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Callosal Disconnection Syndrome Associated with Relapsing Polychondritis
Toru Baba1, Shigenori Kanno, Tomomi Shijo
1Department of Behavioral Neurology & Cognitive Neuroscience, Tohoku University Graduate School of Medicine, Japan.
Relapsing polychondritis can cause meningoencephalitis, affecting the corpus callosum. Prompt diagnosis and immunosuppressive therapy are crucial for managing this rare neurological complication.
Area of Science:
- Neurology
- Immunology
- Rheumatology
Background:
- Relapsing polychondritis (RP) is a rare systemic autoimmune disease targeting cartilaginous tissues.
- Neurological involvement in RP, particularly encephalitis, presents with diverse symptoms based on affected brain regions.
Observation:
- This report details a 72-year-old man with RP who developed acute meningoencephalitis involving the corpus callosum.
- The patient experienced significant neurological deficits, including difficulties with bimanual tasks and dyspraxia, despite initial symptom improvement.
Findings:
- The case highlights a rare instance of callosal disconnection syndrome secondary to relapsing polychondritis-associated meningoencephalitis.
- Immunosuppressive therapy led to clinical improvement, though some motor and coordination deficits persisted.
Implications:
- Recognizing RP as a potential cause of callosal disconnection syndrome is vital for timely diagnosis and treatment.
- Early identification of neurological manifestations in RP can prevent long-term disability and improve patient outcomes.
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