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[Recurrent coma, papular mucinosis and benign dysglobulinemia]
A Verier1, J P Jouet, J P Muller
1Service de Clinique Neurologique, Lille.
Revue Neurologique
|January 1, 1987
Summary
Recurrent comas linked to papular mucinosis may stem from immune system dysregulation and blood hyperviscosity. Early plasmapheresis shows promise in preventing these neurological events.
Area of Science:
- Neurology
- Immunology
- Dermatology
Background:
- Presents a rare case of recurrent comas in a woman with IgG lambda benign monoclonal dysglobulinemia and papular mucinosis.
- Reviews six similar cases, noting coma onset often follows skin lesion exacerbation and is preceded by flu-like symptoms.
Observation:
- The patient experienced three comas between ages 52-57, with preceding influenza-like illness, asthenia, vertigo, and seizures.
- Recovery involved hallucinations and transient hepatic issues; pruritus and hypereosinophilia preceded skin regression.
Findings:
- These comas, potentially fatal in 2/7 cases, typically resolve within 2-20 days without sequelae.
- Evidence suggests an immunologic origin, possibly due to paraprotein-induced blood hyperviscosity affecting brain function.
Implications:
- Blood hyperviscosity, from paraprotein interactions or altered red blood cell deformability, is a likely mechanism.
- Treatment with immunosuppressants and plasmapheresis is recommended; early plasmapheresis may prevent coma recurrence.