Nafiseh Hashemi

Late‐onset Leber hereditary optic neuropathy

While Leber hereditary optic neuropathy typically causes bilateral visual loss in the second through fourth decades, we highlight visual loss from Leber hereditary optic neuropathy in older patients to characterize the clinical features of this cohort.

Leber Hereditary Optic Neuropathy Mimicking Thyroid-related Optic Neuropathy

this, we carefully reviewed the fluorescein angiogram and found in the early phase of the study that there was good choroidal filling (Fig. 1). In the Photo Essay, Figure 2A shows a later stage of the angiogram when filling of the choroid was complete, yet there was no perfusion of the retinal vessels. Furthermore, the appearance of the vascular occlusion in Figure 2A is actually more suggestive of an occlusive vasculitis than a CRAO caused by emboli, adding further support for the diagnosis of Behçet disease (BD). Second, regarding the issue of diagnostic criteria for BD, our patient admitted to recurrent oral ulcers more than 5 times per year. Finally, we agree that CRAO and recurrent papillitis are not listed as minor features in the diagnostic criteria of BD (2), but “vasculitis” and “central nervous system symptoms” are included. If we accept that the vascular occlusion observed in each eye of our patient was caused by vasculitis and that the optic nerve is part of the central nervous system, then we have 2 minor features of BD. While recurrent oral ulcers are a major feature, it is noteworthy that diagnostic criteria for BD vary and have been revised many times. The International Study Group for BD proposed that the diagnosis be considered when recurrent oral ulcers plus 2 other features are present, in the absence of other clinical explanations (3). In our patient, we performed a wide range of ancillary tests to exclude other disorders, which can cause an obliterative vasculitis. Most importantly, we wish to remind clinicians that CRAO and recurrent papillitis can be caused by a vasculitis, such as BD. Prompt treatment can be helpful to prevent further loss of visual function.

Visual hallucinations (Charles Bonnet syndrome) associated with neurosarcoidosis

The Charles Bonnet syndrome (CBS) refers to lucid and complex visual hallucinations in cognitively normal patients with acquired vision loss. It can be associated with any type of vision loss including that related to macular degeneration, corneal disease, diabetic retinopathy, and occipital infarct. Neurosarcoidosis, a multi-systemic inflammatory granulomatous disease affecting both the central and peripheral nervous systems, is rarely associated with CBS. We report a patient with biopsy-confirmed neurosarcoidosis who experienced visual hallucinations following the development of a right seventh-nerve palsy, right facial paresthesia, and bilateral progressive visual loss. This case highlights the importance of recognizing that the CBS can occur in visual loss of any etiology.

Neuroimaging in ophthalmology

In the past three decades, there have been countless advances in imaging modalities that have revolutionized evaluation, management, and treatment of neuro-ophthalmic disorders. Non-invasive approaches for early detection and monitoring of treatments have decreased morbidity and mortality. Understanding of basic methods of imaging techniques and choice of imaging modalities in cases encountered in neuro-ophthalmology clinic is critical for proper evaluation of patients. Two main imaging modalities that are often used are computed tomography (CT) and magnetic resonance imaging (MRI). However, variations of these modalities and appropriate location of imaging must be considered in each clinical scenario. In this article, we review and summarize the best neuroimaging studies for specific neuro-ophthalmic indications and the diagnostic radiographic findings for important clinical entities.

A pox upon your house

Herpes zoster ophthalmicus (HZO) is a common viral infectious disorder affecting the ophthalmic division of the trigeminal nerve. A small subset of HZO patients present with the ophthalmic symptoms, but without an accompanied rash, a condition described as Herpes zoster sine herpete. Although HZO is well known to be associated with other central nervous system abnormalities, encephalitis and cerebral infarction are atypical and uncommon. We report an unusual case of presumed unilateral Herpes zoster ophthalmicus sine herpete that presented with trigeminal pain and uveitis and then progressed to encephalitis and bilateral cerebral infarctions despite treatment with acyclovir and corticosteroids. The diagnosis of HZV was confirmed by polymerase chain reaction testing on the cerebrospinal fluid.

Throat pain as a presenting symptom of giant cell arteritis.

