Psychiatric Symptoms Do Not Correlate With Cognitive Decline, Motor Symptoms, or CAG Repeat Length in Huntington's Disease

Zappacosta, B.; Monza, D.; Meoni, Cesare; Austoni, Laura; Soliveri, Paola; Gellera, Cinzia; Alberti, R.; Mantero, Mario; Penati, G.; Caraceni, T.; Girotti, F.

doi:10.1001/archneur.1996.00550060035012

Cited by 90 publications

(61 citation statements)

References 44 publications

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“…Still others experience marked motor, mood, and cognitive symptoms simultaneously (Brandt and Butters 1986;Folstein 1989;Myers et al 1991;Claes et al 1995;Zappacosta et al 1996; Thompson et al 2002). Interestingly, observations in monozygotic twins who inherited identical HTT genes with the same repeat length exhibit marked differences in their symptom profile (Georgiou et al 1999;Friedman et al 2005;Gomez-Esteban et al 2007).…”

Section: Symptomatologymentioning

confidence: 99%

The Neuropathology of Huntington’s Disease

Waldvogel

Kim

Tippett

et al. 2014

Current Topics in Behavioral Neurosciences

204

138

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The basal ganglia are a highly interconnected set of subcortical nuclei and major atrophy in one or more regions may have major effects on other regions of the brain. Therefore, the striatum which is preferentially degenerated and receives projections from the entire cortex also affects the regions to which it targets, especially the globus pallidus and substantia nigra pars reticulata. Additionally, the cerebral cortex is itself severely affected as are many other regions of the brain, especially in more advanced cases. The cell loss in the basal ganglia and the cerebral cortex is extensive. The most important new findings in Huntington's disease pathology is the highly variable nature of the degeneration in the brain. Most interestingly, this variable pattern of pathology appears to reflect the highly variable symptomatology of cases with Huntington's disease even among cases possessing the same number of CAG repeats.

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Section: Symptomatologymentioning

confidence: 99%

The Neuropathology of Huntington’s Disease

Waldvogel

Kim

Tippett

et al. 2014

Current Topics in Behavioral Neurosciences

204

138

View full text Add to dashboard Cite

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“…49,51,95,125,126,[154][155][156]158,160,[162][163][164] Finally, HD and PD patients are impaired on "frontal" neuropsychological tests, such as the stylus maze, verbal fluency, Tower of Toronto, and the Wisconsin Card Sorting Test. 49,70,81,95,[155][156][157]161,[165][166][167][168][169][170][171] There are, however, some differences between the cognitive deficits in HD and PD, although these are often overlooked. HD but not PD patients are reported to have concentration difficulties.…”

Section: Cognitive Symptomsmentioning

confidence: 99%

“…The most prevalent behavioral disturbances associated with HD include: (1) personality and behavioral changes, which include on the one hand impulsive and erratic behavior, increased irritability, aggressiveness and labile mood, and on the other hand, reduced spontaneity and initiative; 49,50,125,126,193 and (2) definable psychiatric disorders, namely, depressive and manic-depressive mood disorder, schizophreniform psychosis, and less commonly, obsessive compulsive disorder. 50,51,125,126,171,[193][194][195][196][197] Since damage to the frontal cortex or basal ganglia can lead to symptoms similar to those outlined above, 96,[198][199][200] the emotional disturbances in HD were suggested to reflect disturbed frontostriatal functioning. 96,201 As noted above, Litvan et al 101 suggested that the symptoms of agitation, irritation, and euphoria in HD are secondary to underactivity of the indirect pathways which arise from the ventromedial caudate and ventral striatum, respectively.…”

Section: Emotional Symptomsmentioning

confidence: 99%

Open interconnected model of basal ganglia‐thalamocortical circuitry and its relevance to the clinical syndrome of Huntington's disease

Joel

2001

Movement Disorders

145

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The early stages of Huntington's disease (HD) present with motor, cognitive, and emotional symptoms. Correspondingly, current models implicate dysfunction of the motor, associative, and limbic basal ganglia-thalamocortical circuits. Available data, however, indicate that in the early stages of the disease, striatal damage is mainly restricted to the associative striatum. Based on an open interconnected model of basal ganglia-thalamocortical organization, we provide a detailed account of the mechanisms by which associative striatal pathology may lead to the complex pattern of motor, cognitive, and emotional symptoms of early HD. According to this account, the degeneration of a direct and several indirect pathways arising from the associative striatum leads to impaired functioning of: (1) the motor circuit, resulting in chorea and bradykinesia, (2) the associative circuit, resulting in abnormal eye movements, "frontal-like" cognitive deficits and "cognitive disinhibition," and (3) the limbic circuit, resulting in affective and psychiatric symptoms. When relevant, this analysis is aided by comparing the symptomatology of HD patients to that of patients with mild to moderate Parkinson's disease, since in the latter there is similar dysfunction of direct pathways but opposite dysfunction of indirect pathways. Finally, we suggest a potential novel treatment of HD and provide supportive evidence from a rat model of the disease.

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“…Also, MTR and COG were significantly related: Those with worse motor function (i.e., higher motor scores) also had worse cognitive function (i.e., lower cognitive scores). (Zappacosta et al, 1996). The only apparent exception to this general rule appears to be apathy, which does relate to cognitive decline in HD (Baudic et al, 2006).…”

Section: Resultsmentioning

confidence: 99%