Neuropsychiatric aspects of Huntington's disease

Abstract

Objective-Neuropsychiatric symptoms are common in Huntington's disease and have been considered its presenting manifestation. Research characterising these symptoms in Huntington's disease is variable, however, encumbered by limitations within and across studies. Gaining a better understanding of neuropsychiatric symptoms is essential, as these symptoms have implications for disease management, prognosis, and quality of life for patients and caregivers. Method-Fifty two patients with Huntington's disease were administered standardised measures of cognition, psychiatric symptoms, and motor abnormalities. Patient caregivers were administered the neuropsychiatric inventory. Results-Ninety eight per cent of the patients exhibited neuropsychiatric symptoms, the most prevalent being dysphoria, agitation, irritability, apathy, and anxiety. Symptoms ranged from mild to severe and were unrelated to dementia and chorea. Conclusions-Neuropsychiatricsymptoms are prevalent in Huntington's disease and are relatively independent of cognitive and motor aspects of the disease. Hypothesised links between neuropsychiatric symptoms of Huntington's disease and frontal-striatal circuitry were explored. Findings indicate that dimensional measures of neuropsychiatric symptoms are essential to capture the full range of pathology in Huntington's disease and are vital to include in a comprehensive assessment of the disease. (J Neurol Neurosurg Psychiatry 2001;71:310-314)

A dopamine agonist, pramipexole, and cognitive functions in Parkinson's disease

“…In trials calling for semantic category, the patients did not differ from controls. They were provided with superordinate categories to assist retrieval, and maybe this is the reason for the intact category Huntington's disease is characterised by striatal pathology and patients early in the course of the disease demonstrate specifically cognitive impairment caused by frontostriatal dysfunction (20).…”

Apathy in Parkinson's disease: clinical features, neural substrates, diagnosis, and treatment

“…78,143,144 Prevalence of apathy is substantially higher in neurodegenerative diseases with more prominent involvement of the prefrontal cortex and caudate nuclei: it is present in 60-90% of patients with progressive supranuclear palsy, 75-95% of patients with frontotemporal dementia, and 55-90% of patients with Huntington's disease. [145][146][147][148] In these diseases, apathy is one of the fi rst neuropsychiatric symptoms to appear, is predictive of disease onset, 149,150 and is more clearly distinguished from depression than it is in Parkinson's disease. [151][152][153] A consideration of the multidimensional nature of apathy, as presented in this Review, would be useful in understanding the major components of apathy in each of these neurodegenerative diseases.…”

Sleep deficits but no metabolic deficits in premanifest Huntington's disease

“…It is caused by an abnormal expansion of a trinucleotide cytosine–adenosine–guanosine repeat (CAG)3 in exon 1 of the huntingtin gene leading to the ubiquitous expression of mutant huntingtin ( Htt ) 4. It is characterized by progressively worsening motor and nonmotor deficits including cognitive abnormalities (which over time lead to dementia), neuropsychiatric symptoms,5 weight loss,6 and sleep and circadian disturbances (for a review see Videnovic et al7). Although pathogenic pathways are beginning to be unraveled offering targets for treatment,8 the precise pathophysiological mechanisms of HD are poorly understood 9.…”