Literature DB >> 22361499

An unusual case of altered mental status in a young woman.

Rama Mohana Challapalli Rao1, Thejo Chipinapi, Shishira Bharadwaj, Kerri Ann Kissell.   

Abstract

CONTEXT: We describe a case of paraneoplastic neurologic syndrome, namely N-Methyl-D-Aspartic acid receptor antibody associated limbic encephalitis, a rare cause of altered mental status in the young. CASE REPORT: A 28 year old Caucasian female nurse presented with acute onset difficulty with word finding and increasing confusion and agitation. She also had visual hallucinations, transient episodes of unresponsiveness, and lingual dyskinesias. Workup including blood, imaging and regular cerebrospinal fluid (CSF) studies was unremarkable. She subsequently developed complex partial seizures. Computerized Tomography scan of chest/abdomen/pelvis revealed a dermoid cyst of the left ovary and CSF N-Methyl-D-Aspartic acid receptor antibody returned positive confirming the diagnosis of paraneoplastic NMDA receptor antibody associated limbic encephalitis. She was treated with methylprednisolone therapy along with plasmapheresis and a left salpingo-opherectomy was performed. The patient showed significant improvement with respect to her cognitive function and had no more seizures.
CONCLUSION: N-Methyl-D-Aspartic acid receptor antibody associated limbic encephalitis is a rare paraneoplastic neurologic syndrome with symptoms including psychiatric manifestations, seizures, language disturbances and autonomic instability. It develops due to antibody induced decrease in N-Methyl-D-Aspartic acid receptors. There is a significant association with ovarian teratoma in >50% female cases. Treatment includes resection of tumor, glucocorticoids, plasmapheresis and Intravenous Immunoglobulin therapy.

Entities:  

Keywords:  N-Methyl-D-Aspartic acid (NMDA) receptor; Paraneoplastic neurologic syndrome; complex partial seizures; confusion; limbic encephalitis; ovarian teratoma; plasmapheresis

Year:  2011        PMID: 22361499      PMCID: PMC3271409          DOI: 10.4297/najms.2011.3518

Source DB:  PubMed          Journal:  N Am J Med Sci        ISSN: 1947-2714


  10 in total

1.  Treatment-responsive subacute limbic encephalitis and NMDA receptor antibodies in a man.

Authors:  María Elena Novillo-López; Jeffrey E Rossi; Josep Dalmau; Jaime Masjuan
Journal:  Neurology       Date:  2008-02-26       Impact factor: 9.910

2.  Limbic encephalitis associated with antibodies to the NMDA receptor in Hodgkin lymphoma.

Authors:  M S Zandi; S R Irani; G Follows; A M Moody; P Molyneux; A Vincent
Journal:  Neurology       Date:  2009-12-08       Impact factor: 9.910

Review 3.  Clinical experience and laboratory investigations in patients with anti-NMDAR encephalitis.

Authors:  Josep Dalmau; Eric Lancaster; Eugenia Martinez-Hernandez; Myrna R Rosenfeld; Rita Balice-Gordon
Journal:  Lancet Neurol       Date:  2011-01       Impact factor: 44.182

4.  Paraneoplastic anti-N-methyl-D-aspartate receptor encephalitis associated with ovarian teratoma.

Authors:  Josep Dalmau; Erdem Tüzün; Hai-yan Wu; Jaime Masjuan; Jeffrey E Rossi; Alfredo Voloschin; Joachim M Baehring; Haruo Shimazaki; Reiji Koide; Dale King; Warren Mason; Lauren H Sansing; Marc A Dichter; Myrna R Rosenfeld; David R Lynch
Journal:  Ann Neurol       Date:  2007-01       Impact factor: 10.422

5.  Retrospective analysis of NMDA receptor antibodies in encephalitis of unknown origin.

Authors:  H Prüss; J Dalmau; L Harms; M Höltje; G Ahnert-Hilger; K Borowski; W Stoecker; K P Wandinger
Journal:  Neurology       Date:  2010-11-09       Impact factor: 9.910

6.  Anti-NMDA-receptor encephalitis: case series and analysis of the effects of antibodies.

Authors:  Josep Dalmau; Amy J Gleichman; Ethan G Hughes; Jeffrey E Rossi; Xiaoyu Peng; Meizan Lai; Scott K Dessain; Myrna R Rosenfeld; Rita Balice-Gordon; David R Lynch
Journal:  Lancet Neurol       Date:  2008-10-11       Impact factor: 44.182

7.  Anti-NMDA receptor encephalitis: report of ten cases and comparison with viral encephalitis.

Authors:  M S Gable; S Gavali; A Radner; D H Tilley; B Lee; L Dyner; A Collins; A Dengel; J Dalmau; C A Glaser
Journal:  Eur J Clin Microbiol Infect Dis       Date:  2009-08-29       Impact factor: 3.267

8.  Anti-NMDA receptor encephalitis in Japan: long-term outcome without tumor removal.

Authors:  T Iizuka; F Sakai; T Ide; T Monzen; S Yoshii; M Iigaya; K Suzuki; D R Lynch; N Suzuki; T Hata; J Dalmau
Journal:  Neurology       Date:  2007-09-26       Impact factor: 9.910

9.  Neurological response to early removal of ovarian teratoma in anti-NMDAR encephalitis.

Authors:  M Seki; S Suzuki; T Iizuka; T Shimizu; Y Nihei; N Suzuki; J Dalmau
Journal:  J Neurol Neurosurg Psychiatry       Date:  2007-11-21       Impact factor: 10.154

10.  Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis in children and adolescents.

Authors:  Nicole R Florance; Rebecca L Davis; Christopher Lam; Christina Szperka; Lei Zhou; Saba Ahmad; Cynthia J Campen; Heather Moss; Nadja Peter; Amy J Gleichman; Carol A Glaser; David R Lynch; Myrna R Rosenfeld; Josep Dalmau
Journal:  Ann Neurol       Date:  2009-07       Impact factor: 10.422

  10 in total

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