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Relapsing Acute Disseminated Encephalomyelitis (ADEM) 01/09/2014 00:00:00

Relapsing Acute Disseminated Encephalomyelitis (ADEM)

Karuna Thapar, Gaurav Dhawan.
Department of Pediatrics, Government Medical College, Amritsar.

Dr Karuna Thapar, Department of Pediatrics, Government Medical College, Amritsar, India.
ADEM is immunologically mediated inflammatory demyelinating disease of CNS. Highest prevalence is seen in prepubertal boys. Clinical manifestations include abrupt development of irritability and neurological signs in children recovering from a viral prodrome. Changes in long tract signs and mental status are commonly observed. We report a case of ADEM with relapse treated effectively with corticosteroid therapy.
Case Report
A 7 years old male child presented with intermittent fever for 25 days, headache for 15 days, irritability, excessive crying, vomiting, and pain abdomen for 5 days. On examination vitals were normal, neck rigidity was present, pupils were bilaterally reacting, tone was normal and reflexes were present. Hemogram, renal functions, liver functions, Electrocardiogram and Chest X-ray were normal. CSF analyses including viral cultures were normal. MRI revealed few small hyper intense lesions in the sub cortical white matter of cerebral hemispheres, left caudate nucleus and rostrum of corpus callosum on right side suggestive of acute disseminated encephalomyelitis. He was treated with intravenous methyl prednisolone for 3 days with which he showed slight improvement and was discharged on oral steroids. The child was readmitted within three months with complaints of decreased vision for 15 days, headache for 10 days, difficulty in micturition for 4 days and irrelevant talks for 1 day. MRI brain revealed that the previous lesions had increased in number and size with involvement of the basal ganglia and the brainstem. The child was given intravenous methyl prednisolone (30mg/kg/day) for three days and then started on oral methyl prednisolone (1mg/kg/day) for two weeks. The child showed dramatic improvement to this therapy and led to resolution of all his neurological deficits. Follow up for 12 months revealed no neurological deterioration.
ADEM is an uncommon disease. In most patients it follows a viral illness but has been reported after bacterial infection, immunizations, and drug and serum administration (1,2,3).

The clinical syndrome is variable but can range from focal neurological deficits to coma and death. Typically, ADEM is characterized by an abrupt onset of fever, obtundation, seizures, and focal neurological signs (2). Obtundation may progress to coma and up to 20% of patients die.

The diagnosis is often confirmed by MRI, which shows multifocal white matter lesions corresponding to the abnormal neurological findings (4,5,6,7). Diagnosis in this case was made on the basis of clinical picture and MRI features.

Although ADEM is a monophasic disease, relapses have been described mainly as case reports (8-13). To qualify as definite relapse, the new clinical episode required at least an interval of 1 month from the initial symptom (14), in addition to different symptoms and radiologic evidence of new lesions at a different site (11,14). The concept of definite relapse was considered to differentiate biphasic ADEM (10-12,15,16) from patients with steroid dependence that shows reactivation of the same lesions (13,16,17). The authors think that the presented case qualifies for a relapse as new clinical episode occurred at an interval of more than 1 month, with some different symptoms, and radiologic evidence of new lesions at new site.

Differential diagnosis of Multiple Sclerosis (MS) was also kept by the authors but male predominance, relapse timing of less than 6 months, occurring shortly after cessation of corticosteroids, only one relapse observed, and MRI findings of hyperintense lesions, grey matter involvement pointed more towards biphasic ADEM than MS (18).

There is no standard treatment for ADEM though corticosteroid is the most frequently reported therapy, improving recovery in most patients (8,19). Clinical response is usually evident within hours of initiation of treatment, particularly after pulsed IV corticosteroids (20). Many patients have shown rapid improvement with prednisone. The rationale for corticosteroid use is their ability to reduce inflammation, decrease edema, and seal the blood-brain barrier, which should decrease the further influx of active immune cells and humoral factors, contributing to demyelination. The main therapeutic options for ADEM are corticosteroids, plasma exchange, and intravenous immunoglobulin. Plasma exchange and intravenous immunoglobulins were not used in our patient because the patient showed dramatic improvement with corticosteroids.

We think our patient represents a rare case of relapsing (biphasic) ADEM treated effectively with corticosteroids.
Compliance with Ethical Standards
Funding None
Conflict of Interest None
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