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Biomedical subjects

W V Good

Publications and source records attributed to W V Good.

68 records · Page 4Linked to original sources
PubMed1991

Bilateral congenital oculomotor nerve palsy in a child with brain anomalies.

We treated a 3-month-old boy with bilateral congenital oculomotor nerve palsy in whom a magnetic resonance imaging scan demonstrated a developmental brain anomaly in the region of the basal ganglia. The pupil was normal on one side, and there was no aberrant regeneration of the oculomotor nerve. We could find no evidence for a peripheral oculomotor nerve lesion. This demonstrates that congenital oculomotor nerve palsy can be caused by brainstem disease. Embryologically, basal ganglia and oculomotor nuclei develop at the same time, and the Edinger-Westphal nucleus develops later. Thus, pupil sparing does not exclude a central origin for congenital oculomotor nerve palsy.

Basal Ganglia↗
PubMedDate not supplied

Aicardi syndrome: more than meets the eye.

An eight-month-old girl with infantile spasms and apparent blindness had electroencephalographic findings compatible with Aicardi syndrome. In addition to optic nerve hypoplasia, there were multiple congenital retinal malformations in the right eye, including chorioretinal lacunae, anomalous retinal vessels, posterior scleral ectasia, and a peripheral fibrous ridge. Magnetic resonance imaging demonstrated agenesis of the corpus callosum, absence of the septum pellucidum, optic nerve and chiasmal hypoplasia, pachygyria, cortical heterotopias, colpocephaly, and hypoplasia of the cerebellar vermis. This patient illustrates the broad spectrum of cerebroretinal malformations now known to characterize Aicardi syndrome.

Abnormalities, Multiple↗
PubMedDate not supplied

Cortical visual impairment in children.

Cortical visual impairment (CVI) in children is most commonly caused by peri- or post-natal hypoxia-ischemia, but may also occur following other insults, e.g., trauma, epilepsy, infections, drugs or poisons, and certain neurologic diseases. The disorder differs considerably in etiology, physical findings, and, perhaps, prognosis, from the cortical blindness seen in adults. The same event that causes CVI by damaging the geniculate and/or extrageniculate visual pathways may also damage other areas of the brain, or the retina, optic nerves, or chiasm. Thus, children with CVI often have other neurological problems. Diagnosis may require the participation of a multidisciplinary team and the use of special visual testing techniques. Due to the uncertainty concerning the prognosis in CVI, clinicians should remain optimistic about the child's potential for some vision recovery.

Brain Damage, Chronic↗
PubMedDate not supplied

Optic nerve shadow enlargement in the Klippel-Trenaunay-Weber syndrome.

A 10-year-old girl with Klippel-Trenaunay-Weber syndrome (KTWS) showed neuroradiologic signs of enlarged optic nerve and medial rectus shadows. In 3 years of follow-up, her vision has remained normal. This is the first report of optic nerve enlargement in this syndrome. The case demonstrates that enlargement of the optic nerve shadows is not inevitably associated with visual deterioration. Optic nerve enlargement may be part of a hamartomatous process that involves other orbital contents.

Angiomatosis↗
PubMedDate not supplied

Postoperative endophthalmitis in children following cataract surgery.

Three children developed endophthalmitis after cataract surgery. Each had signs and symptoms of either nasolacrimal duct obstruction or upper respiratory infection at the time of surgery. The causative organisms were Staphylococcus aureus, Staphylococcus epidermidis, and Streptococcus pneumoniae. Final visual acuities were 6/24, LP, and NLP, respectively. Endophthalmitis after cataract surgery in infants has never been reported. These three cases drawn from two pediatric ophthalmology practices represent an incidence of postoperative endophthalmitis of 0.45%. Although this incidence report is potentially spurious, it indicates that postoperative endophthalmitis is a very real threat in infants. We recommend a thorough systems review and exam of upper airways and lacrimal system before undertaking intraocular surgery in young children. We also caution against simultaneous bilateral surgery.

Anti-Bacterial Agents↗
PubMedDate not supplied

Mitochondrial disorders: analysis of their clinical and imaging characteristics.

PURPOSE: Investigation of the clinical, imaging, and in vivo MR spectroscopy (MRS) characteristics of disorders of mitochondrial function. METHODS: Clinical, imaging (five CT and 20 MR examinations), and MRS (six studies in five patients) findings in 19 patients with mitochondrial disorders were retrospectively reviewed. Results were critically analyzed and, when applicable, compared with results in the literature. RESULTS: Patients included four with mitochondrial encephalopathy with lactic acidosis and stroke-like episodes (MELAS), two with myoclonus, epilepsy, and ragged red fibers (MERRF), two with Kearns-Sayre syndrome, seven with Leigh syndrome, one with progressive cerebral poliodystrophy (Alpers syndrome), and three with trichopoliodystrophy (Menkes disease). MELAS, MERRF, and Kearns-Sayre tended to occur in older children and adults, whereas Leigh syndrome, Alpers syndrome, and Menkes disease occurred in infants and young children. All diseases involved gray matter early in their course, manifest primarily as T2 prolongation, with the deep cerebral nuclei being involved more often than the cerebral cortex. When T2 prolongation was seen in the white matter (MELAS, MERRF, Kearns-Sayre, Leigh), the peripheral and retrotrigonal white matter showed early involvement. Patients with Menkes disease showed rapidly progressive atrophy accompanied by large subdural hematomas. Proton MRS showed an elevated lactate level in involved regions of the brain; the lactate peak disappeared in old areas of T2 prolongation. CONCLUSIONS: Mitochondrial disorders have a wide range of both clinical and imaging findings. Although no one set of findings is diagnostic of these disorders, the combination of deep gray matter involvement and peripheral white matter involvement in young adults or children should suggest the diagnosis, especially when associated with an elevated lactate level on proton MRS.

Adolescent↗