The syndrome of acute bilateral basal ganglia (BG) lesions in patients with diabetic uremia normally affects Asian patients and usually presents with parkinsonian symptoms. We report two patients with this syndrome suffering from acute generalized choreic movements. The brain MRI of both patients revealed a cytotoxic-type of edema in the bilateral BG during the acute phase of the syndrome. After supportive treatment, they completely recovered from generalized chorea and the period of hyperkinesia corresponded to the acute exacerbation of the metabolic disturbance. Follow-up brain MRI revealed complete resolution of the abnormal signal. Thus, generalized chorea may be the initial presenting symptom of the syndrome and bilateral BG lesions probably originate from cytotoxic-type edema.
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http://dx.doi.org/10.1016/j.jocn.2011.01.021 | DOI Listing |
Nat Commun
December 2024
Department of Theory and Bio-Systems, Max Planck Institute of Colloids and Interfaces, 14476, Potsdam, Germany.
Neurodegeneration in Huntington's disease (HD) is accompanied by the aggregation of fragments of the mutant huntingtin protein, a biomarker of disease progression. A particular pathogenic role has been attributed to the aggregation-prone huntingtin exon 1 (HTTex1), generated by aberrant splicing or proteolysis, and containing the expanded polyglutamine (polyQ) segment. Unlike amyloid fibrils from Parkinson's and Alzheimer's diseases, the atomic-level structure of HTTex1 fibrils has remained unknown, limiting diagnostic and treatment efforts.
View Article and Find Full Text PDFFront Endocrinol (Lausanne)
December 2024
Department of Neurology, First Medical Center of Chinese People's Liberation Army (PLA) General Hospital, Beijing, China.
Background: Diabetic striatopathy (DS) is a rare disorder characterized by clinical manifestations of hemichorea, non-ketotic hyperglycemia, and high signal on T1-weighted MRI or high density on CT scan in basal ganglia, typically associated with poor glycemic control.
Objective: This study aimed to analyze clinical characteristics of patients with diabetic striatopathy to raise awareness amongst physicians, especially endocrinologists, about this rare neurological manifestation in patients with diabetes.
Methods: We retrospectively analyzed the data on clinical presentations, laboratory workups, and cranial CT and MRI of six patients with DS who were admitted to our hospital from October 2013 to June 2022.
Eur J Pharmacol
January 2025
Department of Biotechnology, Indian Institute of Technology Hyderabad, Kandi, Sangareddy, Telangana, 502284, India. Electronic address:
Neurodegenerative disorders are characterized by a progressive decline of specific neuronal populations in the brain and spinal cord, typically containing aggregates of one or more proteins. They can result in behavioral alterations, memory loss and a decline in cognitive and motor abilities. Various pathways and mechanisms have been outlined for the potential treatment of these diseases, where redox regulation is considered as one of the most common druggable targets.
View Article and Find Full Text PDFHeliyon
October 2024
Department of Biochemistry and Molecular Medicine, College of Medicine, Taibah University Madinah, Saudi Arabia.
Chorea-acanthocytosis (ChAc) is a rare inherited disease of the nervous system. In this disease the neurological manifestations are associated with acanthocytosis of the red blood cells. The clinical features appear in the third to fourth decades of life.
View Article and Find Full Text PDFSci Rep
December 2024
Cognition and Brain Plasticity Unit, Bellvitge Biomedical Research Institute - IDIBELL], L'Hospitalet de Llobregat, Barcelona, 08097, Spain.
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