Development of the clinical assessment scale in autoimmune encephalitis.

OBJECTIVE: There is no scale for rating the severity of autoimmune encephalitis (AE). In this study, we aimed to develop a novel scale for rating severity in patients with diverse AE syndromes and to verify the reliability and validity of the developed scale. METHODS: The key items were generated by a panel of experts and selected according to content validity ratios. The developed scale was initially applied to 50 patients with AE (development cohort) to evaluate its acceptability, reproducibility, internal consistency, and construct validity. Then, the scale was applied to another independent cohort (validation cohort, n = 38). RESULTS: A new scale consisting of 9 items (seizure, memory dysfunction, psychiatric symptoms, consciousness, language problems, dyskinesia/dystonia, gait instability and ataxia, brainstem dysfunction, and weakness) was developed. Each item was assigned a value of up to 3 points. The total score could therefore range from 0 to 27. We named the scale the Clinical Assessment Scale in Autoimmune Encephalitis (CASE). The new scale showed excellent interobserver (intraclass correlation coefficient [ICC] = 0.97) and intraobserver (ICC = 0.96) reliability for total scores, was highly correlated with modified Rankin scale (r = 0.86, p < 0.001), and had acceptable internal consistency (Cronbach α = 0.88). Additionally, in the validation cohort, the scale showed high interobserver reliability (ICC = 0.99) and internal consistency (Cronbach α = 0.92). INTERPRETATION: CASE is a novel clinical scale for AE with a high level of clinimetric properties. It would be suitable for application in clinical practice and might help overcome the limitations of current outcome scales for AE. ANN NEUROL 2019;85:352-358.

The impact of motivational interviewing on self-perceived burden in patients receiving therapeutic plasma exchange.

BACKGROUND: Autoimmune disorders and neurodegenerative disorders take a physical and emotional toll on patients that undergo therapeutic plasma exchange (TPE) treatments. Previous literature has shown that these patients may feel a greater sense of self-burden. Motivational Interviewing (MI) is a technique used in various settings that has the potential to decrease feelings of self-burden. MI for patients who receive TPE has not been tested. The purpose of this study was to examine the impact of MI in patients with a neurodegenerative diagnosis (eg, transverse myelitis, myasthenia gravis, multiple sclerosis, and chronic inflammatory demyelinating polyneuropathy) that are undergoing TPE treatments. METHODS: This was a prospective, non-randomized, longitudinal study of the impact of MI with patients at high risk of sense of self-burden who underwent apheresis treatments. Consented patients underwent three to six MI sessions with a trained clinician. Patients completed a self-report baseline and post-test of self-perceived burden. RESULTS: Thirty participants consented to the study; 27 were included in the analysis. The Self-Perceived Burden Scale scores were significantly higher at baseline (m = 26.2) when compared to scores post MI sessions (m = 21.48, P < .05). The number of MI sessions (3, 4, 5, 6 sessions) did not significantly impact the outcome score (r2 = 0.001; P = .901). CONCLUSION: MI is a straightforward technique that is feasible and shown to be effective to be used by bedside clinicians while working with patients who receive TPE to decrease levels of self-perceived burden.

Neuropsychological assessment as an objective tool to monitor treatment response in anti-N-methyl-D-aspartate receptor encephalitis.

We report a 1-year follow-up of a young woman with anti-N-methyl-D-aspartate receptor encephalitis. Management of autoimmune encephalitis remains challenging as objective and clinically relevant biomarkers are sought, which allow for the monitoring of treatment response. While further investigation is required, we believe that this case highlights the importance of following a comprehensive neuropsychological profile as a clinically relevant biomarker to guide therapeutic decision-making. By relying on the neuropsychological assessment of the patient, treatment with more toxic medications was avoided and her antiepileptic drug regimen was simplified.

[Neurological appraisal of children and adolescents with psychotic symptoms]. / Valoracion neurologica de niños y adolescentes con sintomas psicoticos.

INTRODUCTION: Psychotic manifestations in childhood are not infrequent, yet the existing literature dealing with the neurological appraisal of children and adolescents with a clinical picture of psychosis is very scant. AIM: To conduct a non-systematic review of the literature that provides an answer to these three questions: When must a neurological appraisal be performed in a child with psychotic traits? What medical conditions can include signs and symptoms of psychosis in their development? And, what diagnostic procedure should be followed? DEVELOPMENT: The diseases that can present psychotic symptoms at onset or during their course are reviewed and grouped by pathologies: inborn errors of metabolism, genetic diseases, autoimmune and infectious diseases, malformations of the central nervous system, epilepsy, vascular pathology, rheumatologic processes, brain tumours, and psychoactive substances and drugs. A diagnostic regimen is proposed in which both the information obtained from the anamnesis and examination and the findings from each of the diagnostic tests are evaluated. CONCLUSIONS: A huge number of processes can display psychotic symptoms during their course and the key information offered by the anamnesis and examination must be taken into account. This review can help neuropaediatricians and other specialists perform a more systematised appraisal of children and adolescents with psychotic signs and symptoms.

TITLE: Valoracion neurologica de niños y adolescentes con sintomas psicoticos.Introduccion. Las manifestaciones psicoticas en la infancia no son infrecuentes; sin embargo, la bibliografia existente acerca de la valoracion neurologica de niños y adolescentes con cuadros psicoticos es muy escasa. Objetivo. Realizar una revision bibliografica no sistematica que permita responder a estas tres cuestiones: cuando debe llevarse a cabo una valoracion neurologica en un niño con rasgos psicoticos?, cuales son las condiciones medicas que pueden incluir un cuadro psicotico en su evolucion? y cual debe ser el procedimiento diagnostico? Desarrollo. Se revisan las enfermedades que pueden presentar sintomatologia psicotica al inicio o durante la evolucion, y se agrupan por patologias: errores congenitos del metabolismo, enfermedades geneticas, enfermedades autoinmunes e infecciosas, malformaciones del sistema nervioso central, epilepsia, patologia vascular, procesos reumatologicos, tumores cerebrales, y farmacos y sustancias psicoactivas. Se propone una pauta diagnostica en la que se valora la informacion obtenida a partir de la anamnesis y la exploracion y la aportacion de cada prueba diagnostica. Conclusiones. El numero de procesos que pueden manifestar sintomatologia psicotica a lo largo de su evolucion es muy elevado, y hay que considerar las claves que ofrecen la anamnesis y la exploracion. Esta revision puede ayudar a neuropediatras y otros especialistas a realizar una valoracion mas sistematizada de niños y adolescentes con cuadros psicoticos.