1/9. movement disorders associated with chronic GM2 gangliosidosis. Case report and review of the literature.A 24-year-old man presented with dystonia, dementia, amyotrophy, choreoathetosis, and ataxia. Partial hexosaminidase a deficiency was documented in serum and leukocytes and confirmed by rectal biopsy with ganglion cells containing membranous cytoplasmic bodies. A brief review of the literature reveals that tremor, dystonia and choreoathetosis are common but neglected symptoms associated with chronic GM2 gangliosidosis.- - - - - - - - - - ranking = 1keywords = ganglion (Clic here for more details about this article) |
2/9. Paroxysmal kinesigenic choreoathetosis and paroxysmal dystonic choreoathetosis in a patient with familial idiopathic hypoparathyroidism.A patient with familial idiopathic hypoparathyroidism whose major symptoms were paroxysmal kinesigenic choreoathetosis (PKC) and paroxysmal dystonic choreoathetosis (PDC) was reported. CT revealed marked calcification in the basal ganglia, the thalamus and the dentate nucleus of cerebellum. Positron emission tomographic (PET) study showed decreased glucose metabolism in the basal ganglia and thalamus. The paroxysms were eliminated by the normalization of serum calcium level. Thus the paroxysms seemed to be induced by hypocalcemia which stimulated the lesions in the basal ganglia and thalamus, on one occasion, as in PKC, and on the other occasion, as in PDC. Concomitant occurrence of both PKC and PDC in a patient is unique and is of interest in considering the pathomechanisms of these different but related disorders.- - - - - - - - - - ranking = 0.021527544442784keywords = nucleus (Clic here for more details about this article) |
3/9. hyperglycemia-induced hemichoreoathetosis: the presenting manifestation of a vascular malformation of the lenticular nucleus.A 72-year-old diabetic woman developed paroxysmal hemichoreoathetosis during an episode of nonketotic hyperglycemia. The movement disorder abated as the blood glucose normalized. A computed tomographic scan revealed a vascular malformation involving the lenticular nucleus on the side contralateral to the dyskinesia. hyperglycemia has rarely been reported to cause episodic dyskinesias, but there have been no prior reports of patients with striatal vascular abnormalities in whom hyperglycemia seemingly caused a transient movement disorder.- - - - - - - - - - ranking = 0.10763772221392keywords = nucleus (Clic here for more details about this article) |
4/9. Delayed choreoathetosis following acute carbon monoxide poisoning.Ten days after accidental exposure to carbon monoxide, a 17-year-old youth developed transitory choreoathetosis of both arms, face, and neck, with moderate dysarthria. CT revealed symmetric bilateral infarction in the head of the caudate nucleus, the putamen, and the small parts of the anterolateral globus pallidus.- - - - - - - - - - ranking = 0.021527544442784keywords = nucleus (Clic here for more details about this article) |
5/9. Paroxysmal kinesigenic choreoathetosis associated with prenatal brain damage.We describe a 15 year old patient with paroxysmal kinesigenic choreoathetosis. Neurological examinations revealed a paresis of the right arm and hand that was similar to ulnar nerve palsy, a right homonymous hemianopsia and an ocular movement disturbance of smooth pursuit to left. Attacks of dystonic spasms began abruptly, usually following running, and lasted less than 5 min. magnetic resonance imaging displayed a linear area of increased signal in the T2-weighted images along the lateral margin to the left putamen, atrophies of the frontal and temporal opercula and a large porencephalic cyst in the left parieto-temporo-occipital region. A cerebral blood flow study with single photon emission computed tomography showed hypoperfusion of the lenticular nucleus and the regions corresponding to the atrophies and the porencephalic cyst. Electroencephalograms during the attacks could not demonstrate epileptic abnormality. Only the neuronal plasticity of an immature brain could explain the discrepancy between the observed huge lesions of the brain and the minor neurological symptoms present. Attacks of paroxysmal kinesigenic choreoathetosis might occur when the basal ganglia maturate to some extent, even if the lesions in the brain were caused before birth.- - - - - - - - - - ranking = 0.021527544442784keywords = nucleus (Clic here for more details about this article) |
