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De Barsy syndrome is defined by the combination of a progeroid aspect, cutis laxa, cornea clouding, growth retardation, mental retardation and athetoid movements. The clinical symptoms of a male infant are described and compared with all other cases reported in literature. The aetiology of this syndrome is unclear; inheritance is probably autosomal recessive.
A 28-year-old man was admitted to our hospital because of focal seizures probably generated by the right sensorimotor cortex with secondary generalization for 3 months. He had 5 years history of keratoconjunctivitis and iriditis. Physical examination revealed minimal hemiparesis and hyperreflexia in the left side and left papillitis. Cerebrospinal fluid analysis showed 75 cells per cubic-millimeter (all lymphocytes) and 88 mg/dl protein. Computerized tomography of the brain revealed elimination of the sulcus and vague enhancement of the cortex in the right cerebral hemisphere. The symptoms and the laboratory data improved spontaneously in a few months. Slow, athetotic movements were observed in the left forearm extensor muscles for about 10 days period. Electromyography (EMG) of these movements showed bursts of 300-800 sec in duration which repeated almost regularly at a varying frequency of 0.3-0.5 msec. The electroencephalographic (EEG) studies revealed slow background activities over the right hemisphere. The simultaneous recording of EEGs and EMGs demonstrated no EEG activity correlated with the athetotic movements. In the jerk-locked averaging (JLA) recorded with the time constant of 0.3 sec, no spiky or sharp EEG activities were detected before the EMG discharges of the left forearm extensor muscles. However, JLA with the time constant of 3.2 sec disclosed a slow negative shift which preceded the athetotic movement by about 500 msec. This negative potential was fairly localized in the right central area, which was thought to be consistent with the hand area of the motor cortex contralateral to the muscles showing the athetotic movements. The present case had a wide-spread, mainly unilateral lesion in the cerebral hemisphere.(ABSTRACT TRUNCATED AT 250 WORDS)
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Our stereotactic experiences in agreement with the literature showed, that different target points could influence the same motor disturbance. To choose the best target point or target point combinations we generally implant the electrodes into VL, Vim, CM, P, dentate nucleus and motor cortex. To ensure the correct sequence of therapeutic lesions we developed an investigation system, taking into consideration the resting and the working state of the motor system. We elicited events centrally (stimulation of the different target points) and peripherally (reflexes) and recorded the evoked potentials at the non-stimulated sites along with the motor and motor modulation effects in the appropriate muscles. The elicited events depend on the site of stimulation and registration and on the state of muscle activity. The centrally and peripherally elicited events influence each other. With our technique the elicited events and their functional dependency is most explicit within the motor system. The results help to explain some basic motor functions and help to answer some of our therapeutic questions.
The diagnostic problems of extrapyramidal disorders (ED) are reviewed. Many of the wide range of ED occur rarely, and clinical experience is difficult to obtain. Despite great advances in pathophysiology and pathological anatomy the diagnosis of ED is still mainly a clinical diagnosis, and the diagnostic problems are discussed principally in relation to the involuntary movements and partly with regard to muscle tone. Except in a few diseases, biochemical and microbiological analyses, EEG, EMG and X-ray examinations offer little contribution to the solution of diagnostic problems in these disorders.
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There are presented three different forms of chorea, in which we have observed an spectacular response to the administration of carbamazepine. The two first cases are paroxysmal choreoathetosis. Both are free of episodes of choreoathetosis under the administration or very low dosis of carbamazepine. However when a placebo is been used or in an accidental omit of the drug, the crisis reapears. The third case is a Sydenham chorea. It received three months of treatment on a 20 mg/kg dosis, the symptoms dimished on the first week. After a year he has not suffered any relapse.
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34 children with extrapyramidal hyperkinesis were treated with Tiaprid. With 22 children the extrapyramidal syndromes could be reduced considerably or disappeared. A reduction of vigilance in the phase of adjustment was the only side effect.
The authors describe a very rare case of choreoathetotic movements which appeared in an epileptic in strict relationship to a toxic concentration of diphenylhydantoin in the serum. These movements were reversible and disappeared after reduction of drug dose and its serum concentration falling to the therapeutic range.
This uncommon paroxysmal movement disorder featuring attacks of dystonic spasm with athetoid posturing is usually of idiopathic nature, often familial and starting in childhood. There are a few reported examples due to cerebral pathology, and only four previous cases due to brain trauma. We report here a 21 year old man with left-sided motor spasms, choreoathetoid in type, which were clearly caused by a right frontal penetrating injury with contusion and haemorrhage. The attacks were relieved by phenytoin therapy. In this case, it seems that the post-traumatic paroxysmal kinesigenic choreoathetosis may be a form of reflex epilepsy.
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Seven cases in a family of hereditary spino-cerebellar degeneration (SCD) similar to dentatorubro-pallido-Luysian atrophy (DRPLA) were reported. The clinical features of these cases were disturbance of ocular movement (limitation of ocular movement and slow eye movement (SEM], remarkable amyotrophy, choreoathetosis, dementia and sleep apnea. The brain CT's revealed marked atrophy in pons and cerebellum. Amyotrophy had been reported in the case of DRPLA, particular ataxo-choreoathetoid form (by Hirayama). Muscle biopsy was performed in these cases, which showed scattered small angulated fiber, severe atrophic fiber with pyknotic nuclear clump, fiber type grouping and small rounded fiber were mixed. These findings indicates neurogenic change of radiculoneuropathy type (by Tanabe). In many reported cases of DRPLA and SCD with amyotrophy, this type of muscle biopsy had not been recognized. In SCD with amyotrophy, a main lesion had existed on peripheral nerve. In this case, there was no definite clinical findings (sensory disturbance, delay of conduction nerve velocity, peripheral neuropathy in nerve biopsy). In recent years, several unclassified cases of SCD with amyotrophy had been reported, which had multi-system degeneration involving peripheral neuropathy. This case is similar to these cases, which is speculates multi-systemic lesions, not only DRPLA but also peripheral nerve involvement. On neuro-otological study, velocity of saccade was slow and persuit was reserved in proband case. In younger onset case, disturbance of saccade and pursuit was mild. In older progressive case, disturbance of saccade and pursuit was progressive and accompanied with severe limitation of ocular movement. Several autopsy cases of SEM had been reported.(ABSTRACT TRUNCATED AT 250 WORDS)
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Following the seminal works of Canter (152) and Darley, et al. (1) concerning differential diagnosis and classification of dysarthria, considerable strides have been made with regard to characterizing the features of speech that occur with disorders of movement. Research should continue to address the neurophysiologic substrates of the dysarthrias of movement disorders, and most importantly, efficacious means of treating these impairments of speech.