Clinicopathological Features in Morvan's Syndrome: An Autopsy Case Study
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Updated: Jun 25, 2025
Comprehensive Autopsy Program for Individuals with Multiple Sclerosis
Published on: July 19, 2019
Doddamalur Mallikarjuna Sindhu1, Shilpa Rao2, Anita Mahadevan2
1Department of Neurology, National Institute of Mental Health and Neurosciences (NIMHANS), Bengaluru, Karnataka, India.
Abstract:
Morvan's syndrome is a rare, complex autoimmune syndrome comprising peripheral nerve hyperexcitability, dysautonomia, insomnia, and encephalopathy. In this case report, we highlight the clinical and pathological findings of an elderly gentleman who presented to us with clinical features of Morvan's syndrome associated with anti-contactin-associated protein 2 (CASPR-2) antibodies. Histopathology [Figure 3] revealed cortical atrophy with gliosis and mild microglial proliferation. Microglial activation and gliosis were observed in the hippocampus, hypothalamus, and thalamus. Brainstem showed multifocal inflammation. Mild inflammation was observed in the leptomeninges. Morvan's syndrome is an autoimmune disease with antibodies targeted against CASPR within the voltage-gated potassium channel (VGKC) complex. Early diagnosis and treatment play a key role in the management of patients. Most patients show a good response when treated with plasmapheresis and steroids. This patient presented to us late into the illness and succumbed.
Insights
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.
