Search

OBJECTIVES Patients with autoimmune encephalitis (AE) often present with symptoms that are broadly characterized as psychiatric or behavioral, yet little attention is given to the precise symptomatology observed. We sought to more fully define the psychiatric symptoms observed in patients with anti–N-methyl-D-aspartate receptor (NMDAR), anti–glutamic-acid-decarboxylase 65 (GAD65), and anti–voltage-gated-potassium-channel complex (VGKC) antibody-mediated AE using the Diagnostic and Statistical Manual of Mental Disorders, Fifth Edition nomenclature. METHODS We present a case series (n = 25) using a retrospective chart review of 225 patients evaluated for AE in a tertiary care academic medical center between 2014 and 2018. The included patients were ≤18 years old with anti-NMDAR AE (n = 13), anti-GAD65 AE (n = 7), or anti-VGKC AE (n = 5). The frequency of neuropsychiatric symptoms present at the onset of illness and time to diagnosis were compared across groups. RESULTS Psychiatric symptoms were seen in 92% of patients in our cohort. Depressive features (72%), personality change (64%), psychosis (48%), and catatonia (32%) were the most common psychiatric symptoms exhibited. On average, patients experienced impairment in ≥4 of 7 symptom domains. No patients had isolated psychiatric symptoms. The average times to diagnosis were 1.7, 15.5, and 12.4 months for anti-NMDAR AE, anti-GAD65 AE, and anti-VGKC AE, respectively (P < .001). CONCLUSIONS The psychiatric phenotype of AE in children is highly heterogenous. Involving psychiatry consultation services can be helpful in differentiating features of psychosis and catatonia, which may otherwise be misidentified. Patients presenting with psychiatric symptoms along with impairments in other domains should prompt a workup for AE, including testing for all known antineuronal antibodies.

Introduction Autoimmune encephalitis (AE) is an inflammatory condition which may present with encephalopathy, including psychiatric symptoms of psychosis and catatonia. When catatonia becomes life-threatening or refractory to medication, electroconvulsive therapy (ECT) should be considered. This case series represents a compilation of patient care experiences in which ECT served as a highly effective augmentation strategy for catatonia associated with autoimmune encephalitis other than NMDA AE. Methods We performed a retrospective case-study analysis of four patients with autoimmune encephalitis and catatonia who had disease onset prior to the age of 18. Cases were selected from the patients evaluated by the child psychiatry consultation and liaison inpatient service (n=460) in which care overlapped with both the autoimmune brain disease program (n=35) and the ECT program (n=4). Retrospective chart review focused on Bush Francis Catatonia Rating Scores, medication treatments targeting catatonia, ECT protocols, and special ethical considerations in patient care. Results Significant improvement in features of catatonia as evaluated by the Bush Francis Catatonia Rating Score (BFCRS) were seen in each patient treated with ECT. Across these four cases we encountered special considerations related to ECT that were specific to pediatric auto-immune catatonia. Conclusions We recommend ECT as a part of the treatment plan for patients who present with refractory features of catatonia (i.e. non-medication responsive). We advocate for following established practice parameters for the use of ECT in adolescents, with proposed modifications for patients with catatonia from this cause who often present with an increased level of medical complexity.

International audience; Children with a diagnosis of Autoimmune Encephalitis (AE) frequently require multi-disciplinary care in order to mobilize the assessment and treatment necessary for recovery. Institutional and provider practice differences often influence the diagnostic workup and treatment pathways made available to patients. There are a variety of provider coalitions in pediatric rheumatology, internal medicine, and neurology that have been making meaningful progress toward the development of consensus in assessment and treatment approaches to patient care. However, child psychiatry is currently underrepresented in this work in spite of the high psychiatric symptom burden seen in some young patients. The need for consensus is often made visible only with inter-institutional dialogue regarding patient care trajectories. We aim to review key updates in the assessment and treatment of children and adolescents with autoimmune encephalitis during the acute phase, with or without catatonia, and to outline provider perspectives by comparing current treatment models in the United States, Canada, and Europe.

Medical conditions that present with psychiatric symptoms are becoming increasingly well-recognized in response to the emergence of the field of neuroimmunology. As the availability of testing for novel antineuronal antibodies has increased, so too has the clinical awareness of this diagnostic spectrum. Psychiatrists may have little exposure to this area of expertise, yet may be called on to assist in the diagnosis and treatment of patients with complex neuropsychiatric syndromes secondary to autoimmune encephalitis. This article summarizes the evaluation and management of patients with autoimmune encephalitis and describes emerging patterns in phenotype recognition.

Background The recently proposed adult diagnostic criteria for Hashimoto encephalopathy (HE) include a requirement of subclinical or mild thyroid disease. However, reports indicate that most children treated for HE do not have evidence of thyroid disease. We aim to evaluate the impact of applying the current adult diagnostic criteria to pediatric patients. Methods Pediatric patients with HE were evaluated at time of symptom onset and follow-up at least 1 year after initiation of immunomodulatory treatment for degree of impairment within the neuropsychiatric domains of cognition, language, psychiatric disturbance, seizure, movement disorder, sleep disruption, and overall functionality. We compared the response to treatment among patients stratified by the presence or absence of subclinical or mild thyroid disease using the Modified Rankin Scale, the Liverpool Outcome Score, and a novel multidomain scale designed for the population with pediatric autoimmune brain disorders. Results Of 17 pediatric patients treated for HE, 6 met full adult diagnostic criteria, whereas 11 patients did not meet criteria solely owing to the absence of thyroid disease. Using our novel scale, the 6 patients meeting full criteria had statistically significant improvement from time of onset of disease to follow-up in the domain of cognition. The 11 patients who did not meet full criteria based on their absence of thyroid disease exhibited statistically significant improvement from time of onset of disease to follow-up in the domains of cognition, language, psychiatric disturbance, movement, and sleep. Conclusions Rigidly applying the current diagnostic criteria to pediatric patients with suspected HE may result in the failure to treat potential responders. We propose a set of diagnostic criteria for HE in children, which does not require thyroid disease but include abrupt onset cognitive regression with deficits in one or more other neuropsychiatric domains in the setting of antithyroid antibodies.