Hashimoto's encephalitis is a rare cause of encephalitis which is improved by corticosteroid treatment. We report the case of a 42-year-old woman who developed progressive dementia associated with episodes of recurrent discorders of consciousness which rapidly improved with corticosteroids. During these episodes, no sleep activity was recorded on the holter EEG. These discorders were reversible with treatment and a normal EEG sleep pattern reappeared. At physical examination, Hashimoto's encephalitis can mimic Creutzfeld-Jakob disease. Systematic sleep-EEG recordings can be helpful for diagnosis of sleep disorders related Hashimoto's encephalitis. This case illustrates the importance of searching for antithyroid antibodies in patients with unexplained encephalitis.
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Crit Care
January 2025
Department of Neuro-Intensive Care Unit, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, Henan, China.
Background And Objectives: Antibody-negative autoimmune encephalitis (AE) is a form of encephalitis characterized by the absence of detectable autoimmune antibodies, despite immunological evidence. However, data on management of patients with antibody-negative AE in the intensive care unit (ICU) are limited. This study aimed to explore the characteristics and subtypes of antibody-negative AE, assess the effects of immunotherapy, and identify factors independently associated with poor functional outcomes in patients requiring intensive care.
View Article and Find Full Text PDFCNS Neurosci Ther
January 2025
Department of Neurology, Beijing Tiantan Hospital, Capital Medical University, Beijing, China.
BMC 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.
Arq Neuropsiquiatr
December 2024
Hospital Israelita Albert Einstein, Instituto do Cérebro, São Paulo SP, Brazil.
Since the description of autoimmune encephalitis (AE) associated with N-methyl-D-aspartate receptor antibodies (anti-NMDARE) in 2007, more than 12 other clinical syndromes and antibodies have been reported. In this article, we review recent advances in pathophysiology, genetics, diagnosis pitfalls, and clinical phenotypes of AE associated with cell surface antibodies and anti-GAD associated neurological syndromes. Genetic studies reported human leukocyte antigen (HLA) associations for anti-LGI1, anti-Caspr2, anti-IgLON5, and anti-GAD.
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