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Biomedical subjects

W B Wilson

Publications and source records attributed to W B Wilson.

At least 37 records · Page 2Linked to original sources
PubMed1988

Sneddon's disease presenting with visual loss and dementia.

A 51-year-old woman with Sneddon's disease presented with transient right hemifield loss of vision and transient right-sided weakness. Over the preceding decade she had experienced a slow decline in mental function. She also had hypertension, migraine, and a mixed seizure disorder. She had skin changes typical for generalized livedo reticularis but she did not have Raynaud's phenomenon or winter ulcerations. Her disease was not understood until the stroke-related symptoms were associated with the skin abnormalities. We review the neuro-ophthalmic manifestations of Sneddon's disease and add data from our case to the growing body of fact that suggests that Sneddon's disease may be an immunologically mediated vasculopathy.

Cerebrovascular Disorders↗
PubMed1988

Isolated vitamin E deficiency in the absence of fat malabsorption--familial and sporadic cases: characterization and investigation of causes.

We observed four young adults, including three siblings, with a progressive neurologic disorder that developed over the first two decades. Electrophysiologic studies revealed mildly delayed nerve conduction, decreased amplitudes of sensory action potentials, and sensory delay in the posterior columns. Known causes of similar neurologic disorders were excluded. Although vitamin E deficiency was well documented, intestinal absorption and plasma lipoprotein transport of vitamin E were normal. Incubation studies in vitro failed to identify a plasma factor causing destruction of circulating vitamin E. There was no clinical or laboratory evidence of steatorrhea caused by gastrointestinal, hepatic, or pancreatic disease. Plasma lipoproteins, apolipoprotein B, and adipose tissue fatty acid composition were normal. Oral vitamin E therapy restored serum levels to normal and caused neurologic improvement in two patients. We postulate that an inherited defect in hepatocyte secretion of vitamin E into lipoproteins may account for this disorder, which occurs in sporadic cases as well as in siblings.

Absorption↗
PubMed1987

Sudden onset of blindness in patients treated with oral CCNU and low-dose cranial irradiation.

Three patients developed the sudden onset of total blindness several months after treatment with oral CCNU and low-dose whole-brain radiation. The anterior visual system was included in the radiation field in all patients. Radiotherapy was given for a frontal-lobe glioblastoma multiforme, for central nervous system prophylaxis in a patient with oat cell carcinoma of the lung, and for a parietal-lobe glioblastoma multiforme. None of the neoplasms involved the anterior visual system. The radiation dose ranged from 3000 to 4650 rad and the oral CCNU dosage from 300 mg to 1050 mg. Patients 1 and 2 also received other chemotherapeutic agents. Patient 3 who was treated only with oral CCNU and cranial irradiation died. At autopsy the brain showed a widely infiltrating residual high-grade glioma as well as patchy coagulative necrosis with swollen axons and dystrophic calcifications. The optic chiasm showed severe demyelination, axonal loss, and hyalinized vessels. Synergism between oral CCNU and radiation may account for the blindness produced.

Administration, Oral↗
PubMed1981

Electrophysiologic findings in bilateral optic nerve hypoplasia.

Electrophysiologic studies were performed on 17 patients with bilateral optic nerve hypoplasia. Since most patients were infants, the diffuse light visual-evoked response was more helpful than the visual-evoked response obtained with pattern stimulation. In children over 1 year of age, the amplitude with diffuse light stimulation correlated well with other clinical measurements used to assess visual function. Before 6 months of age, it correlated better with visual prognosis than did clinical analysis. Photopic and scotopic electroretinographic measurements were normal in all except two in whom the scotopic b wave was supernormal.

Adolescent↗
PubMed1980

Comparison of the pattern and diffuse-light visual evoked responses in definite multiple sclerosis.

We prospectively studied 100 patients with "definite" multiple sclerosis to determine which of three methods of stimulation most consistently had results that disclosed abnormalities in the visual evoked response when they were compared with those from normal controls. The three methods were diffuse flashes of light, intermittent reversing pattern, and constant luminous but reversing pattern. The most sensitive indicator of abnormality of the visual evoked response was the latency of the major occipital positive peak. Virtually no difference was evident between the results from the two methods of pattern stimulation. Of only slightly less sensitivity was the latency of the major positive peak with diffuse-light stimulation, and this showed a prolonged latency in a greater number of patients when visual acuity was poor. Abnormalities of latency of the first negative peak and of amplitude were not related to the clinical expression of the multiple sclerosis, whether relapsing, progressive, or relapsing and progressive.

