SEARCH · PubMed Health

Results for “OPTIC PATHWAY”

Explore indexed PubMed citations for clinical trials, systematic reviews and public health research. Read source abstracts and follow each citation to its original PubMed record.

Quote a phrase for an exact phrase match. Source license links do not imply unrestricted reuse.

At least 361 records · Page 20Linked to original sources

PubMed1998

Discordant puberty in monozygotic twin sisters with neurofibromatosis type 1 (NF1).

Monozygotic twin sisters are reported who have discordant pubertal growth and sexual development. Although both sisters were physiologically appropriate for chronological age in their pubertal development, their adolescent development was considerably different from that expected of monozygotic twin sisters. The more pubertally advanced sister by magnetic resonance imaging had an optic pathway glioma while none was present in her twin sister. Precocious puberty is not an unexpected complication of neurofibromatosis type 1 and is always associated with the presence of an optic pathway glioma. These sisters emphasize the striking similarity that is expected of monozygotic twins and the need for investigation when intertwin differences in growth and/or development arise.

Birth Weight↗

PubMed2000

Radiation therapy of optico-hypothalamic gliomas (OHG)--radiographic response, vision and late toxicity.

BACKGROUND: Management strategies for optic pathway gliomas include observation, surgery, irradiation, chemotherapy and a combination of these modalities. It has been the policy of our University Hospital to consider radiation as the standard treatment for progressive optic pathway gliomas. This report describes the clinical presentation, treatment patterns and outcome with special emphasis on the long term functional status of patients with optico-hypothalamic gliomas (OHG). PATIENTS AND METHODS: Between 1975 and 1997, 25 patients with OHG were treated by radiation therapy (RT) following surgery or biopsy. All patients received a local RT with a 0.5-1 cm margin around the lesions as depicted on CT or MRI scans. Age adjusted radiation doses ranged from 45 to 60 Gy with a single fraction size of 1.6-2 Gy. Endpoints of the study were: radiographic response, survival, progression-free survival and time to endocrinologic toxicity as well as the visual function during follow-up. The median follow-up time was 9 years (range, 1.5-23 years). RESULTS: A partial response was noted in six (24%) of the patients, 13 (52%) patients had a stable tumour throughout the observation period and six (24%) patients had a tumour progression. Overall survival and progression-free survival rates were 94 and 69% at 10 years, respectively. A significant influence on progression-free survival was noted for age at diagnosis (P=0.04) and total dose (P=0.05). Nine out of 13 (69%) patients aged below 10 years compared with 3/12 (25%) patients aged above 10 years experienced hypothalamic-pituitary deficiency (P=0.008) during follow-up. As for visual acuity, nine patients had an improvement, another 13 patients a stable situation and three patients a measurable deterioration. Visual field deficits improved in three, remained unchanged in 16 patients and worsened in only one patient. CONCLUSION: Postoperative RT with a total dose above 45 Gy should be considered as standard treatment in OHG with documented progression. Close radiographic monitoring and lifelong yearly evaluation for the need of possible hormone replacement are strongly recommended.

Adolescent↗

PubMed1988

Brain tumors in children with von Recklinghausen neurofibromatosis.

We determined the frequency of brain tumors in a series of 133 children (18 years of age or younger) with von Recklinghausen neurofibromatosis who were patients at the Children's Hospital of Zurich from 1971 to 1987. Twenty-four patients (18%) were found to have intraorbital or intracranial tumors. Twenty patients (15%) had an optic-pathway glioma (OPG), including 15 (11%) with only an OPG and 5 (4%) with both an OPG and a tumor in the posterior fossa or brainstem. Another 4 patients (3%) had a tumor outside of the optic pathways. In most cases, magnetic resonance imaging was superior to computerized tomography in demonstrating these lesions. The results of this survey are comparable to those of other published reports.

Adolescent↗

PubMed2003

Segmental neurofibromatosis in childhood.

