High dose cyclophosphamide treatment in Marburg variant multiple sclerosis A case report.
Marburg variant multiple sclerosis (MS) is an acute, fulminant and monophasic variant of MS that usually leads to death within weeks to months. No consistently successful treatment is known. We describe a 26-year-old woman who developed acute and progressive motor and sensory deficits. Demyelinating disease was suspected based on brain and spinal MRI and cerebrospinal fluid results. Multiple treatments including corticosteroids, plasma exchange and intravenous immunoglobulin could not halt her clinical and radiological deterioration. She became near quadriplegic and developed motor aphasia. A diagnosis of Marburg variant MS was considered and she was given high dose cyclophosphamide (HiCy) at 50mg/kg/day for four consecutive days, followed by granulocyte colony-stimulating factor six days after the completion of the cyclophosphamide treatment. HiCy successfully induced neutropenia. She started to show a steady neurological improvement from day 17 of HiCy treatment. MR studies two months after HiCy treatment showed significant decrease in the size and enhancement of the lesions. Five months later she had minimal residual right-sided weakness and was able to ambulate without assistance. The great outcome seen in our case suggests that HiCy should be considered as a potential treatment for patients with Marburg variant MS who fail to respond to standard therapy.
Nozaki
Kenkichi
K
Division of Adult Neurology, Department of Neurosciences, Medical University of South Carolina, Charleston, South Carolina, USA.
Abou-Fayssal
Nada
N
D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000328:Adult; D003520:Cyclophosphamide / Q000627:therapeutic use*; D005260:Female; D016178:Granulocyte-Macrophage Colony-Stimulating Factor / Q000627:therapeutic use; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D008279:Magnetic Resonance Imaging; D009103:Multiple Sclerosis / Q000188:drug therapy* / Q000473:pathology; D010951:Plasma Exchange; D012600:Scoliosis / Q000209:etiology; D013116:Spinal Cord / Q000473:pathology; D016896:Treatment Outcome
D000305:Adrenal Cortex Hormones; D016756:Immunoglobulins, Intravenous; D007166:Immunosuppressive Agents; D016178:Granulocyte-Macrophage Colony-Stimulating Factor; D003520:Cyclophosphamide