The diagnosis of Huntington's disease

Folstein, Susan E.; Leigh, R. John; Parhad, Irma M.; Folstein, Marshal F.

doi:10.1212/wnl.36.10.1279

Cited by 156 publications

(83 citation statements)

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“…We studied 23 HD patients diagnosed by a neurologist taking into consideration the clinical findings and a positive family history for HD, according to the criteria established by Folstein et al 5 . In 15 cases the diagnosis was genetically confirmed.…”

Section: Methodsmentioning

confidence: 99%

Language impairment in Huntington's disease

Azambuja¹,

Radanovic²,

Haddad³

et al. 2012

Arq. Neuro-Psiquiatr.

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Language alterations in Huntington's disease (HD) are reported, but their nature and correlation with other cognitive impairments are still under investigation. This study aimed to characterize the language disturbances in HD and to correlate them to motor and cognitive aspects of the disease. We studied 23 HD patients and 23 controls, matched for age and schooling, using the Boston Diagnostic Aphasia Examination, Boston Naming Test, the Token Test, Animal fluency, Action fluency, FAS-COWA, the Symbol Digit Modalities Test, the Stroop Test and the Hooper Visual Organization Test (HVOT). HD patients performed poorer in verbal fluency (p<0.0001), oral comprehension (p<0.0001), repetition (p<0.0001), oral agility (p<0.0001), reading comprehension (p=0.034) and narrative writing (p<0.0001). There was a moderate correlation between the Expressive Component and Language Competency Indexes and the HVOT (r=0.519, p=0.011 and r=0.450, p=0.031, respectively). Language alterations in HD seem to reflect a derangement in both frontostriatal and frontotemporal regions.

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Section: Methodsmentioning

confidence: 99%

Language impairment in Huntington's disease

Azambuja¹,

Radanovic²,

Haddad³

et al. 2012

Arq. Neuro-Psiquiatr.

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“…3 However, Huntington failed to mention cognitive decline, which is now recognised as a cardinal feature of the disease. 4 …”

Section: Hereditary Causes Of Choreamentioning

confidence: 99%

Chorea and related disorders

Bhidayasiri

Truong

2004

Postgraduate Medical Journal

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Chorea refers to irregular, flowing, non-stereotyped, random, involuntary movements that often possess a writhing quality referred to as choreoathetosis. When mild, chorea can be difficult to differentiate from restlessness. When chorea is proximal and of large amplitude, it is called ballism. Chorea is usually worsened by anxiety and stress and subsides during sleep. Most patients attempt to disguise chorea by incorporating it into a purposeful activity. Whereas ballism is most often encountered as hemiballism due to contralateral structural lesions of the subthalamic nucleus and/or its afferent or efferent projections, chorea may be the expression of a wide range of disorders, including metabolic, infectious, inflammatory, vascular, and neurodegenerative, as well as drug induced syndromes. In clinical practice, Sydenham’s chorea is the most common form of childhood chorea, whereas Huntington’s disease and drug induced chorea account for the majority of adult onset cases. The aim of this review is to provide an up to date discussion of this disorder, as well as a practical approach to its management.

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“…1 Clinical diag nosis is currently based on unequivocal motor signs of chorea and/or bradykinesia; 2 however, there are significant early cognitive deficits that may develop independently of motor signs. 3,4 In the symptomatic stages of Huntington disease (symp-HD), loss of medium spiny neurons and atrophy in the striatum are characteristic neuropathological changes.…”

Section: Introductionmentioning

confidence: 99%