Anti-N-methyl-D-aspartate receptor encephalitis: a patient with refractory illness after 25 months of intensive immunotherapy.

Abstract

IMPORTANCE:

N-methyl-D-aspartate receptor (NMDAR) antibody encephalitis is an autoimmune encephalitis that can be paraneoplastic and usually responds to treatment. It is quickly becoming the most common paraneoplastic encephalitis.

OBSERVATIONS:

We present a case of a woman in her late 30s who developed psychiatric symptoms that progressed to encephalopathy, seizures, autonomic instability, and hyperkinetic movements. The patient was found to have an ovarian teratoma and serum and cerebrospinal fluid NMDAR antibodies. Despite resection of the teratoma and treatment with immunosuppressive therapy, the patient progressed to a minimally conscious state. She was supported medically in our institution for 25 months. During her hospitalization, she was treated with multiple immunosuppressive agents. With each treatment, we analyzed the serum and cerebrospinal fluid for NMDAR antibodies. While there was some initial reduction in the serum antibodies, the spinal fluid antibodies remained persistently elevated. The patient did not have any clinical improvement and eventually died after the family decided to withdraw care.

CONCLUSIONS AND RELEVANCE:

As far as we know, this case represents the longest active treatment without improvement of a patient with anti-NMDAR encephalitis. The patient had persistently high cerebrospinal fluid and serum antibody titers, which may be of prognostic significance.

• Authors: Alissa Thomas, Paula Rauschkolb, Núria Gresa-Arribas, Alan Schned, Josep O Dalmau, Camilo E Fadul
• Journal: JAMA neurology (JAMA Neurol) Vol. 70 Issue 12 Pg. 1566-8 (Dec 2013) ISSN: 2168-6157 [Electronic] United States
• PMID: 24166348 (Publication Type: Case Reports, Journal Article, Research Support, N.I.H., Extramural, Research Support, Non-U.S. Gov't)

Chemical References

• Autoantibodies
• Receptors, N-Methyl-D-Aspartate

Topics

• Adult
• Anti-N-Methyl-D-Aspartate Receptor Encephalitis (blood, cerebrospinal fluid, etiology, immunology)
• Autoantibodies (blood, cerebrospinal fluid)
• Female
• Humans
• Immunotherapy (adverse effects)
• Ovarian Neoplasms (immunology, therapy)
• Receptors, N-Methyl-D-Aspartate (immunology)
• Teratoma (immunology, therapy)