Pilomotor seizures and status in non-paraneoplastic limbic encephalitis.
OBJECTIVE
To describe an unusual clinical presentation of a patient with voltage-gated potassium channel Ab- positive, non-paraneoplastic limbic encephalitis.


METHODS
We performed video-EEG monitoring, structural MRI, (18)F-FDG-PET, (1)H-MRS, neuropsychological testing and antibody serology.


RESULTS
A 42-year-old male patient presented in an acute phase of non-paraneoplastic limbic encephalitis confirmed by MRI, with antibodies to voltage-gated potassium channels. His pilomotor status was pharmacoresistant to antiepileptic drugs, but responded to corticosteroid and azathioprine treatment. The MRI findings improved. The pilomotor seizures recurred when the immunosuppressive therapy was discontinued after 18 months. MRI at that time was consistent with hippocampal sclerosis. Complete seizure control was achieved after reintroduction of steroids.


CONCLUSIONS
Pilomotor seizures were the predominant seizure type in this case of non-paraneoplastic limbic encephalitis. Immunosuppressive therapy may provide recovery including seizure control. However, long-term immunosuppression may be necessary to prevent relapse. Hippocampal sclerosis and chronic epilepsy might evolve as sequelae of limbic encephalitis.
Wieser
Stephan
S
Neurology Clinic, Department of Epileptology and Electroencephalography, University Hospital, Zurich, Switzerland.
Kelemen
Anna
A
Barsi
Peter
P
Vincent
Angela
A
Borbely
Csaba
C
Rasonyi
Gyorgy
G
Mueller
Susanne
S
Hess
Klaus
K
Wieser
Heinz Gregor
HG
Halasz
Peter
P
D000328:Adult; D001323:Autoantibodies / Q000097:blood*; D004569:Electroencephalography; D004833:Epilepsy, Temporal Lobe / Q000175:diagnosis / Q000209:etiology; D006801:Humans; D020363:Limbic Encephalitis / Q000150:complications* / Q000175:diagnosis / Q000276:immunology; D008279:Magnetic Resonance Imaging; D008297:Male; D010863:Piloerection / Q000502:physiology*; D049268:Positron-Emission Tomography; D024642:Potassium Channels, Voltage-Gated / Q000276:immunology; D012640:Seizures / Q000175:diagnosis / Q000209:etiology*; D013702:Temporal Lobe / Q000473:pathology / Q000502:physiology; D014741:Video Recording
D001323:Autoantibodies; D024642:Potassium Channels, Voltage-Gated