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 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

Since the description by Galen in the 2nd Century, A.D., clinical neurology has acknowledged the existence of two types of tremor: that which occurs at rest and that occuring during the execution of movement. With the help of refined methods of analysis, E.M.G. and cinephotography, the authors have carried out a detailed clinical assessment in more than 400 patients. The basic criterion used to define a tremor was the classical definition of Dejerine: "An involuntary, rhythmical and symmetrical movement about an axis of equilibrium." As a result of this study, the conclusion has been reached that there are two types of tremor: postural tremor and tremor of attitude. Both are present while the limb remains immobile, whether by wilful design or when at rest in a position of posture and subject only to the action of gravity. During voluntary movement, tremor is not present. Irregular, asymmetrical and non-rhythmic oscillations may appear however - as in so-called intention tremor, of cerebellar origin - but this abnormal movement can hardly be called a real tremor. It is merely a manifestation of ataxia. As a consequence of this study, it is suggested that further understanding of the basic mechanism of tremor can be reached by the investigation of the central neural structures which are involved in the physiology of posture and attitude.
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PMID:Semiology of Tremors. 109 80

An account is given of a form of hereditary, cerebellar ataxia and photomyoclonus. Eight cases from 5 generations were affected. The disease seemed to be transmitted as an autosomal dominant trait. The age at onset usually varied between 35 to 40 years. The symptoms and signs consisted of a cerebellar ataxia, dysarthria and intention tremor. There was no nystagmus. All patients exhibited photomyoclonus and were extremely sensitive to photic stimuli. Other signs were dementia, kyphosis, pes cavus and lipoma localized in the nape of the neck, shoulders and back. Two patients had a partial syndrome with photonyoclonus and skeletal deformities. None of the patients suffered from epilepsy, In one case, histopathological examination revealed atrophy within the cerebellar cortex, dentate nucleus and the posterior columns of the spinal cord. It is concluded that this syndrome belongs to a groups of hereditary ataxias and myoclonus, and differs from myoclonic cerebellar dyssynergia (Ramsay Hunt) and alos from a variety of familial myoclonus and ataxia (Gilbert et al. 1963);
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PMID:Hereditary ataxia, photomyoclonus, skeletal deformities and lipoma. 113 Jan 71

Human and animal experiments performed recently have resulted in a more detailed understanding of limb movement and body posture disorders associated with cerebellar dysfunction. The delay in movement initiation can be explained by a delay in onset of phasic motor cortex neural discharge owing to decreased input from the cerebellar hemispheres. Disorders of movement termination (dysmetria), which can occur for movements at proximal and distal joints, result from disturbances of the timing and intensity of antagonist electromyographic (EMG) activity necessary to break the movement. Disorders in velocity and acceleration of limb movements result from muscular activity that is smaller in amplitude and more prolonged. The cerebellum is important for control of constant force but not for generation of maximal force. Dysdiadochokinesia is explained by a combination of the above mentioned mechanisms. During complex movements in three-dimensional space, the cerebellum contributes to timing between single components of a movement, scales the size of muscular action, and coordinates the sequence of agonists and antagonists. The basic structure of motor programs is not generated in the cerebellum. Hypotonia can be observed only in acute cerebellar lesions. Cerebellar tremor appears to result from a central mechanism, but is modulated or provoked through increased long-loop EMG responses. The common assumption that cerebellar ataxia of stance does not improve with visual feedback is true only of vestibulocerebellar lesions, not for ataxia resulting from atrophy of the anterior lobe of the cerebellum.
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PMID:Pathophysiology of cerebellar ataxia. 158 45

A 38 year-old laborer experienced solvent intoxication during each of two spray paintings of a dump truck and other heavy equipment in an enclosed, unventilated garage. The paint base consisted primarily of toluene and methyl ethyl ketone. Nausea, headaches, dizziness, respiratory difficulty and other symptoms began after exposures. Over the next several days he developed impaired concentration, memory loss and cerebellar signs including an intention tremor, gait ataxia and dysarthria. MRI of the brain and EGG early in the work-up were normal, although later MRIs demonstrated fluid collection over the left parietal area. Examination by a toxicologist and neurologist revealed likely toxic encephalopathy with dementia and cerebellar ataxia. Three formal neuropsychological assessments over 2 1/2 years quantified cognitive, motor and behavioral changes. Despite similar findings in chronic exposure to these solvents, lasting sequelae following acute exposure have not been widely reported.
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PMID:Chronic neuropsychological and neurological impairment following acute exposure to a solvent mixture of toluene and methyl ethyl ketone (MEK). 174 49

