Progressive supranuclear palsy (PSP): Richardson syndrome and other PSP variants.

Phenotypic heterogeneity of progressive supranuclear palsy (PSP) has been increasingly reported in the literature and can be the source of incorrect clinical diagnosis particularly in the early stages of the disease when the classically associated symptoms of early falls and supranuclear gaze palsy may not be apparent. In addition to Richardson syndrome (RS), several atypical clinical phenotypes have been described. Advances in genetic, neuroimaging, and biochemical/molecular technologies contribute to the identification of these clinical subtypes in the context of typical PSP pathological findings. Our goal is to review the phenomenology reported in the literature that is associated with confirmed histopathological changes consistent with a PSP diagnosis and to highlight the clinical spectrum of PSP. A systematic review of the literature in PubMed through July 2015 using MeSH terms and key words related to PSP was conducted. Articles describing PSP classifications, diagnostic criteria, and case reports were reviewed and summarized. Additional PSP phenotypes not seen in recent clinicopathological studies are included. These include primary lateral sclerosis, pallido-nigro-luysian degeneration, axonal dystrophy, and multiple system atrophy in the spectrum of atypical PSP variants beyond the traditionally classified PSP subtypes. This review is intended to help with the diagnostic challenges of atypical PSP variants. We believe that large multicenter clinicopathological studies will help expand our understanding of etiology and specific mechanisms of neurodegeneration and will aid in the appropriate interpretation of outcomes when conducting clinical and basic science research.

Pulmonary function tests in Parkinson's disease.

Morbidity and mortality are usually caused by respiratory disorders in Parkinson's disease (PD) because of pulmonary functional impairments. The purpose of this study was to determine the effects of PD on ventilatory function and that the use of pulmonary function tests (PFT) may serve as an indicator of PD severity. PFT have been performed in 21 patients with PD (15 non-smoker and six exsmoker with 36.17 +/- 26.54 pack-years of smoking history; mean age 64.67 +/- 10.76 years) and 16 normal age-matched control subjects who never smoked. The clinical disability was indicated by a Hoehn-Yahr (H-Y) scale. MEF25% [maximal flow rate at 25% of remaining forced vital capacity (FVC)] and FEV1 (the volume of air expired during the first second of the FVC) in exsmoker PD group was lower than non-smoker PD group (P < 0.05). The two effort dependent variables' peak expiratory flow (PEF) and the maximal flow rate at 75% of the remaining FVC (MEF75%) percent predicted values were 70.66 +/- 24.15 and 69.05 +/- 24.39 in non-smoker PD group whereas 90.18 +/- 17.24 and 90.00 +/- 18.97% predicted were in control group, respectively (P < 0.05). The maximal voluntary ventilation (MVV) was found to be 52.83 +/- 15.52 and 91.52 +/- 13.80% in PD and control group, respectively (P < 0.0001). MVV was the most effected parameter that was inversely correlated with the PD severity (r=-0.87, P < 0.0001). We concluded that less coordinated and less explosive muscle force has contributed to decrease in PEF and MEF75% values, and MVV decreases in PD as a result of the impaired performance and reduced efficiency during repetitive motor tasks which in part reflects abnormal agonist-antagonist muscle activity. So, spirometric studies may serve as a useful indicator of patients' neurophysiological conditions for the purpose of anticipating and preventing complications because of pulmonary impairment.

Cerebrovascular occlusive disease: hydatidosis.

We reviewed six patients with cerebral hydatid embolism from the heart. Although hydatid disease is becoming less common in the world as a whole, it should be considered in the differential diagnosis of embolic stroke in children, especially in the infested areas where hydatidosis is endemic.

A study on the effect and tolerance of lisuride on Parkinson's disease.

Seventeen idiopathic parkinsonian patients ranging between 47 and 75 years of age were included in this study to investigate the effect and tolerance of lisuride on PD. The duration of this simple clinical study, which had no control group was 12 weeks. There was 50% relief in disability scores and ADL in 13 patients in the first group in the combined therapy for 12 weeks with lisuride added to L-DOPA plus benserazide (p < 0.01). Optimal lisuride doses added to L-DOPA plus benserazide varied between 0.1 and 0.8 mg (mean 0.5 +/- 0.2 mg). With the addition of lisuride to treatment, the L-DOPA plus benserazide dose was reduced in 6 of 13 by 38%. Monotherapy with lisuride resulted in 56% to 57% improvement in disability scores and 47% in relief in ADL. Dry mouth, nausea, weakness, postural hypotension, and headache were the most frequently encountered side effects of lisuride. These adverse effects disappeared in 3 or 4 days, depending on a slight decrease and following increase in the dose of lisuride and/or the development of tolerance. Not only will such a combined therapy contribute to the reduction of the end-of-dose inadequacies, on-off phenomena, wearing off, peak-dose dyskinesias, and similar motor fluctuations, it may also play a prophylactic role in their prevention or delay.