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Query: UMLS:C0030552 (paresis)
 5,831 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A family--mother and 2 sons--with a heredodegenerative neurological disease is described. The disease started with bilateral optic atrophy, central scotoma, and color blindness during the second decade. This was followed by a quiescent period until additional neurological symptoms appeared, around the age of 50 years in the case of the mother and 40 and 30 years, respectively, in the sons. The additional symptoms were ataxia, spastic paresis, clonic jerks, grand mal seizures, psychia lability, and slight dementia. The disease was progressive, resulting in permanent hospitalization within a few years. The mother died at the age of 63 years and the sons at 46 and 43 years of age. Neuropathological examination revealed lesions histopathologically characteristic of subacute necrotizing encephalomyelopathy (SNE, Leigh disease), and their distribution in the brain and brainstem also conformed to this disease. On the basis of the clinical course and neuropathological findings, we consider that these 3 patients represent the first reported familial cases of the adult form of SNE.
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PMID:Familial subacute necrotizing encephalomyelopathy of the adult form (adult Leigh syndrome). 23 Jul 81

A 10-year-old girl developed bilateral blindness and partial third nerve paresis immediately following a closed head injury. Bilateral optic atrophy developed subsequently. This is the first report of an association between second and third nerve injuries after minor head trauma in the absence of a preexisting lesion. The pathophysiology of indirect injury to the optic nerve under these circumstances is uncertain, but the lesions in this patient seemed to be due to ischemia.
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PMID:Total blindness after trivial frontal head trauma: bilateral indirect optic nerve injury. 57 Jun 62

A 12-year-old boy was admitted to our hospital in May 1990. Since two years previously, he became restless and could not adapt himself to his new school mates and teacher. He came to refuse going to school, and his intellectual performance rapidly deteriorated, thereafter. During the following two years, he gradually became mutic and incontinent with dystonic involuntary movements, and was admitted to our hospital. Examination revealed melanoderma, mutism, dementia and grossly normal visual and auditory system except for bilateral optic atrophy. Volitional movements were severely limited because of marked truncal and extreme dystonia and spastic paresis. Urinary 17-KS and 17-OHCS were decreased. Serum cortisol did not increase normally on rapid ACTH test. Very-long-chain fatty acids in serum were increased. Serial CTs and MRI demonstrated marked ventricular dilatation and diffuse white-matter lesions involving the frontal lobes, corpus callosum, internal capsules, and less markedly parieto-occipital regions with slight calcification in the optic radiations. Those in the frontal lobes had been visualised in CTs taken 18 months after the onset. School refusal, the initial symptoms of the patient, is one of serious socioeducational problems in Japan. While the majority of children with school refusal might be of non-organic affections, those with apparent mental deterioration and behavioral abnormalities should undergo non-invasive CT or MRI evaluation during the early stage of the disorder. And if diffuse white matter lesions is suggested even in the frontal lobe, the possibility of adrenoleuko-dystrophy should be considered.
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PMID:[A case of adrenoleukodystrophy having progressed from the frontal lobes]. 142 38

