1/5. 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) |
2/5. Was the ataxia of Pierre Marie machado-joseph disease?: A reappraisal based on the last autopsy case from la Salpetriere Hospital.Nosological placement of l'heredo-ataxie cerebelleuse de Pierre Marie (HAC) has never been established even after several autopsy cases from the original Haudebourg family had been reported. To reappraise the clinical and pathological features of HAC in the current framework of hereditary ataxias, we screened the autopsy records of la Salpetriere hospital and identified a patient with a diagnosis of HAC who underwent an autopsy in 1943. Clinical features included heredity compatible with autosomal dominant inheritance, spasticity, increased tendon reflexes, mask-like face, visual impairment, nuclear ophthalmoparesis, and exophthalmos in addition to progressive ataxia. Pathological lesions included the spinal cord (spinocerebellar tracts, anterolateral fascicles, and posterior column), cerebellar dentate nucleus, pontine nucleus, pallidum, motor neurons including the oculomotor nucleus, and substantia nigra. The cerebellar cortex and inferior olives were preserved. These clinical and pathological features, similar to those described in patients from the Haudebourg family, a core prototype of HAC, are indistinguishable from those of machado-joseph disease. It would then be possible to conclude that some of the patients historically considered to have HAC would today be classified as having machado-joseph disease.- - - - - - - - - - ranking = 1keywords = olive (Clic here for more details about this article) |
3/5. syndrome of palatal myoclonus and progressive ataxia: two cases with magnetic resonance imaging.Two patients are reported with palatal myoclonus, progressive ataxia, and dysarthria, unresponsive to treatment with trihexyphenidyl or L-5-hydroxytryptophan. MRI showed enlargement of the inferior olives in one patient, consistent with the pathology usually associated with palatal myoclonus. The syndrome of progressive ataxia and palatal myoclonus should be distinguished from other ataxias and degenerations that affect the brainstem and cerebellum. pathology and specificity of site of the lesions are distinctive.- - - - - - - - - - ranking = 1keywords = olive (Clic here for more details about this article) |
4/5. Primary position upbeat nystagmus. A clinicopathologic study.eye movements were studied with electro-oculography in a patient with primary position, large amplitude, upbeat nystagmus. The upbeat nystagmus increased in amplitude on upward gaze, decreased on downward gaze, and was not altered by loss of fixation. The patient could not produce smooth pursuit movements upward or to the left, but had normal saccadic and vestibular induced eye movements in all directions. At necropsy, a low grade glioma was found involving primarily the medulla and caudal pons. The inferior olives and prepositus hypoglossal nuclei were diffusely infiltrated with tumor. These results suggest (1) primary position upbeat nystagmus is due to a defect in the upward smooth pursuit system, (2) the lower brain stem at the level of the inferior olives and nucleus prepositus hypoglossi is important in the mediation of vertical pursuit, and (3) primary position upbeat nystagmus can result from damage to several nuclei and interconnecting pathways in the caudal brain stem and midline cerebellum involved in control of vertical smooth pursuit.- - - - - - - - - - ranking = 2keywords = olive (Clic here for more details about this article) |
5/5. A dominant hereditary ataxia resembling machado-joseph disease in Arnhem Land, australia.A dominant ataxia among four families of the Arnhem Land Aboriginal people of Northern australia has many clinical features in common with machado-joseph disease. Neuropathology on one case showed multiple system involvement, with sparing of the inferior olives and cerebellar cortex, consistent with machado-joseph disease. Portuguese ancestry is possible, although not proven.- - - - - - - - - - ranking = 1keywords = olive (Clic here for more details about this article) |