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A 29-year-old woman presented with blurred vision and distal paresthesias. Her initial evaluation revealed severe bilateral optic disc edema with distal lower-extremity sensory and motor deficits and electrodiagnostic evidence of a length-dependent mixed demyelinating and axonal polyneuropathy. The results of routine diagnostic testing, including laboratory tests, magnetic resonance imaging, and lumbar puncture, were nondiagnostic. A targeted biopsy was ultimately required for diagnosis. In this article, we discuss the differential diagnosis and outline the clinical evaluation indicated for a patient presenting with demyelinating polyneuropathy and concurrent papilledema.

An isolated ocular motor nerve palsy is defined as dysfunction of a single ocular motor nerve (oculomotor, trochlear, or abducens) with no associated or localizing neurologic signs or symptoms. When occurring in patients aged 50 or older, the most common cause is microvascular ischemia, but serious etiologies such as aneurysm, malignancy, and giant cell arteritis should always be considered. In this article, the authors review the clinical approach, anatomy, and differential diagnosis of each isolated ocular motor nerve palsy and discuss the clinical characteristics, pathophysiology, and treatment of microvascular ischemia., (Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.)

Background: IgG4-associated orbital and ocular inflammation is a relatively unknown entity characterized by sclerosing inflammation with infiltration of IgG4-positive plasma cells. Some so-called idiopathic inflammation syndromes are being re-classified as IgG4-associated inflammation with histopathologic evaluation., Findings: We report three cases with differing manifestations of IgG4-associated ocular and orbital inflammation: a case of recurrent, treatment-refractory sclero-uveitis that was diagnosed as granulomatosis with polyangiitis with an IgG4-related component, a case of pachymeningitis with optic neuritis that resulted in permanent visual loss, and a case of orbital inflammatory pseudotumor. All three would have been incompletely diagnosed without thorough histopathologic evaluation (including immunohistochemistry)., Conclusions: IgG4-associated disease is an idiopathic, multi-organ inflammatory state that can manifest as chronic, relapsing, sclerosing inflammation in virtually any organ system. There is a wide range of presentations in ocular and orbital inflammation. Ophthalmologists should keep IgG4-associated inflammation in mind when examining chronic, sclerofibrosing inflammation with multi-system involvement. The histology of biopsy specimens is crucial in making the correct diagnosis. Timely assessment may lead to fewer diagnostic tests and more targeted therapy.

Purpose of Review: Toxic, nutritional, hereditary, traumatic, and neoplastic optic neuropathies result in significant disability due to visual dysfunction. Many of these conditions are treatable. Early diagnosis may allow for intervention to stabilize or improve vision and prevent unnecessary testing. These conditions have overlapping clinical features, and careful assessment of the visual system allows for accurate diagnosis and management., Recent Findings: Newer treatment strategies are available for optic neuropathies previously thought untreatable, such as some hereditary optic neuropathies. Many conditions that previously were felt to represent distinct diseases can be linked by a common pattern of mitochondrial dysfunction., Summary: The optic nerve is susceptible to a wide variety of pathologic processes. The correct diagnosis depends on a thorough history and detailed evaluation of the visual system. Certain optic neuropathies selectively affect the papillomacular bundle, and particular attention to this location can considerably narrow the differential diagnosis and subsequent workup.

Head trauma can injure the afferent and/or efferent visual systems, resulting in neuro-ophthalmic deficits. When assessing afferent pathway injuries, a stepwise approach to evaluating visual acuity, pupils, color perception, and visual fields is critical. Traumatic optic neuropathy is of especial importance and its management must be tailored on a case-by-case basis. Efferent pathway injuries should be assessed with attention to abnormalities of ocular alignment and motility, which may occur as isolated deficits or as part of a recognizable syndrome. Concussion or diffuse axonal injuries may also affect ophthalmologic function. Here, we review the extant literature describing the assessment and acute treatment of traumatic neuro-ophthalmic deficits.

The hematopoietic system is a complex mixture of circulating blood cells, plasma, and plasma proteins. Disorders of the hematologic system may result from excess or impaired production of blood cells, destruction of blood cells, or abnormal function of existing blood cells. The afferent and efferent visual systems may both be affected by blood cell disorders, and the specific manifestation is dependent upon which cell line is involved, the type of dysfunction, and the characteristics of the target tissue. This article reviews the ocular and neuro-ophthalmic manifestation of hematologic disorders.