Retrospective, Multicenter Comparison of the Clinical Presentation of Patients Presenting With Diplopia From Giant Cell Arteritis vs Other Causes.

BACKGROUND: Although giant cell arteritis (GCA) is a well-known cause of transient and permanent vision loss, diplopia as a presenting symptom of this condition is uncommon. We compared symptoms and signs of patients presenting with diplopia from GCA to those from other causes. METHODS: This was a multicenter, retrospective study comparing the clinical characteristics of patients presenting with diplopia from GCA with age-matched controls. Demographic information, review of symptoms, ophthalmic examination, and laboratory data of biopsy-proven patients with GCA were compared with those of age-matched controls presenting with diplopia. RESULTS: A total of 27 patients presented with diplopia from GCA, 19 with constant diplopia, and 8 with transient diplopia. All patients with constant diplopia from GCA were matched with 67 control subjects who had diplopia from other etiologies. Patients with GCA were more likely to describe other accompanying visual symptoms (58% vs 25%, P = 0.008), a greater number of systemic GCA symptoms (3.5, GCA vs 0.6, controls, P < 0.001) such as headache (94% [17/18] vs 39% [23/67]; P < 0.001), jaw claudication (80% [12/15] vs 0% [0/36]; P < 0.001), and scalp tenderness (44% [7/16] vs 7% [3/43]; P < 0.001). Ocular ischemic lesions (26% vs 1%, P < 0.001) were also common in patients with diplopia from GCA. Inflammatory markers were elevated significantly in patients with GCA vs controls (erythrocyte sedimentation rate: 91% [10/11] vs 12% [3/25], P < 0.001; C-reactive protein: 89% [8/9] vs 11% [2/19], P < 0.001). CONCLUSIONS: GCA is a rare but serious cause of diplopia among older adults and must be differentiated from other more common benign etiologies. Our study suggests that most patients with diplopia from GCA have concerning systemic symptoms and/or elevated inflammatory markers that should trigger further work-up. Moreover, careful ophthalmoscopic examination should be performed to look for presence of ocular ischemic lesions in older patients presenting with acute diplopia.

Optic Neuropathy Due to Chronic Lymphocytic Leukemia Proven With Optic Nerve Sheath Biopsy.

Central nervous system involvement from chronic lymphocytic leukemia (CLL) occurs infrequently, and manifestations include cognitive and cerebellar dysfunction and cranial nerve palsies. We report a 45-year-old man with CLL believed to be in clinical remission, who presented with vision loss and bilateral optic disc edema. His optic neuropathy due to CLL was proven by optic nerve sheath biopsy, and he experienced visual recovery after treatment with ibrutinib and intrathecal methotrexate.

Neuro-ophthalmologic features of chordoid glioma.

Chordoid glioma is a rare intracranial tumor typically arising in the third ventricle, particularly along the anterior aspect of the hypothalamic wall. We describe the clinical, neuroimaging, and pathologic factors of this neoplasm in a patient presenting with a chiasmal syndrome.

Transverse and Sigmoid Sinus Dural Arteriovenous Fistula Mimicking Idiopathic Intracranial Hypertension and Carotid Cavernous Fistula.

Intracranial dural arteriovenous fistulas (dAVFs) can produce a variety of symptoms depending on fistula location, size, and venous drainage. Although cavernous sinus fistulas (CCFs) classically present with symptoms of orbital venous congestion due to retrograde venous drainage into the superior ophthalmic vein (i.e. an arterialised "red eye") (Miller NR. Neurosurg Focus 2007;23:1--15), dAVFs not localised to the cavernous sinus rarely present with a "red eye" and instead produce increased intracranial pressure, which can mimic idiopathic intracranial hypertension (IIH). The authors present a unique case of an intracranial dAVF with clinical features suggestive of both CCF and IIH. Clinicians should be aware of this possibility to avoid delayed diagnosis of the intracranial dAVF.

Cup-to-Disc Ratio in Idiopathic Intracranial Hypertension without Papilloedema.

This study seeks to characterise potential differences in the cup-to-disc ratio (CDR) of patients with idiopathic intracranial hypertension (IIH) with papilloedema (IIHWP) compared with IIH without papilloedema (IIHWOP). The medical charts, optical coherence tomography (OCT), and digital optic disc photos of 30 patients (59 eyes) with IIHWP and 4 patients (8 eyes) with IIHWOP were reviewed retrospectively. The CDR values of the two groups were analysed using the Wilcoxon-Mann-Whitney test. Results showed a statistically significant smaller CDR in patients with IIHWP as compared with IIHWOP. These findings suggest that structural factors of the optic disc may play a role in the development of or lack thereof of papilloedema in patients with IIH.

