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Query: UMLS:C0004134 (ataxia)
 15,886 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A virus isolated from the CSF of a patient who had amyotrophic lateral sclerosis for 7 years, and prolonged pleocytosis in the CSF, was adapted to suckling mouse brain by subsequent serial blind passages. This Schu virus belongs to the tick-borne encephalitis complex of the genus Flavivirus (Togaviridae). Suckling mouse brain homogenate of the 13th passage was used for transmission experiments in various species of laboratory animals. Golden hamsters infected subcutaneously fell ill after a number of months, lost weight, and had paresis of the legs. Histologically they had petechial hemorrhages in different parts of the CNS and inflammatory changes in the gray substance of the spinal cord. Pilot studies with repeated inoculations of small doses of different flavivirus strains suggest a course of the disease in experimental animals which resembles slow-virus infections insofar as no encephalitis is produced and degenerative changes of the anterior horn cells prevail over inflammatory signs in the spinal cord. After intracerebral application of Schu virus, cynomolgus monkeys developed the typical lesions of togavirus panencephalitis with epileptic seizures, ataxia, and paresis. After subcutaneous application, the virus seems to spread along peripheral nerves to anterior spinal roots and spinal cord, where mainly motor neurons of the anterior horn are damaged, and from there to the brain. The histological findings are such that one may assume the disease of the patient was due to the infection with the virus isolated from his CSF. Therefore, the hypothesis may be advanced that at least some of the cases diagnosed as amyotrophic lateral sclerosis are due to a togavirus infection.
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PMID:Attempts to reproduce amyotrophic lateral sclerosis in laboratory animals by inoculation of Schu virus isolated from a patient with apparent amyotrophic lateral sclerosis. 8 63

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

Samples of cerebrospinal fluid (CSF) of 93 dogs with neurologic diseases were examined by cytomorphologic technique, and the changes in the CSF were correlated with histopathologic examinations of the central nervous system (CNS). It was concluded that CSF examination is a significant aid in obtaining a neurologic diagnosis and that good correlation exists between the CSF changes and the pathologic changes in the CNS. The CSF examination allows making a diagnosis of encephalitis and differentiation between viral and other causes (although in mycotic infection the cell membrane preparation can be used to identify the cause directly), could allow making differentiation between congenital malformations and congenital degenerative disease, and helps in identifying physical spinal cord damage, differentiating it from muscular, neurogenic, or functional disorders clinically presented as spinal ataxia. The CSF cytologic examination can indicate the presence of hemorrhage in the CNS. There is not enough experience available in the diagnosis of brain tumors by means of CSF examination; however, in dogs with lymphosarcoma in the CNS, CSF cytologic changes can be diagnostic.
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PMID:Cerebrospinal fluid cytology in canine neurologic disease. 57 91

Three necropsied cases of chronic, sporadic brainstem encephalitis of unknown aetiology are presented. Since their outstanding symptoms were dementia and ataxia of a progressive nature, a noninflammatory disease of the central nervous system was suspected. Neuropathological studies showed chronic inflammatory changes mainly in the brainstem without the presence of inclusion bodies or viral particles. Compared to cases previously reported as brainstem encephalitis, the clinical and pathological findings observed in these cases have rather peculiar characteristics.
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PMID:Chronic brainstem encephalitis with mental symptoms and ataxia: report of three cases with necropsy. 67 Oct 63

It is suggested that damage by mild trauma, viruses or bone disease to the otic capsule or to the membranes between the cochlea and the middle ear is common, and involved in many syndromes of obscure etiology. The clinical perilymph fistula (PF) syndrome can consist of any combination of the following: tinnitus, deafness, phonophobia, vertigo, ataxia, otalgia, facial palsy, headache, diplopia, blackouts, psychological distress. The following testable hypotheses are proposed: otitis media is due to perilymph in the middle ear, with secondary changes resulting from infection or inflammation: otosclerosis results from a slow leak in the presence of enzymes promoting bone growth: Meniere's syndrome follows reduced perilymph support for the endolymphatic system: Bell's palsy results from a perilymph provoked oedema in the bony facial nerve canal: PFs may be responsible for progressive rubella deafness, and for some cases of migraine, epilepsy, anxiety neurosis and hysteria: psychiatric sequelae of the PF syndrome predominate in the post-concussional syndrome and infantile autism: organisms can pass from the throat into the spinal fluid, causing meningitis or encephalitis. The tinnitus and vertigo are caused by random labyrinthine fluid movements, the headache and diplopia by reduced spinal fluid pressure.
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PMID:Perilymph fistula: a cause of auditory, vestibular, neurological and psychiatric disorder. 78 62

A number of flock diseases in sheep associated with impaired locomotion, which are of importance or may assume importance in the Netherlands, are reviewed. Attention is paid to the following metabolic disorders and deficiencies: acetonaemia, hypocalcaemia, hypomagnesaemia, some forms of osteopathy, enzootic ataxia and cerebrocortical necrosis. The following forms of infectious diseases are described: listeric encephalitis, purulent meningitis following injury to the throat, scrapie, visna, Aujeszky's disease and border disease. Finally, a number of viral forms of encephalitis are discussed, which are of minor importance or negligible in the Netherlands, though they are of importance in neighbouring countries. No attempt was made exhaustively to describe the above diseases. The aetiology and pathogenesis usually were not discussed in greater detail than that required for the diagnosis, treatment and prevention of the disease. Efforts were made to be of some help to the veterinary practitioner in controlling some forms of ovine disease in cases in which this is necessary and possible.
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PMID:[Some forms of disease associated with impaired locomotion in sheep (author's transl)]. 96 67

