Cases reported "Hyperkinesis"

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1/4. Involuntary vocalisations and a complex hyperkinetic movement disorder following left side thalamic haemorrhage.

    A variety of involuntary speech phenomena as for example palilalia have been described as consequences of neurological disorders. Palilalia is the involuntary repetition of syllabels, words and phrases in ongoing speech. We describe a 73 year old woman who suffered from a hypertensive thalamic haemorrhage. MRI revealed that the lesion was predominantly located within the pulvinar, extending to the lateroposterior thalamic nuclei and to the pretectal area with possible involvement of the medial geniculate body. Few months after the event she developed involuntary vocalisations with whole words and meaningless syllables being rapidly reiterated. In contrast to typical palilalia these vocalisations were not meaningfully related to the ongoing speech of the patient. In addition, the patient developed a complex hyperkinetic movement disorder with right-sided painful hemidystonia and bilateral clonic jerks and a right-sided postural tremor.
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keywords = complex
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2/4. Complex hyperkinesia during recovery from left temporoparietal cortical infarction.

    A 51-year-old man with preexistent lacunar infarctions in the basal ganglia and pons developed complex involuntary movements of his right arm 1 day after a purely cortical-subcortical ischemic infarction in the territory of the left middle cerebral artery sparing the basal ganglia. The movements were stereotyped and repetitive, affecting hand and fingers or proximal arm muscles alternatively or together. Although hyperkinesia after basal ganglia lesion has been described many times in the literature, it has not yet been reported after cortical-subcortical ischemic infarctions sparing the basal ganglia.
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keywords = complex
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3/4. The "jerky dystonic unsteady hand": a delayed motor syndrome in posterior thalamic infarctions.

    We report the cases of three patients with a thalamic infarct in the territory of the posterior choroidal artery involving the posterior thalamic nuclei. These patients developed delayed complex hyperkinetic motor syndromes, associating ataxia, tremor, dystonia, myoclonus and chorea, which we call "the jerky dystonic unsteady hand". One patient had a severe myoclonic and ataxic-dystonic choreoathetosis; another showed a so-called rubral tremor (myoclonic ataxia with resting, action, and wing-beating tremor) with dystonia; and the third one had a dystonic and ataxic hand with intermittent mild action myoclonus. All of them had sensory dysfunction; two had also presented with a painful Dejerine-Roussy syndrome. All had CT or MRI-proven infarcts in the territory of the posterior cerebral artery involving the posterior choroidal territory with an abnormal signal in the posterior area of the thalamus (pulvinar nucleus) but sparing the other thalamic, subthalamic and midbrain structures. These delayed myoclonic complex hyperkinetic syndromes have not been reported before, and we did not observe them in other topographic forms of thalamic infarcts. They may thus represent a new entity of movement disorders due to lesions in the posterior thalamic nuclei, with specificity for posterior choroidal artery infarcts.
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ranking = 0.4
keywords = complex
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4/4. Reversible hyperkinesia in a patient with autoimmune polyglandular syndrome type I.

    Autoimmune polyglandular syndrome is characterized by a failure of multiple endocrine organs and the presence of circulating organ-specific autoantibodies targeted against the failing organs. Here we describe a patient with autoimmune polyglandular syndrome type I with the endocrine manifestations of hypoparathyroidism, adrenocortical insufficiency, and insulin-dependent diabetes mellitus. Long-standing hypoparathyroidism led to extensive calcification of the basal ganglia which resulted in the clinical presentation of an extrapyramidal movement disorder (choreoathetotic and hemiballistic hyperkinesia of the left extremities). Interestingly, parallel to rehydration and the initiation of cortisol replacement therapy a complete reversion of the hyperkinetic signs was achieved. This case shows a rare multiendocrine organ failure with complex metabolic interactions resulting in marked neurological signs. Furthermore, this case demonstrates for the first time that a hyperkinetic syndrome--most likely due to hypoparathyroidism-induced basal ganglia calcification--can be reversed solely by adequate treatment of the concomitant endocrine failures.
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ranking = 0.2
keywords = complex
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