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Repeated exposure to MPTP does not produce a permanent movement disorder in cats recovered from MPTP-induced parkinsonism.

The effects of additional MPTP injections on striatal neurochemistry and tyrosine hydroxylase positive cell populations within the ventral mesencephalon in cats recovered from parkinson-like motor deficits resulting from previous MPTP administration was studied. A second or third series of MPTP injections in recovered cats initially reinstated parkinson-like motor deficits. All cats again recovered normalized motor function two to three weeks after MPTP administration. Neurochemical analysis of striatal tissue showed no or only minor differences in dopamine and metabolite levels within most striatal regions sampled between cats which received single or multiple sets of MPTP injections. Cell count results showed no significant differences between the two MPTP conditions for the majority of the regions studied. These results suggest that the mechanisms responsible for motor recovery from MPTP intoxication in cats can be transiently disrupted by further exposure to MPTP and that cats do not develop a permanent parkinsonian syndrome from repeated MPTP exposure.

1-Methyl-4-phenyl-1,2,3,6-tetrahydropyridine↗

Posteroventral pallidotomy in movement disorders.

Since 1992 there has been renewed interest in pallidotomy now that the limitations and adverse effects of long-term dopaminergic therapy have become more apparent and more difficult to control in patients with advanced Parkinson's disease. The authors describe the effect of pallidotomy in 19 patients, sixteen of whom had advanced Parkinson's disease with painful dystonia and/or response fluctuations with severe akinesia while in "off" and dyskinesias while in "on". One patient had cortico-basal degeneration with rigidity, one patient had secondary dystonia and one had dystonic posturing due to Wilson's disease. Fifteen patients underwent unilateral pallidotomy, four patients had a staged bilateral procedure. Follow-up ranged from 3 to 42 months (mean 18 months). All patients with peak-dose dyskinesias and/or dystonia had marked reduction of symptoms, including the cases of Wilson's disease and secondary dystonia. The akinesia and rigidity scores of Parkinson-patients in "off" were greatly reduced, mainly but not only on the contralateral side. Evaluation by the patients showed remarkable improvement of symptoms in 79%, leading to substantially improved functional abilities in 68%. In this series the decrease in dopamine-response fluctuations, dystonia, hypokinesia and rigidity with functional improvement as judged by examiners and patients reflect a significant regain of independence.

Adolescent↗

Neurotrophic factors for the investigation and treatment of movement disorders.

Neurotrophic factors (NFs) are proteins that enhance neuronal survival, differentiation, neurotransmitter function and resistance to neurotoxins and lesions. For these reasons the NFs are considered as a new potential therapeutic tool for the treatment of neurodegenerative disorders, a group of diseases that produce the most important cause for disability in the Western world. Some NFs prevent or even reverse the behavioral, biochemical, pharmacological and histological abnormalities observed in several in vitro and in vivo models of neurodegenerative disorders, namely Parkinson's disease. Several NFs have been investigated in primate models of neurological disorders and some of them have been used for patients with these diseases. The results so far obtained in humans have been disappointing for several reasons, including technical problems for delivery, unbearable side effects or lack of efficacy. Future approaches for the use of NFs in humans should include the following: (1) Investigation of the putative compounds in animal models more related to the pathophysiology of each disease, such as in genetic models of neurodegenerative diseases; (2) New methods of delivery including genetic engineering by viral vectors and administration through implantable devices; (3) More precise methods of continuous response evaluation, including the novel neuroimaging techniques; (4) Investigation of the effects of behavioral stimulation and conventional pharmacotherapy on the metabolism of NFs.

Animals↗

Quality of life in Parkinson's disease: validation study of the PDQ-39 Spanish version. The Grupo Centro for Study of Movement Disorders.

The present multicenter study evaluated the validity of the PDQ-39 Spanish version (PDQ-39 SV) for assessing health-related quality of life (QoL) in Parkinson's disease (PD). A total of 103 PD patients were evaluated by the Hoehn and Yahr stage and by the following scales: Unified Parkinson's Disease Rating Scale (UPDRS), Schwab and England scale, Pfeiffer's SPMSQ, and Geriatric Depression Scale. The Hospital Anxiety and Depression Scale and the PDQ-39 SV were self-completed by patients. For analysis of data we used descriptive statistics, Cronbach's alpha, Spearman's rank correlation coefficients, and the Kruskal-Wallis test. The internal consistency of the PDQ-39 SV dimensions was satisfactory (Cronbach's alpha: 0.63-0.94; item-total correlation: 0.57-0.87, P < 0.001), although Social Support and Cognition items were beneath ideal values. Recorded results for Mobility, Activities of Daily Living, and Communication were highly correlated with PD rating scales (rs = 0.49-0.73, P < 0.001). Emotional Well-Being and Body Pain showed their best correlations with mood measures. Cognition was the only dimension significantly correlated with the SPMSQ (RS = 0.27, P < 0.01). These data and previous studies indicate that the PDQ-39 is a consistent instrument for evaluation of physical, emotional, and psychosocial aspects of QoL in PD patients.