Diseases affecting the cerebellum typically cause ataxia, coupled with dysmetria and tremor. Dysmetria is a condition in which there is improper measuring of distance in muscular acts; hypermetria is overreaching (overstepping) and hypometria is underreaching (understepping). Tremor refers to an involuntary, rhythmic, oscillatory movement of a body part. The tremor of cerebellar disease typically is exaggerated by goal-oriented movements (intention tremor). Cerebellar lesions also often cause loss of the menace response, despite the presence of normal vision. The anatomic basis for this phenomenon is obscure. The principal disease affecting the cerebellum in cats is cerebellar hypoplasia due to in utero infection with the panleukopenia virus. This disease will be discussed here. Neurologic signs of cerebellar involvement also may be seen in association with those diseases that affect the CNS multifocally. In these cats, there may be additional signs indicating involvement of other anatomic areas or the cerebellar deficits may occur alone (see discussion of multifocal diseases in Multiple Neurologic Deficits: Inflammatory Diseases [page 426] and Multiple Neurologic Deficits: Noninfectious Diseases [page 440]).
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PMID:Ataxia, dysmetria, tremor. Cerebellar diseases. 180 62

Four weeks after starting tuberculostatic treatment (with isoniazid, rifampicin, streptomycin and pyrazinamide) a 21-year-old man with pulmonary tuberculosis developed symptoms of a radiculomyelopathy as well as mild renal failure. After isoniazid and streptomycin had been discontinued and ethambutol and high doses of vitamin B6 had been added all signs and symptoms improved. However, 4 weeks later tuberculous meningitis occurred which at first seemed to respond to administration of 5 antituberculosis drugs and dexamethasone. But 3 weeks later the patient sustained a partial hemiparesis. Its cause was proven to be a tuberculoma in the region of the brainstem. During further administration of tuberculostatic drugs and glucocorticoids the symptoms gradually receded over 8 months. A 54-year-old man with pulmonary tuberculosis developed cranial nerve pareses and symptoms of cerebellar involvement (trunk ataxia, intention tremor, dysdiadochokinesia) 3 weeks after starting tuberculostatic treatment. Computed tomography revealed multiple intracerebral tuberculomas which gradually shrank with continuation of the tuberculostatic treatment plus glucocorticoids. These two case reports illustrate that in tuberculosis involvement of the CNS can express itself clinically through complex symptoms, sometimes even after the start of tuberculostatic treatment.
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PMID:[Involvement of the central nervous system in disseminated tuberculosis]. 193 77

A case of acute cryptococcal cerebellar encephalitis with CT enhancement confined to the cerebellum is reported. A 46-year-old man with hepatoma was admitted with chief complaints of headache, fever and dizziness. On admission, cerebellar signs (disturbance of finger-to-nose test and of heel-to-knee test, intention tremor, and truncal ataxia) were neurologically noted. However, there were no brainstem signs. Head CT showed swelling and enhancement of the cerebellar cortex and dilatation of the cerebral ventriculi. Cryptococcus neoformans was detected in a culture of the patient's CSF. Clinical symptoms and signs, and enhancement of the cerebellum on CT gradually diminished after administration of anti-fungal drugs, and CSF became negative for cryptococcal antigen 6 months after admission.
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PMID:[A case of acute cerebellar encephalitis due to Cryptococcus neoformans]. 193 83

Eight cases of intention tremor as a late complication of head injury were investigated. The patients ranged in age from 3 to 24 years. All received severe head injuries and lapsed into coma immediately afterward (Glasgow Coma Scale scores less than or equal to 8). Six patients exhibited decerebration or decortication. Hemiparesis was present in six cases and oculomotor nerve palsy in four. In the chronic stage, all patients displayed some degree of impairment of higher cortical function and five had dysarthria and/or ataxia. Initial computed tomography (CT) scans within 3 hours after the injury were obtained in five cases, of which four showed a hemorrhagic lesion in the midbrain or its surroundings. Other CT findings were diffuse cerebral swelling (four cases), intraventricular hemorrhage (three), and multiple hemorrhagic lesions (two). In the chronic stage, generalized cortical atrophy or ventricular enlargement was noted in five cases. These clinical features and CT findings indicate diffuse brain damage as well as midbrain damage and may reflect shearing injury.
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PMID:[Intention tremor after head injury. Clinical features and computed tomographic findings]. 247 1


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