We examined potential clinical and pathologic correlates of seizures among the 3,291 children in the Childhood Brain Tumor Consortium database. Fourteen percent had seizures prior to their hospitalization for a brain tumor. Among children who had a supratentorial tumor, seizures occurred in 22% of those less than 14 years of age. The prevalence of seizures increased to 68% of older teenagers. Among children with an infratentorial tumor, the prevalence of seizures was relatively constant at 6% over all age groups. The onset of seizures began more than one year prior to surgical tumor removal in over half of the children aged five or more with supratentorial tumors, significantly longer than for those of the same age with infratentorial tumors. Almost all children (98.9%) with an infratentorial tumor and seizures had at least one other symptom and more than three-fourths of them had at least three. Eighty-nine percent of children with a supratentorial tumor and seizures had at least one other symptom and more than one-half had at least three symptoms. Regardless of whether the tumor was above or below the tentorium, confusion or stupor and coma were more common in children with seizures than in children without seizures. Among children with supratentorial tumors, symptoms of a declining academic performance or an abnormality of personality, speech, walking, or sensation were significantly more frequent in children with seizures, while visual symptoms (other than visual loss or diplopia) and nausea or vomiting were less frequent. Among children with supratentorial tumors, those who had seizures were more likely to have paralysis of an arm, hand, or face, confusion or stupor, or coma and less likely to exhibit irritability, papilledema, optic atrophy, decreased visual acuity, pupillary abnormalities, or abducens paresis. Among children with infratentorial tumors, those with seizures were significantly less likely to have truncal ataxia, but more likely to experience confusion, stupor, or coma. In the supratentorial compartment, astrocytoma (nos), protoplasmic astrocytoma, anaplastic astrocytoma, and ependymoma were more frequently associated with seizures than was craniopharyngioma. No infratentorial tumor type was more or less likely to be associated with seizures. All common tumor types that were represented in both the supratentorial and the infratentorial compartment except astrocytoma (nos) were associated with significantly greater rates of seizures when located in the supratentorial compartment. The tumor location with the highest incidence of seizures was, as expected, the superficial cerebrum. More than 40% of the children with such tumors had seizures.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:Epidemiology of seizures in children with brain tumors. The Childhood Brain Tumor Consortium. 154 79

The object of the study are 126 children with internal hydrocephalus treated within the period 1978-1990. The children were preoperatively divided into six groups according to the etiology of the hydrocephalus. Disorders of the visual functions accompanying elevated intracranial pressure (setting sun 51, syndrome of the aqueduct of Sylvius 14, paresis of craniocerebral nerves 9, nystagmus 8, optic atrophy 4) were established preoperatively in 48.4% of the children. 44 of the children were revised because of malfunction of the shunt with the following neuroophthalmological symptoms: setting sun 6, paresis of craniocerebral nerves 9, syndrome of the Sylvius aqueduct 6, papilledema 6, optic atrophy 9 and nystagmus 5. The fact that a great many changes in the visual functions sometimes precede the manifestations of the changes in CT image determines their significance for early diagnosis and treatment.
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PMID:Neuroophthalmological symptoms in children treated for internal hydrocephalus. 184 Aug 20

Observation of neurosarcoidosis in a thirty-year-old black female characterised predominantly by bilateral facial nerve paralysis gave rise to a review of literature since 1978 and also to a comparison with an early study in 1963. As described 1963 the clinical picture is characterized by increased protein content of the CSF (33.8%), facial nerve paresis (25.5%), pleocytosis (23%), diabetes insipidus (21%), hemiparesis (17.2%), organic psychosis (16.9%), papilloedema (15.5%), ataxia (13%), convulsive seizures (12.5%), optic atrophy (12.5%), loss of hearing (12.2%), nystagmus (8.6%) and numerous other symptoms more rarely found. This corresponds to the symptoms of chronic basal meningitis with an infiltration in the neighbouring structures of brain and less frequently the spinal cord. In only 58.7% of the cases (presumably at the onset of sarcoidosis) was the bronchial tract (or the lungs) affected, in 11.5% the skin or the eyes. Although the clinical picture is clear enough the etiology has yet to be determined. Evidence of a pathogen or a pathogenic agent (analogous to berylliosis) has never been established to date. On the other hand there are some indications of a disturbance in the immune system, perhaps of a particular genetic foundation since sarcoidosis strikes black patients with conspicuous frequency. There exist more cases in one family. Exceeding expected random distribution, many patients have the HLA-Factor B 8 (on the chromosome 6) and DR 3. The Kveim-Test was in 71 cases positive, in 12 cases negative. The possibilities of carrying out studies of CSF - analogous to the studies of bronchial lavage - in the most cases of neurosarcoidosis have not been exhausted as to determine the activity of the T-lymphocytes, the interleucines, the angiotensin-converting enzyme while the Gallium 67 scintigraphy and other methods to determine the non-specific activity of the inflammation. The efficiency of the treatment with corticosteroids (Prednison or Triamcinolonacetonid) depends of the phase of the inflammatory process. 12% of the registered cases died.
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PMID:[Neurosarcoidosis. Comparative analysis of the clinical profile based on 537 cases from the world literature up to 1963 and from 1976-1988]. 240 26

