FULMINANT MONOPHASIC MULTIPLE-SCLEROSIS, MARBURGS TYPE

The clinical, neuroradiological and necropsy findings are described in a 49 year old woman with long-standing idiopathic pulmonary haemosiderosis and acute monophasic multiple sclerosis (Marburg's type). Progression of the demyelinating process produced blindness and paraplegia over three weeks. At five weeks, magnetic reasonance imaging (MRI) studies showed lesions in the pons and left occipital lobe. The patient died 10 weeks after onset of symptoms. Necropsy examination revealed acute plaques in the optic chiasm, and the white matter around the lateral and fourth ventricle and spinal cord. Similarities between this and previously described cases of Marburg's disease are discussed.