Biomedical subjects

S Michowitz

Publications and source records attributed to S Michowitz.

6 recordsLinked to original sources

PubMed2000

Visual pathway tumors and hydrocephalus.

The authors evaluated the impact of hydrocephalus on the clinical picture of children with visual pathway tumor (VPT) with or without neurofibromatosis (NF). Charts of children with VPT treated in the authors' center since 1985 were retrospectively reviewed, and those with hydrocephalus were selected and summarized. Thirty-five children with VPT were found, of whom 20 had NF. Hydrocephalus was found in 4 children with NF (20%) and in 5 without NF (33.3%). In 6 of the children, ventricular dilatation with signs of acute increased intracranial pressure already existed at the time of diagnosis and the hydrocephalus was shunted at this time. In the other 3 children, all with NF, the hydrocephalus resulted from slowly developing aqueductal stenosis, leading in 2 to severe visual acuity deterioration. The results suggest that in children with VPT and NF, hydrocephalus, and especially hydrocephalus resulting from aqueductal stenosis, is more frequent than in the general population of NF patients, and less frequent than in VPT patients without NF. The possibility of the indolent development of hydrocephalus should be borne in mind while following children with NF. The optic nerve, when already involved with a glioma, is more vulnerable to increased pressure. Thus, in children with VPT and NF, any ventricular dilatation should lead to a consideration of early shunting.

Child↗

PubMed1998

Diffuse brain stem gliomas. Are we improving outcome?

We reviewed our experience with diffuse brain stem glioma (dBSG) to evaluate whether any improvement of outcome had occurred in our patients over the years. Of the 24 children referred to our department with suspected dBSG from 1981 to 1997, 5 had a different final diagnosis based on the clinical course. Mean survival in the remainder was 16+/-9.8 months from diagnosis. Survival increased with a longer interval from onset of symptoms to diagnosis (12.9+/-9.0 months with an interval of 1-4 weeks; 19.50+/-10.8 months with a longer interval). Visual symptoms at presentation were associated with a poorer prognosis. Survival was better in the 3- to 5-year age group (at diagnosis). Overall, a trend toward a slight improvement in survival was seen over the years, which we presumptively attribute to the introduction of intensive chemotherapy for these patients. We suggest that chemotherapy may be important in the management of dBSG until a better modality is found.

Adolescent↗

PubMedDate not supplied

Pediatric brain stem gliomas: an update.

It has become evident that pediatric brain stem gliomas are a heterogeneous pathology and should be classified according to clinical and radiological criteria. This classification has contributed to better treatment and greatly improved prognosis. Based on a review of the literature, we describe the different types of brain stem astrocytomas reported, which are: cervicomedullary, exophytic, cystic, focal and diffuse. Particular attention is paid to therapeutic modalities. For the first three lesions named the treatment is surgical and oncological therapy should be evaluated only for regrowth of the mass. Focal tumors of the medulla and pons are still treated empirically (surgically and/or with radiotherapy), because a definitive therapeutic protocol has not yet been elaborated; on the other hand it is well established that if the focal mass is in the midbrain this should merely be monitored by means of serial MRI, while radiotherapy should be applied in the event of the tumor's growth. Diffuse gliomas are treated with oncological therapy, and surgery (for biopsy or tumor excision) is not indicated. Five illustrative cases from our department are presented.

Adolescent↗

PubMedDate not supplied

Visual pathway tumor: a heterogeneous tumor with a variable clinical course.

The aim of the study was to delineate the clinical characteristics of visual pathway tumor in children without neurofibromatosis-I. The authors reviewed the charts of all patients meeting these criteria (n = 12) who were followed in their center over a 13-year period. In 8 patients the disease was relentlessly progressive, and imaging showed a chiasmatic/hypothalamic, exophytic globular lesion. The remainder had a benign course with long periods of tumor stability; one showed some spontaneous visual improvement. The lesions of the latter subgroup were multilobular, with elongated posterior extension into the optic tract. This differentiation, according to the imaging findings, may have significant therapeutic implications. In the first type, every effort should be made to arrest the disease and decrease the size of the lesion, whereas in the second, despite decreased visual ability, careful observation in the appropriate approach. There are as yet no known biological markers to better delineate these two types of tumor behavior.

Cranial Nerve Neoplasms↗

PubMedDate not supplied

Successful chemotherapeutic treatment of diencephalic syndrome with continued tumor presence.

A 7-month-old infant with typical features of diencephalic syndrome (DES) associated with a hypothalamic mass, most probably a glioma, was treated with chemotherapy. The tumor showed clear shrinkage, but after more than 2 years regrowth was noted. During the treatment period the child regained normal growth and became free of symptoms. As radiation therapy, especially at a young age, has significant adverse effects and a neurosurgical approach to the diencephalic region also has the potential to cause significant sequelae, a chemotherapeutic option, when it exists, is preferred. Thus, in an infant in whom a glioma is suspected to be the cause of the DES, based on the clinical picture and the neuroimaging appearance, chemotherapy should be considered the primary therapeutic modality. Even if its effect is temporary, its use is well justified. The most appropriate treatment protocol still needs to be determined.

Abducens Nerve Diseases↗

PubMedDate not supplied

Dysembryoplastic neuroepithelial tumor: a curable cause of seizures.

Dysembryoplastic neuroepithelial tumor (DNT) was first characterized in 1988 by Daumas-Duport and Scheithauer [1], who described 39 cases in children with morphologically distinct brain tumors. Partial complex seizures (PCSs), well-defined neuroimages, and typical pathologic findings were characteristic. Surgical resection of the tumor was usually curative. We present a new case of a child with PCSs who was diagnosed and treated for DNT. The clinical presentation and the difficulties of the differential diagnosis are discussed.

Anticonvulsants↗