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.
Increased Cognitive Functioning in Symptomatic Huntington's Disease As Revealed by Behavioral and Event-Related Potential Indices of Auditory Sensory Memory and Attention
