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Nonvasculitic autoimmune inflammatory meningoencephalitis (NAIM): a reversible form of encephalopathy
R J Caselli1, B F Boeve, B W Scheithauer
1Department of Neurology, Mayo Clinic Scottsdale, AZ 85259, USA.
Abstract:
Five patients, age 54 to 80 years, presented between 3 weeks and 18 months after symptomatic onset of progressive cognitive decline, psychosis, and unsteady gait that proved to be due to a steroid-responsive nonvasculitic autoimmune inflammatory meningoencephalitic syndrome. CSF examination showed elevated immunoglobulin (Ig)G index and IgG synthesis rate in all three patients in whom it was checked, and brain biopsy revealed perivascular lymphocytic infiltrates without vessel wall invasion.
Insights
Steroid-responsive autoimmune meningoencephalitis can cause progressive cognitive decline, psychosis, and gait issues. Early diagnosis and treatment are crucial for managing this rare inflammatory brain condition.
Area of Science:
- Neurology
- Immunology
- Neuroinflammation
Background:
- Autoimmune meningoencephalitis is a group of rare inflammatory brain disorders.
- These conditions can mimic neurodegenerative diseases, posing diagnostic challenges.
Observation:
- Five patients (54-80 years) presented with progressive cognitive decline, psychosis, and unsteady gait.
- Symptoms appeared 3 weeks to 18 months after onset.
Findings:
- The condition was identified as steroid-responsive nonvasculitic autoimmune inflammatory meningoencephalitic syndrome.
- Cerebrospinal fluid (CSF) analysis revealed elevated immunoglobulin G (IgG) index and IgG synthesis rate in tested patients.
- Brain biopsy showed perivascular lymphocytic infiltrates without vessel wall invasion.
Implications:
- This syndrome is treatable with immunosuppressive therapy, such as steroids.
- Prompt diagnosis can prevent irreversible neurological damage.
- Further research into specific triggers and long-term outcomes is warranted.
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