Longitudinal follow-up of vision in a neuromyelitis optica cohort.

BACKGROUND: Neuromyelitis optica (NMO) is an inflammatory disease associated with optic neuritis and myelitis. Recently, several studies showed that optical coherence tomography (OCT) could be an interesting method for the evaluation of disease severity; however, to date there are no studies with a longitudinal follow-up of visual function in NMO. The aim of this study was to assess the ability of OCT to evaluate the progression of visual dysfunction in NMO. PATIENTS AND METHODS: A group of 30 NMO patients (thus, 60 eyes), comprised of 20 women and 10 men with a mean age of 43.7 +/- 12.3 years, were prospectively evaluated clinically and by a whole neuro-ophthalmological work-up, including: visual acuity (VA), fundoscopy, visual evoked potential (VEP), visual field (VF) and optical coherence tomography (OCT). All patients were tested at baseline (after a mean disease duration of 6.1 years) and after a mean time of follow-up of 18 months (range: 12-36 months). RESULTS: Mean VA was similar at the two evaluation times (0.77 +/- 0.36 versus 0.77 +/- 0.35). The mean VF defect decreased slightly, but the difference was not significant (-5.9 +/- 1.3 dB versus -5.3 +/- 1.3 dB). In contrast, the mean retinal thickness seen on OCT decreased from 87.4 +/- 23.3 µm to 79.7 +/- 22.4 µm (p = 0.006). These modifications were only observed in eyes with a past or a recent history of optic neuritis (-15.1 µm; p < 0.001) and not in eyes without any history of optic neuritis (-2.4 µm; not significant). Also, they occurred independently of the occurrence of relapses (n = 13) and especially optic neuritis episodes; however, the number of optic neuritis episodes was low (n = 5). CONCLUSION: OCT seems to be a more sensitive test than VA or VF for monitoring ophthalmological function in NMO and it seems to be helpful for the detection of infra-clinical episodes in patients with a past history of optic neuritis. Our results suggest that this easily performed technique should be used in the follow-up of NMO, but complementary studies are warranted to confirm its interest at an individual level.

[A case of bilateral horizontal gaze ophthalmoplegia: The 1+1 syndrome]. / Un cas d'ophtalmoplégie horizontale bilatérale : le syndrome 1+1.

INTRODUCTION: Oculomotor deficiencies in multiple sclerosis (MS) are frequently characterized by internuclear ophthalmpoplegia or isolated abduction or adduction palsies. Complete unilateral conjugate gaze paralysis and the "one and a half" syndrome are rare. Complete bilateral horizontal gaze paralysis has been exceptionally reported. OBSERVATION: Here, we describe an unusual oculomotor paralysis as a suspected first event of MS. A 24-year-old woman with an uneventful medical history presented for sudden onset of binocular diplopia. On examination, abduction and adduction saccades were impossible, whereas vertical eye saccades and convergence were normal. Oculocephalic reflex failed to improve horizontal eye movement. No nystagmus and no other sign of brainstem dysfunction were observed. Visual acuity was 4/10 in the right eye and 6/10 in the left eye. A sign of Marcus Gunn was noted in the right eye. Blood samples and cerebrospinal fluid were normal, no oligoclonal bands were detected. Visual evoked potentials were significantly impaired in both eyes and argued for bilateral optic neuritis. Brain MRI scans showed white matter T2-hypersignal abnormalities, which fulfill Barkhof criteria for MS. A small symmetric lesion was noted in the posterior part of the medial pontine tegmentum. As a first episode of MS was suspected, treatment with methylprednisolone 1000 mg/d for 3 days was started, and was followed by complete recovery of eye movements and visual acuity after 3 weeks. DISCUSSION: To our knowledge, only two cases of complete horizontal bilateral ophthalmoplegia have been reported in the literature. Both were associated with peripheral facial nerve palsy as a first event in MS. In our case report, we describe for the first time a complete bilateral horizontal ophthalmoplegia with no other brainstem dysfunction. By analogy with the "one and a half" syndrome, such complete horizontal gaze paralysis could be named a "one and one" syndrome and seems to be specifically related to a first event of MS.

