The cause of blepharospasm is unknown in most cases.
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High-field strength magnetic resonance imaging is an accurate clinical technique for detecting the relative distribution of ferritin in the brain. In normal adults, iron is found in highest concentrations in the globus pallidus, red nucleus, pars reticulata of the substantia nigra, and dentate nucleus of the cerebellum; its distribution is clearly mapped as signal hypointensity (darkness) on a T2-weighted image due to local-field heterogeneities produced by ferritin. Iron is absent at birth and increases in concentration in the putamen in the elderly. Poorly drug-responsive Parkinson's disease (multiple-system atrophy) is characterized by premature signal hypointensity in the putamen and caudate, while Hallervorden-Spatz disease exhibits abnormal hypointensity in the globus pallidus in children. Dyskinetic disorders often have abnormal signal hyperintensity (whiteness) in the putamen related to gliosis.
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Excluding surgical procedures, this article focuses on clinical pharmacotherapeutic approaches to treatment of parkinsonism and tremor, chorea, dystonia, tic, and tardive dyskinesia.
An eight-year-old girl presented with a bizarre posture and gait and was diagnosed as having hysterical astasia-abasia. She walked with marked lordosis and head hyper-extended, and would walk backwards across the room to a bed or chair for support. She had a midline astrocytoma and the disorder was relieved with removal of the tumour. The unusual symptomatology accompanying many midline lesions is re-emphasized.
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When placed in a tank of water, aged rats (24 to 27 months old) showed marked impairments in swimming. Compared with young adult rats (3 to 4 months old), the older animals moved their limbs less vigorously and were less successful in keeping their heads above water. The young, but not old, rats maintained a position nearly horizontal to the water surface and planed across it. These movement dysfunctions of aged rats resemble those seen in young adult animals that have sustained injury to brain dopamine-containing neurons. The swimming impairments of the aged rats were reversed by the dopamine receptor stimulant apomorphine and by the biosynthetic precursor of dopamine, L-dopa. Thus, age-related alterations in brain dopaminergic systems may be responsible for some of the movement disturbances associated with senescence.
Eye movement recording (EMR) has been performed in 5 asymptomatic myotonic dystrophy (MyD) gene carriers, 7 mildly affected MyD patients, and 23 age- and sex-matched healthy controls. The purpose of the study was to evaluate whether eye movement abnormalities are an early expression of the MyD gene, and to determine the value of this procedure for detection of otherwise asymptomatic gene carriers. EMR did not reveal any abnormalities in the asymptomatic group, but in the mildly affected group showed significantly (P less than 0.01) decreased maximum velocities of the saccades, compared with controls. The results indicate that EMR may aid in the detection of mildly affected MyD patients. However, true presymptomatic diagnosis with EMR has not yet proven possible.
Patients with episodic or paroxysmal movements or postures often are thought to have hysteric or psychosomatic illnesses. Kinesigenic (movement-induced) posturing similarly is usually misinterpreted. This case is notable because of the presence of symptoms of two distinct diseases with similar symptoms and changes from one dystonic posture to another during three different durations of attack. The condition improved with acetazolamide therapy. The effect of acetazolamide on sodium and potassium ionophores is discussed because of new genetic information about these illnesses.
Abnormal protein aggregation has been postulated to explain the molecular basis for many neurodegenerative diseases, including Alzheimer's disease, Parkinson's disease and prion diseases, as well as trinucleotide repeat disorders. The recent findings that mutations in alpha-synuclein lead to autosomal-dominant, early-onset Parkinson's disease in some families and that alpha-synuclein is found in Lewy bodies of all Parkinson's disease patients prompted the hypothesis that the pathophysiology of all Parkinson's disease patients starts with an abnormal folding of alpha-synuclein, producing excessive aggregation that overwhelms the antiaggregation mechanisms of the cell. The genetics of Parkinson's disease and polyglutamine repeat disorders and the evidence of abnormal processing and aggregation of the respective target proteins for the aetiology and pathogenesis in these diseases are reviewed.
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It has long been known that in the last years of his life Maurice Ravel (1875-1937) suffered from a progressive neurological illness. Despite several proposed diagnostic hypotheses, the precise nature of his illness remains elusive.
Chronic neurodegenerative diseases, such as Parkinson's disease or Huntington's disease, have a strong impact on quality of life. Psychosocial problems arise from the disease itself, as well as from those who provide care. When no cure is available, care must take precedence.
On the basis of many years' work experience of ambulatory center of industrial rehabilitation the author proposed the set of technical means necessary for restorative treatment of patients with locomotor disturbances. There are proposed constructions, developed in the center and approved in the process of rehabilitation of patients with segmentary disturbances of locomotory functions.
By means of a biocybernetic approach the pursuit and posture motor control of brain-damaged children with spastic hemiparesis and disturbed motor coordination of mild extent were investigated. The postural motor control system was loaded by pseudostochastic binary torque sequences applied to the wrist. The dynamics of this system were characterized on the basis of the parameters forearm displacement as well as surface-EMG of M. Biceps and Triceps (calculations of weight functions). The pursuit motor control was analyzed by a pursuit tracking method. By this way the motor capacity for fine and rough motor coordination, motor rhythm, motor adaptation, the maximal speed of movements and motor reaction time could be tested. Considering these results a characterization of movement control on different levels of motor regulation was possible (dynamics of muscular, spinal, and supraspinal components of motor control).