Huntington Disease: Difference between revisions
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The clinical manifestations of HD usually present between the ages of 35 and 45 years, but can begin at any age from childhood to old age.<br> | The clinical manifestations of HD usually present between the ages of 35 and 45 years, but can begin at any age from childhood to old age.<br> | ||
The clinical features of Huntington disease (HD) include a movement disorder, a cognitive disorder, and a behavioral disorder. Patients may present with one or all disorders in varying degrees. | The clinical features of Huntington disease (HD) include a movement disorder, a cognitive disorder, and a behavioral disorder. Patients may present with one or all disorders in varying degrees. | ||
=== Movement Disorder === | |||
'''Chorea''' (derived from the Greek word meaning to dance) is the most common movement disorder seen in HD. | |||
Initially, mild chorea may cause the patient to appear restless, as if they are fidgeting. This progresses, and severe chorea may appear as uncontrollable writhing and flailing of the extremities, which interferes with function. | |||
As the disease progresses, chorea coexists with ,and gradually is replaced by, dystonia and parkinsonian features, such as bradykinesia, rigidity, and postural instability. | |||
In advanced disease, patients develop an akinetic-rigid syndrome, with minimal or no chorea. Other late features are spasticity, clonus, and extensor plantar responses. | |||
== Diagnostic Procedures == | == Diagnostic Procedures == | ||
Revision as of 00:36, 14 September 2015
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General[edit | edit source]
Huntington disease (HD) is an incurable, adult-onset, progressive deurodegenerative disorder which presents with involuntary movements, dementia, and behavioral changes[1].
HD is named after George Huntington, the physician who described it as hereditary chorea in 1872[2].
Mechanism of Injury / Pathological Process
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add text here relating to the mechanism of injury and/or pathology of the condition
Clinical Presentation[edit | edit source]
The clinical manifestations of HD usually present between the ages of 35 and 45 years, but can begin at any age from childhood to old age.
The clinical features of Huntington disease (HD) include a movement disorder, a cognitive disorder, and a behavioral disorder. Patients may present with one or all disorders in varying degrees.
Movement Disorder[edit | edit source]
Chorea (derived from the Greek word meaning to dance) is the most common movement disorder seen in HD.
Initially, mild chorea may cause the patient to appear restless, as if they are fidgeting. This progresses, and severe chorea may appear as uncontrollable writhing and flailing of the extremities, which interferes with function.
As the disease progresses, chorea coexists with ,and gradually is replaced by, dystonia and parkinsonian features, such as bradykinesia, rigidity, and postural instability.
In advanced disease, patients develop an akinetic-rigid syndrome, with minimal or no chorea. Other late features are spasticity, clonus, and extensor plantar responses.
Diagnostic Procedures[edit | edit source]
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Outcome Measures[edit | edit source]
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Management / Interventions
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Differential Diagnosis
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Key Evidence[edit | edit source]
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Resources
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Case Studies[edit | edit source]
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Recent Related Research (from Pubmed)[edit | edit source]
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References[edit | edit source]
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