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[Paraneoplastic limbic encephalitis with positive anti-RI antibodies and mediastinal seminoma].
Rev Neurol (Paris). 2008 Jun-Jul; 164(6-7):612-9.RN

Abstract

We report the case of a 49-year-old man who was admitted for progressive behaviorial disorders with frontal elements. There was no sensorial nor motor deficiency. Clinical examination revealed android obesity, cutaneous and mucous paleness, pubic and axillary depilation and gynecomastia. Encephalic MRI found a lesion of the left amygdalian region with high T2 intensity and low T1 intensity associated with gadolinium-enhancement. Cerebrospinal fluid analysis showed a lymphocytic meningitis. Panhypopituitarism was found on the endocrine investigations. Anti-RI antibodies were positive, leading to the diagnosis of paraneoplastic limbic encephalitis. The CT-scan showed a node of the lower part of the thymic area. Surgical resection revealed an ectopic mediastinal seminoma. The evolution consisted of paraneoplastic fever and crossed-syndrome with right hemiparesia and left common oculomotor nerve paralysis. Treatment was completed by two cycles of carboplatin, corticosteroids and substitutive opotherapy. Paraneoplastic fever disappeared, but behavioral disorders and palsy remain unchanged. The patient died two years later in a bedridden state. This case of paraneoplastic limbic encephalitis associated with positive anti-RI antibodies and mediastinal seminoma is exceptional and has not to our knowledge been described in the literature. Cancers usually associated with anti-RI antibody are breast and lung cancer. Paraneoplastic limbic encephalitis is not the classical clinical presentation, which usually is brainstem encephalitis. Hypothalamic involvement, uncommon in paraneoplastic limbic encephalitis is mainly associated with positive antineuronal anti-Ma2 antibodies. Finally, the gadolinium enhancement on encephalic MRI is unusual in paraneoplastic limbic encephalitis.

Authors+Show Affiliations

Service de neurologie, pavillon F, hôpital Pasteur, 30, voie Romaine, B.P. 69, 06002 Nice, France. mael0480@yahoo.frNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Case Reports
English Abstract
Journal Article

Language

fre

PubMed ID

18565362

Citation

Launay, M, et al. "[Paraneoplastic Limbic Encephalitis With Positive anti-RI Antibodies and Mediastinal Seminoma]." Revue Neurologique, vol. 164, no. 6-7, 2008, pp. 612-9.
Launay M, Bozzolo E, Venissac N, et al. [Paraneoplastic limbic encephalitis with positive anti-RI antibodies and mediastinal seminoma]. Rev Neurol (Paris). 2008;164(6-7):612-9.
Launay, M., Bozzolo, E., Venissac, N., Delmont, E., Fredenrich, A., & Thomas, P. (2008). [Paraneoplastic limbic encephalitis with positive anti-RI antibodies and mediastinal seminoma]. Revue Neurologique, 164(6-7), 612-9. https://doi.org/10.1016/j.neurol.2008.02.044
Launay M, et al. [Paraneoplastic Limbic Encephalitis With Positive anti-RI Antibodies and Mediastinal Seminoma]. Rev Neurol (Paris). 2008 Jun-Jul;164(6-7):612-9. PubMed PMID: 18565362.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - [Paraneoplastic limbic encephalitis with positive anti-RI antibodies and mediastinal seminoma]. AU - Launay,M, AU - Bozzolo,E, AU - Venissac,N, AU - Delmont,E, AU - Fredenrich,A, AU - Thomas,P, Y1 - 2008/05/13/ PY - 2007/09/01/received PY - 2008/02/09/revised PY - 2008/02/20/accepted PY - 2008/6/21/pubmed PY - 2008/10/4/medline PY - 2008/6/21/entrez SP - 612 EP - 9 JF - Revue neurologique JO - Rev Neurol (Paris) VL - 164 IS - 6-7 N2 - We report the case of a 49-year-old man who was admitted for progressive behaviorial disorders with frontal elements. There was no sensorial nor motor deficiency. Clinical examination revealed android obesity, cutaneous and mucous paleness, pubic and axillary depilation and gynecomastia. Encephalic MRI found a lesion of the left amygdalian region with high T2 intensity and low T1 intensity associated with gadolinium-enhancement. Cerebrospinal fluid analysis showed a lymphocytic meningitis. Panhypopituitarism was found on the endocrine investigations. Anti-RI antibodies were positive, leading to the diagnosis of paraneoplastic limbic encephalitis. The CT-scan showed a node of the lower part of the thymic area. Surgical resection revealed an ectopic mediastinal seminoma. The evolution consisted of paraneoplastic fever and crossed-syndrome with right hemiparesia and left common oculomotor nerve paralysis. Treatment was completed by two cycles of carboplatin, corticosteroids and substitutive opotherapy. Paraneoplastic fever disappeared, but behavioral disorders and palsy remain unchanged. The patient died two years later in a bedridden state. This case of paraneoplastic limbic encephalitis associated with positive anti-RI antibodies and mediastinal seminoma is exceptional and has not to our knowledge been described in the literature. Cancers usually associated with anti-RI antibody are breast and lung cancer. Paraneoplastic limbic encephalitis is not the classical clinical presentation, which usually is brainstem encephalitis. Hypothalamic involvement, uncommon in paraneoplastic limbic encephalitis is mainly associated with positive antineuronal anti-Ma2 antibodies. Finally, the gadolinium enhancement on encephalic MRI is unusual in paraneoplastic limbic encephalitis. SN - 0035-3787 UR - https://www.unboundmedicine.com/medline/citation/18565362/[Paraneoplastic_limbic_encephalitis_with_positive_anti_RI_antibodies_and_mediastinal_seminoma]_ L2 - https://linkinghub.elsevier.com/retrieve/pii/S0035-3787(08)00209-9 DB - PRIME DP - Unbound Medicine ER -