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Biomedical subjects

N J Schatz

Publications and source records attributed to N J Schatz.

At least 19 recordsLinked to original sources

Optic neuropathy in Hodgkin's disease.

Hodgkin's disease is a rare cause of infiltrative optic neuropathy, which typically evolves late in the disease course. We managed an unusual case of isolated optic neuropathy in a 21-year-old man occurring during clinical remission from Hodgkin's disease. Radiotherapy and treatment with high-dose systemic corticosteroids resulted in dramatic improvement in vision. Even without other evidence of recurrent disease, acute-onset optic neuropathy in a patient with a history of a lymphoproliferative disorder should raise the question of a reemergence of the malignancy.

Adult

Neuroimaging of the optic nerve after fenestration for management of pseudotumor cerebri.

The mechanisms by which optic nerve-sheath fenestration is effective remain speculative. Possibilities include surgical production of a cerebrospinal fluid filtration outlet or scarring in the subarachnoid space around the nerve in the vicinity of the fenestration site, with shifting of the pressure gradient from the nerve head to the retrobulbar portion. Two patients who underwent successful optic nerve-sheath fenestration developed a cystlike structure contiguous to the fenestration site, apparently in direct communication with the optic nerve sheaths. This was shown on magnetic resonance imaging (one patient) and orbital echography (both patients). These previously unreported observations may support the hypothesis that fenestration works by creating a filtration apparatus that controls the intravaginal pressure in the subarachnoid space surrounding the orbital segment of the optic nerve.

Adult

Pseudotumor cerebri from cranial venous obstruction.

Dural sinus hypertension from cerebral venous outflow impairment is a cause of pseudotumor cerebri. The authors documented six such patients: two with unilateral neck dissection, one with surgical ligation of the dominant sigmoid sinus, two with thrombosed central intravenous catheterization, and one with dural sinus thrombosis. The site of cerebral venous outflow obstruction was variable and identified in three patients with computed tomography, conventional magnetic resonance imaging, magnetic resonance angiography, and/or angiography. Magnetic resonance angiography used in two patients characterized the venous flow pattern and identified the site of obstruction, confirming magnetic resonance angiography as an effective noninvasive blood flow technique in diagnosing and following these patients. Three patients were treated successfully with medical therapy and one patient with optic nerve fenestration. The two patients with thrombosed central venous catheters had serious systemic illnesses and suffered permanent visual loss.

Adult

Spontaneous recovery of vision in progressive anterior ischemic optic neuropathy.

Four patients with typical anterior ischemic optic neuropathy experienced progressive deterioration of visual function, to acuity levels of finger-counting in two eyes, 20/400 in one, and 20/50 in the fourth. All subsequently enjoyed significant spontaneous recovery of vision to 20/60, 20/60, 20/40, and 20/25, respectively. No previous reports have documented the details of such improvement in the progressive form of anterior ischemic optic neuropathy. The natural history of this common optic nerve disorder is reconsidered in light of these observations. The potential for spontaneous recovery should be taken into account in the interpretation of visual outcome in medical or surgical therapeutic trials for progressive anterior ischemic optic neuropathy.

Aged

Microsaccadic flutter.

Microsaccadic flutter is a rare symptomatic saccadic oscillation that has been reported only twice previously. Here we describe 5 patients with this disorder. The oscillation is horizontal, has a frequency of 15-30 Hz, an amplitude of 0.1-0.5 degrees, and cannot be seen with the unaided eye. It is usually not associated with any underlying neurological disorder. We hypothesize that microsaccadic flutter is due to malfunction of the brainstem omnipause neurons.

Abducens Nerve

Factors influencing outcome of prednisone dose reduction in myasthenia gravis.

We reviewed retrospectively 114 prednisone dose reduction attempts in 63 myasthenic patients. Dose reduction was considered successful if a patient remained asymptomatic for more than 1 year on no prednisone or a stable low dose of prednisone. Successful dose reduction attempts were more common in patients taking azathioprine, but thymectomy did not influence taper outcome. Slower rate of dose reduction and higher ending dose of prednisone improved the chance of success.

Adolescent

Magnetic resonance imaging of radiation optic neuropathy.

Three patients with delayed radiation optic neuropathy after radiation therapy for parasellar neoplasms underwent magnetic resonance imaging. The affected optic nerves and chiasms showed enlargement and focal gadopentetate dimeglumine enhancement. The magnetic resonance imaging technique effectively detected and defined anterior visual pathway changes of radionecrosis and excluded the clinical possibility of visual loss because of tumor recurrence.

Adenoma

Magnetic resonance imaging of optic nerve meningiomas. Enhancement with gadolinium-DTPA.

Six patients with optic nerve sheath meningiomas were studied with gadolinium-diethylenetriaminepentaacetic acid (Gd-DTPA)-enhanced magnetic resonance imaging (MRI) to evaluate intracranial extension. The intraorbital and intracranial tumors were isointense to cortical gray matter on T1-weighted studies without contrast in all patients. After Gd-DTPA, three patients showed mild enhancement of the intraorbital tumor, whereas five of six patients showed vivid enhancement of the intracranial tumor. In four cases, the intracranial extension could not be definitely seen on MRI without Gd-DTPA. Two patients had proton density and T2-weighted studies; in each case, the intraorbital tumor remained nearly isointense. The intracranial tumor was suggested in one patient on T2-weighted studies, but was poorly defined. Gadolinium-DTPA has proved valuable in detecting intracranial extension of optic nerve meningiomas that are not well visualized on standard MRI without contrast.

Adult

Delayed radiation injury to the retrobulbar optic nerves and chiasm. Clinical syndrome and treatment with hyperbaric oxygen and corticosteroids.