Segmental neurofibromatosis refers to individuals who have manifestations of neurofibromatosis type 1 (NF-1) limited to one area of the body. It results from a post-conceptional mutation in the NF-1 gene leading to somatic mosaicism. Although it is generally considered a rare condition, this report of 39 children with segmental NF-1 demonstrates that it is commonly seen in a pediatric NF-1 referral center. The mean age at diagnosis was 7.8 years (range: 2-25 years). Twenty-nine patients had only pigmentary manifestations of segmental NF-1, including seven who had only café-au-lait macules and 22 who had café-au-lait macules and freckling. Two patients had isolated plexiform neurofibromas; a third patient had a plexiform neurofibroma of the eyelid in addition to ipsilateral dysplasia of the sphenoid wing and Lisch nodules. A 12-year-old girl had an isolated tibial pseudarthrosis. An 8-year-old boy had an isolated optic pathway tumor, which behaved both biologically and radiographically as an NF1-associated tumor. While most children with segmental NF-1 have only localized pigmentary changes, some children will have isolated plexiform neurofibromas, pseudarthroses, or optic pathway tumors. Accurate diagnosis of segmental NF-1 is crucial for both management and genetic counseling.

Adolescent↗

PubMed1998

Brain tumor in the first year of life: a single institute study.

Brain tumors in infants present special diagnostic and therapeutic challenges. To figure out the clinical features, pathological classification of the tumors and the treatment outcome of infantile brain tumors, 458 children (age<16) with brain tumors were reviewed retrospectively. Among them 21 cases (4.6%) were diagnosed during the first 12 months of life. Two tumors were definitely of congenital origin. The majority of infants with brain tumors presented with increased intracranial pressure. Fourteen tumors were located at the supratentorial area. Sixteen cases had neuroepithelial tumors; astrocytoma (optic pathway), supratentorial primitive neuroectodermal tumor (PNET) and medulloblastoma were found in three cases each. There were two treatment-related mortalities. Compared with the outcomes in older children, the treatment outcome was poorer in medulloblastoma and the optic pathway glioma which showed a higher growth potential. Because of the limited application of postoperative adjuvant therapy, radical surgical removal played a more important role in this age group. The prognosis of patients in whom the tumors could not be totally removed, largely depended on the pathological malignancy of the tumors. Though the treatment outcome was not always dismal, immaturity of the brain, higher growth potential, perioperative risks, limitations in adjuvant therapy, and pessimistic attitude on the part of parents made management more challenging.

Adolescent↗

PubMed1990

Evaluation of patients with obvious or suspected degenerative and dystrophic disorders of the retina, pigment epithelium and choroid. Experience from a Swedish referral center.

Between August 1, 1982, and December 31, 1988, 487 patients with clinically established or suspected diagnosis of a hereditary degenerative or dystrophic disorder of the ocular fundus or of a disorder of the optic pathways were evaluated with an extensive set of clinical and electrophysiological tests, 67% of the patients were referred from other eye departments. In order of magnitude the most frequently encountered diagnoses were: 1) 'functional' disturbances (18%), 2) disorders of the optic nerve (9%), 3) retinitis pigmentosa (8%), 4), 5) and 6) progressive cone dystrophy, disorders of the central optic pathways, and fundus flavimaculatus/Stargardt's disease (5% each), and 7) choriocapillaris atrophy (4%). Choriocapillaris atrophy affected older patients than retinitis pigmentosa (P less than 0.001). A male preponderance was observed for juvenile retinoschisis (P less than 0.005) and congenital stationary night blindness (P less than 0.05), and a female dominance in the groups of patients with 'functional' symptoms (P less than 0.01). A specific diagnosis was established in 469 patients. After exclusion of 16 patients referred because of a known hereditary disposition, 196 (43%) of the remaining 453 patients were referred under the same diagnosis as our final diagnosis, 153 (34%) under a different diagnosis and 104 (23%) without any clear diagnosis. Obviously, there is a need for regional centers specialized in these disorders.

Adolescent↗

PubMed2005

Chiasmatic neurons in the ventral diencephalon of mouse embryos--changes in arrangement and heterogeneity in surface antigen expression.