6/9. Pseudochoreoathetosis. Movements associated with loss of proprioception.OBJECTIVE: To describe seven patients with proprioceptive sensory loss and choreoathetoid movements. DESIGN: Case series. SETTING: Outpatient and inpatient university referral. patients: patients with sensory loss and abnormal movements. INTERVENTION: None. MAIN OUTCOME MEASURE: None. RESULTS: One patient had a parietal cortex injury, one had a thalamic infarction, two had spinal cord lesions, two had dorsal root ganglion neuronopathies, and one had an ulnar neuropathy. In each case, the duration of abnormal movements correlated with the duration of proprioceptive sensory loss, and the abnormal movements were restricted to body parts with proprioceptive sensory loss. The movements varied from chorea and athetosis to dystonia. CONCLUSIONS: These cases suggest that proprioceptive sensory loss can lead to a movement disorder, termed pseudochoreoathetosis, which occurs following the appearance of lesions anywhere along proprioceptive sensory pathways, from peripheral nerves to the cerebral cortex. It is hypothesized that pseudochoreoathetosis occurs because of the failure to process limb proprioceptive information in the striatum. Therefore, both choreoathetosis and pseudochoreoathetosis may be manifestations of the failure of the striatum to properly integrate cortical motor and sensory inputs.- - - - - - - - - - ranking = 1keywords = ganglion (Clic here for more details about this article) |
7/9. Choreo-acanthocytosis like phenotype without acanthocytes: clinicopathological case report. A contribution to the knowledge of the functional pathology of the caudate nucleus.Detailed clinical and neuropathological findings in two unrelated patients with a chorea-acanthocytosis-like phenotype (CA) are reported. One case met all the diagnostic criteria of CA and had a deceased brother with the same disease. The second case had a virtually identical phenotype to the former but without acanthocytes. These findings suggest that both patients are affected by the same disease and that acanthocytes are not essential to the diagnosis. Neuropathological autopsy studies on the brain of the second case showed selective atrophy of the caudate nucleus that seemed to correspond to the movement disorder and behavioural abnormalities prominent in this patient. In both subjects, morphometric and ultrastructural examination of the peripheral nerve showed loss of myelinated fibres, more accentuated distally, and cytoskeletal changes in the axoplasm. These findings support the hypothesis that peripheral neuropathy in CA is caused by distal axonopathy.- - - - - - - - - - ranking = 0.10763772221392keywords = nucleus (Clic here for more details about this article) |
8/9. Paroxysmal choreoathetosis: an epileptic or non-epileptic disorder?The pathophysiology of paroxysmal kinesigenic choreoathetosis (PKC) is controversial. Some classify it as a non-epileptic movement disorder, others consider PKC as a form of reflex-epilepsy but postulate that the epileptogenic source is within basal ganglia rather than in the cortex. An extensive invasive longterm monitoring in a girl with PKC demonstrated a consistent ictal discharge arising focally from the supplementary sensory-motor cortex (SMC), with a concomitant discharge recorded from the ipsilateral caudate nucleus, without significant spread to other neocortical areas. An hypothesis is presented to explain how a focal discharge within the supplementary motor cortex, demonstrated for the first time to occur in a patient with PKC, might cause phenomenologies distinct from the habitual SMC seizures and strongly suggesting a basal ganglia semeiology.- - - - - - - - - - ranking = 0.021527544442784keywords = nucleus (Clic here for more details about this article) |
9/9. Choreoathetosis in acquired immune deficiency syndrome patients with cerebral toxoplasmosis.The aim of our study was to evaluate both the incidence and the pathologic and clinical features of extrapyramidal disorders in a population of acquired immune deficiency syndrome (AIDS) patients with cerebral toxoplasmosis. Of 240 AIDS patients evaluated in the 1985-1994 period, 50 of them were diagnosed to have cerebral toxoplasmosis on the basis of the following criteria: occurrence of specific antibodies, computed tomography and/or magnetic resonance imaging (MRI), and regression of the symptoms after specific therapy. Three of 50 (6%) had hemichoreoathetosis. In the first case, the disorder began as a dyskinesia of the left hand that subsequently spread to the whole ipsilateral arm and assumed the features of choreic athetotic movements. The other two cases were characterized by left hemisomatic distal choreic movements. Therapy with pyrimethamine and sulfadiazine led to a complete recovery of the extrapyramidal signs in two cases and to improvement in the third. According to our observations, the onset of these movement disorders could not be related to the dimension of the lesion or to the edema, but to a specific localization in subthalamic nucleus, in subthalamic/pallidal, and pallidal/thalamic pathways. MRI seems the elective tool to perform a more accurate study of the anatomic areas involved in this pathway and to verify their integrity. Cerebral toxoplasmosis in AIDS can be considered as a new etiopathogenic cause of choreoathetosis.- - - - - - - - - - ranking = 0.021527544442784keywords = nucleus (Clic here for more details about this article) |