Ewing sarcoma mimicking a peripheral nerve sheath tumor.

We describe the first patient with an extradural, extramedullary Ewing's sarcoma tumor mimicking a nerve sheath tumor with no overt evidence of metastasis. A 28-year-old woman with no past medical history presented with a progressive 3-year history of low back pain and right-sided lower extremity radiculopathy after having failed conservative therapies. MRI of the lumbar spine revealed a right-sided enhancing, dumbbell-shaped lesion at the right neural foramen appearing to originate from the L4 nerve root, suspicious for a peripheral nerve sheath tumor or schwannoma. The patient and findings are discussed in the context of the literature, including an update on the relatively recent diagnostic redesignation of the Ewing's sarcoma family tumors.

Paraganglioma of the thoracic spinal cord.

Paraganglioma arising in the thoracic extradural space is an uncommon tumor, with only four cases previously reported. The authors review the clinical and pathological features of thoracic paraganglioma and compare them to the more common paraganglioma of the cauda equina.

Malignant meningioma in Gorlin's syndrome: cytogenetic and p53 gene analysis. Case report.

Gorlin's syndrome, also known as multiple basal cell carcinoma syndrome, is a familial tumor condition with autosomal-dominant inheritance. Patients develop multiple basal cell carcinomas beginning in childhood. They also have a typical dysmorphic facies, skeletal malformations, and a particular type of epithelial cyst of the jaws. Recent evidence localizes a Gorlin's syndrome locus on chromosome 9 at band q31. Both tumors and malformations of the central nervous system occur with Gorlin's syndrome. Medulloblastoma is the primary brain tumor most frequently associated with this syndrome; over 40 such cases have been reported. However, only seven cases of meningioma associated with Gorlin's syndrome have been described. The authors report the case of a woman with Gorlin's syndrome whose mother and maternal grandfather also had the condition. The patient was found to have a medulloblastoma at 4 years of age and presented with a large bifrontal meningioma at 19 years of age. The meningioma was histologically malignant and had a complex karyotype with multiple translocations including a t(5;9) with the breakpoint on chromosome 9 located at 9q32. The constitutional karyotype of the mother was normal. No mutations of exons 5 to 9 of the p53 gene were detected using single-stranded conformational polymorphism analysis.

The influence of diabetes mellitus on outcome from subarachnoid hemorrhage.

Diabetes mellitus (DM) has been shown to be a risk factor for subarachnoid hemorrhage (SAH). However, the influence of this disease on outcome from SAH has not been adequately studied. We retrospectively reviewed 150 patients with SAH, including 22 with and 128 without DM. Our results indicate that pre-existing DM does not significantly influence outcome from SAH when examined in conjunction with other chronic diseases and epidemiological factors.

Epidemiological characteristics of subarachnoid hemorrhage in an urban population.

Several risk factors for unfavorable outcome from subarachnoid hemorrhage (SAH) have been identified. The prevalence of such risk factors varies among ethnic groups and among men and women. The influence of ethnic background and gender as factors in the outcome after SAH has not been adequately studied and is the focus of the present investigation. Outcome in 145 consecutive patients was dichotomized as good and moderately disabled vs severely disabled, vegetative, and dead. A multiple logistic regression model was used to examine the factors of gender, ethnic group (white and non-white), age, admission neurological grade, pre-existing hypertension, and intravenous drug abuse. Our data reveal that hypertensive, white males, with a history of intravenous drug abuse, have a high risk of unfavorable outcome following SAH. These observations are important for the design and interpretation of future studies relating to SAH.

Intravenous cocaine abuse and subarachnoid haemorrhage: effect on outcome.

A retrospective study of subarachnoid hemorrhage associated with intravenous cocaine injection was undertaken in a large urban hospital. Patients who used intravenous cocaine had significantly poorer outcomes when compared with subarachnoid haemorrhage patients with no known exposure to the drug.

Evaluation of treatment of normal-pressure hydrocephalus.

