摘要:

Six cases of classic achromatopsia arc presented. The methods of practical clinical diagnosis are discussed, including paradoxical pupillary constriction in darkness, the easily performed Sloan achromatopsia test, and electrophysiologic studies which are useful in voung children. The visual and cosmetic benefits of heavily tinted contact lenses in such patients are stressed.

ISSN:0272-846X    

出版商:OVID    

年代:1982

数据来源: OVID

摘要:

Three patients with optic tract lesions, incomplete homonymous hemianopias, and good vision in both eves are presented. All three had a Marcus Gunn pupil in the eye with temporal field loss (eye contralateral to the tract lesion). This pupillary sign may serve to differentiate acute optic tract from acute ge-niculostriate lesions when the characteristic infrageni-culate optic atrophy has not had time to develop.

ISSN:0272-846X    

出版商:OVID    

年代:1982

数据来源: OVID

摘要:

An adolescent female with downbeat nystagmus and long tract signs was evaluated by a number of diagnostic radiologic procedures. Metrizamide CT cisternography was the procedure that established the diagnosis by showing not only the tonsillar herniation, but also the medullocervical deformity characteristic of the Chiari type II malformation. This case report emphasizes the superior diagnostic capability of this technique with respect to conventional radiography in the diagnosis of Arnold-Chiari malformation.

ISSN:0272-846X    

出版商:OVID    

年代:1982

数据来源: OVID

摘要:

A syndrome including juvenile-onset diabetes mellitus, optic atrophy, diabetes insipidus, and high-frequency sensorineural hearing loss, expressed completely or partially, is being identified with greater frequency. Visual loss may be progressive and accompanied by primary optic atrophy. Concurrent diabetic retinopathy has only rarely been reported. Visual acuity may deteriorate to less than 20/400. The etiology is unknown. However, histopathologic studies indicate that the diabetes insipidus, optic atrophy, and neurosensory hearing loss represent progressive degenerative conditions. Inheritance appears to be autosomal recessive with incomplete penetrance. The syndrome is rare; therefore, a complete neuroophthalmologic and neuroradiologic evaluation is imperative to rule out a mass lesion. This syndrome should be considered in young people with visual loss and optic atrophy of unknown etiology even if they are not known juvenile diabetics.

ISSN:0272-846X    

出版商:OVID    

年代:1982

数据来源: OVID

摘要:

The Sturge-Weber syndrome is characterized by the presence of a leptomeningeal hemangioma variably associated with a facial nevus flammeus, seizure disorder, mental retardation, hemiparesis, homonymous hemianopsia, glaucoma, and other signs and symptoms. The relationship between the cutaneous manifestations and the other features of this disorder was studied in 35 patients. We observed that all patients with the facial nevus flammeus had involvement of the upper eye lid or forehead. Bilateral cutaneous involvement was common and when present, was often associated with extensive lesions of the trunk and extremities. The distribution of the cutaneous lesions appeared to be unrelated to that of the trigeminal nerve. A subgroup of patients, the syndrome's “forme fruste,” were identified. There appeared to be no relationship between the presence and extent of the cutaneous lesions and the observed seizure disorder, difficulty of seizure control, intellectual deficit, hemiparesis, homonymous hemianopsia, and intracranial calcification. Notably, the “forme fruste” patients appeared to be spared from glaucomatous involvement and from choroidal hemangioma. We will also discuss other aspects of this syndrome.

ISSN:0272-846X    

出版商:OVID    

年代:1982

数据来源: OVID

摘要:

The atypical clinical presentations of three patients with an oculomotor (III) nerve palsy are outlined. The first patient is a 49-year-old with a painful, pupil-sparing ophthalmoplegia of sudden onset due to a pituitary adenoma which had eroded into the cavernous sinus. The second patient is a 7-year-old with a traumatic III nerve palsy who despite full recovery of her oculomotility, has been symptomatic due to a permanent internal ophthalmoplegia. The third patient is an 8-year-old who has undergone prolonged optomet-ric therapy for an “atypical esotropia,” but in fact has a congenital III nerve palsy with oculomotor synkinesis and deep amblyopia.

ISSN:0272-846X    

出版商:OVID    

年代:1982

数据来源: OVID

摘要:

Forced downward deviation of the eyes has been described as a useful sign in patients with hysterical coma and seizures. Two patients are described emphasizing the value of this test in prolonged feigned sei-zured states as well as in hysterical syncopal episodes.

ISSN:0272-846X    

出版商:OVID    

年代:1982

数据来源: OVID

摘要:

Basic concepts in neurobiology are reviewed with emphasis on axonal transport and the nerve growth factor. Our current understanding of the regenerative capacity of the mammalian central nervous system, and research designed to minimize neuronal damage following trauma are discussed.

ISSN:0272-846X    

出版商:OVID    

年代:1982

数据来源: OVID

摘要:

A 15-year-old boy with a recurrent third nerve palsy who was found to have a neurinoma of the third nerve is described. Unusual features of his presentation and review of the literature relative to this rare tumor are discussed. Tumors derived from Schwann cells, which wrap around the axons of peripheral nerves, are called neurinomas, neurofibromas, plexi-form neurofibromas, neurilemomas, and schwannomas. Neurinomas are usually localized in the peripheral nerves.1The most frequent site of occurrence of an intracranial schwannoma is the acoustic nerve; however, the tumor may occur along the second, fifth, seventh, 11th, and 12th cranial nerves.2Neurinomas of the oculomotor nerves are extremely rare. In the literature, there are only four reported cases clinically manifested and pathologically verified as neurinomas of the third nerve.

ISSN:0272-846X    

出版商:OVID    

年代:1982

数据来源: OVID

ISSN:0272-846X    

出版商:OVID    

年代:1982

数据来源: OVID