Neuro-ophthalmic Manifestations Research Articles

Ocular Manifestations in Patients with HIV Infection/AIDS who were Referred from the ART Centre, Hassan, Karnataka, India

Ocular involvement in HIV infection/AIDS is very common and it includes various clinical presentations which may be asymptomatic or atypical or they may be the initial manifestations of the underlying disease. The severity of these lesions increases as the immune competency decreases, leading to visual impairment or blindness. The purpose of this study was to determine the prevalence and the types of HIV associated ocular conditions and their effect on the vision in patients with HIV/AIDS. This cross sectional study was based on the patients with HIV infection/AIDS, who were referred to the Ophthalmic OPD, District hospital, Hassan. These patients were referred from the District ART Centre for a complete ophthalmic evaluation, irrespective of their immune status and the presence or absence of symptoms. All the patients underwent a complete ophthalmic examination, which included both anterior and posterior segment evaluation and colour vision assessment. Out of the 553 patients, 66% belonged to the age group of 21-40 years. 87% of the patients had a BCVA of >6/18, whereas 4.7% had very poor vision. 37.6% of the patients had ocular manifestations. Anterior segment, posterior segment and neuro-ophthalmic manifestations were seen in 7%, 9.94% and 5.79% of the patients respectively. The most common anterior segment manifestation was recurrent lid infections, while HIV microangiopathy was the most common posterior segment manifestation. The other unusual findings included an abnormal colour vision in 6.3% of the patients and bilateral lid retraction in 8.5% of the patients. Since ocular manifestations are very common and as they can occur at any time during the course of HIV infection, an awareness on various patterns of the ocular disease and the screening of all the patients with HIV infection/AIDS is a must.

Ocular manifestations of HIV/AIDS: A literature review (Part 2)*

This is the second part of an article on the review of published literature on the ocular manifestations of HIV/AIDS. This part deals with the posterior, orbital, neuro-ophthalmic and iatrogenic manifestations of the disease. (S Afr Optom 2011 70(2) 81-88)

Nasopharyngeal cancer mimicking otitic barotrauma in a resource-challenged center: a case report.

IntroductionNasopharyngeal cancer commonly manifests with cervical lymphadenopathy, recurrent epistaxis and progressive nasal obstruction. Neuro-ophthalmic and otologic manifestations can also occur. Isolated otologic presentations of nasopharyngeal cancer are rare and the diagnosis of nasopharyngeal cancer may not be foremost in the list of differentials.Case presentationWe present the case of a 29-year-old Nigerian woman with bilateral conductive hearing loss and tinnitus after air travel. There were no other symptoms. The persistence of the symptoms after adequate treatment for otitic barotrauma necessitated re-evaluation, which led to a diagnosis of nasopharyngeal cancer.ConclusionIsolated otologic manifestations of nasopharyngeal cancer are rare in regions with low incidence of the disease. There is a need for it to be considered as a possible differential in patients presenting with bilateral serous otitis media.

Isolated sixth cranial nerve palsy as the presenting symptom of a rapidly expanding ACTH positive pituitary adenoma: a case report

BackgroundPituitary adenoma may present with neuro-ophthalmic manifestations and, typically, rapid tumor expansion is the result of apoplexy. Herein, we present the first case of an isolated sixth cranial nerve palsy as initial feature of a rapidly expanding ACTH positive silent tumor without apoplexy.Case PresentationA 44 year old female with a history of sarcoidosis presented with an isolated sixth cranial nerve palsy as the initial clinical feature of a rapidly expanding ACTH positive silent pituitary adenoma. The patient underwent emergent transsphenoidal hypophysectomy for this rapidly progressive tumor and subsequently regained complete vision and ocular motility. Despite tumor extension into the cavernous sinus, the other cranial nerves were spared during the initial presentation.ConclusionsThis case illustrates the need to consider a rapidly growing pituitary tumor as a possibility when presented with a rapidly progressive ophthalmoplegia.

