Psychosis assessment in early-stage Parkinson's disease: comparing Parkinson's psychosis questionnaire with the brief psychiatric rating scale in a portuguese sample.

Psychotic symptoms in Parkinson's disease (PD) are frequent, disabling, and an important prognostic factor. Thus, screening instruments for detecting psychosis in PD are needed. For this purpose, we applied the Parkinson's Psychosis Questionnaire (PPQ), a short structured questionnaire, which requires no specific training, along with the Brief Psychiatric Rating Scale, expanded version (BPRS-E), for rating general psychopathology, including psychotic symptoms. We evaluated, in a cross-sectional study, a Portuguese sample of 36 early-stage PD patients (mean age of 73 years; mean duration of illness of 3.2 years). The PPQ total score correlated with the BPRS-E total score (0.359; P = 0.032) and with the BPRS-E-positive symptoms score (0.469; P = 0.004). The prevalence of psychosis (41.7%) was higher than expected. Sampling bias and detection of minor psychotic phenomena may have contributed to this result. These findings suggest that the PPQ should be further evaluated as a feasible assessment for psychotic symptoms in PD.

Autosomal dominant cerebellar ataxia: frequency analysis and clinical characterization of 45 families from Portugal.

BACKGROUND AND PURPOSE: The relative frequency of the different autosomal dominant cerebellar ataxia (ADCA) varies widely amongst different geographic locations. Here we describe a series of 45 ADCA families from Portugal. METHODS: Patients with progressive cerebellar dysfunction of autosomal dominant transmission underwent a clinical examination protocol and genetic testing for spinocerebellar ataxia (SCA)1 to Machado-Joseph disease (MJD)/SCA3, SCA6, SCA7, SCA10, SCA12, SCA17 and dentatorubral-pallidoluysian atrophy (DRPLA). We registered the clinical characteristics and frequency of each type of ataxia. RESULTS: MJD/SCA3 was the most frequent ADCA (26 families, 57.8% of all families), followed by DRPLA (5 families, 11.2%), SCA7 (2 families, 4.4%), SCA2 and SCA1 (1 family each, 2.2% each); 10 families (22.2%) had no molecular diagnosis. SCA1 and SCA7 patients had African ancestry. DRPLA patients had Portuguese ancestry and were characterized by prominent anticipation and a variable combination of epilepsy, extra-pyramidal symptoms and dementia. Ophtalmoparesis, slow saccades and retinopathy were most distinctive of SCA3, SCA2 and SCA7 cases, respectively. CONCLUSIONS: MJD/SCA3 was the most common ADCA in this group of families. The high frequency of DRPLA and presence of SCA1 and SCA7 cases was unexpected. The presence of these rarer ADCA types probably reflects migration phenomena, posing a challenge for differential diagnosis.

Age-related white matter lesions are associated with reduction of the apparent diffusion coefficient in the cerebellum.

Cerebellar apparent diffusion coefficient (ADC) was found to be increased after acute cerebral hemispheric stroke. There are no data on cerebellar ADC changes in patients with chronic, age-related white matter lesions (ARWML). We aimed to determine longitudinal ADC variations on cerebral hemispheric and cerebellar white matter regions of patients with ARWML in order to study relations between ADC changes in both regions. ADC was measured serially (1-year interval) on lesioned periventricular frontal white matter, frontal and parietoccipital normal appearing white matter and middle cerebellar peduncles, on 19 aged patients with ARWML, which also underwent gait assessment. We compared regional ADC at 0 and 1 year and calculated variation percentages for each region. Correlation analysis was made between ADC variation in cerebellar regions and in contralateral hemispheric regions and between cerebellar ADC at 1 year and walking speed. After 1 year, ADC was higher on lesioned periventricular frontal white matter and lower on cerebellar regions. ADC variations on these regions were negatively correlated. Cerebellar ADC measured after 1 year was positively correlated with walking speed. This suggests a link between vascular disease progression inside frontal lesions and ADC reduction in contralateral cerebellar peduncles. Chronic ischemia in frontal white matter could have interrupted frontal-cerebellar circuits, producing hypometabolism in cerebellar regions (and worse performance on motor tasks), decreased perfusion and hence ADC reduction.

Painful legs and moving toes syndrome associated with Hashimoto's disease.

A patient with Hashimoto' s disease presented with painful legs and moving toes (PLMT). The neurological and electrophysiological findings suggest that, in this case, PLMT syndrome is a variety of dystonic movement and is associated with Hashimoto's disease.