Negative influence of L-dopa on subjectively assessed sleep but not on nocturnal polysomnography in Parkinson's disease.

BACKGROUND: Sleep disorders are highly prevalent among patients with Parkinson's disease (PD). Chronic medication with L-dopa may be one of the factors that contributes to poor sleep quality. The aim of this study was to assess the effects of long term use of L-dopa on objective and subjective measures of sleep quality in PD patients. METHODS: Twenty-seven PD patients (mean age 62.5 ± 8.6 years, mean disease duration 7.3 ± 5.9 years, 11 females) underwent nocturnal polysomnography. Their sleep was rated subjectively using the Parkinson's disease sleep scale (PDSS), and their disease severity was assessed using the unified Parkinson's disease severity scale (UPDRS) standard questionnaire. Doses of L-dopa and other medications were correlated with parameters of sleep quality. The polysomnographic recordings were compared with those from 24 age- and gender-matched normal controls. RESULTS: The patients showed decreased total sleep time (TST) and sleep efficiency (SE), prolonged sleep onset and REM sleep latency and wake after sleep onset (WASO). Parts I-III of the UPDRS scores correlated with TST, SE and WASO but not with PDSS scores. L-dopa dosage and part IV of the UPDRS correlated with PDSS scores but not with polysomnographic parameters. CONCLUSIONS: Higher doses of chronically administered L-dopa correlated with lower sleep quality according to the subjective measures but not according to the polysomnographic parameters, which were related to the severity of PD symptoms. The low sleep quality according to the subjective measurements may result from complications of therapy at high doses of L-dopa.

[Clinical and genetic study of juvenile form of Huntington's disease]. / Badania kliniczne i genetyczne w mlodzienczej postaci choroby Huntingtona.

Huntington's disease (HD) is an autosomal dominant neurodegenerative disorder caused by high instability and extension of CAG sequences within the coding region of IT15 gene. It affects both sexes and age at onset of the disease may be different but usually occurs in midlife. The term Juvenile Huntington's disease is generally applied to 10% of the cases with onset before 20. We present clinical features and results of DNA analysis in 16 patients from 14 families aged 9 to 36. The age of onset was between 5 to 20 years; duration of the disease was from 2 to 16 years. In 10 cases the mutated gene was transmitted by the affected father; only in two cases by the mother. In all cases anticipation manifested by earlier onset of the disease in subsequent generations and expansion of CAG repeats was documented. The number of CAG repeats was between 50 and 92 (mean 67.3). Progressive mental deterioration, declining school performance, hyperactivity and emotional disturbances were the first symptoms of juvenile HD. Neuropsychological assessment showed mean IQ in Wechsler test 59.6 and Mini-Mental State Examination scores 22.8. Rigidity and bradykinesia were predominant features in the cases with juvenile onset, the remaining ones developed choreatic movements. Three persons had epileptic seizures; two (both females) revealed behaviour and psychiatric disturbances. Amplitudes of somatosensory evoked potentials, visual evoked potentials and brainstem auditory evoked potentials were markedly reduced. MRI of the brain showed atrophy of heads of the caudate nuclei, putamen and globus pallidus.