Anti-CASPR2 encephalitis causes limbic encephalitis, Morvan syndrome, and neuromyotonia.
Anti-CASPR2 encephalitis:
- Associated with thymoma (~20%)
- Can be paraneoplastic
- Male predominance
- Good prognosis
- CASPR2 in synaptic zones
- Limbic system involvement
- Memory impairment
- Nerve hyperexcitability
- Motor nerve involvement
- Neuromyotonia
- Autonomic dysfunction
- Hyperhidrosis
- Cardiac involvement
- Contactin-associated protein 2
- Paranodal Kv1 channel
- Nerve conduction
¶ Antibody Effects
- Disrupts channel function
- Nerve hyperexcitability
- Central effects
- Neuromyotonia
- Autonomic dysfunction
- Encephalopathy
- Pain
- Memory impairment
- Seizures
- Psychiatric symptoms
- Peripheral nerve hyperexcitability
- Fasciculations
- Cramps
- When present (thymoma)
- Essential for recovery
- May need surgery
- Corticosteroids
- IVIG
- Plasma exchange
- Second-line agents
- Anti-CASPR2 encephalitis (2022)
- Morvan syndrome (2021)