Relative pupil-sparing oculomotor nerve palsy as the presenting sign of posterior fossa meningioma.

We report a case of relative pupil-sparing oculomotor paresis initially attributed to ischemia because weakness of other cranial nerves was minimal and dismissed as insignificant. Neuroimaging eventually revealed a posterior fossa meningioma. The neurologic symptoms and signs disappeared immediately after resection of the tumor. The third nerve palsy was attributed to deformation of the brainstem. This case reinforces the importance of neuroimaging even in patients who have apparently isolated oculomotor palsy with features not classic for an ischemic etiology.

Objective evaluation of improvement in optic neuropathy following radiation therapy for thyroid eye disease.

PURPOSE: While the literature supports the use of radiation therapy for thyroid eye disease, it does not sufficiently describe in detail the results of radiation therapy for optic neuropathy associated with thyroid eye disease. The objective of this study is to quantify the changes in parameters of optic neuropathy after orbital irradiation for thyroid eye disease. METHODS AND MATERIALS: Twelve consecutive patients with optic neuropathy from thyroid eye disease were followed by a single neuro-ophthalmology practice and treated by one radiation oncologist with radiation therapy from 1991 through 1995. All cases were prospectively followed for visual acuity, color vision, mean deviation, and/or foveal sensitivity and afferent pupillary defect. All patients received 2000 cGy in 10 fractions with megavoltage irradiation to the orbits. RESULTS: Ten of 12 patients were evaluated for follow-up (one moved out of this country and one had a stroke, which confounded interpretation of examination results). An analysis was performed retrospectively while treatment and evaluation remained uniform. Five men and five women formed the basis of this study with a median age of 60 years (35-76 years). Nineteen eyes were evaluated for thyroid optic neuropathy. Improvement in optic nerve function occurred in eight of ten patients. Improvement was seen either during radiotherapy or within 2 weeks of completion. No long-term adverse effects were noted. CONCLUSION: This study objectively demonstrates improvement in optic neuropathy from radiation therapy for thyroid eye disease.

Neuro-ophthalmic manifestations of Lyme disease.

Lyme disease is a multisystem disorder caused by infection with the Borrelia burgdorferi spirochete. The diagnosis of Lyme disease usually is based on several clinical criteria, with supportive data from laboratory testing. The presence of the bullseye skin lesion, erythema migrans, is the single pathognomonic criterion. In the 20 years since the initial description of Lyme disease in the United States, B. burgdorferi has been implicated as an etiologic agent in numerous ophthalmic and neuro-ophthalmic syndromes, involving most structures from the cornea to the cranial nerves. Neuro-ophthalmic and ocular manifestations of Lyme disease include meningitis with papilledema, cranial neuropathies, follicular conjunctivitis, nummular keratitis, and intraocular inflammation. Although an association with Lyme disease has been purported for numerous other syndromes, a definite causal relationship has not been proved in many cases. During a period of rapidly increasing awareness of Lyme disease, a high index of suspicion and poorly defined criteria for its presence have resulted in over-diagnosis of Lyme disease. In the authors' experience, the incorrect diagnosis of Lyme disease initially has been made in patients with allergic conjunctivitis, keratoconus, morning glory syndrome, craniopharyngioma, meningioma, CNS lymphoma, paraneoplastic syndrome, multiple sclerosis, sarcoid, syphilis, and functional illness. Nevertheless, this treatable infection must be an important consideration in the differential diagnosis of certain ocular or neurologic diseases.

Luxury perfusion following anterior ischemic optic neuropathy.

We present five patients who developed luxury perfusion following anterior ischemic optic neuropathy in whom fluorescein angiography was misinterpreted as "capillary hemangioma" or neovascularization of the disc. In each case, the segment of disc hyperemia corresponded to a spared region of visual field. Luxury perfusion represents a reparative autoregulatory reaction to ischemia.

Hemianopic visual field defects in children with intracranial shunts: report of two cases.

OBJECTIVE AND IMPORTANCE: Intracranial shunts are commonly placed in children and frequently require replacement during the child's growing years. Severe signs of increased intracranial pressure often are the first indication of shunt displacement and malfunction. Subtle neuro-ophthalmic signs in children are usually overlooked. CLINICAL PRESENTATION: Two cases are described in which homonymous hemianopsia resulted from unsuspected movement of the shunt catheter in growing children. In one male patient, the catheter tip impaled the optic tract, and in the other male patient the shunt became embedded in the midbrain tegmentum and shunt failure led to compression of the posterior cerebral artery. INTERVENTION: In the presence of optic atrophy, papilledema may go unnoticed without serial examinations. This stresses the importance of detecting other early warning signs of shunt displacement and increased intracranial pressure, including visual field changes and subtle abnormalities of motility. CONCLUSION: Because early diagnosis and shunt revision may allow visual recovery, children with shunts should be followed with visual fields and serial disc photographs.

24-hour blood pressure monitoring in patients with anterior ischemic optic neuropathy.

