Dati normativi dei parametri tomografici OCT Spectral Domain in eta pediatrica.

RIASSUNTO Obiettivo: Riportare i valori normali dello spessore maculare, del volume maculare e dello strato delle fibre nervose (RNFL) dei bambini utilizzando un OCT a dominio spettrale. Metodi: Studio descrittivo in una popolazione preliminare di 94 bambini di età compresa tra i 5 e i 18 anni con anamnesi negativa per patologie sistemiche e locali e con equivalente sferico medio di 0,33 D±1,41 (range ±4 D, astigmatismo <1D). Sono state eseguite scansioni per lo studio maculare e per lo studio del nervo ottico. I dati sono stati analizzati in relazione a età, sesso ed equivalente sferico. Risultati: Valutando i dati in due gruppi (occhio destro e occhio sinistro) non sono state rilevate differenze significative per nessuno dei parametri analizzati (t-test: campioni indipendenti tutti i p>0,17) per cui si è scelto di analizzare l'occhio destro di tutti i pazienti. Lo spessore maculare centrale medio è risultato 274,97±18,28 micron, il volume retinico centrale medio 0,22±0,15 mm3, RFNL inferiore 124,11±16,49 μ, RNFL superiore 126,42±15,78 μ, RNFL nasale 69,96±13,19 μ e RNFL temporale 75,97±11,88 μ. Si è registrata una differenza significativa tra lo spessore maculare centrale e il sesso (t(92)= -2,65; p=0,009) e una correlazione lineare tra lo spessore maculare centrale e l'età dei pazienti (rho=0,37; p<0,001). Conclusioni: Lo studio riporta i valori standard di riferimento per lo spessore maculare, il volume maculare e l'RNFL in età pediatrica

Late spontaneous resolution of a double anterior chamber post deep anterior lamellar keratoplasty

A 31-year-old healthy male underwent deep anterior lamellar keratoplasty with big-bubble technique for treatment of keratoconus in his right eye. One week after surgery, he presented with detachment of the endothelium-Descemet complex with formation of a double anterior chamber, despite the apparent absence of an intraoperative Descemet membrane rupture. A subsequent intervention with the intent to relocate the corneal graft button was not effective, because the detachment appeared again one day later. The authors hypothesized that, at the time of the stromal dissection with big bubble technique, a small amount of air penetrated into the anterior chamber, creating a false pathway through the trabecular meshwork. The aqueous humor then penetrated the graft flowing through the false pathway, causing the endothelium-Descemet detachment. The persistence of that pathway, even after the intervention of graft repositioning, caused the failure of the latter procedure and persistence of the double chamber. We decided to wait and observe. The double anterior chamber spontaneously resolved in approximately three months

Reworsening of Recurrent Guillain-Barré Syndrome Triggered by COVID-19 Infection

Introduction: Guillain-Barré Syndrome (GBS) is an acute, immune-mediated, generalized polyradiculoneuropathy often triggered by a bacterial or viral infection, vaccination, or surgery. During the SARS-CoV-2 pandemic, some patients were reported with GBS associated COVID-19 infection. Case Presentation: We report, herein, a patient who had a recurrent GBS after forty years. Intravenous immunoglobulins (IVIg) induced improvement, but her condition worsened suddenly after twenty days, coinciding with a COVID-19 infection. A second IVIg cycle was administered, and she improved again. Conclusion: The take-home message is that in the current pandemic, any re-worsening or lack of improvement after appropriate treatment of GBS or possibly other autoimmune neurological diseases must be checked to determine if it is related to COVID-19 infection

Amniotic membrane application in complex cases of penetrating keratoplasty surgery

