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Condition/Disorder Synonyms

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  • Huntington's chorea

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ICD-9-CM Code

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  • 333.4 Huntington's chorea

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ICD-10-CM Code

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  • G10 Huntington's disease

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Preferred Practice Pattern

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Key Features

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Description

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  • Degeneration of nerve cells in the brain

  • Hereditary disorder

  • Children of people with Huntington's have 50% of inheriting the gene

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Essentials of Diagnosis

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  • Genetic dysfunction on 4th chromosome

  • Autosomal dominant inheritance

  • Characterized by choreoathetosis and dementia

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General Considerations

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  • Family history and genetic testing to confirm diagnosis

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Demographics

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  • Adult onset generally in 4th or 5th decade of life

  • Early onset, not common but may occur in adolescence

  • Most common in Caucasians of European ancestry

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Clinical Findings

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Signs and Symptoms

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  • Slight but evident alteration in character initially, followed by issues of self-control, eventually leading to failure of all cognitive function

  • Difficulty with attention and concentration, poor mental flexibility

  • Movement abnormalities begin in hands and face, initial slowing of movement and loss of coordination, progressing to chorea, then athetosis

  • Degree and continuous nature of movement causes most patients to experience extreme weight loss and malnutrition

  • Behavioral changes

  • Irritability

  • Rigidity

  • Tremors

  • Paranoia

  • Dementia

  • Unsteady gait

  • Abnormal reflexes

  • Memory loss

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Functional Implications

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  • Loss of ability to interact in society and eventually with family members

  • Loss of ability to care for self mentally, emotionally, physically

  • Loss of functional mobility and associated cognitive processes

  • Difficulty swallowing

  • Limited ambulation

  • Poor communication, speech impairments

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Possible Contributing Causes

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  • Genetic disorder; no other known contributing causes

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Differential Diagnosis

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  • Parkinson's disease

  • Alzheimer's disease

  • Cerebral infection

  • Hyperglycemia

  • Stroke

  • Thyrotoxicosis

  • Tardive dyskinesia

  • Lupus

  • Sydenham chorea

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Means of Confirmation or Diagnosis

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Laboratory Tests

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  • DNA/genetic testing

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Imaging

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  • Head CT may show loss of brain tissue

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Referrals/Admittance

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  • To neurologist for imaging, disease management

    • Medicine: fluoxetine, carbamazepine, anti-psychotic agents

  • To support groups for psychological support

    • Huntington's Disease Society of America

  • To hospice for end-of-life care

  • To geneticist for genetic counseling

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Impairments

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  • Arousal

  • Attention

  • Behavior

  • Cognition

  • Gait

  • Wheelchair mobility

  • Balance

    • Static sit

    • Dynamic sit

    • Static stand

    • Dynamic stand

    • Moving base of support (BOS)

  • Muscle strength

  • Muscle recruitment

  • Coordination

  • Postural control (due to choreoathetosis)

  • Posture (later stages)

  • Range of motion (ROM)

  • Reflexes

    • Deep tendon reflexes (DTR)

    • Babinski

    • Clonus

  • Muscle tone

  • Motor planning

  • Bed mobility

  • Transfers

  • Endurance

  • Aerobic capacity

  • Self-care

  • Home management

  • Fine motor

  • Respiratory compromise

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