CSF findings in patients with voltage gated potassium channel antibody associated limbic encephalitis.
Recently, a new subtype of limbic encephalitis (LE) has been described, serologically characterized by the presence of antibodies against voltage gated potassium channels (VGKC, to be called VGKC-LE). Only little is known about CSF findings in this new disorder. Here we report the results of 29 lumbar punctures in 17 patients with VGKC-LE. Slight pleocytosis, mainly consisting of lymphocytes and monocytes, and elevated total protein concentrations were present in 41 and 47%, respectively. Intrathecal immunoglobulin (Ig) synthesis as defined by the presence of CSF-specific oligoclonal IgG bands, an increased IgG index, or an elevated IgG, IgA, or IgM ratio, was not detected in any of the patients, but dysfunction of the blood-CSF barrier was found in 35%. CSF findings were normal in 23%.


CONCLUSIONS
Unlike paraneoplastic LE, VGKC-LE is not frequently associated with intrathecal Ig production or markedly elevated white cell counts. Thus, normal CSF findings do not preclude the disease. VGKC-Ab should, therefore, be determined whenever LE is clinically suspected, irrespective of the presence or absence of inflammatory CSF changes.
Jarius
S
S
Institute of Clinical Neuroimmunology, Ludwig Maximilians University, Munich, Germany; Neurosciences Group, Weatherall Institute of Molecular Medicine, John Radcliffe Hospital, University of Oxford, United Kingdom.
Hoffmann
L
L
Clover
L
L
Vincent
A
A
Voltz
R
R
D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D005260:Female; D006801:Humans; D007525:Isoelectric Focusing / Q000379:methods; D019344:Lactic Acid / Q000134:cerebrospinal fluid; D007958:Leukocyte Count / Q000379:methods; D020363:Limbic Encephalitis / Q000134:cerebrospinal fluid*; D008297:Male; D008875:Middle Aged; D043025:Oligoclonal Bands / Q000134:cerebrospinal fluid*; D024642:Potassium Channels, Voltage-Gated / Q000276:immunology*; D012189:Retrospective Studies
D043025:Oligoclonal Bands; D024642:Potassium Channels, Voltage-Gated; D019344:Lactic Acid