1/16. syndrome of progressive ataxia and palatal myoclonus: a case report.A 46-year old man presented with progressive cerebellar ataxia for 5 years. physical examination revealed palatal and tongue myoclonus, cerebellar gait, limb ataxia and spasticity of the lower extremities. The imaging studies including CT-scan and MRI of the brain revealed progressive pancerebellar atrophy and bilateral hypertrophic degeneration of inferior olives. The clinical course was slowly progressive. Various medications included anticonvulsants, benzodiazepines and antispasticity failed to abolish the abnormal palatal movement and ataxic syndrome. The syndrome of progressive ataxia and palatal myoclonus is a rare and unique neurodegenerative syndrome. The pathogenesis and treatment are still unknown.- - - - - - - - - - ranking = 1keywords = olive (Clic here for more details about this article) |
2/16. intention myoclonus of multiple sclerosis, its patho-anatomical basis and its stereotactic relief.The typical multiple sclerosis case considered here is especially informative from both the standpoint of its clinical course and on the basis of the autopsy findings. The foci responsible for the severe bilateral intention myoclonus of the trunk and limbs are the nerve cell losses in both red nuclei due to extensive and almost complete demyelination. Thereby the triangle of Mollaret between the red nucleus, inferior olives and dentate nucleus is involved as the patho-physiological circuit responsible for myoclonus. Stereotactic coagulation of dentato-thalamic fibres resulted in complete relief of intention myoclonus. With regard to the triggering of fresh demyelinating foci by stereotactic interventions, our point of view is as follows: Although a stereotactic operation introduces the possibility of triggering new demyelinating foci in less than 10% of the cases, such a possibility does not represent an absolute contra-indication to the stereotactic treatment of action myoclonus in multiple sclerosis, if the patient is informed accordingly.- - - - - - - - - - ranking = 1keywords = olive (Clic here for more details about this article) |
3/16. Palatal myoclonus secondary to vertebral artery compression of the inferior olive.A 47-year-old male with a 5-year history of palatal myoclonus was found on magnetic resonance imaging (MRI) examination to have an ectatic dominant left vertebral artery that compressed the left inferior olive. Microvascular decompression effectively eliminated his symptoms. This case and a similar case presented here with an ectatic vertebral-basilar system illustrate the value of standard MRI in conjunction with magnetic resonance angiography (MRA) in evaluating palatal myoclonus, and they suggest a potential role for decompressive surgery when persistent, highly symptomatic inferior olivary ischemia or compression occurs.- - - - - - - - - - ranking = 5keywords = olive (Clic here for more details about this article) |
4/16. Oculopalatal tremor with tardive ataxia.Oculopalatal tremor consists of palatal tremor and pendular nystagmus and may develop in a delayed fashion after an acute brainstem lesion. Delayed sequelae are generally restricted to the eyes and branchial-derived muscles, such as those of the palate. We report three cases of oculopalatal tremor that subsequently developed disabling delayed-onset ataxia and emphasize the potential for this significant complication after larger bilateral acute brainstem lesions with sparing of the inferior olive.- - - - - - - - - - ranking = 1keywords = olive (Clic here for more details about this article) |
5/16. glucose utilization in the inferior cerebellar vermis and ocular myoclonus.In a patient with symptomatic ocular myoclonus, the authors observed the regional cerebral metabolic rate of glucose use (rCMRGlu) before and after successful treatment with clonazepam. Even after the symptoms resolved, the rCMRGlu in the hypertrophic olive increased persistently, whereas that in the inferior cerebellar vermis contralateral to the hypertrophic olive decreased. The inferior cerebellar vermis, belonging to the vestibulocerebellar system, may be associated with the generation of symptomatic ocular myoclonus.- - - - - - - - - - ranking = 2keywords = olive (Clic here for more details about this article) |
6/16. Palatal myoclonus and inferior olivary lesions: MRI-pathologic correlation.In a patient with palatal myoclonus, MR imaging demonstrated bilateral hyperintense lesions in the ventral part of the medulla. Microscopic examination of the inferior olives showed gliosis, enlargement and vacuolation of the neurons, and demyelinization.- - - - - - - - - - ranking = 1keywords = olive (Clic here for more details about this article) |
7/16. Neuropathologic findings in idiopathic opsoclonus and myoclonus. Their similarity to those in paraneoplastic cerebellar cortical degeneration.The neuropathologic findings in an idiopathic case of the opsoclonus/myoclonus syndrome are reported. Although neurologic dysfunction may have been more widespread, structural lesions were limited to the cerebellum and inferior olives. Severe depletion of purkinje cells with preservation of granular cells was evident throughout the neo- and paleocerebellum; however, groups of Purkinje cells were preserved in the archicerebellum. No abnormalities were evident in the paramedian pontine reticular formation of the caudal pons. inflammation and evidence of anoxic damage were absent. These changes are very similar to those described in paraneoplastic cerebellar cortical degeneration.- - - - - - - - - - ranking = 1keywords = olive (Clic here for more details about this article) |
8/16. 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 = 1keywords = olive (Clic here for more details about this article) |
9/16. Hereditary branchial myoclonus with spastic paraparesis and cerebellar ataxia: a new autosomal dominant disorder.We report a family with branchial myoclonus, spastic paraparesis, and cerebellar ataxia in which six members were affected in two generations and the inheritance appeared to be autosomal dominant. Age at onset ranged from 40 to 50 years. Rhythmic myoclonus involving the palate, pharynx, larynx, and face was followed by truncal ataxia and spastic paraparesis in most patients. CT and MRI revealed mild atrophy of the cerebral and cerebellar cortex and severe atrophy of the medulla and spinal cord. The pons appeared normal and the olives not hypertrophic. CSF studies revealed severe reduction of the serotonin metabolite 5-hydroxyindoleacetic acid. Treatment with 5-hydroxytryptophan and carbidopa at highest tolerated dose mildly improved ataxia but did not modify the myoclonus. Treatment with anticholinergics, benzodiazepines, phenytoin, valproate, carbamazepine, and baclofen was unsuccessful. The clinical symptoms were progressive, leading to death or severe disability 5 to 10 years after the onset of the disease.- - - - - - - - - - ranking = 1keywords = olive (Clic here for more details about this article) |
10/16. 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 = 6keywords = olive (Clic here for more details about this article) |
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