Huntington's chorea: causes, symptoms, diagnosis, polysomnography, treatment

By Cristiano Antonino On Aug 29, 2022

Huntington’s chorea, an overview: extrapyramidal syndromes are neurological disorders involving structures of the central nervous system involved in the control of motor functions

Clinically, these syndromes are classically subdivided into hypertonic-hypokinetic, the paradigmatic picture of which is manifested in Parkinson’s disease, and hypotonic-hypokinetic, exemplified by Huntington’s chorea.

Chronic chorea (Huntington’s chorea) and choreic syndromes

Chronic chorea (Huntington’s chorea) is a hereditary disease, transmitted by an autosomal dominant mechanism with anatomopathological lesions in the putamen and caudate; the choreic syndromes, on the other hand, are a group of diseases with different aetiologies and united by the presence of choreic movements.

Clinical picture

Huntington’s chorea arises between the ages of 30 and 50 and is characterised by hypotonia and hyperkinesias consisting of involuntary, aphinalistic, irregular and asymmetrical movements affecting mainly the distal extremities of the limbs.

Personality disorders are also present (depression with suicidal ideation is frequent in these patients) and mental deterioration that may evolve into a full-blown dementia picture.

With regard to sleep disorders, difficulty falling asleep and nocturnal awakenings have been described (Puca et al., 1988).

Polysomnographic picture

Very few polygraphic investigations have been conducted in this pathology; moreover, they are generally performed during pharmacologically induced sleep and with mixed results.

Winfield and Sparer, during sleep with thiopental, had found in all the syndromes with lesions of the basal ganglia, including the chorea, absence or depression of the spindles; a significant increase in the spindles compared to normal controls had instead been shown, in chronic and acute choreics, during sleep facilitated by secobarbital and chlorpromazine by Puca et al. (1988).

Given the conflicting observations in the literature, Puca et al. (1988) examined spontaneous sleep in chronic choreics.

The choreics showed little sleep, delayed falling asleep, frequent awakening and being awake for a long time.

Their REM sleep appeared poor and slow-wave sleep poorly represented but with great spindling richness (contrary to other dementia forms).