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Related Concept Videos

Disorders of Erythrocytes01:27

Disorders of Erythrocytes

Disorders of erythrocytes, or red blood cells (RBCs), include a range of conditions affecting their number, shape, or function.
Erythrocyte disorders can be broadly categorized into two main types: anemic and polycythemic conditions.
A low oxygen-carrying capacity of the blood due to the loss, lower production, or destruction of erythrocytes is termed anemia. Hemorrhagic anemia, for example, occurs when bleeding from an external wound or internal ulcer reduces erythrocyte counts.
On the other...
Huntington Disease l: Introduction01:21

Huntington Disease l: Introduction

Huntington disease or HD is a progressive, fatal neurodegenerative disorder inherited in an autosomal dominant pattern.PathophysiologyIt is caused by expansion of the CAG trinucleotide repeat in the HTT gene on chromosome 4 (4p16.3), producing an abnormal huntingtin protein with an expanded polyglutamine tract. This misfolded protein disrupts cellular function, leading to neuronal death. Normal alleles have ≤26 repeats, 27–35 are intermediate (risk of expansion), 36–39 show reduced penetrance,...
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Chronic Kidney Disease II: Clinical Manifestations

Chronic Kidney Disease (CKD) progressively impairs multiple body systems due to the accumulation of uremic toxins, which disrupt cellular functions across various organs.Neurologic symptomsNeurologic symptoms often arise early in CKD, as uremic toxin buildup drives changes in cognitive and motor functions. Patients frequently experience fatigue, headache, confusion, difficulty concentrating, and, in severe cases, seizures. Peripheral neuropathy commonly manifests as burning sensations in the...
Alterations in Muscle Tone lll01:11

Alterations in Muscle Tone lll

Rigidity and myotonia are distinct abnormalities of muscle tone that affect resistance and relaxation during movement. Although both involve altered muscle contraction, they arise from different neurological and muscular mechanisms.CharacteristicsRigidity is characterized by uniform resistance to passive movement across the entire range, independent of speed, affecting flexors and extensors equally. It may appear as lead-pipe rigidity (smooth, constant resistance) or cogwheel rigidity...
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Cardiomyopathy II: Dilated Cardiomyopathy

Dilated cardiomyopathy, or DCM, is a progressive myocardial disorder characterized by ventricular chamber dilation and contractile dysfunction.EtiologyVarious factors can cause DCM, including hypertension and heavy alcohol intake, which contribute to the weakening and enlargement of the heart muscle. Viral infections, such as Coxsackievirus B, adenoviruses, and influenza, can lead to DCM by causing inflammation and damage to heart tissue. Certain chemotherapeutic agents, including daunorubicin,...
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Parkinson Disease l: Introduction

Parkinson’s disease is a chronic, progressive neurodegenerative disorder that primarily affects movement. It is characterized by motor symptoms such as resting tremors, muscle rigidity, bradykinesia (slowness of movement), and postural instability. Patients may notice hand tremors at rest, stiffness during movement, or a shuffling gait. In addition to motor features, non-motor symptoms include sleep disturbances, mood and behavioral changes, constipation, and cognitive impairment, all of which...

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High-speed Video Microscopy Analysis for First-line Diagnosis of Primary Ciliary Dyskinesia
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Polycythemia and chorea.

Michael M Marvi1, Mark F Lew

  • 1Department of Neurology, University of Southern California, Los Angeles, CA 90033, USA. michaelmarvi@yahoo.com

Handbook of Clinical Neurology
|April 19, 2011
PubMed
Summary

Polycythemia vera, a blood disorder, can cause neurological issues like chorea. Effective management of this condition requires venesections and chemotherapy for better outcomes.

Area of Science:

  • Hematology
  • Neurology
  • Oncology

Background:

  • Polycythemia vera is a myeloproliferative disorder characterized by increased red blood cell mass.
  • Neurological manifestations, including chorea and ballism, are common due to thrombosis and hyperviscosity.
  • The pathophysiology of polycythemia vera-associated chorea remains unclear.

Purpose of the Study:

  • To review the neurological manifestations of polycythemia vera, focusing on chorea.
  • To explore proposed pathophysiological mechanisms for basal ganglia dysfunction in this condition.
  • To discuss diagnostic findings and management strategies for polycythemia vera-associated chorea.

Main Methods:

  • Literature review of polycythemia vera and its neurological complications.

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  • Analysis of proposed mechanisms for chorea, including hypoperfusion and neurotransmitter alterations.
  • Examination of neuroimaging and pathological findings.

    • Main Results:

    • Chorea in polycythemia vera is typically generalized, involving orofaciolingual and appendicular muscles.
    • Proposed mechanisms include hypoperfusion, receptor hypersensitivity, and altered dopamine metabolism.
    • Neuroimaging studies have not identified a clear basis, but pathology may show cerebral venous thrombosis or demyelination.

    Conclusions:

    • Polycythemia vera can lead to significant neurological symptoms like chorea.
    • Management requires addressing the underlying polycythemia vera with venesections and chemotherapy.
    • While neuroleptics may help symptoms, long-term outcomes depend on disease control, with chorea potentially persisting or remitting spontaneously.