Neuro-ophthalmic sarcoidosis

Almost 100 years after its original description, sarcoidosis remains an enigmatic disease with unclear etiology and capricious symptomology, as well as a diagnostic challenge. This review coalesces current literature on the neuro-ophthalmic manifestations of sarcoidosis and discusses the epidemiology, etiology, clinical presentation, diagnosis, and management of this disease. Recent investigations strongly identify a genetic component as well as a host of candidate antigenic triggers. Certain human leukocyte antigen polymorphisms may influence not only the susceptibility of individuals to sarcoidosis but also the course of the disease. Diagnostic advances include the finding of two additional potential biomarkers of sarcoidosis as well as the use of positron emission tomography technology in localization of disease sites for biopsy. In addition to the concomitant and alternative use of immunosuppressive agents to steroid therapy, disease remission in refractory neuro-ophthalmic sarcoidosis with tumor necrosis factor alpha inhibitors has also been reported. Sarcoidosis can affect any part of the visual system; the most common neuro-ophthalmic presentation is optic neuropathy. Diagnosing the disease is problematic as the clinical presentation is nonspecific which may be associated with many other pathologies and no diagnostic finding is pathognomonic. In recent years, progress has been made in identifying new biomarkers and developing imaging techniques. Although corticosteroids remain the mainstay of therapy, many new pharmacological agents have been added to the treatment arsenal.

Orbital Involvement in Bing-Neel Syndrome

Bing-Neel syndrome (BNS) is defined as intracranial involvement of Waldenström macroglobulinemia (WM). Few cases of orbital involvement have been reported. A 51-year-old man with a history of WM developed bilateral orbitopathy and optic neuropathy. Orbital biopsy, cerebrospinal fluid studies, and neuroimaging confirmed the diagnosis of BNS involving the orbital soft tissues, optic nerves, meninges, and cauda equina. The neuro-ophthalmic manifestations resolved after parenteral and intrathecal chemotherapy in addition to autologous stem cell transplantation. The rare neuro-ophthalmic manifestations of BNS may require a multifaceted approach to therapy.

Ophthalmic segment aneurysmal subarachnoid hemorrhage presenting with contralateral abducens nerve palsy: A false localizing sign

Background Neuro-ophthalmic manifestations of aneurysmal subarachnoid hemorrhage are commonly linked to a palsy of the cranial nerve in close proximity to the aneurysm or to chronic intracranial hypertension. Case Report A patient with subarachnoid hemorrhage secondary to a rupture of an intracranial right ophthalmic segment internal carotid artery aneurysm presented with an acute-onset contralateral abducens nerve palsy. There were no clinical or radiologic features of chronic intracranial hypertension. Magnetic resonance imaging showed a co-existent ectatic basilar artery on the left side of the pons in close relation to the sixth nerve. Conclusion Abducens weakness in the above-50 age group is most commonly caused by a vascular etiology. The authors discuss the pathogenesis of this rare clinical entity and present pertinent literature review.

Third Cranial Nerve Palsy as the Presenting Neuro-Ophthalmic Feature of Nasopharyngeal Carcinoma

