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Clinicopathological Features in Morvan's Syndrome: An Autopsy Case Study
Doddamalur Mallikarjuna Sindhu1, Shilpa Rao2, Anita Mahadevan2
1Department of Neurology, National Institute of Mental Health and Neurosciences (NIMHANS), Bengaluru, Karnataka, India.
Neurology India
|May 31, 2024
Summary
Morvan's syndrome, an autoimmune disorder, involves nerve hyperexcitability and encephalopathy. This case highlights pathology linked to anti-contactin-associated protein 2 (CASPR-2) antibodies, emphasizing the need for early diagnosis.
Area of Science:
- Neurology
- Immunology
- Pathology
Background:
- Morvan's syndrome is a rare autoimmune disorder characterized by peripheral nerve hyperexcitability, dysautonomia, insomnia, and encephalopathy.
- It is associated with antibodies targeting the contactin-associated protein 2 (CASPR-2) within the voltage-gated potassium channel (VGKC) complex.
Observation:
- This case report details an elderly male patient presenting with clinical features of Morvan's syndrome.
- The patient was found to have antibodies against CASPR-2.
Findings:
- Histopathology revealed cortical atrophy with gliosis and microglial proliferation.
- Microglial activation and gliosis were noted in the hippocampus, hypothalamus, and thalamus.
- Brainstem exhibited multifocal inflammation, with mild leptomeningeal inflammation observed.
Implications:
- Early diagnosis and treatment, including plasmapheresis and steroids, are crucial for managing Morvan's syndrome.
- This case underscores the severe pathological consequences, including brainstem inflammation and cortical atrophy, in advanced or untreated Morvan's syndrome.
- The findings reinforce the autoimmune basis of Morvan's syndrome targeting CASPR-2 and the VGKC complex.
