Optic nerve sheath fenestration via a supero-medial eyelid skin crease approach for the treatment of idiopathic intracranial hypertension in a UK population.

BACKGROUND/AIMS: Optic nerve sheath fenestration (ONSF) is a surgical intervention in the management of idiopathic intracranial hypertension (IIH) infrequently performed in the United Kingdom. Numerous surgical approaches have been described, including medial transconjunctival, lateral and endoscopic. We describe our outcomes and complications from ONSF via a supero-medial eyelid skin crease incision in patients with IIH. METHODS: We performed a retrospective review of consecutive patients undergoing ONSF for IIH between January 2011 and December 2017 by a single surgeon. RESULTS: Thirty patients were included in the analysis with a median follow-up of 14.5 months. Bilateral ONSFs were undertaken in 27 (90%). The data from one eye per patient were analysed. The mean kinetic perimetry score in mean radial degrees of the I4e isopter improved from 27.3° to 35.7°, p = 0.04. After removing cases with optic atrophy, the median modified Frisén grade of papilloedema improved from 2.5 to 1.0, p = 0.007. A total of 5/30 (17%) patients had complications: two (7%) had recurrence/late failure (one managed medically and one with cerebrospinal fluid [CSF] diversion surgery), one had transient cotton wool spots post-operatively, one had transient retinal haemorrhages and one patient had a transiently oval pupil. No patients had repeat ONSF, but CSF diversion surgery was subsequently carried out in 4/30 (13%) patients. CONCLUSIONS: ONSF via a supero-medial eyelid skin crease approach is effective at improving visual function in patients with IIH. The complication rates are low when compared with CSF diversion surgery and other surgical approaches for ONSF.

Neurofilament results for the phase II neuroprotection study of phenytoin in optic neuritis.

BACKGROUND: A randomized trial of phenytoin in acute optic neuritis (ON) demonstrated a 30% reduction in retinal nerve fiber layer (RNFL) loss with phenytoin versus placebo. Here we present the corresponding serum neurofilament analyses. METHODS: Eighty-six acute ON cases were randomized to receive phenytoin (4-6 mg/kg/day) or placebo for 3 months, and followed up for 6 months. Serum was collected at baseline, 3 and 6 months for analysis of neurofilament heavy chain (NfH) and neurofilament light chain (NfL). RESULTS: Sixty-four patients had blood sampling. Of these, 58 and 56 were available at 3 months, and 55 and 54 were available at 6 months for NfH and NfL, respectively. There was no significant correlation between serum NfH and NfL at the time points tested. For NfH, the difference in mean placebo - phenytoin was -44 pg/ml at 3 months (P = 0.019) and -27 pg/ml at 6 months (P = 0.234). For NfL, the difference was 1.4 pg/ml at 3 months (P = 0.726) and -1.6 pg/ml at 6 months (P = 0.766). CONCLUSIONS: At 3 months, there was a reduction in NfH, but not NFL, in the phenytoin versus placebo group, while differences at 6 months were not statistically significant. This suggests a potential neuroprotective role for phenytoin in acute ON, with the lower NfH at 3 months, when levels secondary to degeneration of the anterior visual pathway are still elevated, but not at 6 months, when levels have normalized.

The Evolution of an Optic Nerve Head Granuloma Due to Sarcoidosis.

Sarcoidosis can affect the optic nerves by means of optic disc oedema secondary to posterior uveitis, optic disc oedema secondary to raised intracranial pressure, optic neuritis, optic atrophy secondary to compression or infiltration from a primary central nervous system lesion, and primary granuloma of the optic nerve head. The authors report the use of optical coherence tomography in assessing the response to immunosuppression in a 57-year-old woman with an optic nerve head granuloma.

Visual Outcomes from Shunting for Idiopathic Intracranial Hypertension.

