High concentrations of catecholamines in human hypothalamic-hypophysial blood.

While the hypothalamic-hypophysial portal system has been extensively studied in laboratory animals, equivalent studies have not been performed in humans. Here, we present an experimental procedure for collecting suprapituitary blood in man. To solve the question on the origin of such blood we investigated specific markers of hypothalamic secretory activity: the catecholamines (CAs). We found (a) norepinephrine (NE), dopamine (DA), and epinephrine (E) concentrations from approximately 1.5 to 2.5, 3.5 to 4.5, and 6- to 10-fold higher, respectively, in suprapituitary than peripheral blood, (b) different NE/DA and NE/E ratios in favor of DA and E in suprapituitary blood, and (c), a complete (100%) group separation (suprapituitary vs. peripheral) when discriminant analysis included only DA and E. These data indicate that suprapituitary blood composition is different from that of the peripheral blood, and is particularly rich in CAs and claimed differences between DA and E release on one hand and NE release on the other in suprapituitary blood also are observed. We advance the hypothesis of a hypothalamic source of such amines draining via median eminence into portal vasculature, and name this blood "hypothalamic-hypophysial blood." Besides serving as "classical" neurotransmitters, CAs may also have a direct neurohormonal role in the regulation of the human hypothalamic-hypophysial function.

Naloxone potentiation of 2-Br-alpha-ergocryptine (CB 154) effects on GH secretion in man.

The effect of the combined administration of either CB 154 (2.5 mg/osM) or L-DOPA (500 mg/osM) and naloxone have been studied in six normal male volunteers. Naloxone cause a clear-cut potentiation of CB 154 effects on human growth hormone (hGH) secretion and only a slight, statistically non-significant, potentiation of L-DOPA effects on hGH secretion. These results have been discussed in the light of morphofunctional findings showing possible interactions between dopamine (DA) systems and somatostatin-positive and enkephalin-positive nerve terminals.

Primary intrasellar germinoma: case report.

Intracranial germinomas arising primarily within the sella turcica are extremely rare. Preoperative diagnosis is difficult to establish even with sophisticated procedures. Diabetes insipidus is the main clinical manifestation. The authors report a case of an apparently primary intrasellar germinoma causing subclinical pituitary apoplexy in a 12-year-old boy. The transsphenoidal approach and appropriate radiotherapeutic management were employed with a good outcome.

Cerebral aspergillosis following intracranial surgery. Case report.

The authors report a case of cerebral aspergillosis. This is only the fifth case following intracranial surgery noted in the literature. Pathogenesis, angiographic findings, and results of cerebrospinal fluid culture are discussed.

Infratentorial arachnoid cysts.

The infratentorial compartment represents the second most common location of arachnoid malformations. Ten arachnoid cysts of the posterior fossa, operated on between 1970 and 1983, are reviewed. These lesions, although congenital and developmental in nature, may present at any age, and males are more frequently affected. A high rate of birth-related trauma (50% in this series) is conceivably due to fetal macrocranium, and the enlarged head and psychomotor retardation prevail in infancy and childhood. In arachnoid cysts occurring during adulthood, symptoms and signs more clearly indicate a dysfunction of the posterior fossa. Besides computerized tomography, pneumoencephalography and metrizamide techniques are recommended to rule out a Dandy-Walker syndrome in doubtful cases, and to obtain information about the cerebrospinal fluid (CSF) circulation. It is particularly important to establish the presence and type of communication of cysts with the CSF pathways. Although infratentorial cysts often communicate, they can be space-occupying masses because of increasing CSF retention, which may be due to a ball-valve mechanism or to inadequate communication. The frequently associated hydrocephalus (seven of the 10 cases in this series had hydrocephalus) seemed to be dependent mainly upon mechanical factors. The authors discuss the indications for intracranial surgery versus shunting procedures and report the results achieved by direct cyst excision.

Stereotaxic rostral mesencephalotomy in treatment of malignant faciothoracobrachial pain syndromes. A survey of 14 treated patients.

Stereotaxic rostral mesencephalotomy was performed 19 times in 14 patients suffering from intractable pain syndromes due to malignant diseases. The satisfying results in terms of pain relief during a necessarily short follow-up period (mean 4.9 months) are outlined. Undesired side effects were mainly confined to oculomotor disorders, which partly subsided over the months following the operation. Some technical aspects of the procedure and the pertinent literature are briefly discussed.

