Morvan syndrome associated with prominent Tau pathology: A clinicopathological case report

Area of Science:

• Neurology
• Immunology
• Pathology

Background:

• Morvan syndrome is an autoimmune disorder characterized by peripheral nerve hyperexcitability, autonomic dysfunction, and encephalopathy.
• It is often associated with antibodies against LGI1 and Caspr2.
• This case highlights a patient with Morvan syndrome and concurrent tau pathology.

Purpose of the Study:

• To report a case of Morvan syndrome with prominent tau pathology.
• To investigate the neuropathological findings in a fatal case of Morvan syndrome.
• To explore the potential association between autoimmune disorders and tau deposition.

Main Methods:

• Clinical assessment of neurological, autonomic, and cognitive symptoms.
• Serological testing for LGI1 and Caspr2 antibodies.
• Postmortem neuropathological examination focusing on tau pathology in the frontal cortex.

Main Results:

• Autopsy revealed thymoma and extensive tau accumulation in the frontal cortex, involving neurons and astrocytes.
• Neurofibrillary tangles, pretangles, and astroglial tau aggregates were identified.
• The patient had no history of head trauma or contact sports.

Conclusions:

• This case suggests a potential association between Morvan syndrome (LGI1/Caspr2 antibodies) and focal tau pathology.
• The coexistence of autoimmune features and tau deposition raises questions about shared pathophysiological mechanisms.
• Further research is needed to clarify the relationship between autoimmune disorders and tauopathies.