We have investigated the changes in arrangement of the SSEA-1 immunoreactive chiasmatic neurons in the mouse ventral diencephalon from embryonic day (E) 9 to the end of gestation. A regionally specific staining of SSEA-1 was first detected in the ventricular layer of the caudal diencephalon at E10 and later at E11 on the cells in the subventricular layer. At E12, these cells formed the characteristic V-shaped configuration caudal to the optic axons in the chiasm. At E13-E15, this neuronal array changes gradually to a configuration that facilitates contact with the optic axons only at the midline and the initial segment of the optic tract. Colocalization studies showed that CD44 was localized strongly on the neurons in the central but not lateral domains of the array, suggesting existence of heterogeneity in these neurons in terms of surface antigen presentation. This difference between the central and lateral domains raises the possibility that the chiasmatic neurons may regulate the patterning of axon orders at the midline and the optic tract through presentation of distinct combination of guidance cues at these strategic positions in the optic pathway. Furthermore, exogenous Lewis-x/SSEA-1 inhibited neurite outgrowth from the E14 retinal explants; this inhibition was observed in neurites from both ventral temporal and dorsal nasal retina. These findings suggest an action of this surface carbohydrate on the control of axon growth and guidance in the mouse optic pathway.

Age Factors↗

PubMed1994

Follow-up and quality of survival of 67 consecutive children with CNS tumors.

We report the findings at follow-up in 67 consecutive children with central nervous system tumors treated over a 5-year-period at a single institution. The diagnoses were supratentorial astrocytoma (n = 12), cerebellar astrocytoma (n = 10), ependymoma (n = 9), medulloblastoma (n = 9), brain stem glioma (n = 6), optic pathway glioma (n = 5), and others (n = 16). The survival rates were 83% for supratentorial astrocytomas at a median of 46.5 months, 90% for cerebellar astrocytomas and 55% for ependymomas at 40 months, respectively, 55% for medulloblastomas at 22 months, 33% for brain stem gliomas at 23 months, and 80% for optic pathway gliomas at 49 months. With regard to neurological sequelae, 13 patients were treated for epilepsy, 13 patients had mild to moderate neurological deficits, and 4 patients were severely disabled. Seventeen of 37 tested patients performed below average on formal neuropsychometric testing, one-fourth attended special education courses, and at least one-fourth suffered from behavioral and adjustment problems.

Adolescent↗

PubMed2001

Spontaneous regression of optic gliomas: thirteen cases documented by serial neuroimaging.

OBJECTIVE: To demonstrate spontaneous regression of large, clinically symptomatic optic pathway gliomas in patients with and without neurofibromatosis type 1 (NF-1). METHODS: Patient cases were collected through surveys at 2 consecutive annual meetings of the North American Neuro-Ophthalmology Society (NANOS) and through requests on the NANOSNET Internet listserv. Serial documentation of tumor signal and size, using magnetic resonance imaging in 11 patients and computed tomography in 2 patients, was used to evaluate clinically symptomatic optic pathway gliomas. All tumors met radiologic criteria for the diagnosis of glioma and 4 patients had biopsy confirmation of their tumors. In 3 patients, some attempt at therapy had been made many years before regression occurred. In one of these, radiation treatment had been given 19 years before tumor regression, while in another, chemotherapy had been administered 5 years before signal changes in the tumor. In the third patient, minimal surgical debulking was performed 1 year before the tumor began to shrink. RESULTS: Spontaneous tumor shrinkage was noted in 12 patients. Eight patients did not have NF-1. In an additional patient without NF-1, a signal change within the tumor without associated shrinkage was detected. Tumor regression was associated with improvement in visual function in 10 of 13 patients, stability of function in 1, and deterioration in 2. CONCLUSIONS: Large, clinically symptomatic optic gliomas may undergo spontaneous regression. Regression was seen in patients with and without NF-1. Regression may manifest either as an overall shrinkage in tumor size, or as a signal change on magnetic resonance imaging. A variable degree of improvement in visual function may accompany regression. The possibility of spontaneous regression of an optic glioma should be considered in the planning of treatment of patients with these tumors.

Adolescent↗

PubMed2007

Evaluation of pituitary macroadenomas with multidetector-row CT (MDCT): comparison with MR imaging.