Nasopharyngeal carcinoma, the most common carcinoma to involve the skull base, may present with neuro-ophthalmic features. Most patients have multiple cranial nerve dysfunction, the fifth and sixth cranial nerves being most often affected (1-3). We report a case that presented with third cranial nerve palsy as the only neuro-ophthalmic feature. A 48-year-old man with no significant past medical history presented to our clinic with a complaint of diplopia and ipsilateral periocular pain of 3 days’ duration. The patient also reported having noticed a mass in the left submandibular area 6 months earlier. Neurologic examination revealed partial right ptosis and complete absence of adduction, supraduction, and infraduction of the right eye. The pupils in low illumination were equal at 4 mm and symmetrically reactive to light and near targets. Visual acuity, ophthalmoscopy, and cranial nerve and motor examination results were normal. Results of the remaining physical examination were within normal limits except for a painless mass over the left submandibular area. All laboratory values and were within the normal ranges. MRI of the brain and nasopharynx showed a large mass centered at the clivus region and spreading into the nasopharynx, invading the basis of the occipital bone, both sphenoid and posterior ethmoid sinuses, the medial part of the right cavernous sinus, and the petrous apex (Fig. 1A-B). The mass enhanced heterogeneously. The superior and inferior orbital fissures, optic nerves, and other intraorbital structures were spared. Significant bilateral lymphadenopathy of the neck was evident and some lymph nodes showed hypodense centers indicative of necrosis. A digital subtraction angiogram revealed no vascular abnormalities.FIG. 1: A. T2 axial MRI shows a tumor with mixed signal intensity that is invading the sphenoid and posterior ethmoid sinuses. B. Postcontrast coronal MRI shows an enhancing tumor centered at the clivus with partial right cavernous sinus invasion (arrow).Biopsy of the nasopharyngeal mass revealed a nonkeratinizing differentiated carcinoma (Fig. 2). The patient was referred to the oncology department for radiotherapy and chemotherapy.FIG. 2: Histopathology of a nasopharyngeal punch biopsy shows fibrous connective tissue with infiltrating cords of anaplastic cells (hematoxylin and eosin, 340).The third cranial nerve paralysis remained stable without improvement, and no other neurologic symptoms had occurred after 3 months. A retrospective study of 79 patients with nasopharyngeal carcinomas (4) disclosed that one quarter of these patients have neuro-ophthalmic manifestations. In a group of 564 patients with nasopharyngeal carcinomas (1), cranial nerve dysfunction was present in 12%. In 92% of the patients, neurologic deficits were confined exclusively to cranial nerves. Another study (5) showed that the most frequently affected cranial nerves were the fifth and sixth. Our patient is unusual in that the third cranial nerve was the only one involved. The extent of the tumor on MRI fails to indicate why the third cranial nerve was the only affected cranial nerve. Yesim Yetimalar Beckmann, MD Benian Deniz, MD Department of Neurology Atatürk Training and Research Hospital Izmir, Turkey [email protected] Fazil Gelal, MD Department of Radiology AtatÜrk Training and Research Hospital Izmir, Turkey Yaprak Seçil, MD Department of Neurology Atatürk Training and Research Hospital Izmir, Turkey

Neuro-ophthalmic manifestations of a complicated cholesteatoma

Background A cholesteatoma, also known as an epithelioma, is a mass of soft tissue that results from the accumulation of keratin protein debris enclosed by a layer of stratified squamous epithelium. Case Report A patient with cholesteatoma surrounding both internal carotid arteries presented with cerebral ischemic symptoms expressed as visual and ocular symptoms. Because of its location, mass effect, and potential stenosis of the internal carotids, this slow-growing tumor is potentially sight threatening. Asymmetric cupping of the optic nerves associated with symptoms such as dizziness, diplopia, or facial pain should be worked up carefully. Conclusion The primary care optometrist can play an important role in the diagnosis and continued management of a patient with a cholesteatoma, carefully monitoring early signs of progression or change. This case illustrates how an intracranial lesion, away from the eye, can impact a number of ocular and visual functions.

Neuro-ophthalmic manifestations of the syndrome of ophthalmoplegia, ataxia and areflexia: a review.

