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1.
JAMA Neurol ; 78(6): 678-686, 2021 06 01.
Article in English | MEDLINE | ID: mdl-33900360

ABSTRACT

Importance: Idiopathic intracranial hypertension (IIH) causes headaches, vision loss, and reduced quality of life. Sustained weight loss among patients with IIH is necessary to modify the disease and prevent relapse. Objective: To compare the effectiveness of bariatric surgery with that of a community weight management (CWM) intervention for the treatment of patients with active IIH. Design, Setting, and Participants: This 5-year randomized clinical trial (Idiopathic Intracranial Hypertension Weight Trial) enrolled women with active IIH and a body mass index (calculated as weight in kilograms divided by height in meters squared) of 35 or higher at 5 National Health Service hospitals in the UK between March 1, 2014, and May 25, 2017. Of 74 women assessed for eligibility, 6 did not meet study criteria and 2 declined to participate; 66 women were randomized. Data were analyzed from November 1, 2018, to May 14, 2020. Interventions: Bariatric surgery (n = 33) or CWM intervention (Weight Watchers) (n = 33). Main Outcomes and Measures: The primary outcome was change in intracranial pressure measured by lumbar puncture opening pressure at 12 months, as assessed in an intention-to-treat analysis. Secondary outcomes included lumbar puncture opening pressure at 24 months as well as visual acuity, contrast sensitivity, perimetric mean deviation, and quality of life (measured by the 36-item Short Form Health Survey) at 12 and 24 months. Because the difference in continuous outcomes between groups is presented, the null effect was at 0. Results: Of the 66 female participants (mean [SD] age, 32.0 [7.8] years), 64 (97.0%) remained in the clinical trial at 12 months and 54 women (81.8%) were included in the primary outcome analysis. Intracranial pressure was significantly lower in the bariatric surgery arm at 12 months (adjusted mean [SE] difference, -6.0 [1.8] cm cerebrospinal fluid [CSF]; 95% CI, -9.5 to -2.4 cm CSF; P = .001) and at 24 months (adjusted mean [SE] difference, -8.2 [2.0] cm CSF; 95% CI, -12.2 to -4.2 cm CSF; P < .001) compared with the CWM arm. In the per protocol analysis, intracranial pressure was significantly lower in the bariatric surgery arm at 12 months (adjusted mean [SE] difference, -7.2 [1.8] cm CSF; 95% CI, -10.6 to -3.7 cm CSF; P < .001) and at 24 months (adjusted mean [SE] difference, -8.7 [2.0] cm CSF; 95% CI, -12.7 to -4.8 cm CSF; P < .001). Weight was significantly lower in the bariatric surgery arm at 12 months (adjusted mean [SE] difference, -21.4 [5.4] kg; 95% CI, -32.1 to -10.7 kg; P < .001) and at 24 months (adjusted mean [SE] difference, -26.6 [5.6] kg; 95% CI, -37.5 to -15.7 kg; P < .001). Quality of life was significantly improved at 12 months (adjusted mean [SE] difference, 7.3 [3.6]; 95% CI, 0.2-14.4; P = .04) and 24 months (adjusted mean [SE] difference, 10.4 [3.8]; 95% CI, 3.0-17.9; P = .006) in the bariatric surgery arm. Conclusions and Relevance: In this randomized clinical trial, bariatric surgery was superior to a CWM intervention in lowering intracranial pressure. The continued improvement over the course of 2 years shows the impact of this intervention with regard to sustained disease remission. Trial Registration: ClinicalTrials.gov Identifier: NCT02124486.


Subject(s)
Bariatric Surgery/trends , Body Mass Index , Intracranial Pressure/physiology , Pseudotumor Cerebri/diagnosis , Pseudotumor Cerebri/therapy , Weight Reduction Programs/trends , Adult , Female , Humans , Pseudotumor Cerebri/epidemiology , Treatment Outcome , Weight Loss/physiology , Young Adult
4.
Neurogenetics ; 16(1): 69-75, 2015 Jan.
Article in English | MEDLINE | ID: mdl-25159689

