Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0004134 (ataxia)
 15,886 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The value of evoked potentials in studying conduction in the somatosensory pathway was assessed in patients with various neurological disorders. In patients with multiple sclerosis (MS) abnormalities of the cervical response (N14) were found particularly in longstanding cases but also in the early stages of the disease, even in patients without sensory symptoms or signs, and were reversible in some patients. The cortical response was also abnormal in some cases but the two were not always affected together. In Friedreich's ataxia both the cervical and cortical responses were usually abnormal. Subclinical abnormalities of the cervical responses were found in some patients with hereditary spastic paraparesis or mixed forms of spinocerebellar ataxia. The cervical responses were also abnormal in patients with peripheral neuropathy and cervical radiculopathy, and in some patients with brain-stem or thalamic lesions. Cervical and cortical responses were normal in the lateral medullary syndrome, whereas the cortical response was markedly abnormal in patients with high brain-stem or cerebral hemisphere vascular lesions. Cortical and subcortical responses were abnormal in some patients with stereotactic thalamic lesions. Enhanced cortical responses were found in patients with lesions at different levels in the CNS. The most marked enhancement was observed in patients with familial myoclonic epilepsy. Lesser degrees were found in some patients with MS, progressive supranuclear palsy, thalamic lesions, brain-stem encephalitis and syringomyelia. Enhanced responses were usually found in patients with minimal or no clinical sensory involvement. It is postulated that this type of abnormality results from an interference to the inhibitory mechanisms which normally operate at various levels in the somatosensory pathway. It is concluded that evoked potential studies are a valuable adjunct to the clinical evaluation of sensation, and that they may provide useful information on the pathophysiology of conduction in the somatosensory pathway.
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PMID:The contribution of evoked potentials in the functional assessment of the somatosensory pathway. 22 50

The authors report some observations about 228 cases of multiple sclerosis with secure diagnosis subdivided in severe (34,6 %), common (29,8 %) and benign after the tenth year of illness (35,6 %). These observations are in agreement with classical statistic reports concerning the natural disease history : higher female incidence (64 %), average age at onset about 29,5 years, 4 main first signs (ocular [42,5 %], motor [41,5 %], ataxia [36,3 %], sensory [35 %] and remitting course [82,5 %]). Follow up study of 81 "benign" forms after the tenth year of the disease allows us to look at the freqwuency of "secondarily worsening" forms (25,7 %) and "benign remaining" ones until the twentieth year (10,1 %).
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PMID:[Analysis of 228 cases of multiple sclerosis (author's transl)]. 23 40

Electrical stimulation was applied to the spinal cord of 75 patients who had demyelinating and degenerative diseases of the central nervous system, and 3 patients who had sustained spinal cord injuries. The electrical energy was delivered to the central nervous system by the percutaneous technique. The amount of electrical energy required to produce the perception of paresthesias was measured in 11 patients. The minimum power necessary was 76.89 muW, the maximum was 868 muW, and the average was 448.8 muW. The patients were evaluated by 4 examiners by means of routine neurologic examination, videotape movies, and measurement of urinary bladder function. Continued improvement in neurological status, which allowed the patient to live a better lifestyle, occurred in 30 of the 61 patients with multiple sclerosis, and 6 of the 10 patients with ataxia. The patient with transverse Myelitis, the patient with primary lateral sclerosis, and 1 patient with olivopontocerebellar atrophy; also noted similar enhancement of neurological function. The patients with amyotrophic lateral sclerosis and spinal cord injuries had no changes of significance. Thirty-two out of 44 patients who were ambulatory had significant improvement, whereas 10 of the 19 patients who were not ambulatory had improvement. There was no evidence that electrical stimulation of the spinal cord, when applied via dorsally placed percutaneous electrodes and when carried only to the perception of a paresthesias, has any adverse effect on neurological function. It is hypothecated that the electrical current alters neurotransmitters to enhance the transmission along nervous and neurochemical pathways. The exact mechanisms are unknown at the present time.
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PMID:Treatment of demyelinating and degenerative diseases by electro stimulation of the spinal cord. 31 May 8

The first autopsy of a case of multiple sclerosis from the District of Hokuriku was reported. The patient, a 50-year-old house-wife, born in Toyama Prefecture, had noticed a paresthesia of her face, fatigue, numbness and weakness in the right limbs, dimness of vision and gait disturbance at ave 44. Furthermore, in the course of the disease, she had suffered from visual disorder, tetraplegia, hyperreflexia, pyramidal signs and cerebellar syndroms such as dysarthria, nystagmus, intention tremor and ataxia. She also showed symptoms of euphoria and dementia. After a course of six years she died of bronchopneumonia. Remissions and exacerbations were noted four times during her clinical history. Histopathologically, there were many recent and old demyelinating lesions of varying sizes and shapes in all parts of the central nervous system, namely the cerebrum, brainstem, spinal cord and optic nerve. In contrast to the clinical symptoms, the cerebellum itself revealed less plaques than the other areas of the brain. According to the observed distributions of the lesions, our case can be classified as belonging to the optico-cerebro-spinal type in the Ikuta and Zimmerman classification. The demyelinated lesions were characterized by a perivenular distribution of the plaques, lack of tissue necrosis, paucity of inflammatory reaction and marked fibrous gliosis of varying degrees.
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PMID:First autopsy report of a multiple sclerosis case in Hokuriku District of Japan. 53 38

