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Pediatric GAD-65 Autoimmune Encephalitis: Assessing Clinical Characteristics and Response to Therapy With a Novel Assessment Scale.

Publication ,  Journal Article
Harmon, A; Stingl, C; Rikhi, A; Tran, L; Pizoli, C; Malinzak, M; Van Mater, H
Published in: Pediatr Neurol
March 2022

BACKGROUND: Glutamic acid decarboxylase (GAD) encephalitis is a neuroinflammatory disease characterized by a broad range of symptoms including cognitive deficits, behavioral changes, and seizures. Children with this disorder have heterogeneous presentations, and little is known about symptom progression over time and response to immunotherapy. METHODS: This study reports 10 pediatric GAD encephalitis cases and symptoms found at presentation and follow-up. In addition, symptom severity was reported utilizing a novel scale evaluating functional outcomes across the domains affected by autoimmune encephalitis including cognition, language, seizures, psychiatric symptoms, sleep, and movement. Retrospective chart review was conducted for 10 patients aged <18 years, diagnosed with GAD encephalitis, and followed for one year or more. Chart review included clinical, imaging, and laboratory findings at time of diagnosis and at six- and 12-month follow-ups. RESULTS: At presentation, cognitive deficits were found in all patients, seizures in six of 10, and language decline in seven of 10. Psychiatric symptoms were prominent for all but one patient with three of nine patients presenting with psychosis. Fatigue, sleep disruption, and movement disorders were less prominent symptoms, occurring in approximately half of the cohort. Cognition and fatigue improved significantly over time when compared with symptom severity, whereas seizures, psychiatric symptoms, and sleep did not. Language and sleep showed improvement only in early stages. Analysis of seizure frequency and type noted variability mirroring trends noted in adult studies of GAD encephalitis. CONCLUSIONS: This study demonstrated the variability of symptom profiles of pediatric GAD encephalitis and benefits of symptom severity scales. Symptom profiles and progression vary in this population.

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Published In

Pediatr Neurol

DOI

EISSN

1873-5150

Publication Date

March 2022

Volume

128

Start / End Page

25 / 32

Location

United States

Related Subject Headings

  • Symptom Assessment
  • Neurology & Neurosurgery
  • Male
  • Immunotherapy
  • Humans
  • Glutamate Decarboxylase
  • Female
  • Encephalitis
  • Cohort Studies
  • Child, Preschool
 

Citation

APA
Chicago
ICMJE
MLA
NLM
Harmon, A., Stingl, C., Rikhi, A., Tran, L., Pizoli, C., Malinzak, M., & Van Mater, H. (2022). Pediatric GAD-65 Autoimmune Encephalitis: Assessing Clinical Characteristics and Response to Therapy With a Novel Assessment Scale. Pediatr Neurol, 128, 25–32. https://doi.org/10.1016/j.pediatrneurol.2021.12.007
Harmon, Alexis, Cory Stingl, Aruna Rikhi, Linh Tran, Carolyn Pizoli, Michael Malinzak, and Heather Van Mater. “Pediatric GAD-65 Autoimmune Encephalitis: Assessing Clinical Characteristics and Response to Therapy With a Novel Assessment Scale.Pediatr Neurol 128 (March 2022): 25–32. https://doi.org/10.1016/j.pediatrneurol.2021.12.007.
Harmon A, Stingl C, Rikhi A, Tran L, Pizoli C, Malinzak M, et al. Pediatric GAD-65 Autoimmune Encephalitis: Assessing Clinical Characteristics and Response to Therapy With a Novel Assessment Scale. Pediatr Neurol. 2022 Mar;128:25–32.
Harmon, Alexis, et al. “Pediatric GAD-65 Autoimmune Encephalitis: Assessing Clinical Characteristics and Response to Therapy With a Novel Assessment Scale.Pediatr Neurol, vol. 128, Mar. 2022, pp. 25–32. Pubmed, doi:10.1016/j.pediatrneurol.2021.12.007.
Harmon A, Stingl C, Rikhi A, Tran L, Pizoli C, Malinzak M, Van Mater H. Pediatric GAD-65 Autoimmune Encephalitis: Assessing Clinical Characteristics and Response to Therapy With a Novel Assessment Scale. Pediatr Neurol. 2022 Mar;128:25–32.
Journal cover image

Published In

Pediatr Neurol

DOI

EISSN

1873-5150

Publication Date

March 2022

Volume

128

Start / End Page

25 / 32

Location

United States

Related Subject Headings

  • Symptom Assessment
  • Neurology & Neurosurgery
  • Male
  • Immunotherapy
  • Humans
  • Glutamate Decarboxylase
  • Female
  • Encephalitis
  • Cohort Studies
  • Child, Preschool