Anterior ischemic optic neuropathy in moyamoya disease: a first case report.

Neuro-ophthalmological manifestations in moyamoya disease are usually the result of cerebrovascular involvement of the visual pathways. We report a case of ischemic optic neuropathy due to ocular hypoperfusion as a result of moyamoya disease, despite a prior internal to external carotid artery bypass with normal hemisphere perfusion. The blood supply of the optic nerve, a proposed pathogenesis of an anterior ischemic optic neuropathy and complications of the ocular ischemic syndrome are discussed.

Preservation of visual fields after peri-sellar gamma-knife radiosurgery.

Radiosurgical treatment of pituitary and peri-sellar tumors has become an increasingly utilized modality as an alternative to conventional radiotherapy and surgery. Such radiosurgery results in a relatively high dose of radiation to the optic chiasm. The clinical data establishing safe single-fraction doses to the chiasm is immature, although taken together previous literature suggests a recommended maximal dose of 8 Gy. Optic neuropathy, when it occurs, tends to take place within 2 years of treatment. We evaluated the visual fields of 20 sequential patients that received significant doses to the optic chiasm by Gamma-knife radiosurgery. There were 17 cases of pituitary adenoma and 3 cases of meningioma, and two patients refused follow-up testing. Preoperative visual field and cranial nerve examinations were done prior to radiosurgery and in follow-up, with a median follow-up of 24 months. There were no cases of quantitative visual field deficit induced by treatment. No patients developed symptomatic visual deterioration. Radiat. Oncol. Invest. 90:343-350, 2000. 2000 Wiley-Liss, Inc.

Reversible empty sella in idiopathic intracranial hypertension: an indicator of successful therapy?

Idiopathic intracranial hypertension is commonly associated with an empty sella, caused by herniation of subarachnoid cerebrospinal fluid through an absent or patulous diaphragma sellae. We describe the findings in two patients who presented with headache, papilledema, and visual disturbances. Diagnosis of idiopathic intracranial hypertension was made on the basis of clinical symptoms and laboratory data. Initial imaging studies in each patient showed an empty sella. After treatment, one with acetazolamide and the other with lumboperitoneal shunting, the appearance of the sellar contents became normal.

Paraneoplastic retinopathy associated with antiretinal bipolar cell antibodies in cutaneous malignant melanoma.

PURPOSE: It has been shown previously that the sera, from patients with visual paraneoplastic syndrome associated with lung cancer, contain immunoglobulins that are reactive with the tumor and with photoreceptor and large retinal ganglion cells. The purpose of this study is to determine the retinal cell population that reacts with immunoglobulins in the sera of patients with melanoma-associated retinopathy. METHODS: Clinical and electrophysiologic studies were used to determine the locus responsible for the visual defect in each patient. Sera from two patients with melanoma-associated retinopathy, from a patient with herpes zoster, and from a patient who had a colon tumor were obtained. The sera were incubated with sections of retina obtained from a healthy 3-year-old child who had died of asphyxiation. The tissue sections subsequently were incubated with biotin-labeled anti-human immunoglobulin G, and then with streptavidin-labeled peroxidase. Finally, the tissue sections were developed to show peroxidase activity in the targeted retinal cells. RESULTS: Clinical and electrophysiologic studies were consistent with a defect in intra-retinal transmission distal to the photoreceptors. The immunoglobulins from the patients with the melanoma-associated retinopathy reacted selectively with the bipolar cells of the retina; approximately 30% of the bipolar cells were immunoreactive. The sera from the other two patients were not reactive with any of the retinal cells examined. CONCLUSIONS: The sera of patients with the paraneoplastic syndrome, melanoma-associated retinopathy, contain high titer immunoglobulins that are reactive only with a subset of the bipolar retinal cells. The clinical, electrophysiologic, and immunologic studies are all consistent with an intra-retinal transmission defect at the level of the bipolar cells.

Confocal laser scanning ophthalmoscope. Reproducibility of optic nerve head topographic measurements with the confocal laser scanning ophthalmoscope.

BACKGROUND: Glaucoma is an optic neuropathy in which changes in the appearance of both the optic nerve head and the surrounding tissues are important in both diagnosing its presence and progression. Accurate methods to objectively document the appearance of the optic nerve are necessary. The confocal laser scanning ophthalmoscope (Zeiss) is a new prototype instrument that may have the capability to accurately perform this function. METHODS: The authors performed a prospective pilot study evaluating the ability of the confocal laser scanning ophthalmoscope to reproduce three-dimensional optic nerve images. Each retinal image contained 600,000 bytes of information. Thirty discrete images of the right optic nerves of 19 visually normal volunteers were obtained. Depth measurements were compared from the same 100 x 100 micron areas (neighborhoods). RESULTS: Image comparisons found the variability of depth measurements for the entire image were within 102 microns (95% confidence interval). Sixty percent of the depth measurements were reproducible within 100 microns. Variability of the depth measurements was greatest where the neuroretinal rim sloped at the edge of the optic cup and lowest in the peripapillary area. CONCLUSION: The confocal laser scanning ophthalmoscope has the potential to be a safe, rapid, and reproducible method of imaging ocular structures.

