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Monoclonal paraproteinemia with subacute encephalopathy, seizures, and scleromyxedema
Neurology
|June 1, 1987
Abstract:
For 3 months, a 37-year-old man developed memory loss and personality changes, followed by fever, seizures, bilateral upper motor neuron signs, and increased CSF protein with skin induration that was compatible with scleromyxedema. An IgG-type paraproteinemia was identified. He improved with plasmapheresis, but died in acute respiratory and renal failure. Autopsy revealed right temporal demyelination and meningeal inflammation.
Insights
A man with memory loss and skin changes had IgG paraproteinemia. Despite plasmapheresis, he developed neurological and organ failure, with autopsy showing brain inflammation and demyelination.
Area of Science:
- Neurology
- Immunology
- Dermatology
Background:
- Paraproteinemia is an abnormal protein in the blood.
- Scleromyxedema is a rare skin condition associated with paraproteinemia.
Observation:
- A 37-year-old man presented with a 3-month history of cognitive decline, personality changes, fever, seizures, and upper motor neuron signs.
- He also had skin induration consistent with scleromyxedema and elevated cerebrospinal fluid (CSF) protein.
- An IgG-type paraproteinemia was detected.
Findings:
- The patient showed initial improvement with plasmapheresis, a procedure to remove antibodies from the blood.
- However, he subsequently experienced acute respiratory and renal failure.
- Autopsy revealed demyelination in the right temporal lobe and inflammation of the meninges.
Implications:
- This case highlights a rare neurological presentation associated with IgG paraproteinemia and scleromyxedema.
- It underscores the potential for severe, multi-systemic complications.
- Further research is needed to understand the pathogenesis and optimize treatment strategies for such complex cases.