Aged↗

[Dysarthric movement disorders of the velopharynx].

In this study we developed another method for the detection of nasopharyngeal closing patterns. This investigation of dynamic closure patterns was based on flexible video-nasopharyngoscopy and was undertaken in both healthy subjects and patients with dysarthria. Quantitative assessments of pharyngeal wall excursions allowed evaluation of closure patterns which were then matched with a predefined, mathematically-based algorithm. The results showed that a greater velopharyngeal constriction was maintained in the following order: resonants, open vowels, forced expiration, closed vowels, consonants, and swallowing. Variations in phonetic expressions of phonemes mainly interfered with velum excursion, whereas the lateral pharyngeal mucosal wall was minimally involved and the posterior wall portions were unaffected. Velopharyngeal closure patterns varied with different tasks: the velum was mainly moved for phonetic parameters, while lateral and posterior pharyngeal wall motion was observed on a large scale for swallowing. Comparison of subject groups revealed that patients with dysarthrias had reduced velar motility, leading to a more frequent hypernasal sound. Here, lateral pharyngeal walls showed a variable motility that in some cases was even more than in healthy subjects. Both groups exhibited qualitatively comparable velopharyngeal closure patterns, but significant differences were proved in their distributions.

Adult↗

[Neurophysiological and neuroimaging studies for restless legs syndrome and periodic leg movement disorder].

The restless legs syndrome (RLS) is a clinical diagnosis based on the four essential criteria defined by the International Restless Legs Syndrome Study Group (IRLSSG). An idiopathic form can be separated from a symptomatic form. Neurophysiological studies have investigated the pathophysiology of the idiopathic RLS or have been used to exclude a symptomatic cause, in particular polyneuropathy. So far cortical excitability changes, corticomotor, somatosensory and auditory pathways, spinal cord excitability, B-wave rhythm and cycling alternating pattern, as well as reflex mechanisms have been investigated by electroencephalography, evoked potentials, Bereitschaftspotentials, nerve conduction and thermal threshold measurements, electromyography, transcranial Doppler sonography, measurements of the spinal flexor reflex as well as neuroimaging techniques. The etiology of the RLS cannot be revealed by these methods, neurophysiological studies in RLS are, however, useful for a better understanding of the pathophysiology and for exclusion of a polyneuropathy or other symptomatic causes. In addition to neurophysiological investigations, small fiber neuropathy, which seems to be a more common finding in RLS patients than expected to date, may need biopsy for confirmation. This review will focus on investigations of the different systems involved with diverse neurophysiological methods.

Brain↗

Exogenous levodopa is not toxic to elderly subjects with non-parkinsonian movement disorders: further clinical evidence.

We report three women between 80 and 87 years of age who had longstanding essential tremor. Due to an erroneous diagnosis of Parkinsonism they had received levodopa preparations for 13, 16 and 25 years, respectively, with cumulative levodopa equivalent doses of 18.0, 9.0, and 4.1 kg. The patients showed neither dyskinesias nor signs of Parkinsonism. These observations do not support a detrimental role of chronic levodopa exposure in elderly individuals with essential tremor.

Aged↗

Borna disease virus infection in racing horses with behavioral and movement disorders.

Borna disease virus (BDV) is a neurotropic agent with capacity to infect and cause neurological disease in a broad range of warmblooded hosts including horses, sheep, cattle, cats, and possibly also humans. The epidemiology of BDV is largely unknown. However, it is likely that subclinically infected animals may represent potential virus reservoirs. In two groups of Swedish racing horses, one clinically healthy and one consisting of horses with diffuse neurological signs, the BDV seroprevalence was 24.5% and 57.7%, respectively. BDV RNA was detected in peripheral blood mononuclear cells in 8 out of 28 (28.6%) investigated horses, the majority of the BDV RNA-positive horses belonging to the group with neurological signs. There was a close relationship between the Swedish equine BDV isolates and previously reported equine BDVs in Europe. Our results point to an association of BDV infection with atypical disease patterns in horses such as diffuse mental and gait disturbances. These findings may be of importance for the understanding of the epidemiology of BDV infections in animals and man.

Amino Acid Sequence↗