Neuro-ophthalmic manifestations of sarcoidosis: clinical spectrum, evaluation, and management.

OBJECTIVE: To familiarize the reader with the neuro-ophthalmic manifestations of sarcoidosis. MATERIALS AND METHODS: All patients underwent systemic evaluations (chest radiograph, magnetic resonance imaging and/or computed tomography, serum angiotensin-converting enzyme level, and gallium scan). Histologic confirmation was preferred (11 of 15 patients underwent biopsy, ten of whom [82%] had positive biopsies, and four refused). Otherwise, the diagnosis of clinical sarcoidosis was based on laboratory evaluation. RESULTS: We report our experience with 15 patients who had neuro-ophthalmic manifestations of sarcoidosis other than optic neuropathy or chiasmal disease. Eight of 15 (53%) did not have known sarcoidosis at the time of presentation. Thirteen of 15 (87%) patients demonstrated lesions consistent with sarcoidosis on magnetic resonance imaging of the brain. Treatment with corticosteroids and/or other immunomodulatory agents was necessary in all cases. CONCLUSIONS: Neuro-ophthalmic manifestations of sarcoidosis are rare. They may be the presenting signs of otherwise occult disease. Suspicion and inclusion in the differential are a key to establishing the diagnosis. A strategy for the detection and evaluation of these cases is presented.

Reversible blindness resulting from optic chiasmitis secondary to systemic lupus erythematosus.

OBJECTIVE: To report the diagnosis, radiologic findings, and therapy of a 51-year-old female with systemic lupus erythematosus (SLE) who, while on hydroxychloroquine maintenance therapy, presented with a junctional scotoma indicative of chiasmal disease. This visual loss developed after she had been tapered off corticosteroids. MATERIALS AND METHODS: An interventional case report of a female that was given acute therapy with 1-gram daily of intravenous methylprednisolone sodium succinate for 5 days, followed by maintenance methotrexate and a slow taper of oral prednisone. Magnetic resonance imaging (MRI) scans, visual acuity, color vision, and threshold visual fields were performed. RESULTS: The MRI scan showed chiasmal involvement, which may occur in SLE in absence of any other evidence of systemic activity. Therapy led to visual function returning to 20/20 OD and 20/20 OS, with normal Ishihara plates OU and only minimal paracentral depressions OU. She has been able to be weaned off prednisone while on methotrexate maintenance. CONCLUSIONS: Chiasmal involvement may occur in SLE in absence of any other evidence of systemic activity. Maintenance with hydroxychloroquine may not be adequate to prevent this rare cause of visual loss in SLE. Aggressive therapy of chiasmal involvement in SLE, even when the visual loss is profound, may lead to visual restoration, which was virtually complete in this case. Methotrexate may be an alternate agent for patients who break through with optic neuropathy while on hydroxychloroquine.

Neuro-ophthalmologic manifestations of a paraneoplastic syndrome and testicular carcinoma.

The authors report two patients with testicular cancer who exhibited supranuclear gaze disorders as a manifestation of a paraneoplastic brainstem encephalomyelitis. In the first patient, neuro-ophthalmic dysfunction was accompanied by a prominent limbic encephalitis whereas in the second patient, an unusual, mixed pendular and jerk nystagmus was manifested. Neuroimaging revealed an enhancing hypothalamic mass in the first patient and was negative in the second. Blood from both patients contained an antibody previously reported in a patient with limbic encephalitis and testicular cancer.

Annual review of systemic diseases: 1995-1996. Part I.

Systemic illnesses, such as vasculitides and systemic lupus erythematosus, may have ocular symptoms, or they may present with ocular signs and undiagnosed or occult systemic signs. The recently described ophthalmic and neurologic presentations and new diagnostic modalities for Wegener's granulomatosis, giant cell arteritis, and systemic lupus erythematosus are reviewed. Hypercoagulable states and their role in visual diseases are assessed, and strategies for evaluating patients with hypercoagulable states are described.

Optic chiasmal compression by venous aneurysm: magnetic resonance imaging diagnosis.

The overwhelming majority of traumatic carotid cavernous fistulas present with a bruit, arterialization of conjunctival vessels, or an elevated intraocular pressure. Described herein is a patient who presented with progressive deterioration of vision, without any of the above signs. Magnetic resonance imaging demonstrated compression of the optic ch asm by a venous aneurysm arising from a carotid cavernous fistula. This entity should be considered in patients who present with progressive visual loss.

Homonymous field defect as the first manifestation of Creutzfeldt-Jakob disease.

PURPOSE: Although in the early stage of Creutzfeldt-Jakob disease most patients have obvious dementia, we found that the disease can be diagnosed in patients solely on the basis of a visual system disorder. METHODS: We examined three patients who initially complained of a nonspecific, insidious visual disturbance. RESULTS: The three patients were found to have Creutzfeldt-Jakob disease, confirmed by histopathologic analysis. Each patient had a homonymous hemianopsia on the first neuro-ophthalmologic examination. The initial neurologic, neuroimaging, and electrophysiologic examinations were not conclusive. The subsequent rapid deterioration in the neurologic status, including dementia and typical electroencephalographic changes, was suggestive of Creutzfeldt-Jakob disease. CONCLUSION: At onset of Creutzfeldt-Jakob disease, patients may have a homonymous hemianopsia despite normal results of magnetic resonance imaging of the brain and neurologic examination.

Dysgammaglobulinemia in steroid-dependent optic neuritis: response to gammaglobulin treatment.

