Non-ketotic hyperglycemia is an unusual and rare cause of hemichorea-hemiballismus. Correction of the hyperglycemia usually results in total resolution of the signs and symptoms. We present the case and medical imaging findings of a 66-year-old female who presented with steadily worsening choreiform and ballistic movements of the right upper and lower extremities over a 2-week period. Her serum glucose was greater than 600 mg/dL, and no ketones were present. CT scan and MR demonstrated left basal ganglia abnormalities suggesting hyperglycemia-related hemichorea-hemiballismus syndrome. Restoration of euglycemia led to eventual resolution of all symptoms. Knowledge of this disorder is paramount so as to rule out other causes of intracranial pathology.
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http://dx.doi.org/10.14740/jocmr2259w | DOI Listing |
JA Clin Rep
December 2024
Department of Anesthesiology, Niigata University Medical and Dental Hospital, 1-754 Asahimachi-Dori, Chuo-Ku, Niigata, 951-8520, Japan.
Background: Non-ketotic hyperglycinemia (NKH) is a rare autosomal recessive disorder caused by defects in the glycine cleavage system, leading to elevated glycine levels in the central nervous system. NKH manifests in various forms, with the neonatal type being the most severe and often associated with high mortality and significant neurological impairment. This case report highlights the successful uses of desflurane and nitrous oxide for anesthetic management in a patient with NKH.
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.
BJR Case Rep
November 2024
Department of Radiology, Zhongshan Hospital Affiliated to Xiamen University, School of Medicine, Xiamen University, Xiamen, Fujian 361004, China.
Non-ketotic hyperglycaemia (NKH)-induced hemichorea-hemiballismus (HC-HB) is an infrequent reversible condition observed in individuals with poorly controlled diabetes. In this report, we present a case of NKH-induced HC-HB exhibiting distinctive morphological and functional alterations on conventional magnetic resonance imaging (MRI), diffusion-weighted imaging (DWI), and H magnetic resonance spectroscopy (MRS), followed by subsequent monitoring. A 70-year-old male with a 20-year history of diabetes presented with severe unilateral involuntary movement.
View Article and Find Full Text PDFCureus
September 2024
Diabetes and Endocrinology, Peterborough City Hospital, Peterborough, GBR.
Latent autoimmune diabetes (LAD) involves gradual autoimmune destruction of the pancreatic beta cells, leading to reduced insulin production. When it occurs in adults, the term "latent autoimmune diabetes in adults (LADA)" is used, and when it occurs in young people, the term "latent autoimmune diabetes in the young (LADY)" is used. Patients usually present with symptoms suggestive of type 2 diabetes, and test positive for islet cell antibodies, but do not require insulin therapy at diagnosis and for up to six months thereafter.
View Article and Find Full Text PDFCureus
August 2024
General Medicine, Saveetha Medical College and Hospital, Chennai, IND.
Diabetic striatopathy (DS) is an acute hyperkinetic movement disorder arising from non-ketotic hyperglycemia. This condition predominantly affects females and is more common in the elderly, highlighting the interplay between diabetes, striatal pathology, and neurological movement disorders. DS is characterized by involuntary movements, such as hemichorea or hemiballism, and distinctive neuroimaging findings that can be mistaken for more common cerebrovascular events.
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