Background: Hashimoto encephalopathy is a rare form of encephalopathy thought to be of autoimmune etiology. Cognitive changes and seizures are the most commonly reported presenting manifestation. Stroke-like episodes have also been documented in these individuals. We describe a rare adolescent with Hashimoto encephalopathy who presented with stroke-like symptoms.
Patient Description: A previously well 15-year-old girl experienced sudden-onset language disturbance and right hemiparesis. Her symptoms resolved, but weeks later, she began experiencing refractory seizures, episodes of status epilepticus, and cognitive decline. An extensive evaluation was unremarkable, but thyroid peroxidase antibodies were elevated, and a diagnosis of Hashimoto encephalitis was made. Steroid therapy was initiated, and her symptoms resolved with return to baseline cognitive function.
Conclusion: Hashimoto encephalopathy is a highly treatable condition that may be considered in the differential diagnosis of children and adolescents presenting with stroke-like symptoms.
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http://dx.doi.org/10.1016/j.pediatrneurol.2016.02.003 | DOI Listing |
Intern Med
December 2024
Division of Neurology, Department of Internal Medicine, Faculty of Medicine, Saga University, Japan.
Schizophr Res
December 2024
Department of Preventive Intervention for Psychiatric Disorders, National Institute of Mental Health, National Center of Neurology and Psychiatry, Tokyo, Japan; Department of Psychiatry, National Center Hospital of Neurology and Psychiatry, Tokyo, Japan; Japan Health Research Promotion Bureau, Tokyo, Japan. Electronic address:
Background: Cognitive impairment is a cardinal feature in patients with schizophrenia and leads to poor social functioning. Recently, the treatment of schizophrenia has evolved to include the goal of improving quality of life (QoL). However, most of the factors influencing subjective QoL are unknown.
View Article and Find Full Text PDFNat Neurosci
December 2024
Laboratory for Proteolytic Neuroscience, RIKEN Center for Brain Science, Wako, Japan.
Tau pathology is a hallmark of several neurodegenerative diseases, including frontotemporal dementia and Alzheimer's disease. However, the sequence of events and the form of tau that confers toxicity are still unclear, due in large part to the lack of physiological models of tauopathy initiation and progression in which to test hypotheses. We have developed a series of targeted mice expressing frontotemporal-dementia-causing mutations in the humanized MAPT gene to investigate the earliest stages of tauopathy.
View Article and Find Full Text PDFBMC Neurol
December 2024
Department of Neurology, Peking University Shenzhen Hospital, Shenzhen, Guangdong, 518036, China.
Neuronal intranuclear inclusion disease (NIID) is a rare progressive neurodegenerative disease with a characteristic pathological feature of eosinophilic hyaluronan inclusions in the nervous system and internal organs. The identification of GGC-repeat expansions in the Notch 2 N-terminal like C (NOTCH2NLC) gene facilitates the accurate diagnosis of NIID. Due to its rareness and high clinical heterogeneity, the diagnosis of NIID is often delayed or missed.
View Article and Find Full Text PDFLancet Neurol
January 2025
Neuroimmunology Program, Institut d'Investigacions Biomèdiques August Pi i Sunyer/CaixaResearch Institute, Hospital Clínic de Barcelona, Barcelona, Spain; Pediatric Neuroimmunology Unit, Neurology Department, Sant Joan de Déu Children's Hospital, Barcelona, Spain; Institut de Recerca Sant Joan de Déu, Esplugues de Llobregat, Spain; European Reference Networks-RITA. Electronic address:
Background: The usefulness of current diagnostic approaches in children with suspected autoimmune encephalitis is unknown. We aimed to assess the diagnosis of autoimmune encephalitis in clinical practice and to compare the performance of two international diagnostic algorithms (one intended for patients of any age [general], the other intended for paediatric patients), with particular emphasis on the evaluation of patients with probable antibody-negative autoimmune encephalitis because this diagnosis suggests that immunotherapy should be continued or escalated but is difficult to establish.
Methods: We did a prospective cohort study that included all patients (<18 years of age) with suspected autoimmune encephalitis recruited at 40 hospitals in Spain whose physicians provided clinical information every 6 months for 2 years or more.
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