11/34. Opsoclonus-ataxia due to childhood neural crest tumors: a chronic neurologic syndrome.Five children with subacute or acute onset of cerebellar ataxia and opsoclonus are described. Two had cerebrospinal fluid pleocytosis at the onset of ataxia and were initially thought to have acute parainfectious cerebellar ataxia of childhood. All were found to have tumors of neural crest origin (two neuroblastomas, three ganglioneuroblastomas). Tumors were small and only found by computed tomographic techniques. Urinary catecholamine metabolites were elevated in only two of the patients. Four of the five failed to improve neurologically with resection of the tumor. All four have had a steroid-sensitive chronic ataxic syndrome that worsens with acute nonspecific illnesses and has resulted in long-term deficits, particularly in speech and gross motor function. This is a metabolic encephalopathy associated with permanent residual neurologic deficits but without visible lesions on neuroimaging studies. We stress the frequency of cerebrospinal fluid pleocytosis in patients with tumor-associated opsoclonus and the clinical difficulty in separating tumor-associated cases from those due to other causes [corrected].- - - - - - - - - - ranking = 1keywords = ganglion (Clic here for more details about this article) |
12/34. Pathological findings in a case of hypoxic myoclonus treated with 5-hydroxytryptophan and a decarboxylase inhibitor.A 72-year-old woman suffered a respiratory arrest following intoxication with barbiturates. Her examination 27 months after the anoxic incident revealed involuntary jerks of trunk and limb muscles triggered by willed movements. On a regimen of 1 g L-5-HTP and 100 mg l-alpha-methyldopa hydrazine (carbidopa), action myoclonus disappeared completely. This medication had to be discontinued because of a regressive hysterical reaction. Two months later, she was found unconscious; resuscitation efforts were unsuccessful. autopsy showed death was caused by choking on food. Coronal slices of the cerebral hemispheres and transverse section of the brainstem and cerebellum revealed no lesion. No evidence of hypoxic damage could be demonstrated in the cerebral cortex, hippocampus, striatum, pallidum, subthalamus, thalamus, or other diencephalic structures. In the caudal half of the midbrain tegmentum, a marked astrocytic reaction of some duration was encountered in the lateral parts of the supratrochlearis nucleus, the lateral subnucleus of the mesencephalic gray, and the immediately adjacent cuneiform and subcuneiform nuclei. In the former nucleus, sites of presumed nerve cell disintegration were found, but the neuronal populations of this nucleus and of the other raphe nuclei were well maintained. The other brainstem structures and the cerebellum were normal. Our neuropathological findings suggest that hypoxic myoclonus (a) does not seem to be explained by demonstrable neuronal loss in motor structures, such as cerebellum, thalamus, or basal ganglia and (b) does not appear to be causally related to a detectable reduction in the serotonin-containing neurons of the brain but rather to a functional derangement of anatomically intact serotonergic pathways originating perhaps from other, as yet unidentified, damaged neuronal structures.- - - - - - - - - - ranking = 8.2056141589555keywords = nucleus (Clic here for more details about this article) |
13/34. hypertrophy of the inferior olivary nucleus. A clinico-pathological observation.The clinico-pathological findings in a 61-year-old man, who suffered from branchial and skeletal myoclonus, appearing six months after a brainstem infarction are reported. Of all the drugs which are usually thought to be effective in the treatment of myoclonus, only valproic acid brought some relief. The necropsy revealed bilateral hypertrophy of the olives, together with bilateral pontine tegmental and rubral infarctions, without involvement of the olivary pathway.- - - - - - - - - - ranking = 8.2056141589555keywords = nucleus (Clic here for more details about this article) |
14/34. Palatal "myoclonus" and inferior olive hypertrophy with one-sided cerebellar lesion. Clinico-pathological report of one patient.A case of palatal myoclonus and inferior olive hypertrophy is reported. Lesions located other than in the medulla were cerebellar infarction, lymphomatous infiltrates and, supratentorially, progressive multifocal leukoencephalopathy. It is suggested that double innervation of the olives from either side dentate nucleus may be why in the case reported here and in several cases in the literature, one-sided supra-olivary lesions can produce bilateral hypertrophy. As with palatal "myoclonus" and olivary hypertrophy, it is proposed that if the characteristic rhythmical movements occur, lesions besides those along the dentate-olivary pathway and the olivary hypertrophy itself have to be present.- - - - - - - - - - ranking = 2.0514035397389keywords = nucleus (Clic here for more details about this article) |
