ACUTE DISSEMINATED ENCEPHALOMYELITIS (ADEM): A CASE REPORT OF A FAVOURABLE RESPONSE TO DELAYED ADMINISTRATION OF ORAL PREDNISOLONE FOLLOWING POOR RESPONSE TO TREATMENT WITH INTRAVENOUS IMMUNOGLOBULINS (IVIG)

Objectives: To report a novel case of ADEM showing temporal improvement following delayed oral steroid treatment after an initial poor response with IVIG.

Methods: The case report of a 2 year 8 month old boy with ADEM who presented to our centre in February 2004 is described.

Results: A 2 year 8 month boy presented with decerebrate posturing and generalized dystonia, requiring ventilation following a short bout of upper respiratory tract symptoms. MRI Brain showed extensive patchy, asymmetrical hyperintense lesions in T2W and FLAIR sequences in the white matter involving cerebral cortex, brainstem, cerebellum and deep gray matter. He was initially treated with IVIG but had poor response. He was extubated after a week of ventilation but remained severely impaired. With no further progress over the next 2 months, he was treated with oral Prednisolone. He improved dramatically within 2 weeks of initiating steroid therapy. His severe dystonia markedly improved, cortical blindness recovered and he came off nasogastric feeding. He began to interact with family members and could obey simple commands.1year 6 months later, he has residual triplegia sparing his right arm. He is able to sit independently and walk with support. His expressive speech lags significantly behind his receptive speech, but he continues to make slow progress in his milestones.

Conclusion: It is well established that steroids or immunoglobulins should be administered as early as possible in cases of ADEM. However, this case demonstrates that there is possibly a longer window period of response to treatment with steroids, in cases of ADEM, then previously known or reported.