ABSTRACT

Leber hereditary optic neuropathy and autosomal dominant optic atrophy are the two most common inherited optic neuropathies. The latter has been associated with mutations in the OPA1 and OPA3 genes. To date, only six families with OPA3-associated dominant optic atrophy have been reported. In order to identify additional families, we performed Sanger sequencing of the OPA3 gene in 75 unrelated optic neuropathy patients. Affected individuals from two families were found to harbour the c.313C > G, p.(Gln105Glu) change in heterozygous state; this genetic defect has been previously reported in four dominant optic atrophy families. Intra- and interfamilial variability in age of onset and presenting symptoms was observed. Although dominant OPA3 mutations are typically associated with optic atrophy and cataracts, the former can be observed in isolation; we report a case with no lens opacities at age 38. Conversely, it is important to consider OPA3-related disease in individuals with bilateral infantile-onset cataracts and to assess optic nerve health in those whose vision fail to improve following lens surgery. The papillomacular bundle is primarily affected and vision is typically worse than 20/40. Notably, we describe one subject who retained normal acuities into the fifth decade of life. The condition can be associated with extraocular clinical features: two affected individuals in the present study had sensorineural hearing loss. The clinical heterogeneity observed in the individuals reported here (all having the same genetic defect in OPA3) suggests that the molecular pathology of the disorder is likely to be complex.


Subject(s)
Mutation , Optic Atrophy, Autosomal Dominant/diagnosis , Optic Atrophy, Autosomal Dominant/genetics , Proteins/genetics , Adult , Aged , DNA Mutational Analysis , Female , Genes, Dominant , Humans , Male , Middle Aged , Optic Disk/pathology , Pedigree , Visual Acuity/genetics , Young Adult
5.
Clin Kidney J ; 7(4): 387-90, 2014 Aug.
Article in English | MEDLINE | ID: mdl-25852914

ABSTRACT

Hypotension is a commonly encountered complication in haemodialysis patients and is a significant cause of morbidity and mortality. Bilateral visual loss in dialysis induced hypotension remains poorly recognized as a complication by both renal physicians and ophthalmologists. We report 2 cases of patients on renal dialysis who suffered severe longstanding hypotension with bilateral non-arteritic anterior ischaemic optic neuropathy. Both patients experienced bilateral loss of vision over a short time period. We feel that physicians must be aware of patients complaining of painless visual loss in this high risk group, as control of blood pressure may be the most important factor in prevention of this visually devastating condition.

6.
Neuroophthalmology ; 38(5): 278-280, 2014.
Article in English | MEDLINE | ID: mdl-27928314

ABSTRACT

Neurosarcoidosis is seen in 5-15% of patients with systemic sarcoidosis. The most common cranial nerve presentations are optic neuropathy and facial nerve palsy. The authors present a case of sarcoidosis presenting with a pupil-involving third nerve palsy. The patient responded to corticosteroid therapy with resolution of investigations her cranial nerve palsy but progressed to develop cerebellar signs. This is the first documented case of a pupil-involving third nerve palsy occurring as the first presentation of neurosarcoidosis. Although typically a pupil-involving third nerve palsy necessitates urgent neuroimaging to rule out a posterior communicating artery aneurysm, it is important to recognise inflammatory causes in the differential diagnosis.

7.
Strabismus ; 17(1): 41-4, 2009.
Article in English | MEDLINE | ID: mdl-19301193

ABSTRACT

This is a retrospective study of the outcome of all consecutive exotropia surgery performed between the years 1999 and 2007. Excluded were cases of slipped muscles, muscle transpositions, and concurrent cyclovertical procedures. A total of 135 patients of mean age 40 years (range 6-72) were operated with a mean preoperative angle of deviation -40Delta (range -12 to -90). Five were treated with fixed sutures and the rest with adjustables. The surgery included preplanned lateral rectus recessions of mean 6 mm (range 2-9) and maximal possible medial rectus advancements with or without resections of mean 5.6 mm (range 2-11), intraoperatively adjusted to give a resistance of +1 to +2 for abduction on forced duction test (FDT). Adjustable suture patients were seen 4 to 6 hours after surgery and underwent further adjustment, if needed, to achieve the target angle. A further review took place at 2 weeks and 2 months. Children remained under follow-up till 8 years of age. Forty percent needed postoperative adjustment. The mean postoperative angle at final measurement was -2Delta (range +25 to -45). Eighty percent achieved an angle within 10Delta of orthotropia. No gross restrictions of ductions were present postoperatively.


Subject(s)
Diagnostic Techniques, Ophthalmological , Exotropia/surgery , Intraoperative Care , Oculomotor Muscles/surgery , Suture Techniques , Adolescent , Adult , Aged , Child , Humans , Middle Aged , Postoperative Period , Retrospective Studies , Treatment Outcome , Young Adult
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