Multiple skull base defects in the setting of spontaneous cerebrospinal fluid rhinorrhea; a dual institution view.

PURPOSE: Idiopathic intracranial hypertension (IIH) is a condition of high cerebrospinal fluid (CSF) pressure that presents with CSF leak. The implications of multiple skull base defects (SBD) and associated synchronous CSF leaks have not been previously explored. MATERIALS AND METHODS: A dual institutional case-control study examined multiple SBD's and encephaloceles on the risk of CSF leak and postoperative failures post-repair. IIH patients with CSF leaks and IIH controls without leaks were selected retrospectively. Chi square analysis evaluated for statistically significant alterations in probability with CSF leak development. RESULTS: 192 patients were selected with 108 IIH controls and 84 spontaneous CSF leak cases. Signs and symptoms for IIH controls and CSF leak cases respectively were pulsatile tinnitus (60.2 % and 29.8 %), headaches (96.3 % and 63.1 %), papilledema (74.1 % and 12.5 %), visual field defects (60.8 % and 13 %) (p < 0.001). Encephalocele formation in controls was 3.7 % compared to cases at 91.6 % (p < 0.001). Multiple SBD's in controls compared cases was 0.9 % and 46.4 % respectively (p < 0.001). Subgroup analysis of CSF leak cases showed 15 patients with two CSF leak repairs due to a recurrence. 27 (39.1 %) single leak cases had multiple SDB's while 12 (80 %) recurrent leaks had multiple SDB's (p = 0.004). CONCLUSIONS: Patients with radiographic evidence of multiple SBD's and encephaloceles represent a high-risk population with a propensity for CSF leaks. Secondary SBD's are common in patients with spontaneous CSF rhinorrhea and higher in patients with a recurrence.

Unilateral Retinal Vasculitis as the Presenting Manifestation of Parry-Romberg Syndrome (Progressive Hemifacial Atrophy).

Purpose: This work aims to present a case of retinal vasculitis associated with Parry-Romberg syndrome. Method: A case report is presented. Results: A 17-year-old man with new floaters was found to have 20/40 vision with 1+ vitreous cell and retinal vasculitis in the right eye only. Workup for infectious etiologies did not reveal an explanation for the retinal vasculitis. However, magnetic resonance imaging of the head showed areas of linear band-like atrophy and scarring of the scalp and soft tissues as well as areas of gliosis and encephalomalacia in the subcortical white matter, all of which were consistent with Parry-Romberg syndrome. The patient was prescribed oral steroids and methotrexate, and the retinal vasculitis improved. Conclusions: Parry-Romberg syndrome is a rarely reported cause of retinal vasculitis and should be kept in the differential for retinal vasculitis.

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

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.

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.

Chiari in the family: inheritance of the Chiari I malformation.

This report presents three families with Chiari malformation type I that became symptomatic during childhood: a mother and son; a set of monozygotic twins; and two half-siblings and their two maternal cousins. These children presented with various symptoms, including headache, stiff neck, and swallowing difficulty. A review of the relevant literature is presented, with an emphasis on familial examples and proposed inheritance. Less common presentations of Chiari malformation type I are discussed, as well as the possible pathogenesis of Chiari malformation type I and associated syringomyelia.