Pitfalls in diagnosing limbic encephalitis - a case report.
BACKGROUND
The syndrome of limbic encephalitis (LE) is characterized by subacute onset of temporal lobe epilepsy, loss of short-term memory, cognitive confusion and psychiatric symptoms.


OBJECTIVE
We report a patient with pharmacoresistant epilepsy who underwent presurgical video-electroencephalogram (EEG)-monitoring with normal psychiatric and neuropsychological findings.


METHODS
Magnetic resonance imaging (MRI) revealed a hyperintense lesion within the right amygdala but no contrast enhancement. Analysis of cerebrospinal fluid (CSF) showed pleocytosis and positive oligoclonal bands, but all tests for neurotropic viruses or borrelia antibodies were negative. Presurgical evaluation identified a right mesiotemporal focus.


RESULTS
As a tumour was the most likely differential diagnosis, we performed selective amygdalohippocampectomy of the right hemisphere. Subsequent histopathological examination revealed the surprising diagnosis of LE. As a consequence, tumour screening was initiated and a testicular carcinoma with high anti-Ma2-antibody titres was detected. Following surgical and chemotherapeutical treatment, the patient was seizure-free and Ma2-antibodies decreased below detection limits. Conclusion - This case report highlights that LE has to be considered even in patients with atypical clinical presentation, i.e. without neuropsychological deficits, if CSF analysis reveals an inflammatory response. When LE is diagnosed, extensive tumour search is mandatory to detect and treat the paraneoplastic origin of LE. Therapeutic strategies of LE include surgical treatment as well as early immunosuppression.
Kerling
F
F
Epilepsy Center (ZEE), Department of Neurology, University of Erlangen-Nuremberg, Erlangen, Germany. frank.kerling@uk-erlangen.de
Blümcke
I
I
Stefan
H
H
D000328:Adult; D000951:Antigens, Neoplasm / Q000276:immunology; D001323:Autoantibodies / Q000276:immunology; D001932:Brain Neoplasms / Q000150:complications / Q000175:diagnosis* / Q000601:surgery; D002277:Carcinoma / Q000150:complications / Q000175:diagnosis / Q000276:immunology; D003072:Cognition Disorders / Q000209:etiology; D003951:Diagnostic Errors / Q000517:prevention & control*; D004569:Electroencephalography; D004833:Epilepsy, Temporal Lobe / Q000209:etiology* / Q000503:physiopathology / Q000601:surgery; D006801:Humans; D020363:Limbic Encephalitis / Q000150:complications* / Q000175:diagnosis* / Q000503:physiopathology; D008279:Magnetic Resonance Imaging; D008297:Male; D008569:Memory Disorders / Q000209:etiology; D019964:Mood Disorders / Q000209:etiology; D009419:Nerve Tissue Proteins / Q000276:immunology; D019635:Neurosurgical Procedures; D013736:Testicular Neoplasms / Q000150:complications / Q000175:diagnosis / Q000276:immunology
D000951:Antigens, Neoplasm; D001323:Autoantibodies; C119249:Ma2 antigen; D009419:Nerve Tissue Proteins