INTRODUCTION: It is important to have information on cavernous sinus extension and bony destruction in pituitary macroadenomas before surgery, but magnetic resonance (MR) imaging cannot always depict them. In the present study we sought to determine whether multidetector-row computed tomography (MDCT) could provide preoperative information in addition to that provided by MR imaging in pituitary macroadenoma. METHODS: The subjects comprised 33 consecutive patients (15 women, 18 men; mean age 50 years) with surgically proven macroadenoma. For MDCT, using the soft-tissue window and bone window, three orthogonal multiplanar reconstruction images were generated from venous-phase contrast-enhanced 0.5-mm isotropic voxel data. MDCT and MR images were evaluated with regard to: (1) clarity of tumor margins; (2) identification of the normal pituitary gland; (3) identification of erosion or destruction of the sellar floor; and (4) visualization of the adjacent optic pathways. RESULTS: MDCT more clearly demonstrated the lateral tumor margin than MR imaging (P = 0.002). No significant differences in visualization of the normal pituitary gland were noted between MDCT and dynamic MR imaging (P = 0.7). MDCT more clearly demonstrated sellar floor erosion or destruction at the sphenoid sinus than MR imaging (P < 0.001). MR imaging was superior to MDCT for visualizing the adjacent optic pathways (P < 0.001). CONCLUSION: MDCT is superior to MR imaging for assessing lateral tumor margin and the sellar floor at the sphenoid sinus. MDCT offers useful preoperative information in addition to that obtained from MR imaging.

Adenoma↗

PubMed1999

Neurological complications of neurofibromatosis type 1 in adulthood.

Neurofibromatosis type 1 (NF1) is a genetic disease with a wide range of neurological manifestations. To examine these, and to evaluate neurological morbidity in adulthood of patients with NF1, we studied a hospital-based series of 158 patients that included 138 adult patients aged >18 years and 20 children. NF1 evaluation included a multidisciplinary clinical and a clinically oriented radiological investigation. Neurological events occurring during childhood (in both children and adults of the series) and adulthood were recorded. One or several neurological manifestations have been observed in 55% of patients (adults and children) (n = 87). These included: headache (28 patients); hydrocephalus (7); epilepsy (5); lacunar stroke (1); white matter disease (1); intraspinal neurofibroma (3); facial palsy (1); radiculopathy (5); and polyneuropathy (2). Tumours included: optic pathway tumours (20); meningioma (2); cerebral glioma (3); and malignant peripheral nerve sheath tumours (6). Life-threatening complications were observed in five adults and included four malignant peripheral nerve sheath tumours and one meningioma. Pain was the leading symptom in 11 adults and was related to malignant peripheral nerve sheath tumours, complications of intraspinal neurofibromas, subcutaneous neurofibromas and peripheral nerve neurofibromas. NF1 in adults was not associated with other disabling or life-threatening neurological complications. Symptomatic optic pathway tumours, cerebral gliomas, symptomatic aqueductal stenosis and spinal compression due to intraspinal NF were observed exclusively during childhood. In this series, the predominant neurological features of adults with NF1 were chronic pain and malignant peripheral nerve sheath tumours.

Adolescent↗

PubMed2001

Combined intraarterial carboplatin, intraarterial etoposide phosphate, and IV Cytoxan chemotherapy for progressive optic-hypothalamic gliomas in young children.