A case is described in which a presumed vascular accident resulted in long-standing visual difficulties in both reading and the analysis of vectors of moving objects. Clinical examination revealed minimal right optic atrophy with a relative superior altitudinal visual field defect associated with a positive Pulfrich effect. A partial head turn to the right in association with paresis of saccades and pursuit eye movements to the right was also evident. Spectacles for distance which incorporated a neutral density filter before the left eye were prescribed. These practically eliminated the Pulfrich effect and alleviated the problems of vector analysis. A near correction was provided which incorporated prisms with bases to the right. This eliminated the reading difficulties. The visual problems experienced by patients suffering from a positive Pulfrich effect are described and discussed.
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PMID:The Pulfrich phenomenon and its alleviation with a neutral density filter. 261 Nov 82

Report on a 56-year-old woman with relapsing polychondritis, with ocular involvement in the form of bilateral optic atrophy and paresis of the extraocular muscles. The cause of this disorder has not yet been identified, although it is presumed to be an immune disease. Clinically there are some obvious similarities to systemic vasculitis.
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PMID:[Rare form of ocular involvement in relapsing polychondritis (author's transl)]. 725 26

See-saw nystagmus is a pendular nystagmus with two distinct components: a conjugate torsional component and a disjonctive vertical component. The acquired form of nystagmus is usually seen with parasellar mass and less frequently in head injury or brainstem infarction. Almost all patients with parasellar mass or head injury have associated bitemporal hemianopia. We report the case of a 27-year-old man who developed intermittent daily oscillopsia five years after a severe head injury. On neurological examination, the patient showed a congenital left eye divergence with left eye convergence paresis, an anosmia, a right optic atrophy and a bitemporal hemianopia. Visual acuity was 7/10 and 3/10 for the right and left eyes. The rest of the neurological examination was unremarkable. Electroencephalogram during oscillopsia was normal. MRI revealed bilateral orbito-frontal low signal intensity on T1 weighted images. The brainstem, the diencephalon and the ventricular system appeared to be intact. Eye movement recordings exhibited intermittent see-saw nystagmus. Clonazepam treatment resolved the see-saw nystagmus. Slowly withdrawal of clonazepam was done without return of the see-saw nystagmus.
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PMID:[Intermittent sea-saw nystagmus successfully treated with clonazepam]. 767 33

Although ocular manifestations of Lyme disease have long been noted, they remain a rare feature of the disease. The spirochete invades the eye early and remains dormant, accounting for both early and late ocular manifestations. A nonspecific follicular conjunctivitis occurs in approximately 10% of patients with early Lyme disease. Keratitis occurs often within a few months of onset of disease and is characterized by nummular nonstaining opacities. Inflammatory syndromes, such as vitritis and uveitis, have been reported; in some cases, a vitreous tap is required for diagnosis. Neuro-ophthalmic manifestations include neuroretinitis, involvement of multiple cranial nerves, optic atrophy, and disc edema. Seventh nerve paresis can lead to neurotrophic keratitis. In endemic areas, Lyme disease may be responsible for approximately 25% of new-onset Bell's palsy. Criteria for establishing that eye findings can be attributed to Lyme disease include the lack of evidence of other disease, other clinical findings consistent with Lyme disease, occurrence in patients living in an endemic area, positive serology, and, in most cases, response to treatment. Management of ocular manifestations often requires intravenous therapy.
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PMID:Ocular manifestations of Lyme disease. 772 93


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