Controversy regarding the nosological position of the syndrome of ophthalmoplegia, ataxia and areflexia (Miller Fisher syndrome) exists. The oculomotor dysfunction was presumed to represent an unusually symmetrical peripheral cranial nerve dysfunction. To investigate the neuro-ophthalmic manifestations in this rare syndrome we reviewed 109 reports describing 243 cases. The ophthalmoplegia was remarkable in its constant association with a cerebellar type ataxia. It was described to be remarkably symmetrical at all stages of development and recovery. From the early description of the syndrome by Fisher the ophthalmoplegia was observed to evolve as a symmetrical failure of upgaze followed by loss of lateral gaze and last by downgaze, recovery develops in the opposite pattern. Despite the severe nature of the ophthalmoplegia, 58 patients were reported to have sparing of downgaze and 192 (79%) had relative sparing of the eye lids. Active lid retraction and preserved Bell's phenomenon, despite upgaze paralysis, were described in 22 and 15 patients respectively. Upper lid jerks were described in 2, Parinaud's syndrome in 2, convergence spasm in 6, internuclear ophthalmoplegia in 15 and horizontal dissociated nystagmus in 11. Interestingly 23 were reported to present with paralysis of abduction progressing to lateral gaze paralysis and 5 had paralysis of abduction and contralateral gaze paralysis. Four had defective vestibulo-ocular reflex despite recovery of upgaze, 10 had central type nystagmus including rotatory, retractory and rebound nystagmus. Relative preservation of optokinetic nystagmus and preservation of vestibulo-ocular reflex despite an otherwise complete ophthalmoplegia were reported in 6 and 2 patients respectively.(ABSTRACT TRUNCATED AT 250 WORDS)

Neuro-ophthalmic manifestation of nasopharyngeal carcinoma at Ilorin: a five year review.

Patients with Nasopharyngeal Carcinoma (NPC) may present with neuro-ophthalmic symptoms including visual loss. Involvement of the cranial nerves have been found to impact significantly on the individual's 5 year survival rate, which makes an early diagnosis of this condition of great importance in the management of NPC. A retrospective review of all patients with clinical and histological diagnosis of NPC over a five year period between 1999 and 2003 was carried out. Information retrieved from the records include their age, symptoms and signs with emphases on neuro-ophthalmic at presentation. Data were analysed using SPSS statistical package. Twenty records out of the 23 patients with a diagnosis of NPC seen during this period were available for review. The age range was between 20 and 60 years, the Mean was 38.9 years, SD 11.62. Sixty percent of the patients had neuroophthalmic manifestation with symptoms such as ocular pain, double vision, loss of vision and eye protrusion and signs such as ophthalmoplegia, exposure keratopathy and proptosis in various combinations. Neuro-ophthalmic manifestations were commonly found among patients diagnosed as NPC at Ilorin. A high index of suspicion of NPC whenever a patient presents with neuro-ophthalmic signs and symptom is advocated.

Neuroophthalmological findings of sarcoidosis and Vogt‐Koyanagi‐Harada syndrome

AbstractBesides common ocular manifestations neurologic involvement including optic nerve disease, cranial nerve palsies and disorders of the hypothalamus and pituitary gland was also identified in 12% of patients with sarcoidosis. Facial nerve palsy is the most common but usually self‐limited neurological manifestation. Optic nerve involvement is the most common neuroophthalmic manifestation. Papilledema, compressive, ischemic, or neuritic optic neuropathy due to granulamatous infiltration of any part of the optic nerve may be seen. Lacrimal gland involvement is also common and is usually asymptomatic. Extensive granulomas may lead to diplopia. Orbital symptoms may mimic other inflammatory syndromes. Like sarcoidosis Vogt‐Koyanagi Harada is a disorder characterized with bilateral, granulomatous panuveitis associated with central nervous system and auditory manifestations. At prodromal stage lumbar puncture reveals a pleocystosis, ultrasonography shows diffuse thickening of the choroid tissue. At this stage patients typically report severe headaches, nausea, meningismus, dysacusia, tinnitus, and may even have fever, orbital pain and photophobia. Prodromal phase usually lasts for several days, which may persist for several weeks. It is followed by acute uveitic phase, characterized by bilateral choroiditis, vitritis and papillitis. After that chronic phase is seen with vitiligo, poliosis and resolution of exudative retinal detachments. Neuroophthalmological manifestations may help in diagnosis of rare disorders like Sarcoidosis and VKH syndrome.

