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Huntington Disease

Reviewed 06/2019
 


BASICS

DESCRIPTION

  • Inherited neurodegenerative disease characterized by progressive motor and psychiatric dysfunction, dementia, and ultimately death

    • Onset of symptoms typically occurs between 30 and ...

DIAGNOSIS

HISTORY

  • Patients with an affected family member may present requesting investigation in the presymptomatic stage.

  • Physical symptoms are typically noted first. Patients may present with choreif...

TREATMENT

GENERAL MEASURES

  • Genetic counseling

  • Speech, swallow, occupational, and physical therapy to preserve maximal function

  • Psychiatric/psychological consultation

  • Feeding tubes and smoking cessation in...

ONGOING CARE

FOLLOW-UP RECOMMENDATIONS

  • Full activity as long as possible

  • Physical therapy:

    • Balance and walking problems

    • Strength-building exercises

    • Exercise to help combat depression

  • Occupational therapy ...

REFERENCES

1
Gudesblatt M, Tarsy D. Huntington’s disease: a clinical review. Neurol Rev.  2011;(Suppl):S1–S8. [View Abstract on OvidMedline]
2
Pidgeon C, Rickards H. The pathophysiology and pharmaco...

ADDITIONAL READING

  • Reilmann R. Pharmacological treatment of chorea in Huntington’s disease—good clinical practice versus evidence-based guideline. Mov Disord.  2013;28(8):1030–1033. [View Abstrac...

CODES

ICD10

G10 Huntington’s disease 

ICD9

333.4 Huntington’s chorea 

SNOMED

  • 58756001 Huntington’s chorea (disorder)

  • 230299004 Juvenile onset Huntington’s disease

  • 230300007 Late onset Huntington’s disease

  • 230...

CLINICAL PEARLS

  • HD is an autosomal dominantly inherited disease of insidious onset (typically) between 30 and 50 years.

  • There is no known cure.

  • Early symptoms include rhythmic choreiform movements, dyst...

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