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Fulminant monophasic multiple sclerosis, Marburg's type
M D Johnson1, P Lavin, W O Whetsell
1Department of Pathology, Vanderbilt University Medical Center, Nashville, Tennessee 37232-2561.
Abstract:
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.
Insights
This study details a rare case of acute monophasic multiple sclerosis (Marburg
Area of Science:
- Neurology
- Pathology
Background:
- Idiopathic pulmonary haemosiderosis is a rare condition causing iron buildup in the lungs.
- Multiple sclerosis (MS) is a chronic autoimmune disease affecting the central nervous system.
- Marburg's type multiple sclerosis is an aggressive, monophasic form of MS.
Observation:
- A 49-year-old woman with a history of idiopathic pulmonary haemosiderosis presented with rapid neurological decline.
- Clinical progression included blindness and paraplegia within three weeks.
- Magnetic resonance imaging (MRI) revealed acute demyelinating lesions in the brainstem and occipital lobe.
Findings:
- Necropsy confirmed extensive acute demyelinating plaques in the central nervous system, including the optic chiasm, periventricular white matter, and spinal cord.
- The neuropathological findings were consistent with Marburg's type multiple sclerosis.
- The co-occurrence of idiopathic pulmonary haemosiderosis and aggressive MS was noted.
Implications:
- This case highlights the severe and rapid progression possible in Marburg's type multiple sclerosis.
- Understanding the interplay between systemic conditions like pulmonary haemosiderosis and neurological diseases is crucial.
- Further research into rare MS subtypes and their pathological mechanisms is warranted.