Thyroid dysfunctions may be accompanied by numerous neurological and psychiatric disorders. The most well-known is cognitive impairment and depression in hypothyroid patients, as well as an increased risk of cerebrovascular accidents. A separate, although a rare entity, is Hashimoto's encephalopathy. Unlike encephalopathy associated with other conditions, management in Hashimoto's encephalopathy highly responds to steroid treatment and may be associated with normal thyroid profile at presentation. Hashimoto's encephalopathy, while rare, may have been under-recognised since its clinical presentation overlaps several more common disorders, such as depression, seizures or anxiety. We present two cases of hypothyroidism with peculiar presentation. The first case has rapidly progressive neurological dysfunction, normal thyroid function at presentation, normal MRI finding and responds to steroid treatment. The second case has a subacute progressive neurological deterioration with elevated thyroid-stimulating hormone titre at presentation. Both these cases are known hypothyroidism on regular thyroxin replacement therapy with elevated anti-thyroid peroxidase antibodies. We conclude that Hashimoto's encephalopathy can present with a wide spectrum of neurological illnesses in the setting of hypothyroidism. Thyroid status may vary from hypothyroid, normothyroid to even hyperthyroid. This condition usually has an abnormal electroencephalography (EEG) background and usually responds to high dose steroids.
Download full-text PDF |
Source |
---|---|
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3027635 | PMC |
http://dx.doi.org/10.1136/bcr.01.2010.2630 | DOI Listing |
CNS Neurosci Ther
January 2025
Department of Neurology, Beijing Tiantan Hospital, Capital Medical University, Beijing, China.
Background: Metabolomics offers promise in uncovering potential biomarkers and understanding the pathophysiology of autoimmune encephalitis (AE), which is a cluster of disorders with the host immune system targeting self-antigens expressed in the central nervous system (CNS). In this research, our objective was to explore metabolic characterization in cerebrospinal fluid (CSF) from individuals with AE, aiming to shed light on the pathophysiology of AE.
Methods: A targeted approach was applied using an ultra-performance liquid chromatography coupled to tandem mass spectrometry (UPLC-MS/MS) system to study CSF metabolites in patients with AE (n = 18), and control subjects without neurological diseases (n = 17).
Intern Med
December 2024
Division of Neurology, Department of Internal Medicine, Faculty of Medicine, Saga University, Japan.
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.
View Article and Find Full Text PDFEnter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!