Cases reported "Movement Disorders"

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21/37. Electroencephalographic correlates of myoclonus.

    In order to overcome various drawbacks of the conventional polygraphic study of a relationship between myoclonus and EEG, the EEG preceding and following the myoclonic jerk was simultaneously averaged by the CNV program. The subjects were 7 patients presenting with myoclonus of various kinds. The conventional polygraphs showed various paroxysmal EEG activities in 4 patients, but none of those paroxysmal activities was temporally related to myoclonus except for one case. As a result of the present averaging technique, 2 patients with cerebellar ataxia with intention myoclonus showed myoclonus-related EEG spikes or spike-and-slow-waves in the contralateral central or centroparietal region. These myoclonus-related spikes preceded the myoclonus by 10-17 msec, suggesting the presence of a discharging focus in the deep cerebral structures, rather than in the cerebral cortex, in these cases. Two other patients, one with resting myoclonus and the other with postural myoclonus, showed myoclonus-related slow waves on the contralateral hemisphere. This previously undescribed method of averaged polygraphic recording will be very useful in detecting an EEG correlate of spontaneously occurring myoclonus.
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22/37. Galloping tongue: post-traumatic, episodic, rhythmic movements.

    Two patients experienced delayed onset of episodic, rhythmic, involuntary movements of the tongue after head and neck trauma. These three-per-second waves began as posterior midline focal tongue contractions, lasted about 10 seconds in each episode, persisted for 2 to 4 months, and were not accompanied by other body movements or by EEG abnormality.
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23/37. Paroxysmal kinesigenic choreoathetosis and abnormal contingent negative variation. A case report.

    We treated a patient suffering from paroxysmal kinesigenic choreoathetosis (PKC). The etiology and pathophysiologic mechanism of this rare movement disorder are unclear. Like other patients with PKC, our patient experienced attacks more frequently when making anticipated movements. Because anticipation plays an important role in the genesis of the contingent negative variation (CNV), we investigated the CNV in our patient. One of the components of the CNV, the slow negative wave (SNW), repeatedly showed a remarkable enhancement compared with that of controls. After institution of phenytoin sodium therapy, the attacks of PKC subsided and the SNW amplitude came within the range of control values. There may be a relationship between PKC and the abnormal CNV.
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24/37. Selective surgical containment for Legg-Perthes disease: recognition and management of complications.

    Surgical containment is often selected for older children with "at risk" Legg-Perthes disease because healing time is long and abduction bracing is poorly tolerated. This review of 19 older children (mean age, 9.6 years) treated by surgical containment, selected from a total of 167 patients with Legg-Perthes disease, demonstrates the frequency of complications when only older children with severe involvement are surgically contained. The radiographic results (37% good, 26% fair, 37% poor) are mediocre when compared with reported series in which patients were not selected critically for "at risk" factors or in which the means age was lower. Although surgically contained, the femoral head in certain older children with "at risk" Legg-Perthes disease is unable to tolerate full activity. The avascular head responds in much the same manner as the adult femoral head with avascular necrosis (slow repair, marked collapse): therefore, older children treated surgically must be followed closely. Failure of containment, with head collapse, following surgical containment is heralded by a loss of hip motion and must be treated vigorously by reinstituting the usual nonsurgical protocol (hospitalization, traction, muscle release, arthrogram. Petrie casts, abduction bracing). Obviously, prior to surgical containment, the family should be advised of the possible later need for rehospitalization and a return to abduction casts or braces.
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25/37. The "happy puppet" syndrome in two siblings.

    Two siblings with the "happy puppet" syndrome are presented. Their clinical features are quite similar and closely resemble those of previously reported cases. These features include severe mental retardation, epileptic seizures, easily provoked and prolonged paroxysms of laughter, atactic jerky movements, hypotonia, large mandible with prognathia, and 2-3 cps spike and wave activity in the EEG. The occurrence of this syndrome in the two siblings suggests a genetic etiology possible as an autosomal recessive trait.
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26/37. 3-Methylglutaconic aciduria in the Iraqi-Jewish 'optic atrophy plus' (Costeff) syndrome.

