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Reversible limbic encephalitis with antibodies against the membranes of neurones of the hippocampus
  1. H Shimazaki1,
  2. Y Ando1,
  3. I Nakano1,
  4. J Dalmau2
  1. 1Division of Neurology, Department of Internal Medicine, Jichi Medical University, Tochigi, Japan
  2. 2Division of Neuro-Oncology, Department of Neurology, University of Pennsylvania, Pennsylvania, USA
  1. Correspondence to:
 Dr H Shimazaki
 Division of Neurology, Department of Internal Medicine, Jichi Medical University, Tochigi 329-0498, Japan;hshimaza{at}jichi.ac.jp

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Paraneoplastic limbic encephalitis (PLE) is a rare neurological syndrome characterised by short-term memory impairment, seizures and various psychiatric disturbances. It is often associated with small-cell lung cancer, germ-cell tumours of the testis and breast cancer, but rarely with ovarian teratoma.1 Several cases of PLE with ovarian teratoma have been reported, but the autoantigens of this disease remain unknown. Recently, an antibody to the membranes of neurones of the hippocampus (antigens colocalising with exchange factor for ADP-ribosylation factor 6 A (EFA6A)) was reported in association with PLE and ovarian teratoma.2 Here, we report a case of a young Japanese woman who had PLE with ovarian teratoma, and whose serum and cerebrospinal fluid (CSF) contained an antibody against the membranes of neurones of the hippocampus. Immunosuppressive treatments resulted in a rapid improvement.

A 30-year-old woman was admitted to our hospital (Jichi Medical University, Tochigi, Japan) in April 2004 because of headache, fever and disorientation for 3 days. Figure 1A summarises the clinical course of the patient. She had no relevant family or medical history of interest. Her temperature was 37.8°C. Neurological examination on admission showed only recent memory disturbance. Examination of CSF showed increased protein concentration (670 mg/l), an increased number of mononuclear-dominant cells (40/mm3) and 67 mg/dl glucose. CSF cytology was negative for malignant cells. Polymerase chain reaction for herpes simplex virus (HSV) was negative. No marked increase in anti-HSV, varicella zoster virus, human herpes virus type …

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Footnotes

  • Funding: This work was supported by a Jichi Medical University Young Investigator Award.

  • Competing interests: None.

  • Informed consent was obtained from the patient for publication of the features of the case.