No abstract

In this study of public hospitals in Singapore, the incidence of neuro-ophthalmic diseases was higher in Chinese and Indians compared to Malays.

Objective The degree of pneumatization of the temporal bone has implications in the pathophysiology and surgical considerations of many temporal bone disorders. This study aims to identify common pneumatization patterns in the petrous apex, mastoid, and infralabyrinthine compartments of the temporal bone. Variables associated with temporal bone pneumatization were also identified. Study Design Case series with chart review. Setting Single tertiary hospital. Subjects and Methods In total, 299 high-resolution computed tomography scans of the temporal bone performed on patients between 2013 and 2016 were reviewed. Only normal temporal bone scans in patients aged 13 years and older were included. Previously published grading systems were used to classify pneumatization patterns in the petrous apex, mastoid, and infralabyrinthine region. Results The most common pneumatization pattern in the petrous apex was group 2 (less than half of the petrous apex medial to the labyrinth is pneumatized), that in the mastoid was group 4 (hyperpneumatization), and that in the infralabyrinthine region was type B (limited pneumatization), at 54.8%, 55.4%, and 76.0% of patients, respectively. Patients with increased pneumatization of 1 temporal bone compartment tended to have increased pneumatization of the same compartment on the contralateral side and the other compartments on the ipsilateral side ( P < .05). Younger age ( P < .001) and male sex ( P = .001) were associated with increased pneumatization in the petrous apex and infralabyrinthine compartments. Conclusion The degree of temporal bone pneumatization varies among the different compartments. Age and sex have a significant association with the degree of pneumatization of the petrous apex and infralabyrinthine compartment.

images in clinical medicineT h e ne w e ngl a nd jou r na l o f m e dic i ne n engl j med 360;9 nejm.org february 26, 2009 e12 A 50-year-old woman with hypertension had an acute pontine hemorrhage, as seen on computed tomography (Panel A, arrow), which resulted in quadriplegia. Thirty months later, she reported having difficulty reading because of oscillopsia. The physical examination revealed pendular nystagmus (two cycles per second) with a predominantly vertical component and some horizontal and torsional eye movements (Video 1). She also had palatomyoclonus, seen as rhythmic, involuntary contractions of the soft palate and pharyngopalatine arch (one to two cycles per second) (Video 2). T 2 -weighted magnetic resonance imaging of the brain showed hyperintensity and enlargement of the inferior olivary nuclei, findings that were compatible with a diagnosis of hypertrophic olivary degeneration (Panel B, arrowheads). The condition, which may be caused by trauma, infection, demyelination, neoplasm, or vascular injury in the components of the dentatorubroolivary pathway, results in olivary hypertrophy rather than atrophy. Myoclonic contractions of the soft palate and nystagmus represent the effects of transsynaptic degeneration of the inferior olivary nuclei. After treatment with trihexyphenidyl, there was some improvement in the patient's visual symptoms.

Idiopathic intracranial hypertension (IIH) is uncommon in the paediatric population. Papilloedema is the hallmark sign and patients can suffer permanent vision loss as a consequence. We describe the role of optical coherence tomography (OCT) in the follow-up of two paediatric patients with newly diagnosed IIH. Patient A presented with vomiting and examination showed ophthalmoplaegia and papilloedema. She was treated with acetazolamide, furosemide and therapeutic lumbar punctures. Patient B presented with incidental papilloedema and was treated with acetazolamide and she reported intermittent headache during follow-up. Fundoscopic examinations for both patients showed persistent blurred disc margins but OCT examinations documented improvement of average retinal nerve fibre layers. OCT may be of value in monitoring for recurrence in paediatric IIH.