W e read with great interest the state-of-the-art review on giant cell arteritis (GCA) by Weyand (1). We recently saw a 76-year-old man who presented with severe throat pain to the point where he had difficulty swallowing. He was treated with doxycycline for “strep throat”, although swabs and culture were not performed. He failed to improve and developed hoarseness and unintentional weight loss of 10–15 pounds. Three months after developing a sore throat, the patient experienced jaw claudication without headache, scalp tenderness, or night sweats. Two months later, he had several episodes of transient monocular vision loss in the left eye, reporting “snowflakes” in the superior left visual field. Five days later, he experienced abrupt vision loss in the left eye. His medical and surgical histories, medication use, and review of systems otherwise were negative. Neuro-ophthalmic examination revealed visual acuity of 20/40, right eye, because of a cataract, and hand motions, left eye. There was a left relative afferent pupillary defect, ocular motility was normal, and funduscopy showed right optic disc hyperemia and the left disc had pallid edema. Fluorescein angiography confirmed bilateral optic disc leakage while choroidal and retinal filling times were normal. Erythrocyte sedimentation rate (ESR) was 84 mm/h. The patient was treated with intravenous methylprednisolone (1,000 mg) and then switched to 80 mg of oral prednisone per day. A temporal artery biopsy revealed chronic inflammation, histiocytes, and giant cells within the vessel wall with destruction of the internal elastic lamina. Over the next 2 weeks, the patient’s sore throat, hoarseness, and jaw claudication resolved. His visual acuity remained unchanged and after 6 weeks of treatment, his ESR was 5 mm/h, and he was clinically stable. Our patient presented with respiratory tract symptoms that, we believe, were due to inflammation of branches of external carotid arteries (ascending pharyngeal arteries) causing reversible ischemic injury to pharyngeal tissue. Larson et al (2) reported that 9% of GCA patients present with prominent respiratory symptoms, such as cough, sore throat, and hoarseness, whereas respiratory tract symptoms are the initial complaints among 4% of the patients with GCA (3). These symptoms usually resolve quickly with steroid treatment. Other respiratory symptoms of GCA include nonproductive cough, changes in voice, fatigue and pain in the tongue, and dyspnea with orthopnea (3–6). Aortitis might also produce hoarseness in GCA from involvement of the recurrent laryngeal nerve (7). Our patient’s initial presentation of isolated throat pain resulting from GCA is unusual (8,9). Subsequently, he developed more classic manifestations of the disorder, including jaw claudication and arteritic anterior ischemic optic neuropathy. Our case serves as a reminder to the clinician of the diverse clinical presentations of GCA.

Magnetic Resonance Imaging in Acute Unilateral Optic Neuropathies in Adults

Non-arteritic ischaemic optic neuropathy (NAION) and optic neuritis may have overlapping presentations, making differentiation between the two difficult. All adults presenting with acute unilateral optic neuropathy (symptoms <3 weeks of onset) between January 2011 and January 2012 underwent complete neuro-ophthalmic examinations and were diagnosed with either NAION or ON. Patients underwent pre- and post-contrast magnetic resonance (MR) with gadolinium, diffusion-weighed imaging (DWI), T2, short tau inversion recovery (STIR), and gadolinium-enhanced fast imaging employing steady state acquisition (FIESTA) sequences of the brain and orbit. A blinded neuroradiologist analysed the images as normal, consistent with NAION, consistent with ON, or unrelated incidental findings and the results were correlated to clinical diagnoses on initial presentation. Sixteen patients were included, 4 of whom had optic neuritis and 12 had NAION. Concordance rate for the blinded neuroradiological diagnosis and the final clinical diagnosis was seen in only 3 of the 16 patients. Nonspecific or unrelated findings were seen in 6 cases. Our study demonstrates a low concordance rate between radiographic and final clinical diagnoses, with only 3/16 patients correctly diagnosed by blinded neuroradiological evaluation. Despite the limitations of our study, we believe that neuroradiologists can assist clinicians in the final diagnosis of in patients in whom the clinical presentation is uncertain. Future work is necessary to determine whether more rapid imaging in these cases might expand the utility of DWI in optic neuropathy.

Visual hallucinations: A review for ophthalmologists

Visual hallucinations (VH), as opposed to illusions (which are misperception of a real visual stimulus), are a false sense of perception without external visual stimulation. The content of VH is highly variable and can range from simple unformed images (e.g., flashing lights or steady spots and colored lines) or shapes (geometric hallucinations) to formed hallucinations (e.g., vivid objects, flowers, animals or even people). In this review, we describe the common etiologies and the differentiating features for VH of interest to ophthalmologists.