[Optic neuropathy and meningioma: a diagnostic trap]. / Méningiome et neuropathie optique : un piège diagnostique.

INTRODUCTION: Meningiomas are benign primary meningeal tumors. Their diagnosis may be incidental or in response to a work-up for neurological or ophthalmological symptoms. PATIENTS AND METHODS: The clinical course of five patients with ophthalmological symptoms leading to the diagnosis of meningioma is described. RESULTS: The case reports consist of five women (48 to 54 years old - mean 52 years at the onset of symptoms), all suffering from a progressive unilateral decrease in visual acuity with a normal initial fundus examination and ipsilateral visual field changes. Ancillary testing, in particular MRI and CT-scans, had to be repeated to make the diagnosis of meningioma, which was delayed from 18 months to 4 years. DISCUSSION: The clinical presentation of these five cases was that of a retrobulbar optic neuropathy, which biased the work-up towards an inflammatory disease of the central nervous system such as multiple sclerosis. However, the atypical character of the neuropathy, which did not respond to intravenous steroids, caused the diagnosis to be questioned and radiological examinations repeated. The iso-intense appearance of meningiomas on T1 MR imaging and only slightly hyperintense appearance on T2 may result in a diagnostic delay if the exam is not performed and interpreted by an experienced professional. Gadolinium contrast, fat suppression and centration on the anterior visual pathways are essential to a proper MRI examination. CONCLUSION: When confronted with a progressive, painless optic neuropathy unresponsive to steroid treatment, the diagnosis of meningioma of the anterior visual pathways must be considered. This diagnosis is enabled by a targeted MRI of the anterior visual pathways.

[Serious fireworks-related eye injuries in Alsace (France)]. / Blessures oculaires graves liées à l'utilisation d'artifices de divertissement en Alsace.

AIM: The purpose of the study was to review cases of serious fireworks-related eye injuries presented in the Strasbourg (France) University Hospital ophthalmology emergency department and to analyze epidemiological and clinical data such as visual outcomes and risk factors. METHOD: A 13-year retrospective study (1994-2007) including fireworks-related ocular and adnexal injuries requiring hospitalization was conducted. RESULTS: Thirty-nine patients were reported; 95% were male. The mean age was 19.38 years old. There was 61.5% contusions, 33.3% eye and adnexal burns, and 15.4% of the patients suffered from a penetrating injury. More than half of the patients required emergency ophthalmic surgical procedures. Twenty-six percent of the patients had final visual acuity of 20/400 or less. CONCLUSIONS: Fireworks are a cause of severe eye injury in France. These injuries are potentially preventable, especially in young males.

[Diplopia complicating scleral buckling surgery for retinal detachment]. / Diplopie dans les suites du traitement chirurgical de décollement de rétine par cryo-indentation.

INTRODUCTION: Diplopia is a well-known complication of scleral buckling for retinal detachment. It is generally caused by ocular motility disturbances. SUBJECTS AND METHODS: We conducted a retrospective study on patients operated on for retinal detachment between 1998 and 2006 at Strasbourg University Hospital (France) who secondarily complained of diplopia. Patient history, scleral buckling type, and early and late postoperative complications were described. RESULTS: Among 821 patients who underwent scleral buckling, 12 developed diplopia lasting more than 3 months. Six of the scleral buckling operations were vertical, three horizontal, and three oblique. Examination of the 12 patients showed seven cases of hypertropia, two cases of hypertropia associated with esotropia, one case of hypertropia with exotropia, and two cases of exotropia. The scleral buckle was removed in first intention in all 12 patients; binocular single vision was restored in six cases. Secondarily, prism correction restored binocular vision in three additional patients. Strabismus surgery was necessary for the remaining three patients. DISCUSSION: Diplopia following retinal detachment usually responds to simple measures. In our study, the removal of the buckle restored binocular vision in the majority of cases, a prism correction was useful for the remaining patients. Strabismus surgery was often very efficient. CONCLUSION: Diplopia complicating scleral buckling generally responds to simple treatments that can eliminate a handicapping functional deficit.