Steroid-Responsive Optic Neuropathy (SRON) in Anti-neutrophil Cytoplasmic Antibody–Negative Wegener Granulomatosis with False-Positive Indicators of Giant Cell Arteritis and Sarcoidosis

A 65-year-old woman with rheumatological symptoms and diplopia was diagnosed as suffering from giant cell arteritis. Four years later she presented to us with a steroid responsive optic neuropathy. A presumptive diagnosis of neurosarcoidosis was made because of a gallium-67 single-photon emission tomography scan indicating a positive ‘panda sign’. Subsequent chest computerised tomographic imaging revealed cavitating lung lesions and an open biopsy established the diagnosis of Wegener granulomatosis despite the negative anti-neutrophilic cytoplasmic antibody. At the time of diagnosis the patient had a steroid responsive optic neuropathy and had recently developed a bilateral uveitis. Treatment with cyclophosphamide and corticosteroids produced complete remission with no further progression of disease.     

Brainstem Infarction in Pupil-Sparing Oculomotor Nerve Palsy Detected by Diffusion-Weighted Magnetic Resonance Imaging

The usefulness of diffusion-weighted magnetic resonance imaging studies in pupil-sparing oculomotor nerve palsy due to midbrain infarction is illustrated in two interventional case reports. Two cases are presented illustrating the superior sensitivity of diffusion-weighted magnetic resonance imaging in detecting midbrain infarction in this clinical situation.     

Abducens Nerve Palsy Following Expansion Cranioplasty with Distraction Osteogenesis

Abstract

Childhood abducens nerve palsy can occur as a result of trauma, tumour, vasculopathic disease, elevated intracranial pressure, infection, inflammation, and congenital or idiopathic causes. The authors present two cases of unilateral abduction deficit secondary to a recent trans-sutural distraction osteogenesis (TSuDO) operation for craniosynostosis. After distractor removal, the problem resolved spontaneously over 2–4 months in both cases. This is a first reported case of sixth nerve palsy as a complication of TSuDO operation.     

Medullated retinal nerve fibers

Abstract. Myelinated retinal nerve fibers may cause fluctuating functional defects. In cases of association with neuro-ophthalmological disease, functional loss due to the medullated nerve fibers and due to the concomitant disease, may be difficult to evaluate.     

A shape statistic for visual field evaluation: Utility in minor optic neuropathy

Minor deviations from the normal threshold surface are difficult to identify in static perimetry. Numerical assessment of surface shape is a possible remedy. An easily calculated statistic that depends on an extended isopter concept is presented. It is capable of detecting small aberrations from normal. Sensitivity was superior to that of pattern standard deviation in subjects with resolved optic neuritis. Specificity was estimated at 95%.     

Chemotherapy for Optic Nerve Glioma in A Child with Neurofibromatosis Type-1

The majority of optic pathway tumors associated with Neurofibromatosis type-1 (NF-1) are benign, slow-growing lesions: however, rapidly growing tumors, which cause proptosis and visual loss, can sometimes occur. Optimal management of these tumors is still unclear. We report the case of a 12-year-old male, affected by NF-1 who was found to have a rapidly growing right optic nerve glioma, treated with carboplatin and vincristine. During chemotherapy, optic disc swelling rapidly disappeared; the orbital tumor decreased in size, with magnetic resonance imaging (MRI) evidence of tumor shrinkage. There was a progressive improvement in visual acuity and Visually Evoked Potential (VEP) amplitudes. Children with NF-1 associated optic pathway tumors should not receive chemotherapy unless there is documented progressive disease. Nevertheless, in contrast with previous studies, this case demonstrates that chemotherapy can be a safe and effective treatment for NF-1-associated optic nerve glioma, and should not be reserved only for patients with optic chiasm or tract involvement.     

Neuro-Ophthalmic Literature Review

This review article is dealing with the clinically relevant pupil research work, during the eighties and nineties and its consequences for the neuro-ophthalmologist. Suggestions for pupil examinations including pharmacological testing and handling of various pupillary disorders are given. The article covers the following items: (1) The pupil and the afferent visual system, (2) Parasympathetic pupillary dysfunction, (3) Disorders of the sympathetic pupillary innervation (Horners syndrome), (4) Other issues, including the pupil in diabetes mellitus, iris related pupillary disorders, the pupil as an indicator of vigilance, and the pupillary near reflex.     