Neuro-ophthalmic Sarcoidosis: The University of Iowa Experience

Purpose: To report a case series of neuro-ophthalmic sarcoidosis manifestations from a predominantly Caucasian Midwest population. Design: Retrospective non-comparative case series and literature review. Participants: Twenty patients with biopsy proven sarcoidosis cases and neuro-ophthalmic manifestations. Methods: We reviewed 67 consecutive charts with the clinical diagnosis of neurosarcoidosis at the University of Iowa Hospital and Clinics (UIHC) Department of Ophthalmology database in Iowa City, Iowa, seen from 1984 to 2006. Main Outcome Measures: Charts were reviewed for the following: 1) demographic information; 2) neuro-ophthalmic findings; 3) biopsy location and results; 4) pre-existing sarcoidosis; 5) neuroimaging studies (e.g., cranial magnetic resonance imaging and computed tomography scans); 6) cerebrospinal fluid results; 7) sarcoid related testing (serum angiotensin converting enzyme, chest radiograph, chest computed tomography scans, Gallium scan, bronchoalveolar lavage, pulmonary function testing); 8) treatment; and 9) course of disease. Results: Twenty of the 67 charts (30%) had biopsy proven sarcoidosis and neuro-ophthalmic manifestations. Of the 20 included cases, 4 (20%) were men and 16 (80%) were women. Six (30%) patients were African-American and 14 (70%) were Caucasian. The average age at diagnosis was 43.1 years with a standard deviation of 14.1 and a range of 22 to 80 years. Neuro-ophthalmic manifestations included optic neuropathy (14), cranial neuropathy (4), Horner's Syndrome (1), tonic pupil (1), and optic tract involvement (1). Of the 14 patients presenting with optic neuropathy, 8 had optic disc edema, 5 had optic disc pallor and 1 had an optic disc granuloma. Contrast cranial magnetic resonance imaging (MRI) showed pathologic contrast enhancement (16 of 19 cases) involving optic nerve (9), optic chiasm (1), optic radiations (1), cavernous sinus (1), leptomeninges (3), and cerebral parenchyma (3). Chest imaging was abnormal in the course of disease for 12 of 18 and serum angiotensin-converting enzyme was only elevated in 5 of 15 patients tested. All 20 patients were treated with corticosteroids but five required additional immunosuppressive therapy to control disease activity. The neuro-ophthalmic course was relapsing and remitting in 8 cases, stable or resolved in 7, and chronic in 5 patients. After treatment of patients with optic neuropathy, visual acuity at last follow-up visit was improved in 5, worsened in 5, and stable (i.e., within one Snellen acuity line of baseline) in 4. Conclusion: In our Midwest retrospective case series of biopsy proven neuro-ophthalmic sarcoidosis, patients were predominately white females with a wide age range. Consideration for the diagnosis of neurosarcoidosis should therefore not be limited by age, gender, or race. Optic neuropathy was the most common manifestation, typically presenting with optic disc edema and severe visual loss. No light perception vision was relatively common and should be considered a “red flag” for the diagnosis. Contrast cranial MRI frequently shows pathologic enhancement of the visual pathway. Serum angiotensin converting enzyme and chest radiography had relatively poor sensitivity for detecting biopsy proven disease in our study and therefore additional testing for tissue diagnosis might still be necessary for extrapulmonary neuro-ophthalmic sarcoidosis. Corticosteroids are the mainstay of therapy but some patients may require additional immunosuppressive therapy.

Ophthalmic Manifestations of HIV Infections in India in the Era of HAART: Analysis of 100 Consecutive Patients Evaluated at a Tertiary Eye Care Center in India

