Course and outcome of acute limbic encephalitis with negative voltage-gated potassium channel antibodies.
BACKGROUND
Limbic encephalitis is a potentially treatable immunological condition. The presence of voltage-gated potassium channel antibodies (VGKC-Ab) in the cerebrospinal fluid (CSF) and serum of patients with the condition is a marker of the disease associated with a non-paraneoplastic form and good response to treatment. Recent work has highlighted absent serum VGKC-Ab and distinct immunology in patients with the paraneoplastic form of limbic encephalitis.


METHODS
The cases of four patients with the typical clinical presentation, neuropsychological features and brain imaging of acute limbic encephalitis, in the absence of any evidence for associated cancer during a follow-up of at least 18 months are described here.


RESULTS
All patients had negative testing for VGKC-Ab measured during their acute presentation. All patients made some recovery, although they were left with marked cognitive deficits and persistent seizures.


CONCLUSIONS
These cases demonstrate that the absence of VGKC-Ab in limbic encephalitis does not necessarily imply a paraneoplastic form. Further work is required to establish the immunological basis for the disorder in these patients, and the optimal treatment regimen.
Samarasekera
S R
SR
Royal Victoria Infirmary, Newcastle upon Tyne, UK.
Vincent
A
A
Welch
J L
JL
Jackson
M
M
Nichols
P
P
Griffiths
T D
TD
D000208:Acute Disease; D000328:Adult; D000906:Antibodies / Q000134:cerebrospinal fluid*; D003072:Cognition Disorders / Q000209:etiology; D005260:Female; D006801:Humans; D020363:Limbic Encephalitis / Q000134:cerebrospinal fluid / Q000150:complications / Q000276:immunology* / Q000628:therapy*; D008279:Magnetic Resonance Imaging; D008297:Male; D010257:Paraneoplastic Syndromes; D024642:Potassium Channels, Voltage-Gated / Q000276:immunology*; D011237:Predictive Value of Tests; D012640:Seizures / Q000209:etiology; D016896:Treatment Outcome
D000906:Antibodies; D024642:Potassium Channels, Voltage-Gated