Aimee Szewka

Idiopathic Intracranial Hypertension: Relation Between Obesity and Visual Outcomes

Background:Increased body mass index (BMI) has been associated with increased risk of idiopathic intracranial hypertension (IIH), but the relationship of BMI to visual outcomes in IIH is unclear. Methods:A retrospective chart review of all adult cases of IIH satisfying the modified Dandy criteria seen at our institution between 1989 and 2010 was performed. Demographics, diagnostic evaluations, baseline visit and last follow-up examination data, treatment, and visual outcome data were collected in a standardized fashion. Groups were compared, and logistic regression was used to evaluate the relationship of BMI to severe visual loss, evaluating for interaction and controlling for potential confounders. Results:Among 414 consecutive IIH patients, 158 had BMI ≥40 (World Health Organization Obese Class III) and 172 had BMI 30–39.9. Patients with BMI ≥40 were more likely to have severe papilledema at first neuro-ophthalmology encounter than those with a lower BMI (P = 0.02). There was a trend toward more severe visual loss in 1 or both eyes at last follow-up among those patients with BMI ≥40 (18% vs 11%, P = 0.067). Logistic regression modeling found that 10-unit (kilogram per square meter) increases in BMI increased the odds of severe visual loss by 1.4 times (95% confidence interval, 1.03–1.91, P = 0.03) after controlling for sex, race, diagnosed hypertension, and diagnosed sleep apnea. Conclusion:Our finding of a trend for severe papilledema and visual loss associated with increasing BMI suggests that very obese IIH patients should be closely monitored for progression of visual field loss.

New observations in the fragile X-associated tremor/ataxia syndrome (FXTAS) phenotype

Purpose: Fragile X-associated tremor/ataxia syndrome (FXTAS) was originally defined as tremor, ataxia, cognitive decline, and parkinsonism in individuals who carry between 55 and 200 CGG repeats in the promoter region of the fragile X mental retardation 1 (FMR1) gene. This paper describes a series of patients who meet the definition of FXTAS who presented for care between 2009 and 2014. Methods/Results: Retrospective chart review of patients seen in the FXTAS clinic at Rush University in Chicago. Conclusions: Patients with FXTAS may present with a progressive supranuclear palsy-like phenotype and other eye movement abnormalities are common in these patients as well. Rapid worsening of gait abnormalities in FXTAS may be due to a secondary spinal issue and should be aggressively treated to regain function. Finally, the FXTAS Rating Scale score does not reliably inform the certainty of diagnosis or CGG repeat size in these patients.

Pediatric idiopathic intracranial hypertension and extreme childhood obesity: a comment on visual outcomes.

BMI < 25 kg/m 2 , 6.5%: BMI 25-29.9 kg/m 2 , 14.5%: BMI 30-39.9 kg/m 2 , and 17.7%: BMI

Teaching NeuroImages: Numb chin syndrome in an edentulous patient

40 kg/m 2 ; P < .05 for linear trend). 2 BMI remained an independent predictor of severe visual loss after controlling for sex, race, hypertension, and sleep apnea. Given the similarity between the postpubertal patients with IIH of Brara et al and the published studies of adults with IIH, it would be interesting to know whether there was a similar relationship between the degree of obesity and visual outcome in their cohort. Such a finding would further emphasize the likelihood that increasing degrees of obesity not only places children at a higher risk of developing IIH but also of developing important, lifelong visual loss from IIH.

Linear scleroderma en coup de sabre presenting with positional diplopia and enophthalmos.

A 49-year-old woman presented with isolated chin hypoesthesia. She had no history of malignancy or systemic symptoms and has worn dentures for 24 years. Results of a neurologic examination were unremarkable except …

Incidental visual field loss: ethical considerations in assessing and reporting ability to drive.

A 28-year-old woman with alopecia since childhood presented with 4 years of progressive intermittent diplopia and right eye getting “stuck” with upward gaze. Maddox rod test revealed a subtle intermittent right hypotropia and right enophthalmos (figure 1). Asymptomatic T2 hyperintensities were noted on MRI (figure 2). Scalp morphea was confirmed pathologically. Formal rheumatologic evaluation was otherwise unremarkable. Linear scleroderma en coup de sabre presenting as positional diplopia, unilateral enophthalmos, and focal neuroimaging findings is rare and is suspected to represent a chronic local inflammatory process.1,2 The brain lesions tend to develop adjacent to the skin atrophy.1

Chiari in the Family: Inheritance of the Chiari I Malformation

Driving safety is an issue frequently encountered in the neurology clinic, particularly as it pertains to cognitive decline, vision loss, and motor limitations. This case illustrates the ethical dilemmas associated with determining driving safety, particularly those associated with an incidentally found congenital visual field abnormality. The authors discuss the issues involved with overruling patient autonomy for the principle of beneficence and the ethics of reporting patients with unsafe vision to authorities.