Pourfour du Petit Syndrome: A Rare Association With Cluster Headache.

Pourfour du Petit syndrome is an uncommon cause of eyelid retraction, associated with unilateral mydriasis and hemifacial hyperhidrosis. This syndrome is caused by hyperactivity of the ipsilateral oculosympathetic pathway and needs to be recognized because it has an opposite clinical presentation but the same topographic and diagnostic value as Horner syndrome. The authors report a rare case of Pourfour du Petit syndrome associated with cluster headache and discuss pathophysiological hypotheses, clinical presentation, complementary exams, pharmacologic testing, treatment options, and prognosis. Early detection of these symptoms may lead to swift diagnosis and treatment.

Evisceration with autogenous scleral graft and bioceramic implantation within the modified scleral shell: 133 cases over 17 years.

INTRODUCTION: To present long-term follow-up data on evisceration performed with autogenous scleral grafting and ceramic implantation in a modified scleral shell. METHODS: This was a retrospective analysis of all consecutive eviscerations performed in the Department of Ophthalmology, Montpellier University Hospital, France, between February 1998 and October 2015. For all patients, the technique used was a conventional anterior evisceration after total keratectomy, disinsertion of the medial rectus muscle, sectioning of the optic nerve and excision of sclera centered on the papilla. The scleral graft was then sutured just behind the sutured keratectomy, and the bioceramic implant was inserted by posterior way in the scleral shell. Demographic characteristics, implant size and type, cosmetic results from pictures of all patients and complications were recorded. This study was performed with Ethics Review Committee Approval, and in compliance with the Declaration of Helsinki. RESULTS: In total, 133 patients (36.6% women) were identified during the study period. The mean (SD) implant size was 17.32 (1.84) mm. The median follow-up after evisceration was 57.43 (24.7, 68.3) months. Two cases of implant exposure (1.5%) were recorded. For 24 patients (17.9%), additional surgeries were performed for ptosis (2.2%), conjunctival cyst (1.5%), or post-evisceration socket syndrome (6.7%). Cosmetics results were excellent for 50.1% of cases, good for 33.3% and fair for 16.6%; using a grading scale based on the superior sulcus deformity. CONCLUSION: Evisceration with autogenous scleral grafting and ceramic implantation can result in a high volume of restoration, good cosmetic results, and low risk of exposure of the implant.

Retinal vascular fractal dimension and cerebral blood flow, a pilot study.

PURPOSE: Ocular and brain microcirculation share embryological and histological similarities. The retinal vascular fractal dimension (FD) is a marker of retinal vascular complexity of the vascular tree. The purpose of this study was to explore the relationship between cerebral blood flow (CBF), retinal vascular FD and other retinal vascular markers. METHODS: Cross-sectional analysis comprising 26 individuals ≥65 years old from the Cognitive REServe and Clinical ENDOphenotype (CRESCENDO) cohort of relative healthy older adults. Retinal vascular FD was measured from fundus photographs by using the semi-automated Singapore Eye Vessel Assessment (SIVA) software. CBF was estimated using a 2D pulsed ASL MRI sequence. Associations between blood flow and retinal parameters were analysed using linear regression models adjusted for age and sex. RESULTS: Cerebral blood flow was positively associated with venular FD (R2  = 0.32, p = 0.03). This association was stronger in the anterior versus posterior brain territories (R2  = 0.35 [p = 0.001] versus R2  = 0.16 [p = 0.07], respectively). Global CBF was correlated with arteriolar branching angle (R2  = 0.23, p = 0.01) and tortuosity (R2  = 0.20, p = 0.02). Global CBF was not correlated with other SIVA parameters. CONCLUSIONS: Retinal venular complexity summarized by the FD was associated with cerebral blood flow as well as retinal arteriolar tortuosity and branching angle. Larger prospective clinical studies are needed to confirm these results.

Myasthenia Gravis Induced by Immune Checkpoint Inhibitors.

Immune checkpoint inhibitors deeply improved the prognosis of metastatic melanoma or other types of cancer, but their related adverse effects (AEs) can be very severe, especially when the neurological system is touched, as in myasthenia gravis (MG). It is a rare immune AE that can be life-threatening and can be revealed by several symptoms. We report a case of our experience and review the current literature of MG exacerbated or occurring during immunotherapy to describe characteristics of this AE, warn the oncologist about this toxicity, and summarize the treatments conducted. Thirty-four cases of MG were reported, mostly with anti-programmed cell death protein 1 checkpoint inhibitor, and with melanoma. Onset was quick after the first or second infusion. Treatment comprised corticosteroids, prostigmine, and more or less plasmapheresis or immunoglobulins. Prognosis is poor, as 13 patients died after MG. MG is a rare immune-related AE that must be rapidly evoked and treated in case of neurological symptoms emerging after immunotherapy.