|
Age (years)
|
18 to 50
|
Less than 50
|
Less than 50
|
60 to 70
|
Less than 50
|
|
Sex (% female)
|
75%
|
66%
|
Greater than 50%
|
50%
|
Less than 50%
|
|
Etiology
| | | | | |
|
Paraneoplastic (%, and commonly occurring cancers)
|
9% to 56% have ovarian teratoma, predominately females less than 18 years of age
|
10% to 30%; low titers; SCLC; thymoma; CASPR2>>LGI-1
|
50% to 70%; SCLC, breast carcinoma; thymoma
|
50%; SCLC thymoma
|
Rarely associated with cancer
|
|
Nonparaneoplastic
|
Approximately 50%
|
70%; high titers
|
30% to 50%
|
50%
|
Frequent
|
|
Anatomical subtype
| | | | | |
|
Limbic encephalitis
|
Less common
|
Typical
|
Typical
|
Typical
|
Typical
|
|
Panencephalitis
|
Typical
|
Rare (involving basal ganglia)
|
Unclear
|
Unclear
|
Less common
|
|
CSF abnormal (%)
|
90%
|
40%
|
90%
|
80%
|
20%
|
|
Psychiatric Features
|
Common and pronounced: Anxiety, agitation, paranoid delusions, perceptual changes, erratic behavior, speech changes, severe psychosis
|
Agitation, anxiety, panic-attacks, depression, psychosis, hallucinations, delusions, delirium, confabulation
|
Atypical psychosis, which can be isolated
|
Paranoia, behavioral changes
|
Depression, atypical psychosis (case reports)
|
|
Neurological Features
|
Early features: seizures, cognitive/memory impairment; Late features: catatonia, orofacial and limb dyskinesia, dystonia, autonomic dysfunction, reduced level of consciousness, aphasia, central hypoventilation
|
LGI1: limbic encephalitis (more common): amnesia temporal lobe seizures, tonic seizures, and hypernatremia. Extrapyramidal symptoms (choreoathetosis) and extra-temporal (faciobrachial dystonic) seizures (less common). CASPR2: limbic encephalitis, Morvan's syndrome (neuromyotonia, REM disorder, insomnia, and autonomic dysfunction).
|
Memory impairment, temporal lobe seizures
|
Prominent temporal lobe seizures, memory impairment, concomitant glutamic acid decarboxylase autoantibodies
|
Stiff-person syndrome, cerebellar ataxia, cognitive/memory impairment, epilepsy (often mesial temporal)
|
|
Response to treatment
|
Highly responsive to immune therapy and removal of ovarian teratoma
|
Highly responsive to immune therapy
|
Moderately responsive to immune therapy
|
Moderately responsive to immune therapy
|
Often refractory to immune therapy
|
|
Relapse risk
|
20% often with psychiatric signs; may indicate tumor reoccurrence
|
Rarely relapses
|
Tendency to relapse (based on small case series)
|
Tendency to relapse (based on small case series)
|
Tendency to be chronic and relapse
|