At the age of 12, a prematurely born boy with an otherwise unremarkable past medical history developed bilateral optic neuritis associated with transverse myelopathy. Over the ensuing 3 years, recurrent bouts of optic neuritis OU, with dyschromatopsia, and acuity and field loss (arcuate, central, and paracentral scotomas) were controlled with increasing doses of corticosteroids. However, the patient became steroid-dependent and experienced recurrent optic neuritis during multiple attempts at tapering the steroids. He developed optic atrophy and steroid complications, including cushingnoid features and growth maturation delay. Immunoglobulin G subclass 2 and 3 deficiencies were the only serologically detectable abnormalities. Administration of intravenous gammaglobulin (25 g monthly) allowed discontinuation of steroids without further ophthalmic or neurologic disease. Following steroid withdrawal and institution of gammaglobulin, the patient grew 6 inches within 2 years, regaining his vision, retrieving his stature, and normalizing his psychosocial development.

Low-dose cyclosporine therapy of granulomatous optic neuropathy and orbitopathy.

Four patients with granulomatous disease of the anterior visual pathway presented with optic neuropathy between July 1986 and February 1987, secondary to an orbital pseudotumor (n = 1) and sarcoidosis (n = 3). All but one became resistant to corticosteroid therapy, and all were subsequently treated for 1 to 2 years with low-dose cyclosporine alone or in combination with tapering doses of prednisone. Two patients experienced pronounced recovery of visual function, and visual function was stabilized in the other two; in one of these, magnetic resonance imaging showed substantial regression of intracranial lesions. Steroid-induced complications of uncontrolled hyperglycemia (n = 2) and obesity (n = 4) were controlled with cyclosporine. The authors conclude that low-dose cyclosporine is a safe and effective therapeutic alternative in ocular granulomatous diseases.

Temporal arteritis in blacks.

Clinicians should be aware that temporal arteritis in blacks has a clinical presentation similar to that found in the white population. Heightened clinical awareness of the possibility of temporal arteritis in black patients should lead to earlier diagnosis and initiation of immunosuppressive therapy, thus helping prevent the severe sequela of blindness that has been documented in about half of the cases of untreated temporal arteritis.

Long-term results: botulinum for sixth nerve palsy.

Eight patients with intracranial malignancies or vascular lesions and sixth nerve palsies were treated with botulinum toxin chemodenervation of the antagonist medial rectus muscle. Primary deviation ranged from 20 to 75 prism diopters (pd) of esotropia. Six were treated acutely (within 3 months of onset) and two, which demonstrated partial recovery of lateral rectus function but with residual esotropia and diplopia, were treated after 6 months. After a mean follow-up of 20.6 months, seven were diplopia-free with excellent rotations. Five had complete resolution of the esotropia and diplopia, with near complete recovery of abduction. One had 6 pd residual esotropia, while another, whose sixth nerve had been resected, required a modified Jensen procedure, resulting in full rotations. The single case of bilateral sixth nerve palsy had a functional improvement but was lost to follow-up. One patient had a vertical strabismus induced with the injection and had a gradual return of the esotropia.

Autoimmune optic neuropathy: evaluation and treatment.

Fourteen patients, 12 of whom were women, with an age range from 26 to 56 years, presented with progressive or recurrent optic neuropathy, despite conventional doses of corticosteroid, and laboratory evidence of collagen vascular disease. The visual loss was severe and most had an acuity less than 20/200. Megadose corticosteroid therapy improved the vision in 11 of the 12 patients. Continued oral prednisone and cytotoxic drugs were necessary to maintain vision in nine patients. Patients with autoimmune optic neuropathy must be differentiated from cases with idiopathic optic neuritis or multiple sclerosis to facilitate the appropriate therapy.

Visual system toxicity following intra-arterial chemotherapy.

We studied the effects of intra-arterial chemotherapy on the visual system of 29 consecutive patients with gliomas. As expected, infra-ophthalmic carotid infusion of cisplatin or carmustine (BCNU) was associated with clinically apparent anterior visual pathway lesions. Electroretinography revealed retinal dysfunction in patients without clinical abnormalities. Supra-ophthalmic carotid infusion of cisplatin or BCNU caused no retinal or optic nerve lesions. Electroretinography was abnormal in only one of these patients. Our results indicated that BCNU and cisplatin cause ischemic damage and are toxic to both retinal and neural tissue in patients with gliomas.

Congenital unilateral adduction deficit with simultaneous abduction. A variant of Duane's retraction syndrome.

Congenital adduction deficit not associated with other signs of oculomotor nerve weakness occurs infrequently. Type 2 Duane's retraction syndrome accounts for some of these cases. The authors identified three children with unilateral adduction deficits and simultaneous abduction of the eyes on attempted lateral gaze into the field of action of the apparently paretic medial rectus muscle. This is the first report of a series of patients with this condition previously termed simultaneous or synergistic divergence. All patients had a large exotropia, and two had a horizontal face turn away from the involved eye. A large (14 mm) lateral rectus muscle recession done on one of these children reduced the face turn but did not eliminate the simultaneous abduction. Clinical, intraoperative, and electromyographic data suggest that this condition is an unusual variant of type 2 Duane's syndrome.

Intracranial extension and bone destruction in orbital pseudotumor.

Three cases of surgically proved pseudotumor of the orbital apex with intracranial extension occurred. All demonstrated bony destruction. Varying degrees of ophthalmoplegia and visual loss were present in all three. Previous computed tomographic descriptions of the patterns of orbital pseudotumor have not included bone destruction. Further, intracranial extension has been reported in only one patient. These three cases are reported to emphasize the fact that while these manifestations may be rare, it is appropriate to include orbital pseudotumor in the differential diagnosis of orbital apex lesions that are associated with both bone destruction and/or intracranial extension.