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Biomedical subjects

R Restuccia

Publications and source records attributed to R Restuccia.

8 recordsLinked to original sources
PubMed2003

Early visual function impairment in CADASIL.

The authors carried out genetic analyses and visual electrophysiologic evaluations in six asymptomatic sons and daughters of patients with symptomatic cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL). Three subjects showed Notch3 Cys146Tyr missense mutation and a dysfunction of the outer, middle, and innermost retinal layers, with normal neural conduction in postretinal visual pathways, whereas in the remaining subjects without genetic mutations, no electrophysiologic abnormalities were found. An early vascular retinal impairment in CADASIL may precede the onset of clinical manifestations.

Adolescent↗
PubMed2001

Morphological and functional retinal impairment in Alzheimer's disease patients.

OBJECTIVE: Our study aims to assess the optic nerve fiber layer thickness in vivo, the function of the innermost retinal layer and whether a correlation exists between morphological and functional parameters in patients affected by Alzheimer's Disease (AD). METHODS: Seventeen AD patients (mean age 70.37+/-6.1 years, best corrected visual acuity >8/10 with refractive error between +/-3 sf, intra-ocular pressure (IOP)<18 mmHg) were enrolled. They were compared to 14 age-matched controls. Nerve fiber layer (NFL) thickness was measured by optical coherence tomography (OCT). Three different measurements in each quadrant (superior, inferior, nasal, and temporal) were taken and averaged. The data in all quadrants (12 values averaged) were identified as NFL Overall. Retinal function was assessed by pattern electroretinogram (PERG) recordings using high-contrast (80%) checkerboard stimuli subtending 15 min of the visual arc and reversed at the rate of two reversals/s. RESULTS: In AD eyes, there was a significant (P<0.01) reduction in NFL thickness in each quadrant and in the NFL Overall evaluation compared with the values observed in control eyes. PERGs showed a significant (P<0.01) delay in N35, P50 and N95 implicit times, and reduction in N35-P50 and P50-N95 amplitudes. NFL Overall values were significantly correlated (P<0.01) to the PERG P50 and N95 implicit times and P50-N95 amplitude. No correlations (P>0.01) between NFL values and other PERG parameters (N35 implicit time, N35-P50 amplitude) were found. CONCLUSIONS: Our results suggest that in AD patients, there is a reduction of NFL thickness evaluated in vivo by OCT and this morphological abnormality is related to a retinal dysfunction as revealed by abnormal PERG responses.

Aged↗
PubMed2000

Visual electrophysiological responses in subjects with cerebral autosomal arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL).

OBJECTIVES: To evaluate visual electrophysiological responses in subjects with cerebral autosomal arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL). METHODS: Three subjects (one male and two females, mean age 55.3+/-2.9 years) belonging to an Italian family already diagnosed with CADASIL through clinicopathological and genetic studies and 14 control subjects (6 males and 8 females, mean age 52.7+/-3.6 years) were enrolled in the study. Flash electroretinogram (ERG), oscillatory potentials (OPs) and simultaneous recordings of pattern electroretinogram (PERG) and visual evoked potentials (VEPs) were assessed in all 3 subjects with CADASIL and age-matched controls. RESULTS: Subjects with CADASIL showed: reduced ERG, OP and PERG (N35-P50, P50-N95) amplitudes with respect to our normal limits; delayed PERG (N35, P50) and VEP (P100) implicit times when compared with our normal limits; and VEP (N75-P100) amplitudes and retinocortical times within our normal limits. CONCLUSIONS: Subjects with CADASIL present a dysfunction in the outer, middle and innermost retinal layers when the index of neural conduction in the postretinal visual pathways is normal. The delay in visual cortical responses observed in subjects with CADASIL may be ascribable to retinal impairment with a possible functional sparing of the postretinal visual structures.

Dementia, Multi-Infarct↗
PubMed1999

Correlation between morphological and functional retinal impairment in multiple sclerosis patients.

PURPOSE: To assess whether a correlation exists between optic nerve fiber layer (NFL) thickness and the retinal or visual pathway function in multiple sclerosis (MS) patients previously affected by optic neuritis. METHODS: Fourteen patients with a diagnosis of definite MS were examined. All had been affected by optic neuritis (MSON) with complete recovery of visual acuity (14 eyes included in study). These were compared with 14 eyes from 14 age-matched control subjects. NFL thickness was measured by optical coherence tomography (OCT). Three different measurements in each quadrant (superior, inferior, nasal, and temporal) were taken and averaged. The data in all quadrants (12 values averaged) were identified as NFL Overall, whereas the data obtained in the temporal quadrant only (3 values averaged) were identified as NFL Temporal. Retinal and visual pathway function was assessed by simultaneously recording pattern electroretinograms (PERGs) and visual evoked potentials (VEPs) using high-contrast (80%) checkerboard stimuli subtending 15 minutes and 60 minutes of the visual arc (min arc) and reversed at the rate of two reversals per second. RESULTS: In MSON eyes there was a significant (P < 0.01) reduction in NFL thickness in both NFL Overall and NFL Temporal evaluations compared with the values observed in control eyes. PERG, (15-min arc checks) and VEP (15-min arc and 60-min arc checks), showed a significant (P < 0.01) delay in latency and reduction in amplitude. NFL Overall and NFL Temporal values were significantly correlated (P < 0.01) to the PERG P50 latency and P50 to N95 amplitude recorded with 15-min arc checks. No correlations (P > 0.01) between NFL values and the other electrophysiological data (PERG recorded with 60-min arc checks and VEP recorded with 15-min arc and 60-min arc checks) were found. CONCLUSIONS: There is a correlation between PERG changes and NFL thickness in MS patients previously affected by optic neuritis, but there is no correlation between VEP changes and NFL thickness.