15/34. Olivary hypertrophy in a case with palatal myoclonus: light- and electron-microscopic study.This is a report on the ultrastructural finding of the olivary hypertrophy in a case with palatal myoclonus. By light microscopy two types of neuronal changes were observed in the inferior olivary nucleus, i.e. the central chromatolysis and cytoplasmic vacuolation. Both types were also recognized by electron microscopy and the cytoplasmic vascuolation was identified as the vesiculated endoplasmic reticulum. In the reactive astrocytes, mitochondria were strikingly proliferated.- - - - - - - - - - ranking = 2.0514035397389keywords = nucleus (Clic here for more details about this article) |
16/34. The association of neuroblastoma and myoclonic encephalopathy: an imaging approach.Myoclonic encephalopathy is a unique clinical syndrome of infants and children that is associated with a neurogenic tumor of ganglion-cell origin in approximately half of cases. A review of the literature as well as our personal experience with two recent infants suggests an imaging approach that includes chest radiography and abdominal computed tomography (CT). ultrasonography, neck CT, and chest CT myelography may be required in selected patients. An expedient clinical and imaging evaluation of infants and children with opsomyoclonus is important to detect those patients with neurogenic tumors.- - - - - - - - - - ranking = 1keywords = ganglion (Clic here for more details about this article) |
17/34. Postmortem studies on posthypoxic and post-methyl bromide intoxication: case reports.In two cases of action myoclonus following hypoxic or shock encephalopathy, neuropathological examination disclosed mild or moderate scattered changes involving thalamus, griseum centrale mesencephali, and nucleus centralis superior. Other areas were affected only in one of these cases (striatum, nucleus subthalamicus or hippocampus, nuclei pontis, and cerebellar cortex). In another case (an alcoholic patient), the changes, which involved only corpus mamillare and thalamus, were those of Wernicke-Korsakoff encephalopathy. In one case of oscillatory myoclonus following septic shock, there were marked cerebellar changes involving deep nuclei and mild abnormalities in the thalamus and inferior olive. The last case of action myoclonus following acute methyl bromide intoxication was characterized by marked changes in the inferior colliculi and moderate or mild abnormalities of thalamus, griseum centrale mesencephali, nucleus centralis superior, nucleus reticularis tegmenti pontis, nuclei pontis, and dentatus. The findings are compared with the data of seven previously reported neuropathological examinations in action myoclonus following hypoxic encephalopathy.- - - - - - - - - - ranking = 8.2056141589555keywords = nucleus (Clic here for more details about this article) |
18/34. Myoclonic syndrome and dentate nucleus lesion after excision of giant acoustic neurinoma.The authors describe one case of a Myoclonic syndrome appeared after the excision of a giant acoustic neurinoma. On the basis of autoptic studies, Myoclonic Syndromes, similar to the one reported here, have been shown to be associated to lesions of the Dentate Nucleus. Cranial computed tomography indicates, in our case, a cerebellar lesion localized in the Dentate Nucleus region. This figure allows the authors to correlate "in vivo" the anatomo-clinical data.- - - - - - - - - - ranking = 8.2056141589555keywords = nucleus (Clic here for more details about this article) |
19/34. Hereditary myoclonus and progressive distal muscular atrophy.myoclonus occurs in a variety of pathological conditions, some inherited. We recently evaluated 3 members of a louisiana-texas family with an autosomal dominant disorder manifested by adult-onset, generalized, stimulus-sensitive myoclonus and slowly progressive distal muscle weakness and wasting. The analyses of cerebrospinal fluid homovanillic acid and 5-hydroxyindoleacetic acid before and after probenecid provided some evidence of impaired turnover of central dopamine and serotonin. Treatment with clonazepam resulted in complete and lasting improvement of the myoclonus. A postmortem examination in 1 member of the family revealed chiefly neuronal degeneration of the anterior horn cells, Clark's nucleus, and the lower cranial nerve nuclei. A similar syndrome has not previously been reported.- - - - - - - - - - ranking = 2.0514035397389keywords = nucleus (Clic here for more details about this article) |
20/34. Postanoxic myoclonus. Treatment of a case with 5-hydroxytryptophane and a decarboxylase inhibitor.Postanoxic myoclonus was first accepted as being related to a dysfunction of the ventrolateral thalamic nucleus. Several stereotaxic studies have invalidated this hypothesis. The neurochemical approach, in particular the measure of 5-hydroxyindolacetic acid in the cerebrospinal fluid, has opened new theoretical and therapeutic possibilities involving serotoninergic pathways. A typical case is presented who improved markedly under a combined therapy with 5-hydroxytryptophan and a decarboxylase inhibitor. A review of the pathogenesis and therapeutic approach to postanoxic myoclonus is presented.- - - - - - - - - - ranking = 2.0514035397389keywords = nucleus (Clic here for more details about this article) |
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