Thirteen patients with delayed radiation injury to the optic nerves and chiasm were treated with hyperbaric oxygen (HBO) and corticosteroids. These patients experienced painless, abrupt loss of vision in one (6 patients) or both (7 patients) eyes between 4 and 35 months after receiving radiation doses of at least 4500 cGy to the region of the chiasm. Diagnostic evaluation including neuro-imaging and lumbar puncture showed no recurrent tumor and no other cause for visual loss. No patient's vision improved during treatment or follow-up lasting between 1 and 4 years. There were no serious complications of treatment.

Adrenal Cortex Hormones

Visual evoked responses in pernicious anemia.

We describe three newly diagnosed and untreated cases of pernicious anemia (PA) with clinical features suggestive of subacute combined degeneration. Visual evoked responses (VERs) were evaluted in all three patients. In each instance, delayed responses were obtained, suggesting that involvement of the visual pathways may be an early and perhaps not uncommon manifestation in PA, even in the absence of clinical stigmata of visual impairment. The need for excluding PA in cases of myelopathy with impaired VERs is stressed.

Anemia, Pernicious

Optic atrophy in acute intermittent porphyria.

A 24-year-old woman developed bilateral blindness after recovery from coma secondary to acute intermittent porphyria. Gradual return of vision in the right eye with a permanent unilateral visual field defect and optic atrophy followed. We believe the pathophysiologic mechanism was spasm of the vessels supplying the optic disk leading to ischemia and infarction of the optic nerve.

Acute Disease

Clinical experiences with the use of an automated perimeter (Octopus) in the diagnosis and management of patients with glaucoma and neurologic diseases.

This is a study of 59 patients, 42 with glaucoma and 17 with neurological disease, to compare the Octopus automated perimeter with the conventional Goldmann and Tubinger perimeters for detection and assessment of visual field defects. We believe the Octopus automated perimeter is an excellent method for screening patients suspected of glaucoma and can be expected to identify over 90% of the patients actually having visual field loss due to glaucoma. In patients with neurological disease, the Goldmann perimeter was superior to either the Octopus or Tubinger perimeters in providing clinically useful information.

Adolescent

Trans-sphenoidal removal of a Rathke's cleft cyst.

Symptomatic Rathke's cleft cysts are uncommon. We present a case with suprasellar extension manifested by hypopituitarism and visual disturbances. The treatment was trans-sphenoidal evacuation and partial removal of the capsule. We suggest that the trans-sphenoidal approach to these lesions is usually adequate and that radical removal of the capsule is not necessary.

Confusion

E-rosette formation in Graves' ophthalmopathy.

We investigated the lymphocyte characteristics of 77 Graves' disease patients with and without infiltrative ophthalmopathy. Thirteen patients with infiltrative ophthalmopathy without prior antithyroid therapy and 20 euthyroid patients with progressive ophthalmopathy demonstrated decreased percentages of active and total erythrocyte rosette-forming lymphocytes compared to thyrotoxic patients without eye disease and to a control population (p less than 0.001). There was no significant difference in rosette-forming cells between untreated thyrotoxic and treated euthyroid patients with ophthalmopathy. No lymphocytotoxic antibodies or rosette inhibitory factor was present in the sera of patients with infiltrative ophthalmopathy. Untreated and treated patients with lid retraction and mild proptosis without extraocular muscle disease had decreased active rosette-forming cells (p less than 0.001) but normal total rosette-forming cells. Five patients with infiltrative ophthalmopathy who failed to improve with systemic corticosteroids demonstrated elevated active but normal total rosette-forming cells. Differences in rosette formation between ophthalmic and nonophthalmic Graves' disease may represent an associated cell-mediated abnormality that may explain why control of the thyrotoxic state need not correlate with the ophthalmic manifestations of the disorder.

Adult

Axial myopia. A neglected cause of proptosis.

Ten patients who had unilateral proptosis ranging from 2.5 to 4.0 mm were found to have axial myopia confirmed by ultrasonography and, in one case, by computerized tomography. The historical features emphasized include an acquired painless bulging eye, amblyopia, and a stable visual acuity. Noteworthy features on ophthalmologic examination include a difference in visual acuity and refraction between the two eyes, a difference in the lenses on inspection of the patient's glasses, and an ipsillateral myopic fundus. Careful history taking, examination, ultrasonographic evaluation, and inspection of old photographs will separate this group of patients and avoid unnecessary endocrine studies, angiography, and costly neuroradiologic investigations. Finally, it must be recognized that coexisting disease must be evaluated as if the proptosis did not exist.

Adolescent

Optic tract syndrome. A review of 21 patients.

Twenty-one patients with lesions compromising the optic tract were reviewed. The involvement of the optic tract may be diagnosed in the presence of highly incongruous hemianopia, an afferent pupillary defect, and characteristic atrophy of the optic discs. Behr's pupil, hemianopic pupillary reaction (Wernicke's sign), and associated major neurologic deficits were encountered rarely.

Adolescent

The vasculature of the diaphragma sellae. A postmortem injection study.

The postmortem injection study demonstrated that the main blood supply to each quadrant of the competent diaphragma sellae appears to come from the following vessels: posterior quadrant, inferior hypophyseal arteries; right and left marginal quadrant, tributaries of the intracavernous portion of the carotid artery; anterior marginal quadrant, anterior capsular arteries. When the diaphragm is incomplete or absent the inferior hypophyseal arteries are the main source of blood supply. Microscopic analysis of the vascular bed in the diaphragma sellae reveals that there are fine arterial filaments mostly in the superior layer, while venules are situated in the inferior part of the diaphragm.

Adult