Following are excerpts from the latest studies on Pediatric-Acute Onset Neuropsychiatric Syndrome (PANS), along with case reports of a strep-induced movement disorder in an adult and the role of cytokines in autoimmune and infectious encephalitis.

Case report: SARS-CoV-2/COVID-19 Associated Pediatric Acute-onset Neuropsychiatric Syndrome: A Case Report of Female Twin Adolescents

“To our knowledge, this is the first case report of twin adolescents with COVID-19-associated PANS.” ¹

“Dizygotic twin sisters in late teens, with abrupt and acute onset of severely restrictive food intake, weight loss, OCD, anxiety with intermittent auditory and visual hallucinations, depression, attention deficit, and sleep disturbances, simultaneously accompanied by milder neurologic symptoms such as hand tremor, tinnitus, dizziness, headache, and weakness of proximal muscles [were admitted to a psychiatric unit].”

“The only relevant agent underlying those neuropsychiatric and somatic complaints was COVID-19, and it was validated with laboratory testing…”

“Treatment options consisted of psychotropic agents, antibiotics, antiepileptic, and intravenous immunoglobulin transfusion finely treated the neuropsychiatric symptoms.”

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Case report: Identification of ultra-rare genetic variants in pediatric acute onset neuropsychiatric syndrome (PANS) by exome and whole genome sequencing

“In the current study, we used next generation sequencing to search for ultra-rare genetic variants in patients who met diagnostic criteria for PANS as established by the PANS Consensus Conference.” ²

“Mutations were identified in 21 patients in 11 genes that separate into two broad functional categories: those that affect peripheral innate and adaptive immune pathways, and those that are expressed primarily in cortical neurons, where they function as synaptic regulators and have all been implicated in other neurological and neurodevelopmental disorders.”

“In addition, several genes are expressed in the choroid plexus and brain vasculature, suggesting they might contribute to a breach in the blood-CSF barrier and blood–brain barrier (BBB) that accompanies inflammation and infection.”

“In conclusion, we identified ultra-rare genetic variants in PANS patients that appear to function at multiple levels of the neuroinflammatory circuit, including peripheral and central innate immunity, synaptogenesis, the blood-CSF barrier, and perhaps the enteric nervous system.”

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Case report: Group A Beta-Hemolytic Streptococcus-Induced Tic-Like Movement Disorder in an Adult

“We present a unique case of a 31-year-old female admitted with involuntary movements of the upper extremities of three weeks duration. Her movements start as twitching before progressing to one hand hitting the other or hitting her face.” ³

“After a detailed workup, including a negative magnetic resonance imaging (MRI) of the brain with and without contrast, MRI of the spine, computed tomography of the chest, abdomen, and pelvis for neoplasia, as well as blood work for autoimmunity, infections, and paraneoplastic syndrome, the serology came back strongly positive for antistreptolysin O and antideoxyribonuclease B titers.”

“There are several limitations to our study. First, since our patient presented two months after the onset of symptoms, we cannot definitively establish the temporal relationship between the movement disorder and GABHS. She had considerable overlap between the symptoms and signs of organic and functional/psychogenic tics, providing diagnostic difficulty. In addition, after discharge, she was lost to follow-up; therefore, subsequent progression of symptoms could not be studied, including the development of any neuropsychiatric symptoms and exacerbation or remission of movements.”

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Case report: Cytokines and chemokines profile in encephalitis patients: A meta-analysis

“Encephalitis is caused by autoimmune or infectious agents marked by brain inflammation. Investigations have reported altered concentrations of the cytokines in encephalitis. This study was conducted to determine the relationship between encephalitis and alterations of cytokine levels in cerebrospinal fluid (CSF) and serum.” ⁴

“The cytokines IL-6, IL-8, IL-10, CXCL10, and TNF-α were significantly higher in the CSF of patients compared to controls. Also, there was a significant difference between infectious and autoimmune encephalitis regarding CSF levels of IL-10 and CXCL9. Increased serum levels of TNF-α were observed in patients with encephalitis.”

“Moreover, it was observed that IL-10 had higher levels in autoimmune encephalitis compared to [infectious] encephalitis. Contrastingly, CXCL9 had a higher concentration in infectious encephalitis. Accordingly, the interleukin antagonists could be investigated as a potential adjunctive treatment in encephalitis.”

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