Adult↗
PubMed1980

Acute angle-closure glaucoma secondary to an aneurysm of the posterior communicating artery.

A 57-year-old woman developed pain behind and above her left eye. She had a partial oculomotor nerve paresis manifest by slight blepharoptosis and a dilated pupil. Both anterior chamber angles were narrow but the left was partially occluded. There was no photophobia or corneal edema. Though the moderately increased intraocular pressure was controlled by medication, pain persisted and the oculomotor nerve palsy became complete 12 hours later. Cerebral angiography was done. She had an aneurysm of the left posterior communicating artery. The aneurysm was treated by neurosurgical techniques and the oculomotor paralysis resolved within a few months except for misdirection in regeneration.

Acute Disease↗
PubMed1980

Cerebral blindness and oculomotor nerve palsies in toxoplasmosis.

We studied the clinicopathologic and neuro-ophthalmic manifestations of central nervous system toxoplasmosis in two patients who were being treated with immunosuppressive chemotherapy, one for renal transplantation and the other for systemic lupus erythematosis. Both pateints had oculomotor nerve palsies and later developed visual loss from cerebral involvement. Multifocal central nervous system toxoplasmosis appeared as a complication of their systemic diseases.

Adult↗
PubMed1979

Cerebral mucormycosis: an unusual case.

A 36-year-old obese woman with hyperglycemia and immunosuppression died of bilateral internal carotid artery occlusion associated with mucormycosis. This report describes a rare case in which cerebral mucormycosis occurred without the usual preceding clinical evidence of nasal and orbital infection.

Adult↗
PubMed1979

Extraocular muscle biopsy in chronic progressive external ophthalmoplegia.

A quantitatives assessment of the pathological changes in extraocular muscle is presented in 8 patients with chronic progressive external ophthalmoplegia (CPEO). Serial cross-sections of extraocular muscle were stained with a battery of histochemical and immunohistochemical techniques and compared with 36 normal extraocular muscles and 1 muscle from a patient who had longstanding third nerve plasy with anomalous reinnervation. Several of the patients had a striking increase in the number of ragged-red fibers in extraocular muscle, particularly if frequent ragged-red fibers also were found on limb muscle biopsy. One patients demonstrated extrajunctional acetylcholine receptor (AChR) in a small percentage of fibers, although this finding was not present in the reinnervated muscle. Numerous darkly staining central regions were noted in the ocular muscle fibers of a patient with Stephens syndrome (CPEO, peripheral neuropathy, and cerebellar disease) and in the reinnervated muscle. A patient with myotubular myopathy had single central nuclei in both limb and ocular muscle. All patients demonstrated in their extraocular muscles variation in both the size and distribution of each of the three histochemical fiber types. Extraocular muscle biopsy proved to be a safe, reliable technique. As a similar quantitative analysis is applied to the study of further patients, a better understanding of the pathogenesis of CPEO should be possible.

Adolescent↗
PubMed1979

Meningiomas confined to the optic canal and foramina.

We present two cases, each with a small meningioma arising from the sheath of an optic nerve in the canal. Both cases demonstrate the paucity of clinical and radiographic signs occurring with these tumors. The diagnosis must be strongly suspected when a middle aged white woman slowly and progressively loses vision in one eye in the absence of remission or other neurological findings. We review similar cases reported in the literature that have been proven by biopsy.

Adolescent↗
PubMed1978

Histochemistry of human extraocular muscle.

A reliable method for evaluating biopsy specimens of human extraocular muscles is presented to better understand the pathological responses of these highly organized striated muscles. Three muscle fiber types and their distribution are described with morphological and histochemical measurements used commonly for limb muscle. The granular and fine fibers have single end plates and may be comparable to limb-twitch fibers (type 2 and type 1 fibers). The coarse fibers have multiple end plates and may correspond to multiple end plated tonic fibers found in avian and amphibian limb muscles. The fibers of extraocular muscles are arranged in three concentric zones. Because of the zonal arrangement, a complete cross section should be evaluated in diseases of the ocular muscles to estimate any changes in fiber type distribution.

Adult↗