Treatment of aberrant facial nerve regeneration with botulinum toxin A

purpose To assess the effect and efficacy of botulinum toxin type A (BTX-A) at reducing and maintaining eyelid synkinesia in aberrant facial nerve regeneration, while concurrently observing for the presence of side effects to differing treatment doses. methods A prospective interventional study of five patients with eyelid synkinesia resulting from aberrant regeneration of the facial nerve. Patients were treated with injections of either 120, 80 or 40 units of BTX-A (Dysport) into the orbicularis oculi. Objective and subjective reduction in synkinesia, maintenance of response and presence of side effects were recorded. results All five patients had improvement of the synkinesia with BTX-A treatment. Lower doses were found to be as effective as higher doses. Mean duration of abolished synkinesia was three months. Two patients developed a ptosis which resolved spontaneously. None of the patients treated with the lowest dose of 40 units developed a ptosis. conclusion Low-dose BTX-A has a lower incidence of ptosis and is effective in the treatment of aberrant facial nerve regeneration.     

Mesenchymal progenitor cells derived from traumatized muscle enhance neurite growth

The success of peripheral nerve regeneration is governed by the rate and quality of axon bridging and myelination that occurs across the damaged region. Neurite growth and the migration of Schwann cells is regulated by neurotrophic factors produced as the nerve regenerates, and these processes can be enhanced by mesenchymal stem cells (MSCs), which also produce neurotrophic factors and other factors that improve functional tissue regeneration. Our laboratory has recently identified a population of mesenchymal progenitor cells (MPCs) that can be harvested from traumatized muscle tissue debrided and collected during orthopaedic reconstructive surgery. The objective of this study was to determine whether the traumatized muscle‐derived MPCs exhibit neurotrophic function equivalent to that of bone marrow‐derived MSCs. Similar gene‐ and protein‐level expression of specific neurotrophic factors was observed for both cell types, and we localized neurogenic intracellular cell markers (brain‐derived neurotrophic factor and nestin) to a subpopulation of both MPCs and MSCs. Furthermore, we demonstrated that the MPC‐secreted factors were sufficient to enhance in vitro axon growth and cell migration in a chick embryonic dorsal root ganglia (DRG) model. Finally, DRGs in co‐culture with the MPCs appeared to increase their neurotrophic function via soluble factor communication. Our findings suggest that the neurotrophic function of traumatized muscle‐derived MPCs is substantially equivalent to that of the well‐characterized population of bone marrow‐derived MPCs, and suggest that the MPCs may be further developed as a cellular therapy to promote peripheral nerve regeneration. Copyright © 2012 John Wiley & Sons, Ltd.     

细胞外三磷腺苷对失神经大鼠腓肠肌细胞中FoxO3a通路的影响

目的 探讨细胞外三磷腺苷(ATP)对失神经大鼠腓肠肌细胞中FoxO3a/MAFbx通路的影响.方法 雌性成年Wistar大鼠54只随机分为3组:失神经对照组,ATP治疗组,健康对照组.其中失神经对照组和ATP治疗组在右侧梨状肌下缘切断坐骨神经,制作失神经动物模型.术后ATP治疗组于右侧腓肠肌内注射ATP0.1mg/d,左侧腓肠肌内注射等量生理盐水;失神经对照组双下肢注射等量的生理盐水;健康对照组不做任何处理.在术后0、2、7、14、28d分别处死一组大鼠,取其两侧腓肠肌,称肌湿重,计算肌失重比(右侧/左侧),采用实时荧光定量PCR和Western Blot检测大鼠腓肠肌FoxO3a和MAFbx的mRNA和蛋白表达水平以及253位磷酸化的FoxO3a(p-FoxO3a)蛋白表达.结果 ATP治疗组肌湿重比高于失神经对照组,差异有统计学意义(P＜0.01);ATP治疗组FoxO3a的mRNA和蛋白表达略低于失神经对照组,差异无统计学意义(P＞0.05);MAFbx的mRNA和蛋白表达低于失神经对照组,差异有统计学意义(P＜0.05);p-FoxO3a的蛋白表达量较对照组明显增加,不同时段差异均有统计学意义(P＜0.05).结论 细胞外ATP可能通过磷酸化FoxO3a进而抑制MAFbx蛋白的表达来延缓大鼠骨骼肌失神经萎缩.