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Pitfalls in diagnosing limbic encephalitis - a case report.
Acta Neurol Scand. 2008 Nov; 118(5):339-42.AN

Abstract

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.

AIM

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.

Authors+Show Affiliations

Epilepsy Center (ZEE), Department of Neurology, University of Erlangen-Nuremberg, Erlangen, Germany. frank.kerling@uk-erlangen.deNo affiliation info availableNo affiliation info available

Pub Type(s)

Case Reports
Journal Article

Language

eng

PubMed ID

18384457

Citation

Kerling, F, et al. "Pitfalls in Diagnosing Limbic Encephalitis - a Case Report." Acta Neurologica Scandinavica, vol. 118, no. 5, 2008, pp. 339-42.
Kerling F, Blümcke I, Stefan H. Pitfalls in diagnosing limbic encephalitis - a case report. Acta Neurol Scand. 2008;118(5):339-42.
Kerling, F., Blümcke, I., & Stefan, H. (2008). Pitfalls in diagnosing limbic encephalitis - a case report. Acta Neurologica Scandinavica, 118(5), 339-42. https://doi.org/10.1111/j.1600-0404.2008.01020.x
Kerling F, Blümcke I, Stefan H. Pitfalls in Diagnosing Limbic Encephalitis - a Case Report. Acta Neurol Scand. 2008;118(5):339-42. PubMed PMID: 18384457.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Pitfalls in diagnosing limbic encephalitis - a case report. AU - Kerling,F, AU - Blümcke,I, AU - Stefan,H, Y1 - 2008/03/27/ PY - 2008/4/4/pubmed PY - 2009/4/10/medline PY - 2008/4/4/entrez SP - 339 EP - 42 JF - Acta neurologica Scandinavica JO - Acta Neurol Scand VL - 118 IS - 5 N2 - 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. AIM: 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. SN - 1600-0404 UR - https://www.unboundmedicine.com/medline/citation/18384457/Pitfalls_in_diagnosing_limbic_encephalitis___a_case_report_ L2 - https://doi.org/10.1111/j.1600-0404.2008.01020.x DB - PRIME DP - Unbound Medicine ER -