Cat scratch neuroretinitis: the role of acute and convalescent titers for diagnosis.

Cat scratch neuroretinitis (CSN) is a clinical diagnosis supported by serological testing. We present 2 cases of CSN in which initial acute titers were negative or equivocal for Bartonella henselae while convalescent titers were shown to be positive. We report these cases to emphasize that a single acute negative titer is insufficient to exclude the diagnosis of CSN and that convalescent titers should be obtained in patients for whom there is a high clinical suspicion of the disease.

Bilateral pseudo-internuclear ophthalmoplegia in a patient with myasthenia gravis.

PURPOSE: To report a case of myasthenia gravis presenting with a false localizing sign, a bilateral pseudo internuclear ophthalmoplegia. OBSERVATIONS: A 61 year-old male presented with a five-week history of painless binocular oblique diplopia that was associated with fatigue, vocal hoarseness and bilateral ptosis, the latter two of which worsened through the course of the day. Exam was remarkable for an apparent bilateral internuclear ophthalmoplegia (INO). MRI of the brain with and without contrast and MRA of the head and neck were within normal limits. Lab work was positive for anti-AChR binding, blocking, and modulating antibodies, as well as anti-striated muscle and anti-titin antibodies. The patient was initiated on therapy for myasthenia gravis. CONCLUSION AND IMPORTANCE: Although myasthenia gravis often presents with ptosis or diplopia, rarely patients may develop pseudo-INO secondary to extraocular muscle weakness. True INO occurs with damage to the medial longitudinal fasciculus, a myelinated tract of fibers that controls yoked horizontal eye movements. Clinicians should be suspicious of the false localizing sign of a pseudo-INO associated with myasthenia gravis when more common causes of INO have been excluded.

Evaluation and neuroimaging of the Horner syndrome.

OBJECTIVE: To define the efficacy, safety, and cost-effectiveness of a single centre's approach to evaluating Horner syndrome (HS) including a simplified single neuroimaging protocol. DESIGN: Case series study. PARTICIPANTS: Medical records of 34 patients diagnosed with HS at Houston Methodist Hospital (HMH) were reviewed after obtaining Institutional Review Board approval. METHODS: A retrospective chart review was performed for all patients presenting with the diagnosis of HS at the HMH from January 2010 to November 2013. All patients had diagnostic imaging with contrast-enhanced brain magnetic resonance imaging (MRI) extending to the T2 level in the chest. They had either documented causative diagnosis for HS or were "idiopathic." Efficacy and cost-effectiveness of the proposed neuroimaging technique were analyzed compared with other recommended protocols. RESULTS: We initially reviewed 34 charts with presumed diagnosis of HS; 27 charts were included in the analysis. The average age of patients was 46.6 years. Eleven patients (41%) had a final diagnosis of HS secondary to a proven cause, and 16 patients (59%) were diagnosed as "idiopathic." Ten patients (63%) in the idiopathic group had follow-up, and none of those with follow-up had an alternative cause. The estimated cost of our recommended MRI protocol was US$667.76 without magnetic resonance angiography (MRA) or US$1501.71 with MRA. CONCLUSIONS: A single contrast-enhanced brain MRI extending to the T2 level in the chest is an effective and simple means of ruling out life-threatening and other causative factors of HS. Compared with previous imaging recommendations, this proposed protocol may be simpler for clinicians to use and more cost-effective.

Neuro-Ophthalmology Annual Review.

The aim of this study was to update the practicing ophthalmologist on the English-language neuro-ophthalmology literature from the prior year. This study is a review of English-language literature from August 1, 2012, to August 1, 2013. The authors searched PubMed articles published from August 1, 2012, to August 1, 2013, limited to English-language publications including original articles, review articles, and case reports and excluding letters to the editor, unpublished work, and abstracts. We researched the following topics: pupillary abnormalities, eye movement dysfunction, neuromuscular diseases, optic neuropathies, optic neuritis and demyelinating disease including multiple sclerosis, lesions of the chiasm and posterior primary visual pathways, elevated intracranial pressure, tumors and aneurysms affecting the visual pathways, vascular diseases, higher visual functions, and neuroimaging advances. We intend to share clinically relevant literature of the past year with the practicing ophthalmologist. We aimed to highlight remarkable and interesting literature rather than exhaustively including all new neuro-ophthalmological publications of the year. We reviewed literature in the past year with a focus on relevance and novelty. This review updates the comprehensive ophthalmologist on neuro-ophthalmic topics.

Neuro-ophthalmology as a career.