BACKGROUND AND PURPOSE: Optic pathway and/or hypothalamic astrocytomas in children are often quiescent, but in some cases, more aggressive tumors may cause progressive visual, endocrine, and neurologic deterioration. The initial treatment of these gliomas includes surgery and IV chemotherapy. Radiotherapy is not recommended in young children because of its severe adverse effects on cognitive and neuroendocrine function. This report suggests a new approach using combined intraarterial and IV carboplatin-based chemotherapy for patients for whom first line treatment has already failed. METHODS: Six children (mean age, 57 months) with the diagnosis of optic pathway hypothalamic gliomas, who had tumor progression after surgery and underwent IV chemotherapy, were treated monthly with intraarterially administered carboplatin, intraarterially administered etoposide phosphate, and IV administered Cytoxan. Four of the children had histologically verified pilocytic astrocytomas, and in two cases, diagnosis was made on the basis of clinical findings. Administration of the intraarterial chemotherapy required catheter placement in both internal carotid arteries at the level of C2-C3 and into one of the vertebral arteries at the level of C6-C7, with the patient under general anesthesia. RESULTS: Four of six patients had partial radiographic response, one had stable disease, and one had progressive disease after one cycle. Three patients showed clinical improvement. There were no serious complications associated with the angiographic procedures. Toxicities included bronchospasm that resolved after 3 to 4 minutes in one patient. One patient showed mild ototoxicity, and four patients needed platelet transfusion because of hematologic toxicity of drugs. CONCLUSION: These results suggest that this modality of chemotherapy (administered after failure of systemic [ie, IV] chemotherapy), of progressive optic-hypothalamic astrocytomas in young children may be an effective treatment prior to radiotherapy.

Antineoplastic Agents↗

PubMedDate not supplied

From the archives of the AFIP: pilocytic astrocytoma: radiologic-pathologic correlation.

Pilocytic astrocytoma is the most common pediatric central nervous system glial neoplasm and the most common pediatric cerebellar tumor. This tumor has a noteworthy benign biologic behavior that translates into an extremely high survival rate-94% at 10 years-that is by far the best of any glial tumor. Most patients present in the first 2 decades, and clinical symptoms and signs are usually of several months duration and directly related to the specific location of the tumor. The cerebellum, optic nerve and chiasm, and hypothalamic region are the most common locations, but the tumor can also be found in the cerebral hemisphere, ventricles, and spinal cord. Surgical resection is the treatment of choice for all tumors, except for those involving the optic pathway and hypothalamic region, which may be treated with radiation therapy and chemotherapy. Cross-sectional imaging often demonstrates a classic appearance: a cystic mass with an enhancing mural nodule. Less common appearances are quite nonspecific. Surrounding vasogenic edema is rarely present, and this feature provides a valuable clue to the correct diagnosis. Accurate interpretation of imaging studies plays an essential role in directing treatment of these tumors, particularly when they arise in the optic pathway of patients with neurofibromatosis type 1. Disseminated disease and recurrence are extremely rare.

Adult↗

PubMed1989

Visual evoked potentials in Prader-Willi syndrome.

Oculocutaneous, electrophysiological, and cytogenetic factors were evaluated in 14 patients with Prader-Willi syndrome and in three controls, two albinos and a normal observer. In a substantial number of PW patients chromosomal anomalies, particularly deletions of the long arm of chromosome 15 as well as hypopigmentation of hair, skin, and eye have been identified. In the genetic condition of albinism, hypopigmentation related to neural ectoderm derivatives is associated with reduced visual acuity, foveal hypoplasia, and aberrant retinogeniculocortical projections. The latter can be observed by visual evoked potential (VEP) assessment of hemispheric response symmetry. To determine the possible neural ectodermal origin of hypopigmentation and its involvement in ocular development and optic pathway integrity, the potential distributions of the pattern onset/offset VEP were examined. Our results show hypopigmentation in 13 of our 14 PW patients and a chromosome abnormality in 6; no correlation between these two features was found. None of the PW patients showed the characteristic contralateral hemispheric asymmetry seen in albinism. On the other hand their VEP profiles were found to be atypical, rendering waveform and cortical topography difficult to interpret. Analysis suggests that in the absence of VEP evidence for optic pathway misprojection, PW hypopigmentation is probably of neural crest origin.

Adolescent↗

PubMed1996

[Monocular photopsia preceding with neovascular glaucoma due to internal carotid artery occlusion; a case report].

