1/8. Acquired convergence-evoked pendular nystagmus in multiple sclerosis.Nystagmus seen only with convergence is unusual. We describe four cases of acquired convergence-evoked pendular nystagmus in patients with multiple sclerosis. The nystagmus was horizontal and asymmetric in all patients. Eye movement recordings in one subject showed a conjugate rather than a convergent-divergent relationship of the phase of movement between the two eyes. All patients had evidence of optic neuropathy and cerebellar dysfunction. Occlusion of either eye during fixation of near targets led to divergent drift of the covered eye and a decrease in nystagmus. Intravenous scopolamine reduced nystagmus in one patient. Base-in prisms alleviated symptoms of oscillopsia at near and improving reading visual acuity. Convergence-evoked pendular nystagmus may be more common than currently appreciated, particularly among patients with multiple sclerosis.- - - - - - - - - - ranking = 1keywords = multiple sclerosis, sclerosis (Clic here for more details about this article) |
2/8. Cerebellar hypoplasia-endosteal sclerosis: a long term follow-up.Cerebellar hypoplasia with endosteal sclerosis is an infrequent entity that has been described in only four cases. Major clinical symptoms are cerebellar hypoplasia causing ataxia, hypotonia, mild to moderate developmental delay, microcephaly, growth retardation, endosteal sclerosis, tooth eruption disturbances, and hip dislocations. We report on a girl with this entity, whom we followed for 11 years. The endosteal sclerosis remained stationary over time, as were the clinical neurological symptoms, but neuroadiological symptoms were slowly progressive. We provide a short review of this probably autosomal recessively inherited disorder. (c) 2005 Wiley-Liss, Inc.- - - - - - - - - - ranking = 0.12978434819025keywords = sclerosis (Clic here for more details about this article) |
3/8. Spinocerebellar degeneration with prominent involvement of the motor neuron system: autopsy report of a sporadic case.A sporadic case of spinocerebellar degeneration with prominent involvement of the motor neuron system is reported. A Japanese male without contributing family history, developed cerebellar ataxia at the age of 52, followed by generalized amyotrophy and ophthalmoplegia, and died aged 58. The clinical findings were pathologically verified as degeneration of the spino-ponto-cerebellar system and the motor neuron system, the latter almost identical to those of amyotrophic lateral sclerosis. Additional subclinical changes were found in the dentate nucleus and substantia nigra. brain-stem nuclei subserving eye movements were well preserved, suggesting a supranuclear basis for the ophthalmoplegia. This unusual combination of system degenerations has on rare occasions been reported in the heredofamilial cerebellar disorders. As a sporadic case, however, this may be the first autopsy case of spinocerebellar degeneration with severe concurrent involvement of the motor neuron system.- - - - - - - - - - ranking = 0.018540621170035keywords = sclerosis (Clic here for more details about this article) |
4/8. The cerebellum of epileptics.Five cases are presented with different types of cerebellar lesions which may be encountered in epileptics. Case 1 represents typical postical lesions, Case 2 perinatal anoxic-ischemic damage, Case 3 transneuronal degeneration causing crossed cerebellar atrophy, Case 4 iatrogenic cerebellar atrophy due to phenytoin toxicity. Case 5 illustrates the interaction of two mechanisms, postictal lobular sclerosis and transneuronal degeneration. It is suggested that by attention to the type and distribution of the cerebellar lesions, to the clinical history and to the distribution of lesions in other parts of the brain the pathogenetic mechanisms can be elucidated in most cases.- - - - - - - - - - ranking = 0.018540621170035keywords = sclerosis (Clic here for more details about this article) |
5/8. tuberous sclerosis: unusual associations in four cases.tuberous sclerosis is a rare disease with classic primary or secondary changes affecting mainly the cerebrum, skin, kidneys, and heart. Such lesions are generally hamartomatous and thus display malignant features only in rare cases. This paper describes four cases of tuberous sclerosis which were unique in their association with certain unusual congenital, metabolic, and tumorous conditions.- - - - - - - - - - ranking = 0.11124372702021keywords = sclerosis (Clic here for more details about this article) |
6/8. Tumoral multiple sclerosis of the cerebellum in a child.We present a case of cerebellar tumoral multiple sclerosis in an 11-year-old girl and emphasize these two features: (1) Tumoral multiple sclerosis can occur within the posterior fossa and should be strongly considered in the differential diagnosis of mass lesions of the posterior fossa when typical white matter lesions are seen on T2-weighted images. (2) Tumoral multiple sclerosis can demonstrate ring enhancement on MR.- - - - - - - - - - ranking = 1.1666666666667keywords = multiple sclerosis, sclerosis (Clic here for more details about this article) |
7/8. MR imaging findings of tremors associated with lesions in cerebellar outflow tracts: report of two cases.Two cases with severe tremors were studied by means of electromyograms using surface electrodes and also by magnetic resonance (MR) imaging. The first case was associated with multiple sclerosis and demonstrated a severe postural cerebellar tremor and an alternate activation of antagonist muscles in the right arm. The second case, with hemorrhage in the brainstem, demonstrated a severe tremor at rest and mixed synchronous and alternating activation of antagonist muscles in the left forearm. MR imaging studies localized lesions possibly responsible for these tremors. In the first case a lesion was located in the superior cerebellar peduncle just under the decussation, and in the second case a lesion was found between the red nucleus and the thalamus, with possible involvement of both the cerebellothalamic and nigrostriatal pathways. The first case accords with the theory that a lesion located in the dentate nucleus and its projection can cause severe postural cerebellar tremor. The lesion demonstrated in the second case may be responsible for "cerebellar tremor at rest" or "static (resting) cerebellar tremor."- - - - - - - - - - ranking = 0.16666666666667keywords = multiple sclerosis, sclerosis (Clic here for more details about this article) |
8/8. Methyl iodide poisoning: report of two cases.Two workers were poisoned following exposure to methyl iodide with inadequate protective devices. Their cases are presented together with a review of literature. Both patients developed symptoms and signs of cerebellar lesions and damage of the third, fourth, or sixth cranial nerve pathways. spinal cord lesions producing motor and sensory disturbances were present in one. Late psychiatric disorders were observed in both patients. Although these symptoms were very similar to those reported in the nine published cases of methyl iodide poisoning, the toxicological diagnosis was delayed in one case: as repeated overexposure produced recurrent attacks of multifocal neurological dysfunction, multiple sclerosis was initially diagnosed, although several of the features observed are unusual in this disease. The manifestations of methyl iodide poisoning are similar to those of intoxication with other monohalomethanes. All these compounds probably share the same mechanisms of action. This mechanism and its therapeutic consequences are discussed.- - - - - - - - - - ranking = 0.16666666666667keywords = multiple sclerosis, sclerosis (Clic here for more details about this article) |