Multiple recurrences of meningiomas.

The authors report a case of a patient with a right sphenoid wing and a right parasagittal fronto parietal meningioma diagnosed 25 years after a right sphenoid wing meningioma had been removed. There was no evidence of Von Recklinghausen disease. The two tumors were benign. The problems concerning multiple meningiomas and recurrences of meningioma are briefly discussed.

Pineal germinoma with massive supratentorial extension. Case report.

An uncommon case of huge pineal germinoma without associated ventricular enlargement is presented. The tumor extended supratentorially in dumb-bell form to affect both cerebral hemispheres, simulating a glioma at neuroradiological examinations. Subtotal removal through a bilateral parieto-occipital approach plus radiation therapy were followed by relief of symptoms for over one year since surgery. The misleading patterns of the tumor and the delayed onset of symptoms in our case are outlined.

Surgical management of cerebral cavernous angiomas causing epilepsy.

Epilepsy is the most frequent presenting symptom in patients with cerebral cavernous angiomas. Surgical removal of these vascular malformations causing epilepsy is usually recommended for patients with disabling, intractable seizures, while conservative management is indicated for neurologically intact patients with well controlled seizures. In this paper is reported a retrospective series of 36 patients suffering epilepsy from cerebral cavernous angiomas, and admitted at our Institution between 1975 and 1992. All patients were surgically treated; mortality and morbility were absent. The mean follow-up period was 5.9 years, since the 36 patients were alive and available for a control review in December, 1993. Seizure outcome of the patients resulted in a complete cure of the epileptic disorders in 9 (25%), improved seizure control with decreased medication in other 11 (30.5%), and epilepsy control with the same preoperative therapy in the remaining 16 (44.5%) patients. Furthermore, patients with preoperative neurological signs associated to epilepsy resulted improved. The authors conclude that surgery, where it is unlikely to cause neurological deficits, may allow a definitive cure of epilepsy in patients with cerebral cavernous angiomas, preventing the possible risks from hemorrhage and mass effect.

Bilateral traumatic third nerve palsy.

Bilateral palsy of the third cranial nerve in a head-injured patient is described. Delayed computed tomography scanning demonstrated a midline necrotic lesion within the mesencephalon ventral to the aqueduct. The lesion, possibly the sequela of a focal contusion, involved both third nerve nuclear complexes and caused paralysis of their voluntary as well as autonomic functions. Gaze mechanisms and long tracts appeared to be less heavily damaged. The literature dealing with third nerve palsy, particularly bilateral cases, from traumatic and other origins is briefly reviewed.

Extraneural metastases from primary pineal tumors. Review of the literature.

Extraneural metastases from primary pineal tumors are extremely rare: only 15 such cases could be found in th literature. Another case is presented of a 10-year-old boy who developed multiple pulmonary and skeletal metastases from a presumed primary pineal germinoma. The incidence and pathogenesis of distant dissemination in the different types of pineal tumors are discussed, with special comment on the controversial role of the direct surgical approach and shunting of cerebrospinal fluid.

Arachnoid cysts of the middle cranial fossa: a clinical and radiological study of 25 cases treated surgically.

Arachnoid cysts of the middle cranial fossa are probably not so infrequent as previously thought. Twenty-five cases are reported together with the most relevant clinical and radiological findings. The authors emphasize the importance of computerized tomography (CT) in providing pre- and postoperative anatomical and physiopathological information. The potential morbidity of these lesions is stressed and surgical indications are discussed with preference for a radical craniotomy rather than a shunt procedure.

CT scan and metrizamide CT cisternography in arachnoid cysts of the middle cranial fossa: classification and pathophysiological aspects.

Thirty-one cases of arachnoid cysts of the middle cranial fossa were investigated by CT scan, 7 of them undergoing additional metrizamide CT cisternography. In this paper we analyze and correlate the different findings from these two examinations and propose a classification of arachnoid cysts of the middle cranial fossa into three basic types. Pathophysiological and surgical implications are discussed.

Stereotaxic biopsy and radioactive implantation for interstitial therapy of tumors of the pineal region.

Stereotaxic biopsy was performed in 10 patients with tumors of the pineal region. On the basis of intraoperative tissue diagnosis, low-energy radioactive sources (125I) were implanted in seven patients for interstitial irradiation during the same stereotaxic procedure. Results were good in five cases. This therapeutic modality appears to be indicated in cases of proven low-grade malignancy, inasmuch as the implantation of radioactive sources in a highly malignant lesion carries the risk of severe, often irreversible, damage to the surrounding brain, because of possible seed migration.

