vomiting
4. meningismus
CLINICAL FEATURES
• febrile illness occurs in 50 to 75 percent of children
1. Encephalopathy ,
I. lethargy →coma
II. Confusion
3. Behavioral changes
4. Seizures
2. Acute hemiparesis
3. Cerebellar ataxia
4. Cranial neuropathies
5. cranial neuropathies including optic neuritis
2. movement disorders
3. sensory deficits
CLINICAL FEATURES
• New clinical symptoms may develop during
hospitalization.
• The arms can be involved if the demyelinating lesion is in the cervical cord.
• Respiratory failure may occur with high cervical lesions that extend into the brainstem.
variants of ADEM
hyperacute variants of ADEM
o These include:
• predominantly ; lymphocytosis
rubella.
• Evidence of inflammation
1. white and red blood cells
2. increased protein concentration
3. increase in myelin basic protein.
• CSF myelin basic protein concentration level, reflecting demyelination, is frequently
elevated in ADEM.
Lumbar puncture
• Some patients with ADEM have oligoclonal bands in CSF.
• Oligoclonal bands
2. viral syndromes
3. neuropathies.
The cerebrospinal fluid
• Results of CSF immune profile testing (eg, CSF:serum
immunoglobulin G [IgG] index, CNS IgG synthetic rate,
oligoclonality) employing age-appropriate normative data
are positive in fewer than 10% of prepubertal children
with ADEM
The MRI abnormalities
• The MRI abnormalities are best defined by
1. T2-weighted images
• Almost all patients have multiple lesions in the deep and subcortical
white matter, characteristic of demyelination.
• The periventricular white matter is often spared.
MRI abnormalities
• Typical lesions of ADEM include
– centrifugal at the junction of the deep cortical gray and
subcortical white matter.
– thalamus (30-40%),
– brainstem (45-55%),
– cerebellum (30-40%),
– low-density abnormalities
– but this technique is far less sensitive than MRI for the
encephalopathy
may be prolonged.
DIAGNOSIS
• The diagnosis of ADEM is based upon the
1. clinical
2. radiologic features
2. Excessive irritability
observed in ADEM,
excluded
Diagnostic Criteria for Pediatric ADEM (All Required)
Characteristic Patterns on MRI
Multiphasic ADEM
• Individuals who have experienced typical ADEM are at
risk for recurrence.
– As many as 10% of children with an initial diagnosis of ADEM
experience another ADEM attack
– typically within the first 2-8 years after the initial attack.
Monophasic ADEM
1. A single clinical episode of ADEM may evolve over as
long as three months
2. Any new and fluctuating symptoms occurring within
three months of the initial event
3. symptoms that appear during glucocorticoid taper or
4. within one month of completing a glucocorticoid
taper
Recurrent And Multiphasic ADEM
• Recurrent ADEM and multiphasic ADEM are the
two relapsing forms of the disease.
1. more than three months after the initial event
and
2. more than one month after completion of
glucocorticoids.
– By definition for ADEM, both must include a clinical
presentation with encephalopathy.
Recurrent ADEM
1. three or more months after the first ADEM event
3. The MRI must show new lesions compared with the first attack
and demonstrate complete or partial resolution of the lesions
associated with the first ADEM episode.
Differential Diagnosis
• other inflammatory demyelinating disorders should be
considered. These include:
1. Multiple sclerosis
2. Optic neuritis
3. Transverse myelitis
4. Neuromyelitis optica (Devic's disease)
5. Other rare conditions
Differential diagnosis
1. CNS Infection: Acute bacterial or viral infections
2. Mitochondrial disease
3. Leukodystrophies: Metachromatic leukodystrophy, X-linked
adrenoleukodystrophy, Alexander's disease
4. Vitamin deficiency
B12, folate
5. CNS malignancy: Lymphoma, high grade glioma
6. Granulomatous diseases
Neurosarcoidosis, Wegener's granulomatosis
7. Inflammatory disease
ADEM/MDEM, SLE, Antiphospholipid antibody
syndrome, Sjogren's disease, Behcet's disease
Diagnosis of demyelinating clinical event
ADEM: acute disseminated encephalomyelitis
MDEM: multiphase ADEM
CIS: clinically isolated syndrome
MS: multiple sclerosis
NMO: neuromyelitis optica
ON: optic neuritis
TM: transverse myelitis
MRI algorithm
TREATMENT
• It is important to first consider a treatment with
antibiotics and/or acyclovir until an infectious cause is
ruled out
• A high dose of intravenous corticosteroids, for 3-5 days
is the primary and most common first treatment of
ADEM
TREATMENT
1. high-dose intravenous (IV) glucocorticoids.
2. intravenous immune globulin
3. plasma exchange
ADEM: Treatment
• High Dose Steroids
– High dose IV Methyl Prednisolone 30 mg/kg/day for 3-5
days
• IVIG (<1 yr, No improvement in 48-72 hrs , AHLE, Recurrent)
– 1 gm/kg/day iv for 2 days
• Plasmapheresis
• Symptomatic Rx
TREATMENT
• For children with ADEM, we recommend
immunosuppressive treatment
• We suggest initial therapy with high-dose glucocorticoids
• IV methylprednisolone (30 mg/kg per day, up to a
maximum dose of 1000 mg per day) for five days without
a taper.
TREATMENT
• For children with ADEM who have an insufficient response to IV
globulin treatment
• Those children who do have residual symptoms are reported to have symptoms
from:
• Transverse myelitis
• Recurrent headaches
• Behavioral problems
PROGNOSIS
• Long-term clinical follow-up and sequential imaging by
MRI are normally required to confirm a diagnosis of
ADEM.
PROGNOSIS
• Development of a relapse with new lesions, it is not
compatible with a diagnosis of monophasic ADEM
PROGNOSIS
• Depending on the clinical and imaging features, it likely
suggests the correct diagnosis being either multiphasic
ADEM or MS.
PROGNOSIS
• Depending on the clinical and imaging features, it likely
suggests the correct diagnosis being either multiphasic
ADEM or MS.
PROGNOSIS
• The prognosis for survival and recovery of
neurologic function is worse for the hyperacute
hemorrhage variants of ADEM, such as acute
hemorrhagic leukoencephalitis, than for typical
ADEM
PROGNOSIS
• Complete recovery in 10 (77%) of the survivors
• Relapsing disease in 2 (15%)
• Mortality less than 2%
– fulminant cervical transverse myelitis or brain
swelling. Children younger than 2 years
• Acute disseminated encephalomyelitis
• Presents with
– Altered mental status/encephalopathy
(irritability to obtunded)
– Acute/subacute onset of focal symptoms
based on lesions
(max neuro symptoms over 4-7 days)
– Typically still during febrile illness
(typically URI)
– 1/3 with seizures
• Workup
– Labs: none are diagnostic
– CSF: pleocytosis, elevated protein, elevated IgG index; rule out infection!
– Imaging: MRIbrain with contrast
• Treatment: IV steroids (ok to start while r/o infection and prophylactic antibiotics
are on board) > IVIg > PLEX; PT
• Outcome
– Recovery over 4-6 weeks- 60-90% with no residual defecits
– Repeat MRI 6-12 months later to assess for lesion resolution