Purpose: To evaluate ophthalmic manifestations in patients with Human Immunodeficiency Virus (HIV) infection in the era of highly active antiretroviral therapy (HAART) at the apex institute for eye healthcare in India. Method: This prospective study was undertaken between October 2004 and December 2005. A complete ophthalmological and systemic examination was performed on each patient. Relevant investigations were carried out in selected patients. Results: One hundred consecutive HIV infected patients (199 eyes) were examined for ophthalmic manifestations. Of these 17% (17/100) had Category A HIV infection (asymptomatic or acute HIV or persistent generalized lymphadenopathy), 23%(23/100) had Category B HIV infection (symptomatic, not A or C), 60%(60/100) had Category C HIV infection (AIDS indicator condition).76%(70/100) were male and 24%(24/100) were female. The median age of patients was 34 years and 52%(52/100) were in the fourth decade. 68%(68/100) patients were on HAART. 45% (45/100) patients had ophthalmic manifestations, the most common being cytomegalovirus (CMV) retinitis (20%) (20/100). Retinal detachment was seen in 70% (14/20) of CMV retinitis patients. HIV vasculopathy was seen in 11% (11/100) of patients. Other lesions included immune recovery uveitis (IRU) (5%)(5/100), acute retinal necrosis (ARN) (3%)(3/100), choroiditis (2%)(2/100), neuro-ophthalmic manifestations (12%)(12/100), complicated cataract (6%)(6/100), keratouveitis (1%)(1/100) and corneal ulcer (1%)(1/100). 7%(7/100) patients presented to us with ophthalmic manifestation as the only presenting sign of HIV infection. Amongst those who had ophthalmic involvement, about 50% (19/40) patients had CD4 count below 100 cells/micro liter and 70% (28/40) patients had CD4 count below 200 cells/micro liter. Conclusions: CMV Retinitis (20%) (20/100) is still the most common manifestation of HIV infection in this series, even in the era of HAART, and is more common than HIV vasculopathy. Immune recovery uveitis is appears to be more common with the introduction of HAART in absence of affordable anti CMV therapy in India. 7% (7/100) of patients present with ophthalmological features as the initial manifestation of HIV. As before, most (70%) (28/40) of the ophthalmic manifestations of HIV infection are present when CD4 count is less than 200 cells/micro liter.

Mitochondrial disorders with significant ophthalmic manifestations

Mitochondrial diseases are a clinically hetyerogenous group of disorders. They can be caused by mutations of nuclear or mitochondrial DNA (mtDNA). Some affect a single organ, but many involve multiple organ systems and often present with prominent neurologic and myopathic features. The eye is frequently affected, along with muscles and brain, but multisystem disease is common. Ophthalmic manifestations include cataract, retinopathy, optic atrophy, cortical visual loss, ptosis and ophthalmoplegia. Kearns-Sayre Syndrome (KSS), Mitochondrial Encephalopathy, Lactic Acidosis Stroke (MELAS), Myoclonic Epilepsy and Ragged Red Fiber myopathy (MERRF) and Lebers Hereditary Optic Neuropathy (LHON) are well known clinical entities that are secondary to mtDNA abnormalities, which has ophthalmic manifestations. Mitochondrial Dysfunction should be considered in the differential diagnosis of progressive multisystem disorder and specifically if there is associated neuro-ophthalmic manifestations, which may be the presenting symptom of these disorders.

Bilateral Abducens Palsies and Facial Weakness as Initial Manifestations of a Chiari 1 Malformation

Chiari I malformations are rare, congenital anomalies involving the caudal herniation of the cerebellar tonsils into the upper cervical spinal canal. Osseous abnormalities of the skull permit the extension of the hindbrain below the foramen magnum and engender syringohydromyelia formation. Neuroophthalmic manifestations are not uncommon; nevertheless, this is the first report of concomitant bilateral abducens palsies and facial nerve impairment as presenting manifestations of a Chiari I malformation. A 30-year-old black female presented with a recent history of blurred vision and binocular diplopia in both left and right gazes. Headaches, dizziness, and orofacial sensorimotor impairments were noted in conjunction with the onset of her diplopia. No history of surgery, trauma, or systemic illness was elicited. Extraocular motility testing confirmed a complete bilateral abduction deficit. Additional cranial nerve testing revealed bilateral facial nerve weakness and tactile hypesthesia in the perioral region. No taste disturbances were reported. Nuclear magnetic resonance imaging studies revealed a Chiari I malformation with a syringohydromyelia formation in the vicinity of the patient's sixth cervical vertebrae. Foramen magnum decompression surgery was performed. Two months after surgery, abduction capacity was restored along with resolution of symptoms of perioral numbness and signs of facial weakness. Bilateral sixth nerve palsies are rare clinical commodities especially when they do not occur in isolation. Intracranial neoplams, trauma, subarachnoid hemorrhaging, demyelinating disease, and meningeal infarcts have all been shown to induce mixed cranial neuropathies. The concomitant bilateral abducens palsy and concomitant facial nerve weakness reported in this case represents an atypical and previously unreported presentation of a Chiari I malformation.