INTRODUCTION. The aim of the study is to investigate the ability of an amniotic membrane implant combined with penetrating keratoplasty to reduce early and mid-term complications in complex cases such as penetrating burns, infective ulcers, keratitis, or corneal graft failure. MATERIALS AND METHODS. Fifty patients: 12 with keratitis, 24 with infective ulcers, 10 with penetrating trauma, and four with a history of corneal graft failure, were divided into two groups. The first group of 25 patients (Group A) underwent penetrating keratoplasty alone, while the second group of 25 patients (Group B) received penetrating keratoplasty associated with an amniotic membrane implant. Amniotic membrane implantation was performed in a ‘patch modality’, and the membrane was sewn to the graft with the epithelium/basement membrane side facing inwards. All patients were evaluated, respectively, 3, 15, 30, 90, and 180 days after surgery. At each visit a slit-lamp examination was performed together with corneal thickness and endothelial cell count assessment. All the data were subjected to statistical analysis with Student’s t-test. RESULTS. At the slit-lamp examination in Group A 48% of patients showed Descemet folds at one and three months, respectively, while 80% of patients of Group B did not show any Descemet folds. In Group A we registered two cases of early graft failure and two cases of shallow anterior chamber, while none of these complications appeared in Group B. In Group A the average corneal thickness at 15, 30, 90, and 180 days post-operatively was, respectively, 695 ± 43 μm, 658 ± 31 μm, 588 ± 12 μm, and 518 ± 20 μm, while in Group B it was found to be, respectively, 667 ± 12 μm, 632 ± 17 μm, 562 ± 16 μm, and 516 ± 10 μm. Differences in corneal thickness between Group A and Group B were found to be statistically significant (p = 0.05). Endothelial cell count was in Group A 2582 c/mm2 at 15 days, 2500 c/mm2 at one month, 2335 c/mm2 at three months, and 2111 c/mm2 at six months, while the average count for Group B patients was 2607 c/mm2, 2503 c/mm2, 2299 c/mm2, and 2086 c/mm2, respectively. Differences in endothelial cell count between the two groups did not show any statistical significance (p &gt; 0.05). CONCLUSIONS. Amniotic membrane implantation associated with penetrating keratoplasty reduces early and mid-term complications of corneal grafts in patients with high risk of failure. This positive effect may be due to the anti-inflammatory, neurotrophic, and anti-angiogenetic properties of the amniotic membrane. Longer observation and larger case series can be useful in evaluation of the functional outcome of grafts in these patients

A new counterintuitive training for adult amblyopia

International audienceObjectives: The aim of this study was to investigate whether short-term inverse occlusion, combined with moderate physical exercise, could promote the recovery of visual acuity and stereopsis in a group of adult anisometropic amblyopes. Methods: Ten adult anisometropic patients underwent six brief (2 h) training sessions over a period of 4 weeks. Each training session consisted in the occlu-sion of the amblyopic eye combined with physical exercise (intermittent cycling on a stationary bike). Visual acuity (measured with ETDRS charts), stereoacuity (measured with the TNO test), and sensory eye dominance (measured with binocular rivalry) were tested before and after each training session, as well as in follow-up visits performed 1 month, 3 months, and 1 year after the end of the training. Results: After six brief (2 h) training sessions, visual acuity improved in all 10 patients (0.15 AE 0.02 LogMar), and six of them also recovered stereopsis. The improvement was preserved for up to 1 year after training. A pilot experiment suggested that physical activity might play an important role for the recovery of visual acuity and stereopsis. Conclusions: Our results suggest a noninvasive training strategy for adult human amblyopia based on an inverse-occlusion procedure combined with physical exercise

Health-related quality of life and functional changes in DMD:A 12-month longitudinal cohort study

Family caregivers of people with amyotrophic lateral sclerosis (ALS) live stressful lives in which they spend most of their time caring for their loved ones and managing difficult situations, thereby reducing the time spent in taking care of themselves. This situation may last several years. Previous literature has widely highlighted that this situation reduces caregivers' quality of life and increases their psychological distress and risk of health problems, but there is a lack of studies that focus on psychological interventions for these situations. This qualitative study examined a pilot experience of two mutual support groups for family caregivers of people with ALS. The aim was to identify caregivers' needs, the prominent aspects of their experience, and to understand whether and how this intervention strategy might help them. Six partners (four men and two women) and six adult children (five women and one man) participated in the groups, which were conducted in northern Italy. After the support groups finished, participants underwent semi-structured interviews. The authors conducted a content analysis of the transcripts of the interviews and the 20 group sessions. The thematic areas identified were "caregiving," "being the son/daughter of a person with ALS," "being the partner of a person with ALS," "group experience" and "group evaluation." The caregiving experience was profoundly different depending on whether the caregiver was a son/daughter or a partner of a patient with ALS. Moreover, comparison with peers and mutual support helped participants to better cope with ALS and its consequences, to improve their care for their relatives and to overcome typical caregiver isolation. These results suggest the usefulness of involving communities in caregiver support in order to create new networks and activate personal and social resources for well-being

Amniotic membrane application in complex cases of penetrating keratoplasty surgery