Surgical management of cavernous angiomas in children.

Cavernous angiomas are vascular malformations affecting any part of the central nervous system (CNS). The management of asymptomatic cavernous angiomas is still debated due to their poorly understood natural history, although more data are now available regarding results of surgical treatment in symptomatic cases. The authors report their surgical experience with 18 pediatric patients operated on for symptomatic CNS cavernous angiomas. The children ranged in age from 10 months to 17 years, without a relevant sex difference. Cavernous angiomas were intracranial in 17 cases: 15 being in the supratentorial compartments and two in the cerebellum. Clinical manifestations were as follows: seizures in 11 cases, focal neurologic deficits in five, and headache in one. The 18th case was observed in a girl showing paraparesis in the spinal subdural-extramedullary space at T8-T9 level. Excision of four deep cerebral lesions was performed after stereotactic localization through non-eloquent cortex. Pathologic confirmation of cavernous angiomas was obtained in all patients. Mortality from surgical procedures was absent in this series. The follow-up period ranged from 1 to 16 years. All 11 epileptic patients obtained seizure control; improvement or stabilization of neurologic symptoms was observed in the remaining seven patients.

N-myc and c-myc oncogenes amplification in medulloblastomas. Evidence of particularly aggressive behavior of a tumor with c-myc amplification.

N-myc and c-myc amplification was investigated in 27 medulloblastomas. DNA was extracted from 19 formalin fixed and paraffin embedded tumors and from fresh frozen tumor tissue in 8 other cases. The results showed no evidence of amplification of N-myc oncogene and only 1 case had a 27 fold amplification of c-myc. Cytogenetically, this neoplasm presented numerous double minute chromosomes (DMs). Moreover, it had an unusual rapidly aggressive course with massive cerebrospinal fluid dissemination unresponsive to intrathecal chemotherapy. Our results indicate a low incidence of N-myc and c-myc gene amplification in medulloblastomas, suggesting that the oncogenic mechanism in these neoplasms is not closely related to DNA gene amplification. C-myc amplification, although not frequently observed, may however provide a growth advantage for medulloblastoma cells in vivo, favoring their rapid dissemination. Medulloblastomas with c-myc activation may represent a subgroup of tumors with a more aggressive behavior.

Establishment of a human medulloblastoma cell line (BO-101) demonstrating skeletal muscle differentiation.

A permanent cell line, BO-101, was derived from a classic vermian medulloblastoma in a 9-year-old child. This line grew in vitro in adherent cultures and grew in athymic mice as serially transplantable intracranial and subcutaneous xenografts. Intracranial neoplasms grew as masses of small cells, which focally showed large cells with intense immunoreactivity for desmin, myoglobin and alpha-striated actin. The rhabdomyoblastic nature of these cells was confirmed ultrastructurally. The primary neoplasm showed immunoreactivity for synaptophysin, neuron-specific enolase and vimentin. A large panel of monoclonal antibodies and antisera against neuronal and glial antigens failed to show glial and neuronal immunoreactivity in the cell culture and xenografts. Despite the marked genotypic and phenotypic differences, the original neoplasm and the cell line share a common chromosomal marker del (12) (p 13.1). The BO-101 line differs phenotypically and genotypically from previously established medulloblastoma cell lines and further supports the heterogeneous biologic properties of the cell populations that constitute these neoplasms.

[Neuroblastoma metastatic to the skull. Radiologic aspects and therapeutic problems]. / Neuroblastoma metastatico al cranio. Aspetti radiologici e problemi terapeutici.

Clinical-radiological findings, treatment and prognosis of cranial involvement in 19 cases of neuroblastoma stage IV have been considered. The most frequent clinical features were periorbital ecchymosis and intracranial hypertension. The radiographic aspects, graduated according to the type of the lesion, showed a close correlation with clinical findings and sometimes preceded them. Computerized tomography, carried out in 12 cases, was more reliable than plain films in identifying the site and extent of cranial lesions and the presence of cerebral extensions, as occurred in 2 patients. The presence of cranial involvement at diagnosis was an unfavorable prognostic sign. The 19 children were treated in various ways so that firm conclusions cannot be drawn, but cranial radiotherapy in combination with chemotherapy appeared to be more efficacious than chemotherapy alone.