This essay was written to discuss the reasoning behind the personal decisions made by 2 current neuro-ophthalmology fellows to pursue neuro-ophthalmology as a career. It is meant to enlighten the reader about what role neuro-ophthalmologists play in clinical practice, what makes neuro-ophthalmology unique to all other sub-specialties, and how this contributes to making neuro-ophthalmology not only one of the most medically interesting, yet rewarding sub-specialties in ophthalmology.

Controversies: Optic nerve sheath fenestration versus shunt placement for the treatment of idiopathic intracranial hypertension.

BACKGROUND: Idiopathic intracranial hypertension (IIH) has been increasing in prevalence in the past decade, following the obesity epidemic. When medical treatment fails, surgical treatment options must be considered. However, controversy remains as to which surgical procedure is the preferred surgical option - optic nerve sheath fenestration (ONSF) or cerebrospinal fluid (CSF) shunting - for the long-term treatment of this syndrome. PURPOSE: To provide a clinical update of the pros and cons of ONSF versus shunt placement for the treatment of IIH. DESIGN: This was a retrospective review of the current literature in the English language indexed in PubMed. METHODS: The authors conducted a PubMed search using the following terms: Idiopathic IIH, pseudotumor cerebri, ONSF, CSF shunts, vetriculo-peritoneal shunting, and lumbo-peritoneal shunting. The authors included pertinent and significant original articles, review articles, and case reports, which revealed the new aspects and updates in these topics. RESULTS: The treatment of IIH remains controversial and lacks randomized controlled clinical trial data. Treatment of IIH rests with the determination of the severity of IIH-related visual loss and headache. CONCLUSION: The decision for ONSF versus shunting is somewhat institution and surgeon dependent. ONSF is preferred for patients with visual symptoms whereas shunting is reserved for patients with headache. There are positive and negative aspects of both procedures, and a prospective, randomized, controlled trial is needed (currently underway). This article will hopefully be helpful in allowing the reader to make a more informed decision until that time.

Neuro-ophthalmology Annual Review.

PURPOSE: To provide a clinical update of the neuro-ophthalmology literature over the last twelve months. DESIGN: This is an annual review of current literature from August 1, 2011 to August 1, 2012. METHODS: The authors conducted a one year English language neuro-ophthalmology literature search using PubMed from August 1, 2011 to August 1, 2012 using the following search terms: pupil abnormalities, eye movements, diseases of muscle and musculoskeletal junction, optic nerve disorders, optic neuritis and multiple sclerosis, chiasm and posterior primary visual pathway lesions, increased intracranial pressure and related entities, tumors (e.g., meningioma) and aneurysm affecting the visual pathways, vascular diseases, higher visual functions, advances in neuroimaging, and miscellaneous topics in neuro-ophthalmology. The authors included original articles, review articles, and case reports, which revealed the new aspects and updates in neuro-ophthalmology. Letters to the editor, unpublished work, and abstracts were not included in this annual literature review. We propose to update the practicing clinical ophthalmologist on the most clinically relevant literature from the past year. However, this review is not meant to be all-inclusive and highlights only the literature most applicable to the practicing clinical ophthalmologist. RESULTS: We reviewed the literature over the past year in neuro-ophthalmology of potential interest and relevance to the comprehensive ophthalmologist. CONCLUSION: This annual review provides a brief update on a number of neuroophthalmic conditions that might be of interest to the practicing clinical ophthalmologist.

Nonorganic visual loss in children.

NOVL in children is a relatively common problem for the comprehensive ophthalmologist. Although the etiology and pathophysiology of NOVL are variable, rare cases are because of physical or sexual abuse. Clinical tests can make the diagnosis of NOVL, and nonorganic is our preferred terminology for NOVL. Clinicians should be careful to establish a normal eye examination and prove that the vision is better than claimed before labeling a patient with NOVL. Rarely, the clinical examination is insufficient to establish the diagnosis of NOVL and electrophysiology may be helpful. Reassurance is generally all that is necessary for most children with NOVL, but some patients might benefit from formal psychiatric evaluation. The possibility of nonorganic overlay should also be considered in children with NOVL, and the combination of organic and NOVL is relatively common.

Papilloedema due to Chiari I malformation.

The Chiari I malformation is a congenital abnormality characterised by downward displacement of the cerebellar tonsils through the foramen magnum into the cervical spine. It presents clinically most often in young adult women. Known ocular manifestations linked to Chiari I consist primarily of oculomotor paresis with cranial nerve VI palsy and convergence/divergence abnormalities. Papilloedema is a rare manifestation of Chiari I with a clinical presentation often similar to that of idiopathic intracranial hypertension. To highlight this unusual complication, the authors report a 64-year-old female who developed papilloedema as the only presenting neurological symptom resulting from a Chiari I malformation.