The economic burden of mental illness.

Mental illness imposes a substantial burden on individuals and society. Using data from national surveys and a newly developed methodology for calculating costs, the authors estimate that in 1985 the total economic costs of mental illness were $103.7 billion. Of this total, direct treatment and support costs were $42.5 billion, or 11.5 percent of total personal health care spending for all illnesses. Morbidity costs--the value of reduced or lost productivity--amounted to $47.4 billion. Mortality costs--the lost value of productivity due to premature death resulting from mental illness--were estimated to be $9.3 billion, or 5.1 percent of total productivity losses for all deaths. Other related costs, including the cost of caregiver services, amounted to $4.5 billion.

Optic nerve decompression surgery improves visual function in patients with pseudotumor cerebri.

Papilledema from pseudotumor cerebri can cause severe loss of visual acuity and visual field. We performed optic nerve decompression surgery on 17 patients with pseudotumor cerebri who, despite maximum conventional therapy, developed progressive loss of visual acuity and/or visual field. Postoperatively, visual acuity improved or stabilized in 33 of 34 eyes (97%). Visual fields improved in 20 of 21 eyes that underwent surgery. Optic nerve decompression surgery relieves local cerebrospinal fluid pressure on the optic nerve. Progressive loss of visual function associated with pseudotumor cerebri can be reversed or stabilized with optic nerve sheath decompression surgery.

Modified optic nerve decompression in patients with functioning lumboperitoneal shunts and progressive visual loss.

Chronic papilledema from pseudotumor cerebri can cause progressive visual loss despite functioning lumboperitoneal shunts. The authors performed modified optic nerve sheath decompression in 12 patients (16 operations) with functioning lumboperitoneal shunts and progressive visual loss. All of the patients demonstrated improvement in visual function. Optic nerve sheath decompression allows release of localized cerebrospinal fluid (CSF) pressure. By performing optic nerve sheath decompression, progressive visual loss secondary to chronic papilledema can be halted and reversed.

Estimates of economic costs of alcohol and drug abuse and mental illness, 1985 and 1988.

The high prevalence of alcohol and drug abuse and mental illness imposes a substantial financial burden on those affected and on society. The authors present estimates of the economic costs from these causes for 1985 and 1988, based on current and reliable data available from national surveys and the use of new costing methodology. The total losses to the economy related to alcohol and drug abuse and mental illness for 1988 are estimated at $273.3 billion. The estimate includes $85.8 billion for alcohol abuse, $58.3 billion for drug abuse, and $129.3 billion for mental illness. The total estimated costs for 1985, $218.1 billion, include $51.4 billion for direct treatment and support costs; $80.8 billion for morbidity costs, the value of reduced or lost productivity; $35.8 billion for mortality costs, the value of foregone future productivity for the 140,593 premature deaths associated with these disorders, based on a 6 percent discount rate and including an imputed value for housekeeping services; and $47.5 billion in other related costs, including the costs of crime, motor vehicle crashes, fire destruction, and the value of productivity losses for victims of crime, incarceration, crime careers, and caregiver services. The cost of acquired immunodeficiency syndrome associated with drug abuse is estimated at $1 billion, and the cost of fetal alcohol syndrome is estimated at $1.6 billion. The estimates may be considered lower limits of the true costs to society of alcohol and drug abuse and mental illness in the United States.

Optic nerve sheath decompression for nonarteritic ischemic optic neuropathy improves multiple visual function measurements.

Optic nerve sheath decompression was performed in seven patients with nonarteritic anterior ischemic optic neuropathy. Visual function was evaluated by measurement of visual acuity with standardized Early Treatment Diabetic Retinopathy Study charts, color vision testing, quantitation of relative afferent pupillary defects with neutral-density filters, and Goldmann and Humphrey perimetry. Visual acuity improved markedly in all patients (at least doubling of the visual angle); the peripheral visual field expanded by at least 20 degrees (as measured by Goldmann perimetry) in six patients. Three patients also experienced marked improvement in color vision, relative afferent pupillary defect, and foveal sensitivity. Our experience supports the possible beneficial effect of optic nerve sheath decompression in patients with nonarteritic anterior ischemic optic neuropathy.