Ten patients with dementia due to normal-pressure hydrocephalus were evaluated prospectively according to a planned, longitudinal protocol for 4 to 12 months. Information recorded at each visit included clinical history, medical and neurological examination, psychometric scoring by Mini-Mental Status Questionnaire, measurement of ventricular size and local cerebral blood flow, and partition coefficients (local lambda changes) (1 lambda) by xenon contrast computerized tomography scanning. Cerebrospinal fluid shunting was carried out in eight cases. Serial evaluations were repeated at intervals up to 8 months after shunting, and demonstrated that the ventricles decreased in size and periventricular hypodensities decreased. White matter 1 lambda values and blood flows and cortical gray matter flows progressively increased for 3 months after shunting, and remained increased except for one case complicated by chronic alcoholism. Clinical recovery correlated with improved cerebral perfusion. There were returns of urinary continence and improvements in gait and usually in activities of daily living. Mentation was the last factor to improve. Factors negatively influencing cerebral perfusion and clinical recovery were shunt failures and various contributing causes of dementia.

Pathogenesis of normal-pressure hydrocephalus--preliminary observations.

Eight cases with well-documented normal-pressure hydrocephalus were studied prospectively for 6 months by history, neurological examinations, Mini-Mental Status tests, xenon-contrast computed tomography measurements of local cerebral blood flow, and cerebral xenon solubility expressed as partition coefficients. Local cerebral blood flow and local partition coefficients were reduced throughout frontal and temporal lobes, basal ganglia, and thalamus. Cerebrospinal fluid shunting procedures were carried out in seven cases. As a result, local cerebral blood flow and local partition coefficients increased toward normal, particularly in frontal white matter, frontotemporal cortex, and basal ganglia. Ventricular size became reduced and mental status improved. Local partition coefficient values were reduced by increased tissue water because low values confirmed cerebrospinal fluid diffusion into white matter, which resolved after shunting. Patients likely to benefit from shunting, including shunt failures requiring revision, were detected.

Steroid hormone binding macromolecules in meningiomas. Failure to meet criteria of specific receptors.

Cytoplasmic estrogen and progesterone binding proteins were assayed in 26 meningiomas using an assay developed for the measurement of estrogen and progesterone receptors in human target tissue. The steroid specificity of the binding proteins was studied in both individual and pooled meningioma tissue by competitive binding assays. Eight meningiomas contained significant amounts of estrogen-binding protein and 18 contained significant amounts of progesterone-binding protein. There was no correlation between the amount of hormone-binding protein and patient age, sex, menstrual status or presentation, or tumor occurrence, location, or pathologic features. The competition studies demonstrated a lack of steroid specificity for these hormone-binding proteins. Therefore, the authors conclude that, contrary to recent reports, the hormone-binding proteins found in meningiomas are unlikely to be specific steroid receptors.

Operative repair of lumbosacral myelomeningocele. Technical note.

A technique is described for closure of lumbosacral myelomeningoceles. The pathological anatomy of these lesions is examined, and the junction of the skin and dura is identified as the "junctional zone." This zone permits maximal preservation of the available dura for watertight closure after operative dissection. The junctional zone also serves as an anchor for traction sutures, permitting skin closure without tension. Seventy consecutive repairs have been completed by the authors without significant complications. There have been no instances of cerebrospinal fluid leaks, meningitis, or wound dehiscence. In all cases the repair was carried out rapidly and in a single stage.

Giant intracranial and extracranial meningioma: case report and review of the literature.

Large extracranial extensions of intracranial meningiomas are rare. We report the case of a 63-year-old man with a giant parietal occipital meningioma, which was removed successfully without significant complications. The tumor's total 2600-g mass makes it one of the largest of central nervous system origin ever resected. We detail the problem of dealing with such a tumor and review similar cases.

Intrathecal and intraventricular morphine for pain in cancer patients: initial study.

Intractable pain in six cancer patients was treated with lumbar intrathecal morphine (two patients) and intraventricular morphine (four patients). Daily percutaneous injections of morphine through Ommaya reservoirs were made. Initially, 1 mg of lumbar intrathecal morphine resulted in pain relief for 10 to 14 hours, and 2.5 to 4.0 mg of intraventricular morphine gave relief for 12 to 24 hours. This treatment was continued for 3 to 7 months in three of the adults. Morphine requirements gradually increased. Side effects were minimal, and there were no complications.