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Uncontrolled movements

Hard to walk

My hands and feet are clumsy

Involuntary movements

Walking difficulties

Poor coordination

Abnormal involuntary movements

Not seeing your symptoms? No worries!

What is Spiny Red Cell Chorea?

Spiny red cell chorea, also known as chorea-acanthocytosis or Levine-Critchley syndrome, is a very rare neurological disorder characterized by high numbers of misshapen, spiny red blood cells (acanthocytes) circulating in the bloodstream, combined with rapid, involuntary, purposeless movements (chorea). Additional symptoms can include include seizures and odd behavior. The disorder is caused by a mutation on the VPS13A gene and runs in families.

Typical Symptoms of Spiny Red Cell Chorea

Diagnostic Questions for Spiny Red Cell Chorea

Your doctor may ask these questions to check for this disease:

  • Do you have uncontrollable movements in your hands, legs, or face?
  • Do you have difficulty walking normally?
  • Are you having difficulty moving your arms and legs?
  • Do you have difficulty with fine motor control or coordination in your hands and feet?
  • Do you have repetitive movements like shoulder jerking, facial muscle twitching, or eye blinking?

Treatment of Spiny Red Cell Chorea

Currently, there is no treatment to cure, prevent, or slow the progression of Spiny red cell chorea or Chorea-acanthocytosis. Treatment primarily focuses on symptom management and support.

Reviewed By:

Benjamin Kummer, MD

Benjamin Kummer, MD (Neurology)

Dr Kummer is Assistant Professor of Neurology at the Icahn School of Medicine at Mount Sinai (ISMMS), with joint appointment in Digital and Technology Partners (DTP) at the Mount Sinai Health System (MSHS) as Director of Clinical Informatics in Neurology. As a triple-board certified practicing stroke neurologist and informaticist, he has successfully improved clinical operations at the point of care by acting as a central liaison between clinical neurology faculty and DTP teams to implement targeted EHR configuration changes and workflows, as well as providing subject matter expertise on health information technology projects across MSHS. | Dr Kummer also has several years’ experience building and implementing several informatics tools, presenting scientific posters, and generating a body of peer-reviewed work in “clinical neuro-informatics” – i.e., the intersection of clinical neurology, digital health, and informatics – much of which is centered on digital/tele-health, artificial intelligence, and machine learning. He has spearheaded the Clinical Neuro-Informatics Center in the Department of Neurology at ISMMS, a new research institute that seeks to establish the field of clinical neuro-informatics and disseminate knowledge to the neurological community on the effects and benefits of clinical informatics tools at the point of care.

Shohei Harase, MD

Shohei Harase, MD (Neurology)

Dr. Harase spent his junior and senior high school years in Finland and the U.S. After graduating from the University of Washington (Bachelor of Science, Molecular and Cellular Biology), he worked for Apple Japan Inc. before entering the University of the Ryukyus School of Medicine. He completed his residency at Okinawa Prefectural Chubu Hospital, where he received the Best Resident Award in 2016 and 2017. In 2021, he joined the Department of Cerebrovascular Medicine at the National Cerebral and Cardiovascular Center, specializing in hyperacute stroke.

From our team of 50+ doctors

Content updated on Jan 14, 2025

Following the Medical Content Editorial Policy

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References