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Anti-N-Methyl-D-Aspartate Receptor Encephalitis with Decrease in Blood Flow in Cerebellum.

Koji Obara1, Tomoko Ono1, Itaru Toyoshima1

  • 1Department of Neurology, National Hospital Organization, Akita National Hospital, Yurihonjo, Japan.

Case Reports in Neurology
|February 22, 2021
PubMed
Summary

Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis can cause cerebellar damage. This case study highlights cerebellar hypoperfusion and suggests oral methotrexate as a potential treatment for refractory NMDAR encephalitis.

Keywords:
CerebellumEncephalitisMethotrexateN-methyl-D-aspartate receptorSPECT

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Area of Science:

  • Neurology
  • Immunology

Background:

  • Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is an autoimmune disorder often associated with severe neurological sequelae, including progressive cerebellar atrophy.
  • Cerebellar dysfunction in NMDAR encephalitis can manifest as motor deficits, cognitive impairments, and speech problems, significantly impacting patient recovery.

Observation:

  • A 15-year-old female presented with consciousness disturbance, initially suspected as influenza encephalopathy.
  • The patient received immunotherapy including glucocorticoid pulse therapy, intravenous immunoglobulins, and plasma exchange, followed by oophorectomy for an ovarian teratoma due to positive anti-NMDAR antibodies.

Findings:

  • Despite aggressive treatment, the patient exhibited persistent neuropsychiatric symptoms and marked cerebellar hypoperfusion on serial single-photon emission computed tomography (SPECT) scans.
  • Oral methotrexate (MTX) administration led to slow but significant recovery, with residual mild impairments in working memory and verbal fluency.
  • Profound cerebellar hypoperfusion, particularly in lobules VI and VII, was correlated with the patient's persistent working memory deficits and speech difficulties.

Implications:

  • Cerebellar hypoperfusion is a significant factor contributing to long-term sequelae in anti-NMDAR encephalitis.
  • Oral methotrexate may represent a viable and promising therapeutic option for refractory cases of anti-NMDAR encephalitis, especially those with prominent cerebellar involvement.
  • This case underscores the importance of monitoring cerebellar blood flow in NMDAR encephalitis patients and considering novel treatment strategies for refractory disease.