Mental disorders in Huntington’s disease

Клюшников, С.А.; Юдина, Е. Н.; Illarioshkin, S. N.; Иванова-Смоленская, И.А.

doi:10.14412/2074-2711-2012-2508

Neurology, Neuropsychiatry, Psychosomatics

2012

DOI: 10.14412/2074-2711-2012-2508

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Mental disorders in Huntington’s disease

С.А. Клюшников

Е. Н. Юдина

S. N. Illarioshkin

et al.

Abstract: Болезнь (хорея) Гентингтона (БГ) -наследственное хроническое прогрессирующее нейродегенеративное забо-левание с мультисистемным поражением головного мозга, передающееся по аутосомно-доминантному типу с практи-чески 100% пенетрантностью мутантного гена, отличающе-еся особой тяжестью и фатальностью течения. Впервые описано в 1872 г. американским врачом Дж. Гентингтоном [1]. Распространенность БГ в мире составляет в среднем 7-12 случаев на 100 тыс. населения [2,3]. Дебют заболева-ния приходится обычно на 4-5-е де… Show more

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Cited by 3 publications

References 41 publications

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Clinical features of vascular cognitive impairment

Martynova,

Vakhnina,

Zakharov

2024

RJTAO

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Vascular cognitive impairment (VCI) can occur in cerebrovascular disease of varying degree of severity, which requires further investigation.Objective: to identify specific features of attention deficit and executive function (EF) impairment in VCI and to determine the relationship between cognitive, neurological and radiological signs of the disease.Material and methods. The study involved 80 patients (51 with VCI – the main group and 29 healthy volunteers – the control group). All participants underwent clinical and neuropsychological examination and MRI of the brain. VCI was diagnosed according to 2014 VASCOG criteria. The neuropsychological examination included an assessment of the general cognitive function (MoCA test, MMSE); general EF (FAB, EXIT-25); episodic memory (12-word memory test and Benton Visual Retention Test); semantic memory (categorical association method); initiation and productivity of cognitive activity (literal associations); attention [TMT-A, Symbol-Digit Modalities Test (SDMT)]; cognitive flexibility (TMT-B); cognitive control (Stroop test).Results. As a result of the study, three subgroups of patients with VCI were identified: 1) predominantly attention deficit (AD); 2) AD and significant decrease in cognitive flexibility (CFD); 3) AD in combination with a significant cognitive control deficiency (CCD). The TMT-A and SDMT tests were significantly worse in all subgroups of VCI compared to the control group, with the worst SDMT score in subgroup 2. Subgroup 2 also had the longest time to complete the TMT-B test. Subgroup 3 was characterized by the worst Stroop test results, and uncontrolled arterial hypertension was more common in this group. Gait disturbances were significantly more frequent in subgroup 2, and increased reflexes of oral automatism and grasp reflex were observed in subgroup 3. White matter hyperintensity (WMH) was more pronounced in subgroup 2, and lacunar infarcts (LI) were more frequent in subgroup 3. Prevalence of LI and severity of WMH were lowest in subgroup 1.Conclusion. The main features of VCI are attention deficit and EF impairment, characteristics of EF disorders depend on the severity and type of vascular lesions of the brain. Progression of WMH is associated with more pronounced CFD and an increase in the number of LI is associated with CCD. There is a correlation between CFD and gait disturbances and between CCD and lack of primitive reflexes inhibition.

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Clinical features of vascular cognitive impairment

Martynova,

Vakhnina,

Zakharov

2024

RJTAO

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Huntington’s disease in psychiatric practice

Gasenko¹,

Arslanova²

2021

Z. nevrol. psikhiatr. im. S.S. Korsakova

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Chorea without chorea or mental disorders as a mask of Huntington’s disease

Кривоножкина

Chernaya

Miroshnikova

et al. 2021

РНЖ

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Huntington’s disease (HD) is an orphan disease with an average incidence of 5.5 cases per 100 000 population. It is characterized by a high degree of penetrance, variable expressivity, manifestation in different age periods. This disorder, transmitted in an autosomal dominant way, does not have gender differences. The disease often progresses slowly but steadily over many years, eventually leading to severe disability. The clinical picture of this disease has a pathognomonic combination of neurological (extrapyramidal) and neuropsychiatric disorders. With the addition of mental disorders, especially dementia, the diagnosis of HD becomes more likely and obvious. However, if disorders in the mental sphere, including irritability, irascibility, apathy, abulia, delirium, hallucinations, impaired memory and attention, become the debut of the disease, then they largely mask clinical picture. In such cases, patients are hastily made other diagnoses: psychopathy, schizophrenia, bipolar disorder, Alzheimer’s disease, and others. You should be wary of the possible secondary nature of neuropsychiatric disorders in patients, especially young patients.

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Mental disorders in Huntington’s disease

Cited by 3 publications

References 41 publications

Clinical features of vascular cognitive impairment

Clinical features of vascular cognitive impairment

Huntington’s disease in psychiatric practice

Chorea without chorea or mental disorders as a mask of Huntington’s disease

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