There are inherent difficulites in the rehabilitation of patients with multiple sclerosis (MS). The clinical manifestations of the disease vary tremendously, as does its natural course. The many theories on the etiology, pathogenesis and treatment of MS are presented. However, at the current time, the patient with MS may be treated with the realistic goal of minimizing the effects of the disease. This is accomplished best in a rehabilitation setting. Diagnosis, prognosis, and factors associated with precipitaiton or exaggeration of symptoms, are discussed. Specific techniques of rehabilitation of decreased motor power, spasticity, ataxia and intention tremor, speech and optic defects, and impaired sensation are presented. Decubiti and ulcerations, bladder and bowel problems, and sexual dysfunction are recognized as common problems and attention must be directed to these. The importance and wide array of emotional difficulties in patients with MS is emphasized, and those patients who may benefit from psychotherapy are discussed.
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PMID:Rehabilitation of patients with multiple sclerosis. 68 58

The author presents a case report of a manic-depressive patient who developed dysarthria and ataxia while on lithium maintenance. These symptoms were erroneously attributed to lithium toxicity occurring at therapeutic serum levels. However, the symptoms persisted despite diminution in lithium dosage and a neurological consultation revealed the diagnosis of multiple sclerosis. The author concludes that lithium therapy is effective with manic-depressive patients, that adverse side effects are infrequent and can occur at toxic or therapeutic serum lithium levels, and that the exceptional patient may have a second illness incorrectly attributed to lithium toxicity.
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PMID:Multiple sclerosis masquerading as lithium toxicity. 69 Jun 25

A description of 2 cases of disseminated sclerosis during exacerbations and paroxysms of dysarthria and ataxia in the clinical picture is given. These attacks are connected with processes of demyelinization and are differentiated with epilepsy. On the basis of neurophysiological studies the mechanisms of paroxysmal dysarthria and ataxia are discussed. The attacks were arrested under the influence of carbamazepine. The action of the drug is explained by its manifold properties.
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PMID:[Paroxysmal attacks of dysarthria and ataxia in multiple sclerosis]. 72 59

Two postmortem case of multiple sclerosis treated by sterotactic operations for the intention shaking of limbs, trunk, and head, and for the action myoclonus are analyzed to determine the location of the substrate of myoclonic and ballistic movements, the location of the coagulations for relief of these movements, and whether fresh demyelinating foci are elicited by intracerebral interventions. In the first case of a clinically typical multiple sclerosis, the foci responsible for the severe action myoclonus and intention ataxia of the trunk are demyelinations in the right and left red nucleus resulting in nerve cell damage and loss and an almost complete destruction of myelinated fibers. The restricted foci in the white matter of the cerebellum which do not involve the cerebellar nuclei are not extensive enough or old enough to be the cause of the action myoclonus but may, perhaps, sustain the pathogenesis. - In the second case of cerebral palsy and combined multiple sclerosis (detected post mortem), the combination of the severe damage of putamen and caudate nucleus by status marmoratus and the extensive nerve cell and fiber damage due to demyelinating foci in the substantia nigra are probably the substrate of the jactitation and intention myoclonus of the left limbs. The stereotactic coagulation of the dentatothalamic and pallidothalamic fibers in the base of V. o.p. and V.o.a. at the point where they pass through the zona incerta (location confirmed post mortem) resulted in a nearly complete relief of hyperkinetic movements. In the first case, fresh demyelinating foci are present in both hemispheres with stereotactic interventions; these foci are located, amongother places, around the coagulation and the electrode track. In the second case, post mortem serial brain sections demonstrate that stereotactic operations even in subacute multiple sclerosis can be carried out without eliciting any exacerbation of demyelination foci. Therefore, the danger exists that stereotactic intervention in cases of multiple sclerosis may precipitate fresh demyelinating foci. As our clinical experience [Riechert and Richter, 1972a, b] indicates, however, this occurred in markedly less than 10% of the cases.
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PMID:Pathologic-anatomical findings and cerebral localization in stereotactic treatment of extrapyramidal motor disturbances in multiple sclerosis. 109 87

Nine cases of multiple sclerosis with paroxysmal disorders were treated with acetazolamide. In most cases a brain-stem origin of the seizures was suggested by their particular pattern: crossed syndromes (facial spasm associated with contralateral weakness of the arm and leg, paroxysmal paraesthesiae in one side of the face and weakness of the contralateral leg), paroxysmal dysarthria, and ataxia. One patient with a Brown-Sequard syndrome complained of paroxysmal paraesthesiae in the lower limbs, for which a spinal origin was admitted. In all patients the paroxysmal disorders were promptly suppressed or markedly reduced by acetazolamide.
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PMID:Treatment with acetazolamide of brain-stem and spinal paroxysmal disturbances in multiple sclerosis. 115

Twelve years following the diagnosis of a histologically proved Sarcoidosis, a patient developed a central paresis of one lower limb. Two years later, tetraparesis, bladder dysfunction, ataxia and an internuclear bilateral ophthalmoplegia occurred. In CSF oligoclonal IgG without dysfunction of the blood brain barrier was found. On the basis of these data multiple sclerosis was diagnosed.
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PMID:[Simultaneous occurrence of sarcoidosis and MS or sarcoidosis within the clinical picture of multiple sclerosis?]. 152 36


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