Introduction: Subacute adult-acquired hemichorea is a striking presentation with a broad differential, including ischemic, metabolic, and inflammatory causes.
Case: We encountered a 74-year-old woman with rapid onset of hemichorea and associated encephalopathy. Following a thorough workup without identification of clear imaging or laboratory abnormalities, we empirically treated with IVIg. Her hemichorea dramatically improved. Due to relapses of hemichorea, she required repeat immunotherapy with IVIg or high dose steroids followed by maintenance mycophenolate.
Discussion: This case of seronegative autoimmune hemichorea highlights the importance of a high index of suspicion for an inflammatory etiology of chorea when other causes are ruled out and performing an immunotherapy trial.
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http://dx.doi.org/10.1002/acn3.52052 | DOI Listing |
Parkinsonism Relat Disord
August 2024
Division of General Neurology and Ataxia Unit, Department of Neurology and Neurosurgery, Universidade Federal de São Paulo, Sao Paulo, SP, Brazil. Electronic address:
Ann Clin Transl Neurol
May 2024
Division of Neuroimmunology and Neuroinfectious Diseases, Department of Neurology, Massachusetts General Hospital, Boston, Massachusetts, USA.
Introduction: Subacute adult-acquired hemichorea is a striking presentation with a broad differential, including ischemic, metabolic, and inflammatory causes.
Case: We encountered a 74-year-old woman with rapid onset of hemichorea and associated encephalopathy. Following a thorough workup without identification of clear imaging or laboratory abnormalities, we empirically treated with IVIg.
Cureus
March 2024
Neurology, Yasumi Hospital, Morioka, JPN.
Though rare, autoimmune paraneoplastic and non-paraneoplastic chorea can be leading causes of adult-onset acute/subacute chorea. Here, we report a case of acute-onset chorea with suspected autoimmune-mediated mechanisms in a 79-year-old female who exhibited acute-onset choreiform movements on the right side of her body. She tested positive for anti-centromere antibodies (ACAs) without displaying symptoms of scleroderma.
View Article and Find Full Text PDFJ Med Case Rep
March 2024
Department of Neurology, Institute of Medical Science, Banaras Hindu University, Varanasi, India.
Background: Diabetic striatopathy, also known as hyperglycemic hemichorea-hemiballismus, is a rare movement disorder associated with nonketotic hyperglycemia in patients with poorly controlled diabetes mellitus. The pathophysiology is not fully elucidated but may involve hyperviscosity, ischemia, and alterations in basal ganglia neurotransmitters.
Case Presentation: We present a case of a 64-year-old Asian female patient with longstanding poorly controlled type 2 diabetes mellitus who developed abrupt-onset right-sided hemichorea-hemiballismus.
SAGE Open Med Case Rep
January 2024
Colombo South Teaching Hospital, Kalubowila, Sri Lanka.
Diabetic striatopathy is a rare neurological complication of diabetes mellitus that presents with sudden onset hemichorea or hemiballismus and is associated with hyperglycemia and striatal abnormality, either by hyperdensity on non-contrast computer tomography or hyperintensity on T1-weighted magnetic resonance imaging. Here we report a 55-year-old female, from Sri Lanka, who presented with involuntary movements of the left upper and lower limbs. Her past medical history included diabetes mellitus and she was on warfarin 5 mg daily for a mechanical mitral and tricuspid valve replacement.
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