We described a 67-year-old right handed man with a left internal carotid artery occlusion, who developed monocular photopsia that supervened neovascular glaucoma. He had an antecedent transient motor aphasia. His photopsia, exaggerated by light, persisted intermittently. Orbital bruit was obtained on the left, more clearly during the photopsia. Brain MRI, cerebral angiography, and duplex sonography of carotid and ophthalmic arteries indicated left internal carotid artery (ICA) occlusion with collateral circulation through the ophthalmic artery. Visual evoked potentials (VEPs) revealed a prechiasmal disturbance of the optic pathway of the left side. The patient had carotid endarterectomy of the left ICA, and his visual disturbance has gradually improved. Ocular symptoms due to ICA ischemia are commonly transient visual loss with dark background known as amaurosis fugax. Neovascular glaucoma is sometimes complicated with carotid artery occlusion. However, photopsia associated with carotid artery occlusion is rare. Photopsia mimics scintillating scotomata, but the latter precedes migraine and is biocular and homonymous, ascribable to spreading depression from the occipital lobe. Retinal or prechiasmal optic pathway might be influenced by poor circulation of the ophthalmic artery. In addition, disturbance of light adaptation due to retinal hypoperfusion may be possible reason. Neovascular glaucoma is intractable, once developed. Therefore, atypical scintillating visual disturbance must be recognized as a sign of carotid artery insufficiency and supervened glaucoma to prevent it.

Aged↗

PubMed1983

Bilateral reorganization of the rat optic tract following enucleation of one eye at birth.

In albino rats with one eye removed at birth (NE rats), electron microscopic studies were made on the optic tract (OT) to count the fibers and to measure their thickness. In addition, experiments were made in NE rats to know the physiological properties of the optic pathway such as conduction velocity, synaptic delay and so on. The number of fibers in the OT of NE rats was compared with that constituting the crossed and uncrossed pathways of normal adult rats. In NE rats the fiber count increased by about 30,000 in the OT ipsilateral to the remaining eye while it decreased by about the same amount in the contralateral OT. The axonal cross section area of OT fibers was measured as an index for fiber thickness. No marked abnormalities were found in the OTs of NE rats with regard to the morphological dimension. Relay cells (P-cells) of the LGN were recorded by electrical stimulation of the optic pathway. The uncrossed projection of NE rats to the LGN was characterized by the following points: (1) P-cells responding to stimulation of the uncrossed fibers were encountered in NE rats much more frequently than in normal adult rats. (2) Synaptic delays assumed to be involved in trans-synaptic activation of P-cells by the uncrossed fibers were calculated at larger values than for P-cells activated by the crossed fibers in NE rats and normally grown rats.

Aging↗

PubMed1990

Study of visual evoked potentials in diabetics without retinopathy: correlations with clinical findings and polyneuropathy.

To evaluate central optic pathways' involvement in diabetics, visual evoked potentials (VEP), in particular the latency of positive peak (LP100), were studied in 35 patients without retinopathy (4 insulin-dependent, 31 non-insulin-dependent) and 35 normal controls using reversal pattern stimulation. LP100 was significantly delayed in diabetics at both binocular and monocular stimulation. Furthermore, the delay in LP100 was significantly longer in the diabetics with polyneuropathy than in those without, particularly after binocular stimulation. A positive correlation was found between latencies of VEP and HbA1, duration of disease, and neurologic score. VEP measurement seems a simple and sensitive method for detecting early alterations in central optic pathways in diabetics.

Adult↗

PubMed1985

Abnormal visual-evoked potentials in leukemic children after cranial radiation.

Visual-evoked potentials (VEPs) were studied in 55 asymptomatic children with leukemia or solid tumors in remission in order to detect subclinical demyelination of the optic pathway after CNS prophylaxis. In group I (11 patients with ALL studied prospectively), VEP latency was increased in ten after cranial radiation (CR) as compared with previous values. Group II (18 patients with ALL in maintenance) and group III (16 patients with ALL off therapy) were studied retrospectively and VEP latency was found above normal limits in 33 and 31%, respectively. In group IV (four patients with solid tumors and six with leukemia, all of whom received no CR), VEP latency was normal despite periodical intrathecal methotrexate administrations to five of them. We conclude that CR determines a slowing of conduction on VEP test, probably due to demyelination of the optic pathway, in a high proportion of patients. The future clinical significance of these findings must be established throughout a prolonged follow-up period.

Brain↗