A retrospective notes review was conducted for 50 consecutive patients who underwent shunt surgery for idiopathic intracranial hypertension (IIH). The decimal visual acuity and the mean radial degrees (MRD) of the I4e isopter of the Goldmann visual field were measured pre-operatively and after a mean follow-up period of 1123 days (range: 13-3551 days). A ventriculo-peritoneal shunt was the first procedure in 38 patients and a lumbo-peritoneal shunt in 12. The mean decimal visual acuity of the worse affected eye improved from 0.75 to 0.84, p = 0.011. The MRD score of the worse affected eye improved on average from 25.6° to 35.5°, p < 0.0001. In those with significant pre-operative visual impairment in their worse affected eye (defined as an MRD score ≤30°), the MRD score improved on average from 10.3° to 26.5°, p = 0.0008. The mean number of surgical procedures for each patient was 2.8 (range: 1-15). Taking all surgical procedures into account, post-operative complications were experienced by 30 patients. At last follow-up, 28 patients still complained of headache, 8 of whom had the intervention performed primarily for headache. Shunting can improve visual function in patients with IIH. There is significant post-operative morbidity and often the need for repeated procedures. Headache also commonly remains in these patients. There is a need for a randomised controlled trial of operative interventions in IIH. Sample size calculations for such a trial to treat significant vision loss are presented.

Neuro-ophthalmological Complications of Chronic Inflammatory Demyelinating Polyradiculoneuropathy.

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) can lead to prominent nerve hypertrophy, which can mimic other forms of neuropathy radiologically. Neuro-ophthalmological complications can also occur in CIDP, either at presentation or chronically in the disorder. This can also cause diagnostic difficulties. We report three cases of neuro-ophthalmological complications of CIDP: two cases of papilloedema and one case of proptosis. In all three cases cranial nerve hypertrophy was present. CIDP should be considered in neuro-ophthalmological presentations associated with cranial/spinal nerve root hypertrophy.

The incidence and prevalence of idiopathic intracranial hypertension in Sheffield, UK.

BACKGROUND AND PURPOSE: The purpose of this study was to identify the incidence and prevalence of idiopathic intracranial hypertension (IIH) in Sheffield, UK. METHODS: A retrospective review of case notes was conducted to identify cases of IIH seen between 1 January 2007 and 31 December 2008. RESULTS: Sixteen (15 women and 1 man) new patients were identified to give an incidence within Sheffield of 1.56/100,000/year and 2.86/100,000/year for women. The incidence of IIH in obese women was 11.9/100,000/year. The prevalence of IIH was calculated as 10.9/100,000, and 85.7/100,000 in obese women. CONCLUSION: A higher incidence of IIH than previously reported UK data was found, which may be because of increasing obesity within the population, or improved case ascertainment.

Diffusion MRI in multiple sclerosis.

Diffusion imaging is a quantitative, MR-based technique potentially useful for the study of multiple sclerosis (MS), due to its increased pathologic specificity over conventional MRI and its ability to assess in vivo the presence of tissue damage occurring outside T2-visible lesions, i.e., in the so-called normal-appearing white and gray matter. The present review aims at critically summarizing the state-of-the-art and providing a background for the planning of future diffusion studies of MS. Several pieces of evidence suggest that diffusion-weighted and diffusion tensor MRI are sensitive to MS damage and able to detect its evolution over relatively short periods of time. Although a significant relationship between diffusion-weighted MRI findings and MS clinical disability was not found in the earliest studies, with improved diffusion imaging technology correlations between diffusion abnormalities and MS clinical aspects are now emerging. However, the best acquisition and postprocessing strategies for MS studies remain a matter of debate and the contribution of newer and more sophisticated techniques to diffusion tensor MRI investigations in MS needs to be further evaluated. Although changes in diffusion MRI indices reflect a net loss of structural organization, at present we can only speculate on their possible pathologic substrates in the MS brain. Postmortem studies correlating diffusion findings with histopathology of patients with MS are, therefore, also warranted.

Imaging spinal cord damage in multiple sclerosis.

During the past 2 decades, the considerable improvement of magnetic resonance (MR) technology and the development of new MR strategies capable of providing an in vivo overall assessment of multiple sclerosis (MS) pathology have allowed us to obtain important novel pieces of information on disease evolution in the brain. However, despite this, the correlation between brain MR imaging metrics and clinical disability are still suboptimal. A reason for this discrepancy might be the involvement of clinically eloquent structures, such as the spinal cord, which owing to technical challenges have not been extensively studied using MR imaging until very recently. An objective and accurate estimate of the presence and extent of spinal cord damage might indeed contribute to increasing the strength of the correlations between clinical and MRI metrics. This review summarizes the main results obtained from the application of conventional and modern MR-based techniques for the evaluation of spinal cord damage in MS.