Orbital roof "blow-in" fractures.

Seven patients with significant frontal trauma involving the supraorbital region associated with orbital roof "blow-in" fractures were admitted to the Maryland Institute for Emergency Medical Services Systems (MIEMSS) at the University of Maryland Medical System over a 16-month period. High-resolution computed tomography (HRCT) with three-dimensional reconstructions demonstrated a downward displacement of the orbital roof in the absence of any orbital rim discontinuity. Associated findings included contussive and hemorrhagic injury to the ipsilateral frontal and parietal lobes, proptosis, ptosis, chemosis, orbital hematoma, and optic nerve contusion. The only long-term ocular pathology in this group of patients was mild to severe optic atrophy. It is recommended that all patients with frontal bone trauma have thin-cut computed tomographic evaluation of the orbit.

Septo-optic dysplasia in two siblings.

We treated two siblings, a sister and brother, who had features of septo-optic dysplasia, including bilateral optic nerve hypoplasia, absent septum pellucidum, and partial pituitary insufficiency. Additionally, midline central nervous system abnormalities of the corpus callosum and cerebellum were shown. The familial occurrence of this disorder raises the possibility of autosomal-recessive inheritance.

Traumatic trochlear nerve palsy diagnosed by magnetic resonance imaging: case report and review of the literature.

Although head trauma is the leading cause of acquired trochlear nerve dysfunction, it receives little attention in the neurosurgical literature. A case is reported of closed head injury that resulted in a right superior oblique palsy in association with incoordination on the left side. Diagnostic imaging revealed a normal cranial computed tomographic scan and a left dorsal midbrain lesion on magnetic resonance imaging scan. The relevant anatomy is reviewed, as well as the action of the superior oblique muscle, its agonists and antagonists, and the clinical manifestations of superior oblique dysfunction. This case is one of the few we are aware of in which a relatively isolated trochlear nerve palsy is the result of a lesion that can be documented by diagnostic imaging, and the first in which the imaging modality is magnetic resonance imaging scan.

Orbital "blow-in" fractures: clinical and CT features.

During a 16 month period, seven patients were admitted to our trauma center with "blow-in" fractures of the orbital roof. This injury results from a significant direct blunt force applied to the supraorbital region of the frontal bone with transmission of energy to the thin orbital plate of this bone and displacement of bone fragments downward into the superior orbit. High resolution CT with multiplanar reformation and three-dimensional display proved very useful in identifying and characterizing the bone and soft tissue abnormalities found in our patients. Our experience suggests that CT demonstration of supraorbital frontal bone fractures and/or frontal lobe cerebral contusions is an indication for thin section CT of the entire orbit, even when there is no evidence of a superior orbital rim fracture. Demonstration of an orbital blow-in fracture should prompt careful clinical assessment of the globe, optic nerve, and extraocular muscles to identify and appropriately manage underlying injuries to these structures.

A prospective ophthalmic evaluation of patients with acute myeloid leukemia: correlation of ocular and hematologic findings.

We report the results of a prospective 2-year study of the ocular manifestations of myeloid leukemia. Fifty-three patients underwent complete ophthalmic evaluation prior to the initiation of treatment as well as during the course of their disease. All ocular abnormalities were confined to the retina and optic nerve and were present in 34 patients, 30 of whom had either hemorrhages or cotton-wool spots alone or in combination. These findings were unrelated to age, sex, French-American-British (FAB) classification, and pretreatment leukocyte count or hematocrit. Patients with retinopathy had significantly lower platelet counts than those without retinopathy. Three patients had funduscopic evidence of optic nerve edema. None of these had clinical evidence of CNS leukemia. The presence of retinopathy was unrelated to therapeutic response. There was complete resolution of all ocular findings in those patients surviving the induction phase of therapy.

Measuring comorbidity and overlap in the hospitalization cost for alcohol and drug abuse and mental illness.

This paper measures the cost of short-stay hospitalization in 1985 for alcohol and drug abuse and mental illness (ADM). We identify and quantify two measurement problems: comorbidity in which ADM disorders may play a secondary role to other diagnoses and "overlap" in which ADM disorders occur together. Using data from the National Hospital Discharge Survey, we estimate the ADM short-stay hospital costs to total $13.1 billion. Almost four-fifths are costs of care for patients with primary ADM diagnoses and the remaining one-fifth is attributed to ADM comorbidity. About one-fifth of both the ADM discharges and the days of care represent overlap for hospitalized patients.