Socio-Psychiatric Consequences of Huntington's Disease

Dewhurst, Kenneth; Oliver, Jack; McKnight, Anthony

doi:10.1192/bjp.116.532.255

Cited by 168 publications

(85 citation statements)

References 3 publications

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“…There was no evidence of cognitive decline coinciding with the initial psy-chiatric presentation in either of our two cases. Mood symptoms and disorders in HD have been well described in the literature (4,18). One study found that in a HD population, up to 32.5% met DSM III-R criteria for major affective disorders and 9% for dysthymia (4).…”

Section: Discussionmentioning

confidence: 99%

Early Psychiatric Manifestations of Huntington's Disease: More Than Just a Movement Disorder

Leroi¹,

Michalon²

1997

DMJ

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Huntington's disease (HD), a neurodegenerative condition of the central nervous system, poses a major challenge for clinicians. The clinical presentation is variable and consists of cognitive, psychiatric and motor symptoms. The psychiatric manifestations often appear many years before the movement disorder. Lack of recognition of the psychiatric mani-festations may lead to delays in diagnosis and multidisciplinary interventions. The aim of this article is to highlight the psychiatric aspects of HD, particularly focusing on the early presenta-tion. Two cases of HD with prominent psychiatric symptoms are used to illustrate the preva-lence of these aspects of the disease. In the absence of treatment directly influencing the course of HD, psychiatrists remain central to the multidisciplinary team diagnosing and treating this debilitating condition.Huntington's disease (HD), which was first described by George Huntington in 1872, is a neurodegenerative disorder which presents as a syndrome of motor abnormalities, cognitive decline and neuropsychiatric symptoms. HD has an autosomal dominant pattern of in-heritance with complete penetrance. The gene for the disease was identified in March, 1993 (1,2), enabling predictive and prenatal testing for those at risk of inheriting the disease (3). The disease affects 5 to 8 per 100,000 in North America (4,5). The average age of onset is be-tween 35 and 40 years of age, however, juve-nile forms of the disease can result in symp-toms as early as age 4 (6).The course of HD is progressive and is marked by increasing motor difficulties, in-cluding chorea, dystonia and athetosis, as well as bradykinesia, dysarthria, dysphagia, and urinary incontinence (7). Death results an average of 17 years after onset of the disease and is caused by cardiac and pulmonary complica-tions, suicide, nutritional deficiencies and self-destructive behaviours (8).The HD gene (referred to as IT15), found on the short arm of chromosome 4, partly consists of an unstable triplicate repeat (CAG) sequence. The number of triplicate repeats is higher (more than 38 repeats) in those with HD than in the normal population, which has 11-38 repeats. The mRNA transcribed from the HD gene can be found in all organs of an affected person and codes for a protein which has been called "huntingtin". The relationship between this protein and the development of the pathological changes in HD is still unclear, although several studies have attempted to define the neurophysiology and neuropathology. Neuropathology reveals early and promi-nent degeneration of the caudate nucleus. Later cell loss occur in the globus pallidus, subthalamic nucleus, nucleus accumbens, cerebellum and parts of the cortex (9). Within the caudate nucleus, abnormalities of small-to-medium sized spiny neurons result in loss of the inhibitory neurotransmitter gamma aminobutyric acid (GABA) as well as substance P and enkephalins. There is relative sparing of somatostatin and dopamine (10).HD is generally considered a "neurologic" illness, falling ...

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Section: Discussionmentioning

confidence: 99%

Early Psychiatric Manifestations of Huntington's Disease: More Than Just a Movement Disorder

Leroi¹,

Michalon²

1997

DMJ

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“…Although an early study described anxiety as the most common prodromal symptom, occurring in 12% of 102 patients (Dewhurst 1970), it is the symptoms of anxiety that are more often seen rather than a primary anxiety disorder. This group of symptoms is likely to be secondary to underlying cognitive changes and should be considered as a separate neuropsychiatric behaviour disorder.…”

Section: Anxiety Disordersmentioning

confidence: 99%

Psychiatric and behavioural manifestations of Huntington's disease

Jauhar¹,

Ritchie²

2010

Adv. psychiatr. treat

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SummaryPatients with Huntington's disease can be a challenge to psychiatrists and to psychiatric services. We present a patient whose case history illustrates this and underlines the need for prompt and adequate treatment of psychiatric problems in this population. We discuss the psychiatric and behavioural manifestations of the illness, the treatments available and the role of psychiatric services in providing care for people with Huntington's disease.

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“…[7] Three main theories exist as to why some HD patients present with psychosis while others do not: (i) genetically, these patients have a different subtype of HD, [7] possibly linked to the length of the CAG repeats; [9,10] (ii) cooccurrence in a 'vulnerable' brain [7] and (iii) developmental effect due to the stress of growing up in a disrupted family structure secondary to the presence of serious illness, of which one has a 50% chance of developing. [11][12][13] Currently, although we are unable to distinguish clinically between the psychosis of HD and schizophrenia, there are various possible pointers. A recent review on secondary psychosis suggests that several questions can be asked to determine whether the psychosis is as a result of a general medical condition (GMC).…”

Section: Diagnostic V Predictive Testingmentioning

confidence: 99%