Adult↗
PubMed1998

Impaired VEP after photostress response in multiple sclerosis patients previously affected by optic neuritis.

The aim of our work was to evaluate if an optic nerve involvement (multiple sclerosis patients previously affected by optic neuritis) may induce any change in visual evoked potential (VEP) after photostress response. VEP in basal conditions and after photostress were assessed in 10 patients with defined multiple sclerosis without a history of optic neuritis (MSWO); in 14 patients with defined multiple sclerosis previously affected by optic neuritis but with complete recovery of the visual acuity (MSON) and in 14 age-matched controls. In order to complete the investigation of the retinal function, Transient Pattern electroretinogram (PERG) and steady-state focal-ERG (counterphased gratings presented at 8 Hz in the macular region) were performed in MSON patients only. In MSWO eyes VEP parameters in basal condition and after photostress did not undergo significant changes compared to controls (ANOVA; P > 0.05). In MSON eyes we observed basal VEP with delayed P100 peak latency and reduced N75-P100 amplitude when compared with the control ones (P < 0.01). In MSON eyes the parameters of VEP after photostress underwent large changes and longer recovery time (RT) than in control and MSWO eyes (P < 0.01). In addition; in MSON eyes we found increased transient PERG P50 latency (P < 0.01) and reduced P50-N95 amplitude (P < 0.01); Focal-ERG (that displays a major component at 16 Hz; 2nd harmonic:2P) with reduced 2P amplitudes and delayed 2P phases (P < 0.01). Our results indicate that patients previously affected by optic neuritis present an abnormal VEP after photostress response and this may be ascribed predominantly to an involvement of the inner retinal layers as indicated by the concomitant impairment of PERG and focal-ERG responses.

Adult↗
PubMed1996

The corporate practice of health care ... a panel discussion.

The pros and cons of treating health care as a profit-making business got a lively airing in Boston May 16, when the Harvard School of Public Health's "Second Conference on Strategic Alliances in the Evolving Health Care Market" presented what was billed as a "Socratic panel." The moderator was Charles R. Nesson, J.D., a Harvard Law School professor of 30 years' standing whose knack for guiding lively discussions is well known to viewers of such Public Broadcasting Service series as "The Constitution: That Delicate Balance. "As one panelist mentioned, Boston was an interesting place for this conversation. With a large and eminent medical establishment consisting mostly of traditionally not-for-profit institutions, the metropolis of the only state carried in 1972 by liberal Presidential candidate George McGovern is in one sense a skeptical holdout against the wave of aggressive investment capitalism that has been sweeping the health care industry since the 1994 failure of the Clinton health plan. In another sense, though, managed care-heavy Boston is an innovative crucible of change, just like its dominant HMO, the not-for-profit but merger-minded Harvard Pilgrim Health Care. Both of these facets of Beantown's health care psychology could be discerned in the comments heard during the panel discussion. With the permission of the Harvard School of Public Health--and asking due indulgence for the limitations of tape-recording technology in a room often buzzing with amateur comment--MANAGED CARE is pleased to present selections from the discussion in the hope that they will shed light on the business of health care.

Community-Institutional Relations↗
PubMed1982

Serial changes in pulmonary functions in children hospitalized with cystic fibrosis.

Pulmonary functions were serially measured in 17 children hospitalized with cystic fibrosis (CF) to determine how much and when pulmonary functions improved during 14 days of inpatient treatment. Absolute lung volumes and forced expiratory flows were recorded every other day while patients received chest physiotherapy, antibiotics, and bronchodilators. Vital capacity first improved after the fifth hospital day coincidentally with a reduction in residual volume and an increase in peak expiratory flow rate. Significant improvements in other pulmonary functions developed later in the hospitalization. Improvement in most pulmonary functions continued throughout the hospitalization and was maintained 2 wk after discharge. Improvement was less and uniformly developed later in those children with severe lung disease on admission (NIH score less than 50) compared with those with moderate respiratory involvement (NIH score greater than 50). Pulmonary function measurements may prove useful in determining the optimal duration of treatment for patients who are hospitalized with CF.

Adolescent↗