Imaging the optic nerve in multiple sclerosis.

Although multiple sclerosis (MS) frequently involves the optic nerves, imaging this structure is not yet performed routinely in clinical practice. The recent improvement of magnetic resonance (MR) technology and the development of new MR strategies, capable of providing an, in vivo, overall assessment of MS pathology has allowed objective metrics to be obtained for monitoring disease evolution, essentially in the brain. However, despite this progress, the correlation between brain MR metrics of the disease and clinical disability are still disappointing. An objective and accurate estimate of the presence and extent of optic nerve involvement might help to overcome this clinical/MRI paradox. This review summarizes the main results obtained from the application of conventional and modern MR-based techniques for the evaluation of optic nerve damage in MS.

The optic nerve sheath on MRI in acute optic neuritis.

Optic nerve sheath dilatation or gadolinium-enhancement on magnetic resonance imaging in acute optic neuritis have been previously reported but have been thought to be rare occurrences. This study recruited 33 patients with acute unilateral optic neuritis. All had their optic nerves imaged with fat-saturated fast spin-echo (FSE) imaging, and 28 had imaging before and after triple-dose gadolinium-enhanced fat-saturated T(1)-weighted imaging. Follow-up imaging was performed on 20 patients (15 following gadolinium). A dilated subarachnoid space at the anterior end of the symptomatic optic nerve on FSE imaging was seen in 15/33 cases. In three of these cases, dilatation was visible on short-term follow-up. Optic nerve sheath enhancement was seen in 21/28 cases acutely: seven at the anterior end of the lesion only, five at the posterior end only and nine at both ends. Optic sheath enhancement was seen in 13 patients on follow-up. This study suggests that optic nerve sheath dilatation on FSE images and optic nerve sheath enhancement on triple-dose gadolinium-enhanced images are common findings in acute optic neuritis. Optic nerve sheath dilatation may be due to inflammation of the optic nerve, with its associated swelling, interrupting the communication between the subarachnoid space of the diseased optic nerve and the chiasmal cistern. Optic nerve sheath enhancement suggests that meningeal inflammation occurs in optic neuritis, in agreement with pathological studies of both optic neuritis and multiple sclerosis.

Cervical spinal cord MTR histogram analysis in multiple sclerosis using a 3D acquisition and a B-spline active surface segmentation technique.

The application of a three-dimensional magnetization transfer (MT) sequence and B-spline active surface segmentation method to produce MT histograms of the cervical spinal cord in a pilot study of controls and multiple sclerosis (MS) patients is presented. Subjects' cervical spinal cords were imaged with (a) a volume-acquired inversion-prepared fast spoiled gradient echo sequence and (b) a volume-acquired noninversion-prepared fast spoiled gradient echo MT sequence. The images were segmented using the B spline active surface technique and MT histograms were produced from the MT images. The method was sensitive enough to detect differences between seven MS patients and 10 controls in mean MT ratio (42.4 pu versus 44.0 pu, p = 0.03) and peak location (45.2 versus 46.8, p = 0.03). The spinal cord volumes obtained from the two sequences were associated with each other (parameter estimate 0.972, 95% confidence intervals 0.742, 1.202, p < 0.001).

Serial magnetization transfer imaging in acute optic neuritis.

