Gerstmann's Syndrome in a Patient Double-positive for Antibodies against the N-methyl-D-aspartate Receptor and NH2-terminal of α-enolase.

Abstract

We herein report a case of anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis concurrent with NH2-terminal of α-enolase (NAE) antibodies. A 36-year-old Japanese woman presented with Gerstmann's syndrome followed by jerky involuntary movements, seizure, autonomic instability, and consciousness disturbance. NAE antibodies were detected in the serum; however, NMDAR antibodies were identified in the cerebrospinal fluid with a cell-based assay, confirming the diagnosis of anti-NMDAR encephalitis. This case highlights the fact that Gerstmann's syndrome can be a manifestation of anti-NMDAR encephalitis and that NAE may be identified concurrently with NMDAR antibodies, suggesting that the diagnosis of Hashimoto encephalopathy requires the reasonable exclusion of alternative diagnoses, including anti-NMDAR encephalitis.

• Authors: Atsuhiko Sugiyama, Masahide Suzuki, Tomoki Suichi, Tomohiko Uchida, Takahiro Iizuka, Keiko Tanaka, Makoto Yoneda, Satoshi Kuwabara
• Journal: Internal medicine (Tokyo, Japan) (Intern Med) Vol. 60 Issue 9 Pg. 1463-1468 (May 01 2021) ISSN: 1349-7235 [Electronic] Japan
• PMID: 33229813 (Publication Type: Case Reports, Journal Article)

Chemical References

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

Topics

• Adult
• Anti-N-Methyl-D-Aspartate Receptor Encephalitis (diagnosis)
• Autoantibodies
• Female
• Gerstmann Syndrome
• Humans
• Phosphopyruvate Hydratase
• Receptors, N-Methyl-D-Aspartate