Lymphoplasmacyte-Rich Meningioma Mimicking Idiopathic Hypertrophic Pachymeningitis

A 24-year-old man presented with long-term headache and progressive visual loss. Neuro-ophthalmic manifestations included finger counting acuity in both eyes, weakly reactive pupils, pale optic discs, and increased deep tendon reflexes. Brain MRI showed meningeal thickening that involved the optic nerves and chiasm and enveloped and displaced the brainstem as far caudally as the foramen magnum. The diffuse extensive nature of the lesion suggested an inflammatory process such as idiopathic hypertrophic pachymeningitis (IHP), but anterior temporal brain biopsy disclosed a relatively high proportion of meningothelial cells with islands of polyclonal inflammatory reaction consistent with a diagnosis of lymphoplasmacyte-rich meningioma (LRM), a rare variant. Among the 19 reported cases of LRM, none has shown as extensive a mass as seen in our patient. Distinguishing between LRM and IHP is important because these entities are treated differently.

Intracranial aneurysms

To review the neuroophthalmic manifestations of cavernous and posterior communicating artery aneurysms as well as the diagnosis and treatment options for patients with these kinds of aneurysms. The natural history of cavernous aneurysms has recently been systematically followed. Comparison of coiling and clipping for posterior communicating artery aneurysms has yielded new suggestions on how to best treat these patients. The review discusses how to follow these cases, diagnose and treat, and assess for complications after treatment decisions have been made.

Concurrent Intermediate Uveitis and an Enhancing Intracranial Lesion as the Initial Manifestation of Sarcoidosis

Posterior segment involvement has been described to be associated with central nervous system involvement in sarcoidosis as a result of direct sarcoid tissue infiltration or mass effect of a cerebral lesion. However, isolated intermediate uveitis occurring concurrently with central nervous system involvement prior to extensive systemic disease is rare. We describe a patient with neuro-ophthalmic manifestations of intermediate uveitis and an enhancing basal ganglia lesion at initial presentation, in the absence of extensive systemic disease. He was treated with high-dose systemic steroids which was progressively tailed down over 6 months. There was prompt resolution of vitritis with good preservation of visual acuity. The difficulties of the initial diagnosis of sarcoidosis and the indications for initiation of steroid therapy are illustrated. We use this case to emphasise the need for a high clinical suspicion of sarcoidosis in the presence of similar unusual and seemingly unrelated combinations of neurological manifestations so as to facilitate the prompt institution of appropriate treatment when indicated.

Neuro-Ophthalmic Manifestations of Intracranial Cavernous Hemangiomas

To describe the neuro-ophthalmic manifestations of patients with intracranial cavernous hemangiomas (cavernomas). A retrospective review of all patients with intracranial cavernomas with neuroophthalmic manifestations who were treated at the Royal Adelaide Hospital in Australia between 1994 and 2004. There were nine patients (three men and six women), with a mean age of 39 years (range 22-61). There was one cerebellar lesion, two thalamic, four pontine, one midbrain/pontine, and one midbrain. Ophthalmic presentations included internuclear ophthalmoplegia (one patient), third cranial nerve (CN) palsy (one patient), fourth CN palsy (one patient), and sixth CN palsy (six patients). Three patients underwent extraocular muscle surgery, and six were treated medically or observed. In five patients the diplopia resolved, in three it was only mild, and in one patient no significant change was noted during the follow-up period. Diplopia is the main neuro-ophthalmic manifestation of intracranial cavernomas. Sixth CN palsy is the most common cause. Neurosurgical or conservative treatment leads to improvement in most cases, and later use of spectacles or extraocular muscle surgery may lead to further improvement.