In serial studies of multiple sclerosis lesions, reductions in magnetization transfer ratio (MTR) are thought to be due to demyelination and axonal loss, with later rises due to remyelination. This study followed serial changes in MTR in acute optic neuritis in combination with clinical and electrophysiological measurements to determine if the MTR changes over time mirror the picture in multiple sclerosis lesions, further validating MTR as a marker of tissue integrity. Twenty-nine patients were recruited who had acute optic neuritis for a median of 13 days (range 7-24 days) since the onset of visual symptoms. A clinical examination and measurement of visual evoked potentials (VEP) was performed on each patient. Their optic nerves were imaged with a fat-saturated fast spin echo (FSE) sequence and a magnetization transfer sequence. Twenty-one had multiple subsequent examinations over the course of 1 year. In addition, 27 control subjects had their optic nerves imaged up to three times over 1 year. A blinded observer segmented the optic nerves from the MTR maps. Lesions were defined on the acute FSE images and, from the coordinates, the ratio of mean lesion MTR : healthy nerve MTR (lesion ratio) was calculated for each dataset. The time-averaged mean MTR in control optic nerves was 47.7 per cent units (pu). In diseased optic nerves, baseline mean MTR was 47.3 pu, with a mean lesion ratio of 0.98. The diseased optic nerve MTR and lesion ratio declined over time with a nadir at about 240 days at a mean MTR value of 44.2 pu and mean lesion ratio of 0.91. Subsequently, diseased optic nerve MTR appeared to rise; after 1 year the diseased optic nerve mean MTR was 45.1 pu (mean lesion ratio 0.93), although the difference was not significant compared with the nadir value. For each 0.01 increase in time-averaged lesion ratio logMAR visual acuity recovery improved by 0.03 (95% CI, 0.002, 0.08, P = 0.02). Time-averaged VEP central field latency was shorter by 6.1 ms (95% CI 1.5, 10.7, P = 0.012) per 1 pu rise in time-averaged diseased optic nerve MTR. The early fall in diseased optic nerve MTR is consistent with demyelination and Wallerian degeneration of transected axons. The late nadir compared with studies of multiple sclerosis lesions may have been due to slow clearance of myelin debris. Remyelination may have influenced subsequent MTR changes. The observations support using MTR to monitor symptomatic demyelinating lesions.

Management of acute optic neuritis.

Optic neuritis is a common condition that causes reversible loss of vision. It can be clinically isolated or can arise as one of the manifestations of multiple sclerosis. Occasional cases are due to other causes, and in these instances management can differ radically. The treatment of optic neuritis has been investigated in several trials, the results of which have shown that corticosteroids speed up the recovery of vision without affecting the final visual outcome. Other aspects of management, however, are controversial, and there is uncertainty about when to investigate and when to treat the condition. Here we review the diagnostic features of optic neuritis, its differential diagnosis, and give practical guidance about management of patients. The condition's association with multiple sclerosis will be considered in the light of studies that define the risk for development of multiple sclerosis and with respect to results of trials of disease-modifying drugs in these individuals.

Continuing optic nerve atrophy following optic neuritis: a serial MRI study.

To investigate optic neuritis as a model for atrophy in multiple sclerosis (MS) lesions we performed serial magnetic resonance imaging (MRI) on 10 patients with a history of optic neuritis using a fat saturated short-echo fast fluid-attenuated inversion recovery (sTE fFLAIR) sequence. The first study was performed a median of 19.5 months after the onset of optic neuritis and the second 1 year later. Using a computer-assisted contouring technique, a blinded observer calculated the mean area of the intro-orbital optic nerves. The mean area of affected optic nerves decreased over 1 year by 0.9 mm2 from 11.1 to 10.2 mm2 (p = 0.01). Poor visual acuity and decreased visual-evoked potential (VEP) amplitude were associated with atrophy. These findings suggest that atrophy is a feature of focal demyelinating lesions, it may evolve over several years, and may have functional significance. Optic neuritis provides a model to study the effect of inflammatory demyelination through the ability to accurately measure visual function and to visualize and measure the optic nerves using magnetic resonance imaging.

Quantification of spinal cord atrophy from magnetic resonance images via a B-spline active surface model.

A method is presented that aims at segmenting and measuring the surface of the spinal cord from MR images in order to detect and quantify atrophy. A semiautomatic segmentation with very little intervention from an operator is proposed. It is based on the optimization of a B-spline active surface. The method allows for the computation of orthogonal cross-sections at any level along the cord, from which measurements are derived, such as cross-sectional area or curvature. An evaluation of the accuracy and reproducibility of the method is presented.