OBJECTIVE: To define parameters of ambulatory diurnal blood pressure in patients who had experienced anterior ischemic optic neuropathy (AION) in a case-controlled study. PARTICIPANTS AND METHODS: Twenty-four patients with AION and 24 control subjects who were matched for age, gender, medical diagnoses, and medications underwent ambulatory automated blood pressure monitoring for 24 hours. RESULTS: The overall diurnal pattern of blood pressure appeared to be normal in all subjects, showing lower blood pressures at night than during the day, an overnight nadir, and an ascending blood pressure curve in the morning to reach daytime levels. Also, patients with AION did not differ from control subjects with respect to the nighttime diastolic nadir or daytime peak systolic blood pressure. However, during the daytime, patients with AION had lower mean systolic and diastolic blood pressures than did matched control subjects. The widest difference between their blood pressure curves occurred after awakening in the morning, when patients with AION had a less steep and more irregular rise of blood pressure. Patients who had signs of vertebrobasilar insufficiency in addition to AION had lower mean diastolic blood pressure during both daytime and nighttime and a lower minimum daytime diastolic blood pressure. CONCLUSIONS: On ambulatory measurements of diurnal blood pressure, patients with AION consistently had a lower mean blood pressure than did control subjects and a lag in the usual rise in blood pressure in the morning to meet increasing daytime demands for perfusion. Chronic hypoperfusion of small end-arterial vessels that supply the optic nerve head may predispose to AION, and may be caused by relative hypotension owing to overtreated hypertension or to abnormal vascular autoregulation. Internists should be asked to monitor blood pressure carefully when treating hypertensive patients who are at risk for AION, to avoid hypotension, especially on awakening in the morning.

Recovery from ocular ischemic syndrome after treatment with verapamil.

Vasospasm has been implicated as a cause of amaurosis fugax, which can be controlled by administration of the calcium channel blockers nifedipine or verapamil. However, vasospasm has not previously been thought to be involved in chronic ocular ischemia. We report a patient with ocular ischemic syndrome, which may have had vasospasm as a contributing cause, since the patient also developed amaurosis fugax despite daily aspirin therapy. An 80-year-old man with chronic open-angle glaucoma developed chronic ocular ischemia characterized by progressively decreased visual acuity, pain, rubeosis, and hypotony, as well as transient visual dimming. Medical evaluation revealed no evidence of carotid stenosis, thromboembolism, or vasculitis as the cause of ocular ischemia. When the calcium channel blocker verapamil was administered, the episodes of transient visual dimming ceased immediately. In addition, soon thereafter, visual acuity improved, the rubeosis partially regressed, and the hypotony reversed. This case indicates that the calcium channel blocker verapamil may be effective in treating cases of ocular ischemic syndrome, when vasospasm is a contributing cause.

Resolution of chronic cluster headache after resection of a tentorial meningioma: case report.

Cluster headache is almost always idiopathic, but, in rare cases, associated intracranial lesions have been found. We describe a patient who had chronic cluster headache for more than 20 years. The headache immediately resolved upon resection of a tentorial meningioma. Prior reports of cluster headache as a manifestation of structural disease are briefly reviewed. In the patient described, the pain was referred from the right tentorium cerebelli to the right side of the face, in accordance with reported studies on the subjective localization of pain referred from posterior fossa structures. The accompanying abnormalities of autonomic function may have been mediated by central autonomic reflexes that are also involved in the pathogenesis of idiopathic cluster headache.

Lid opening apraxia is associated with medial frontal hypometabolism.

We used [18F]fluorodeoxyglucose with positron emission tomography to explore the functional basis of lid opening apraxia (LOA). We studied four patients with LOA without associated pyramidal or extrapyramidal motor dysfunction (mean age 65.5 +/- 4.7 years). We compared measures of glucose metabolism with those calculated for 10 age-matched normal volunteers (mean age 60.6 +/- 11.4). All four patients demonstrated significant metabolic reductions (> 2 SD) in the medial frontal lobe: bilaterally in two, unilaterally in two. Significant abnormal unilateral metabolic reductions in the left basal ganglia were evident in two patients. These findings suggest that medial frontal lobe hypometabolism is associated with LOA. Abnormal striatofrontal interactions may be involved in the pathogenesis of this condition.

Neuro-ophthalmic complications of intracranial catheters.

We report four patients who sustained direct injury to the brain after insertion of intraventricular shunts and pressure monitoring lines, which resulted in permanent neuro-ophthalmic deficits. These included hemianopsia from an optic tract lesion, esotropia and residual bilateral facial paresis from dorsal pontine injury, unilateral blindness from damage to the optic nerve, and dorsal midbrain syndrome from catheter compression in the region of the posterior commissure. Although presumably rare, such injuries should be considered in diagnosing patients with neuro-ophthalmic complaints after insertion of such devices.

Peripapillary hemorrhage.

On routine examination, a 61-year-old man was found to have a deep peripapillary hemorrhage surrounding his left optic nerve head. Further examination revealed a left sixth nerve paresis, a subtle right homonymous quadrantanopia, and jerky pursuit to the left. The diagnostic work-up was delayed by the patient because of business commitments. He returned confused and obtunded. Neuro-imaging showed a large frontal mass, which turned out to be an anaplastic astrocytoma. Diagnosis of the lesion had been obscured by three false localizing signs. Discussion deals with the definition of Terson's syndrome and the occurrence of peripapillary hemorrhages. Other causes of peripapillary hemorrhages are illustrated.

Paralysis of downgaze in two patients with clinical-radiologic correlation.

Selective downgaze paralysis was correlated with discrete bilateral lesions at the mesencephalic-diencephalic junction in a 9-year-old girl following severe pneumococcal meningitis, and in a 64-year-old man who suffered an embolic infarction. Magnetic resonance imaging demonstrated bilateral lesions in the region of the rostral interstitial nucleus of the medial longitudinal fasciculus. Clinical-radiologic correlation allowed identification of the likely vascular cause in both patients.