Introduction: The Optic Neuritis Treatment Trial (ONTT) has established that the magnetic resonance imaging (MRI) findings at the time of presentation of optic neuritis (ON) is the strongest indicator of the development of multiple sclerosis (MS). Reports from Singapore as well as other Asian countries have indicated that these abnormalities are less frequently encountered compared to that reported by the ONTT. This paper aims to describe systematically the brain MRI as well as the optic nerve abnormalities in patients after an episode of acute optic neuritis. Materials and Methods: Patients who presented with acute optic neuritis were retrieved from our prospective optic neuritis study and their MRI scans were reviewed and graded in accordance with the standardised classification employed in the ONTT. Results: Fifteen of 24 patients had MRI brain and optic nerves performed during the acute episode. In the evaluation of brain abnormalities, 40% were classified as grade 0, 20% grade I, 20% grade II, 6.7% grade III and 13.3% grade IV. Optic nerve abnormalities were observed in 80% of cases. At study entry, 10 patients had idiopathic (monosymptomatic) ON, 3 had multiple sclerosis (MS), one each with infective and autoimmune optic neuritis, respectively. The single patient who developed MS at study completion presented with grade II brain abnormalities at the initial MRI. For those with idiopathic ON, our study revealed a higher percentage of grade 0-I brain changes as well as a lower lesion load compared to the ONTT. Lesion load and grade was also lower in anterior optic neuritis compared with retrobulbar disease. Conclusion: Our study revealed a lower percentage of grade II-IV brain MRI abnormalities as well as less lesion load in idiopathic ON compared to the ONTT. This may be related to the lower prevalence of MS in our predominantly Asian population. As diagnostic tests and understanding of neuromyelitis optica or Devic’s disease improves, we may see more patients being diagnosed with this condition, which may also explain our findings. Our data also showed that MRI grade and lesion load in cases of anterior ON was lower than for retrobulbar disease. MRI in ON has an essential role in characterising the disease, evaluating for associated brain lesions, and assessing prognosis in retrobulbar disease but may be less useful in anterior disease. Key words: Multiple sclerosis, Optic nerve enhancement, Periventricular plaques

Pediatric neuro-ophthalmology is a diverse and challenging field. As we strive to provide excellent care to these patients, we will use the results of basic science, genetic, and neurobiological research.

Background: Optic neuritis (ON) may be the initial manifestation of neuromyelitis optica spectrum disorder (NMOSD). Aquaporin-4 antibody (AQP4 Ab) is used to diagnose NMOSD. This has implications on prognosis and is important for optimal management. We aim to evaluate if clinical features can distinguish AQP4 Ab seropositive and seronegative ON patients. Methods: We reviewed patients with first episode of isolated ON from Tan Tock Seng Hospital and Singapore National Eye Centre who tested for AQP4 Ab from 2008 to 2017. Demographic and clinical data were compared between seropositive and seronegative patients. Results: Among 106 patients (120 eyes) with first episode of isolated ON, 23 (26 eyes; 22%) were AQP4 Ab positive and 83 (94 eyes; 78%) were AQP4 Ab negative. At presentation, AQP4 Ab positive patients had older mean onset age (47.9 ± 13.6 vs 36.8 ± 12.6 years, P , 0.001), worse nadir VA (OR 1.714; 95% CI, 1.36 to 2.16; P , 0.001), less optic disc swelling (OR 5.04; 95% CI, 1.682 to 15.073; p = 0.004), and higher proportions of concomitant anti-Ro antibody (17% vs 4%, p = 0.038) and anti-La antibody (17% vs 1%, p = 0.008). More AQP4 Ab positive patients received steroid-sparing immunosuppressants (74% vs 19%, p , 0.001) and plasma exchange (13% vs 0%, p = 0.009). AQP4 Ab positive patients had worse mean logMAR VA (visual acuity) at 12 months (0.70 ± 0.3 vs 0.29 ± 0.5, p = 0.051) and 36 months (0.37±0.4 vs 0.14 ± 0.2, p = 0.048) follow-up. Conclusion: Other than older onset age and retrobulbar optic neuritis, clinical features are non-discriminatory for NMOSD. We propose a low threshold for AQP4 Ab serology testing in inflammatory ON patients, particularly in high NMOSD prevalence populations, to minimize diagnostic and treatment delays.