A patent, who suffered from nonprogressive athetotic-myoclonic hyperkinesia of the left arm and spastic paresis of the underdeveloped left leg since early childhood, experienced a febrile episode at the age of 23, after which a weakness and ataxia of the right limbs with frequent falling persisted; at the age of 28, he developed a right-sided rigidity, tremor at rest and mask-like face as sequelae of encephalitis disseminata exacerbations. The violent intention myoclonus of the left side of the body could be abolished by stereotactic coagulation in the V.o.p and V.o.a and zona incerta until death 11 days later. The athetotic myoclonic hyperkinesia is the consequence of a left-sided severe status marmoratus of the right putamen with extensive loss of nerve cells and shrinkage. The additional nerve cell loss in the right substantia nigra due to demyelinating encephalitic foci did not produce Parkinson symptoms, because these require a normal striatum. This loss did, however, exaggerate the original hyperkinesia to a violent intention myoclonus, which was abolished by interruption of denatatothalamic afferents to the V.o.p nucleus and of pallidothalamic afferents to the V.o.a nucleus. The Parkinson syndrome of the right side is due to demyelinating foci of different ages. The Parkinson symptoms were manifest on the right side, because the left status marmoratus did not severely reduce the striatal nerve cells. In this case, there is no indication that the introduction of the stereotactic electrode has precipitated new demyelinating foci.
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PMID:Stereotactic treatment of action myoclonus in a case of combined status marmoratus and multiple sclerosis. A contribution to the pathophysiology of basal ganglia with multiple lesions in both the striatum and the substantia nigra. 109 75

Three cases (case 1, female, aged 30; case 2, male, aged 32; case 3, male, aged 34) of benign brainstem encephalopathy with truncal ataxia were reported. Two patients had prodromal symptoms Neurological examination revealed truncal ataxia in all cases. As additional neurological signs, anisocoria, mydriasis, nystagmus, ptosis, transient opsoclonus, and facial palsy were seen. There was neither drowsiness nor myoclonus in the three cases. On laboratory examinations, cold agglutination test revealed significant elevation in two cases. The examination of cerebrospinal fluid showed a moderate rise of proteins in one case, but did not revealed pleocytosis in any of the cases. Magnetic resonance imaging of one patient revealed an area of high intensity in the left pontine tegmentum by T2-weighed imaging. The prognosis for all these cases was good, and the reappearance of neurological signs was not present until now. Our cases were different from brainstem encephalitis (Bickerstaff's encephalitis) because of an absence of disturbed consciousness and no pleocytosis in the cerebrospinal fluid. Our cases were also different from "myoclonus-opsoclonus syndrome" because of an absence of myoclonus. We discussed a possibility of a new clinical syndrome which we call "benign brainstem encephalopathy with truncal ataxia".
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PMID:[Benign brainstem encephalopathy with truncal ataxia--a clinical study of 3 cases]. 128 89

The authors describe the course of herpetic encephalitis in 52 patients aged 16 to 64 years. Five types of the initial manifestations of herpetic infection of the CNS were revealed. In 44.3% of cases the disease started from the general cerebral symptomatology and consciousness disturbance; in 13.6% it started in a brain stroke-like manner followed by the development of the comatose status; in 13.6% of cases from memory disorder and unmotivated actions; in the same percentage of cases, the disease onset was marked by the dominance of dizziness, diplopia, ataxia and central hemiplegia ; in 15.9% the disease started from pains in the stomach, loin and lower limbs. Hemispheric and pseudotumorous stem encephalitides (48.1 and 13.6% respectively) were predominant; in 25% meningoencephalitides and in the remainder, encephalomyelitis running their course in the form of disseminated encephalomyelitis (5.7%), focal myelitis (5.7%) or opticomyelitis (1.9%). The data presented attest to the pleomorphism of the clinical picture of herpetic lesions of the CNS.
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PMID:[Clinical forms of acute herpetic infections of the central nervous system in adults]. 132 73

Five hundred and forty four cases of cerebral palsy were studied to find the etiology. Male to female ratio was 1.9:1. Prenatal, natal and postnatal factors were found in 42 (7.72%), 238 (43.75%) and 142 (26.1%) cases respectively. Only 79 (14.52%) cases were found to have more than one factor which could have contributed to brain damage. In 43 (7.9%) cases the prenatal, natal and postnatal history were normal and the cause was not known. Toxemia (1.29%) cases and microcephaly (1.84%) cases were the most common etiological factors in the prenatal category. Among the natal causes, birth anoxia was the most common etiological factor and was observed in 24.45% cases. Infections of the central nervous system comprised the major etiopathogenetic factors of the postnatal causes--11.95% cases had encephalitis, while 5.15% cases had meningitis. In cases where more than one etiology was present, the most frequent causes were a combination of prematurity or birth anoxia in association with toxemia, antepartum hemorrhage, prolonged labour, twins, forceps or caesarean delivery. Anoxia was consistently the most common etiological factor in those cases of monoplegia paraplegia, quadriplegia, diplegia, and ataxia, i.e. in 0.55%, 1.29%, 11.76%, 6.07% and 0.55% cases respectively. The present study reveals that majority of the cases were found to have natal or post natal etiology.
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PMID:Cerebral palsy--an etiological study. 134 Aug 61


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