Metabolic, pediatric, and systemic ophthalmology (New York, N.Y. : 1985)最新文献

A 25-year-old-healthy man, with previous history of parenteral drug abuse, developed a left homonymous hemianopsia. Computed tomographic scanning showed a single ring enhancing lesion in the right parieto-occipital area. After the demonstration of seropositivity for human immunodeficiency virus and high serum immunoglobulin G antibody titer against Toxoplasma gondii, a diagnosis of cerebral toxoplasmosis in an AIDS patient was made. An isolated visual field defect revealing cerebral toxoplasmosis may be the first presentation of AIDS. Opportunistic cerebral lesions should be always considered in subjects at risk for AIDS who present focal neurological signs.

We recorded flash electroretinograms (ERGs), flash visual evoked potentials (VEPs), pattern ERGs and pattern VEPs in two siblings with adult form galactosialidosis. The amplitude of b-wave was decreased in flash ERGs. The peak latency of flash VEPs was within normal range. The pattern ERGs and VEPs were nonrecordable. These results suggested the damage of retinal ganglion cell and inner nuclear layer in these cases.

Since some authors have considered useless a differentiation between hemicentral retinal vein occlusion (HCRVO) and hemispheric retinal vein occlusion (HSRVO), we have conducted a prospective research in order to evaluate the clinical and prognostic features of these diseases. We have followed prospectively 26 cases of HCRVO and 25 cases of HSRVO. The most important risk factors were hypertension, diabetes mellitus and glaucoma in HCRVO, and hypertension in HSRVO. In the HCRVO group 20 cases (76.9%) were of the non-ischemic type and six cases (23.1%) were ischemic-type, whereas in the HSRVO seven (28%) were non-ischemic type and 18 cases (72%) were ischemic-type. Our results demonstrate that the two retinal vein occlusions are quite different with regards to pathogenesis, clinical evolution and visual outcome and point out the necessity to achieve a precise diagnosis.

A delayed or immature transmission dynamics of an otherwise diffuse mesolimbic catecholamine circuit is suggested to be the reason behind lack of pupillary activity and reflex often observed in infants less than one year of age. This deficiency is known to be fully compensated in later years following a growing curve of neuronal metabolic change and as well as a curve for synaptoogenesis.

Type B Niemann-Pick Disease (NPB) is a rare lysosomal storage disease resulting from diminished activity or deficiency of sphingomyelinase and is characterized by multi-system involvement with visceromegaly. Rare ocular involvement (the Macula Halo Syndrome) has been reported. Eight patients (ages 4-36) with NPB underwent complete ophthalmologic evaluations. All patients had periorbital fullness, a hitherto unreported clinical feature. Two patients had a classic Macula Halo Syndrome. One patient developed peri-macular granular deposits forming an incomplete Macula Halo over 5 years. Another patient had macular granular deposits and developed deterioration of central vision and abnormal visual evoked potentials. Ophthalmologic involvement in NPB is more common than previously described. Complete ophthalmologic evaluation is recommended in all patients suspected to have NPB.

Whole blood viscosity (WBV) and haematocrit (Hct) were measured in 8 very low birth weight premature neonates developing acute severe retinopathy of prematurity (ROP) and 7 control neonates without retinopathy or mild ROP. Single samples were collected at 32-34 weeks conceptual age. There was no statistically significant difference in WBV or Hct between the two groups. The results of this preliminary report suggest that WBV does not appear to play a major role in the development of advanced ROP, though larger studies are required to investigate this further.

The activity of galactokinase in red blood cells of 30 patients affected by idiophatic presenile and senile cataract, and of 20 age-matched controls with perfectly transparent lenses, was investigated. The results obtained show a partial deficiency of the galactokinase activity in the patients affected by presenile idiopathic cataract. There is, moreover, a significant relationship between the cortical form of cataract and enzymatic reduction.

A 41 year old male is presented who suffers from both advanced retinitis pigmentosa and active discoid lupus erthematosus. A possible association between the two pigmenting disorders is discussed as well as the treatment of the discoid lupus with potentially retinotoxic hydroxychloroquine.

Retinoblastoma is the most common intraocular malignancy of childhood. Trilateral retinoblastoma is a syndrome characterized by bilateral ocular retinoblastoma in conjunction with an intracranial neuroblastic neoplasm in the pineal body, or in supraseller or parasellar location. We report the case of a three year old girl with trilateral retinoblastoma and review the literature on this subject.