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Progressive solitary sclerosis presented with diplopia: A case report
Mohammad Ali Sahraian1, Masoud Ghiasian2, Abdorreza Naser Moghadasi1
1MS Research Center, Neuroscience institute, Tehran University of Medical sciences, Tehran, Iran.
Multiple Sclerosis and Related Disorders
|December 31, 2018
Summary
This case study highlights progressive solitary sclerosis, a rare condition causing progressive quadriparesis after diplopia. Despite extensive workups, the solitary pontomedullary lesion persisted, showing slow progression over six years.
Area of Science:
- Neuroscience
- Neurology
- Demyelinating Diseases
Background:
- Diplopia and progressive quadriparesis are significant neurological symptoms.
- Solitary pontomedullary lesions require careful differential diagnosis.
- Demyelinating diseases can present with varied clinical courses.
Observation:
- A 24-year-old woman experienced transient diplopia followed by progressive quadriparesis.
- A single, persistent pontomedullary lesion was identified on imaging.
- Extensive diagnostic workup yielded no other abnormalities.
- The lesion remained stable in appearance over a 6-year follow-up despite clinical deterioration.
Findings:
- The clinical presentation and imaging findings were consistent with progressive solitary sclerosis.
- Standard treatments like corticosteroid pulse therapy and rituximab showed limited efficacy.
- Partial improvement was observed after the addition of cyclophosphamide.
Implications:
- Progressive solitary sclerosis can mimic multiple sclerosis but without dissemination in time and space.
- This rare entity should be considered in the differential diagnosis of demyelinating lesions presenting as solitary brainstem lesions.
- Long-term monitoring and tailored immunosuppressive therapy may be necessary for managing this condition.
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