Case of the Week
Section Editors: Matylda Machnowska1 and Anvita Pauranik2
1University of Toronto, Toronto, Ontario, Canada
2BC Children's Hospital, University of British Columbia, Vancouver, British Columbia, Canada
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July 14, 2016
Bulbar and Spinal Cord Marburg Disease
- Background:
- Marburg disease is an acute, fulminant, monophasic variant of multiple sclerosis in which the diagnosis may be difficult due to the absence of previous neurologic symptoms. Necropsy shows extensive axonal loss and necrosis.
- Clinical Presentation:
- This entity is characterized by rapid progression that typically leads to death in 12 months. There is a monophasic course, with slowly progressive motor and sensory involvement, leading to death from brain stem involvement-related complications.
- Key Diagnostic Features:
- Confluent white matter hyperintensities on T2 and FLAIR sequences. Lesions can be small or large, irregular or confluent, affecting any part of the neuroaxis.
- Lesions usually enhance but can also be a mild or absent enhancement.
- We could not find other cases of isolated bulb and spinal cord involvement reported in the literature.
- Most cases are diagnosed in autopsy studies.
- Differential Diagnoses:
- Diffuse astrocytoma: Enhancing infiltrating mass expanding cord, usually less than 4 segments. The cervical spinal cord is affected more than the thoracic. Rapid progression is not expected.
- ADEM (acute disseminated encephalomyelitis): More common in children and young adults. It usually follows an upper respiratory tract infection. Supratentorial parenchymal involvement should be present. Has a good response to steroids, usually leaving little or no symptoms.
- Transverse myelitis: Imaging characteristics may be similar. The diagnosis criteria includes a disease less than 21 days.
- Neuromyelitis optica: Bilateral optic neuritis should be seen with longitudinal extensive myelitis; the disease is most often caused by autoantibodies to aquaporin-4.
- Venous congestive myelopathy: Flow voids are the key for the diagnosis of spinal AV shunts.
- Treatment:
- Steroids, immunosuppressive therapy
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