    Eleven new patients of Iraqi-Jewish origin with bilateral optic atrophy, neurological abnormalities ('optic atrophy plus' syndrome) and 3-methylglutaconic aciduria (type III) are described. Clinical abnormalities in decreasing order of frequency were bilateral optic atrophy, extrapyramidal signs, spasticity, ataxia, dysarthria and cognitive deficit. An association with age was found only for spasticity. Spasticity, extrapyramidal signs and optic atrophy frequently led to major disability, in contrast to ataxia, dysarthria and cognitive deficit. The combined excretion of 3-methylglutaconic and 3-methylglutaric acid ranged between 9 and 187 mmol/mol creatinine. The primary enzymatic defect possibly may reside in the mitochondrial respiratory chain.
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27/37. Abnormal projection of corticospinal tracts in a patient with congenital mirror movements.

    A 31 year-old woman with familial congenital mirror movements not associated with other neurological defects underwent a detailed neurophysiological evaluation including: voluntary electromyographic activity recorded from upper limbs in response to acoustic stimuli, motor evoked potentials from the thenar muscles to focal transcranial magnetic stimulation, F waves from upper extremities, scalp somatosensory evoked potentials and long-latency responses from thenar muscles to electric stimulation of the median nerve. The results were consistent with the presence of fast-conducting pathways connecting each hand motor cortex with both contra- and ipsilateral spinal motoneurones.
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28/37. Congenital lacticacidemia caused by lipoamide dehydrogenase deficiency with favorable outcome.

    A 5-year-old boy had recurrent vomiting and lethargy with lacticacidemia and ketoacidemia since birth. Lipoamide dehydrogenase deficiency was found in muscle and fibroblasts. Therapy with sodium dichloroacetate, thiamine, and carnitine was associated with reduction of the severity and frequency of the decompensation episodes and near normal plasma lactate levels. At 5 years of age, the patient has normal cognitive function and moderate motor impairment.
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29/37. Epileptic negative myoclonus.

    Five patients with partial epilepsy of diverse etiology insidiously developed action-activated jerks. The disorder was limited to one arm in two patients and to the legs in another, and was multifocal in the remaining two. Each jerk was related to an EMG silent period lasting 100 to 400 msec, causing a lapse followed by resumption of posture. Simultaneous EEG-EMG recording showed each postural lapse to be time-locked with a sharp or spike and slow-wave transient over the contralateral sensorimotor cortex, where almost continuous paroxysmal activity occurred. The three patients who were able to cooperate during neurologic evaluation also exhibited motor neglect in the most affected body segment and decreased awareness of the disorder. In three patients, the phenomenon was medically resistant, and in two of them it was continuous and could be defined as epilepsia partialis continua. In the other two, medical treatment induced remission of EEG, motor, and neuropsychological abnormalities. This disabling movement disorder can be classified as "epileptic negative myoclonus" and may result from focal-discharge-related transient disruption of cortical function in the sensorimotor cortex.
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30/37. Disorders of movement in Leigh syndrome.

    Leigh syndrome (LS) is the clinical prototype of a genetically-determined mitochondrial encephalopathy. Twenty-two of 34 patients with LS had evidence of a movement disorder (MD). dystonia, the most common MD, was present in 19 cases, rigidity in 4, tremor in 2, chorea in 2, hypokinesia in 2, myoclonus in 1, and tics in 1. dystonia was most commonly multifocal at onset and showed progression in six patients. In half of the cases an enzymatic defect was detected, most commonly cytochrome C oxidase. The neuroradiologic findings showed prominent basal ganglia lesions in 20/21 patients. putamen, caudate, substantia nigra and globus pallidus were involved in this order of frequency. This experience was reflected in a literature review encompassing 284 cases of LS. However, only 26.4% had MD. Eleven patients, including one of our cases, presented as the primary torsion dystonia phenotype. There are clinical and pathological similarities between LS and other metabolic diseases affecting the central nervous system. The enhanced vulnerability of the nervous system to metabolic stress and the resemblance in the distribution of the pathology of these diverse conditions suggests a common pathogenetic mechanism. An excitotoxin-mediated mechanism is favored, one which might account for the frequent involvement of the basal ganglia in LS.
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