Abstract

Background:
Fulminant variants of multiple sclerosis (MS) are usually monophasic illnesses that lead to rapid and continuous progression of disabilities. Very high mortality and morbidity has been reported with these variants however in our series patients treated aggressively with immunotherapy have shown complete or near complete recovery.

Methods:
We report a case series of 4 patients with fulminant variants of MS seen between January 2008 and February 2011 at Shifa International Hospital, Islamabad. Data was collected retrospectively through chart review, and follow up information was obtained from clinic visits.

Results:
Four patients, three females, with mean age of 27(range 13-40) years. Onset was acute with significant progression over 2 weeks in all cases. Pertinent presenting symptoms were hemiparesis and speech difficulty in all 4 patients, 3 had visual impairment, and 2 had headache. CSF pleocytosis and raised CSF protein was seen in 3 patients each. Visual evoked potentials were abnormal in one patient. Based on the characteristic MRI findings, 3 patients were diagnosed as Balo’s concentric sclerosis and one patient with Marburg’s variant of MS. All patients were initially treated with intravenous methylprednisolone followed by immunomodulatory therapy. Two patients received intravenous immunoglobulins (IVIG) and mitoxantrone, one received IVIG only and one received mitoxantrone only. On follow up, all 4 patients had shown marked improvement with independence in activities of daily living.

Conclusion:
Aggressive treatment with IVIG and mitoxantrone resulted in significant improvement in this small case series. Early and aggressive immunosuppression should be considered in patients with fulminant variants of MS.

Key words: Fulminant, variants, multiple sclerosis, outcomes, Pakistan