Cognitive and psychiatric disturbances are common manifestations of the neurodegeneration that occurs in Huntington's chorea. These may be the initial presenting features of the disorder.


Cognitive changes:

(1) subcortical dementia with impaired executive functioning (planning, organizing, judging, etc)

(2) impaired working memory (forgetfulness) with preserved long-term memory

(3) disordered sleep

(4) loss of concentration

(5) inflexibility (fixed thoughts)

(6) anosognosia (lack of awareness of defects)



• Lesions in the caudate nucleus disconnect the frontal cortex and result in the deterioration in executive functioning.


Psychiatric changes:

(1) personality change

(2) anxiety

(3) depression (with tendency to suicide)

(4) mania

(5) psychosis

(6) irritability and aggression

(7) disinhibition

(8) agitation

(9) loss of interest and apathy


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