INTRODUCTION. The aim of the study is to investigate the ability of an amniotic membrane implant combined with penetrating keratoplasty to reduce early and mid-term complications in complex cases such as penetrating burns, infective ulcers, keratitis, or corneal graft failure. MATERIALS AND METHODS. Fifty patients: 12 with keratitis, 24 with infective ulcers, 10 with penetrating trauma, and four with a history of corneal graft failure, were divided into two groups. The first group of 25 patients (Group A) underwent penetrating keratoplasty alone, while the second group of 25 patients (Group B) received penetrating keratoplasty associated with an amniotic membrane implant. Amniotic membrane implantation was performed in a 'patch modality', and the membrane was sewn to the graft with the epithelium/basement membrane side facing inwards. All patients were evaluated, respectively, 3, 15, 30, 90, and 180 days after surgery. At each visit a slit-lamp examination was performed together with corneal thickness and endothelial cell count assessment. All the data were subjected to statistical analysis with Student's t-test. RESULTS. At the slit-lamp examination in Group A 48% of patients showed Descemet folds at one and three months, respectively, while 80% of patients of Group B did not show any Descemet folds. In Group A we registered two cases of early graft failure and two cases of shallow anterior chamber, while none of these complications appeared in Group B. In Group A the average corneal thickness at 15, 30, 90, and 180 days post-operatively was, respectively, 695 ± 43 μm, 658 ± 31 μm, 588 ± 12 μm, and 518 ± 20 μm, while in Group B it was found to be, respectively, 667 ± 12 μm, 632 ± 17 μm, 562 ± 16 μm, and 516 ± 10 μm. Differences in corneal thickness between Group A and Group B were found to be statistically significant (p = 0.05). Endothelial cell count was in Group A 2582 c/mm2 at 15 days, 2500 c/mm2 at one month, 2335 c/mm2 at three months, and 2111 c/mm2 at six months, while the average count for Group B patients was 2607 c/mm2, 2503 c/mm2, 2299 c/mm2, and 2086 c/mm2, respectively. Differences in endothelial cell count between the two groups did not show any statistical significance (p > 0.05). CONCLUSIONS. Amniotic membrane implantation associated with penetrating keratoplasty reduces early and mid-term complications of corneal grafts in patients with high risk of failure. This positive effect may be due to the anti-inflammatory, neurotrophic, and anti-angiogenetic properties of the amniotic membrane. Longer observation and larger case series can be useful in evaluation of the functional outcome of grafts in these patients

Benefits of glucocorticoids in non-ambulant boys/men with Duchenne muscular dystrophy: A multicentric longitudinal study using the Performance of Upper Limb test

The aim of this study was to establish the possible effect of glucocorticoid treatment on upper limb function in a cohort of 91 non-ambulant DMD boys and adults of age between 11 and 26 years. All 91 were assessed using the Performance of Upper Limb test. Forty-eight were still on glucocorticoid after loss of ambulation, 25 stopped steroids at the time they lost ambulation and 18 were GC naive or had steroids while ambulant for less than a year. At baseline the total scores ranged between 0 and 74 (mean 41.20). The mean total scores were 47.92 in the glucocorticoid group, 36 in those who stopped at loss of ambulation and 30.5 in the naive group (p <0.001). The 12-month changes ranged between -20 and 4 (mean -4.4). The mean changes were -3.79 in the glucocorticoid group, -5.52 in those who stopped at loss of ambulation and -4.44 in the naive group. This was more obvious in the patients between 12 and 18 years and at shoulder and elbow levels. Our findings suggest that continuing glucocorticoids throughout teenage years and adulthood after loss of ambulation appears to have a beneficial effect on upper limb function. (C) 2015 The Authors. Published by Elsevier B.V

Laminin α2 controls mouse and human stem cell behaviour during midbrain dopaminergic neuron development

Development of the central nervous system requires coordination of the proliferation and differentiation of neural stem cells. Here, we show that laminin alpha 2 (lm-α2) is a component of the midbrain dopaminergic neuron (mDA) progenitor niche in the ventral midbrain (VM) and identify a concentration-dependent role for laminin α2β1γ1 (lm211) in regulating mDA progenitor proliferation and survival via a distinct set of receptors. At high concentrations, lm211-rich environments maintain mDA progenitors in a proliferative state via integrins α6β1 and α7β1, whereas low concentrations of lm211 support mDA lineage survival via dystroglycan receptors. We confirmed our findings in vivo, demonstrating that the VM was smaller in the absence of lm-α2, with increased apoptosis; furthermore, the progenitor pool was depleted through premature differentiation, resulting in fewer mDA neurons. Examination of mDA neuron subtype composition showed a reduction in later-born mDA neurons of the ventral tegmental area, which control a range of cognitive behaviours. Our results identify a novel role for laminin in neural development and provide a possible mechanism for autism-like behaviours and the brainstem hypoplasia seen in some individuals with mutations of LAMA2