Abstract Details

Title Autoimmune Glial Fibrillary Acidic Protein (GFAP) Astrocytopathy Following Herpes Simplex Encephalitis (HSE) in a pediatric patient

Topic Child Neurology and Developmental Neurology

Presentation(s) P4 - Poster Session 4 (5:30 PM-6:30 PM)

Poster/Presentation
Number 7-038

Objective To describe a pediatric case of autoimmune encephalitis associated with anti-GFAP antibody in cerebrospinal fluid (CSF) following Herpes simplex encephalitis (HSE), presenting as a subacute neurocognitive decline followed by acute worsening of behavior.

Background Autoantibodies to GFAP in the CSF and/or serum have recently been implicated in cerebral astrocytopathy presenting predominantly with autoimmune meningoencephalomyelitis.
Herpes simplex encephalitis (HSE) has been shown to trigger autoimmune encephalitides, most commonly anti-NMDAR. A recent report described a case of GFAP autoimmune astrocytopathy in an adult patient following HSE. To our knowledge, this is the only case report to date linking HSE to GFAP astrocytopathy. No data is available in current literature to suggest that this association is present in pediatric population.

Design/Methods NA

Results A 13 year old with history of ADHD developed mood and behavioral changes a few months after recovering from HSE. His symptoms include anxiety, impulse control difficulties such as disinhibition and hypersexuality, hoarding, emotional lability, and aggression, which progressively worsened prior to his hospital admission. Neuropsychological testing revealed significant declines in neurocognitive and behavioral functioning. MRI brain did not reveal new signal abnormalities other than evolution of cystic encephalomalacia from his prior HSV encephalitis. His CSF was positive for antibody against GFAP, and he was diagnosed with GFAP astrocytopathy. He was started on immunotherapy with intravenous corticosteroids and immunoglobulins, and is currently being monitored for improvement.

Conclusions Autoimmune GFAP astrocytopathy is a novel autoimmune disease which can present in children with primary neurocognitive and psychiatric manifestations. This is the first pediatric case suggesting an association between GFAP astrocytopathy and a viral trigger such as HSE. Further studies are needed to investigate the association between GFAP astrocytopathy and HSE, and to further delineate the clinical course of this disorder.

Authors/Disclosures
Maureen Handoko-Yang, MD (Rocky Mountain Neurodiagnostics) PRESENTER	Dr. Handoko has nothing to disclose.
William I. Hong, MD	No disclosure on file
Danielle Takacs, MD (Baylor College of Medicine/Texas Children's Hospital)	Dr. Takacs has received publishing royalties from a publication relating to health care.
Edward J. Espineli, MD (Baylor College of Medicine)	No disclosure on file
	No disclosure on file
Eyal Muscal	Eyal Muscal has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for sobi. An immediate family member of Eyal Muscal has stock in pfizer.
Sarah R. Risen, MD	Dr. Risen has nothing to disclose.

��ɫ��

��ɫ��