Gangliocytoma of third ventricle: hyperphagia, somnolence, and dementia.

A 55-year-old man had hydrocephalus caused by a third-ventricular tumor. Mentation improved after ventricular shunting and radiation therapy. Progressive hyperphagia, obesity, memory impairment, and hypersomnolence developed 13 months later, and he died 2 years after diagnosis. At necropsy, a gangliocytoma filled the third ventricle, resulting in compression and necrosis of the ventromedial and posterior hypothalamic nuclei. Clinical manifestations were attributed to these lesions.

The relation of cerebral vasospasm to the extent and location of subarachnoid blood visualized by CT scan: a prospective study.

In 41 cases of verified ruptured saccular aneurysm, we prospectively predicted the presence or absence of delayed symptomatic cerebral vasospasm. CT criteria quantifying the extent and location of subarachnoid blood (developed in our previous retrospective study) were used in this prospective series of patients. Twenty-two patients had recognizable subarachnoid clots larger than 3 X 5 mm or layers of blood more than 1 mm thick (measured on reproduced images). In 20 of the 22 patients with severe significant clot or thick layer, severe vasospasm was correctly predicted and localized (2 false positives). In 19 patients with no blood, or diffuse blood, or blood outside the subarachnoid space, the absence of severe vasospasm was correctly predicted in 14 (5 false negatives). All of the false-positive and false-negative cases could be explained by inadequate CT technique. The data indicate that the extent and location of blood in the subarachnoid space determine the severity and location of vasospasm and that patients in jeopardy of developing symptomatic cerebral vasospasm can now be identified. Early preventive measures may now be assessed more accurately.

Techniques for positron scintigraphy of the brain.

Positron scintigrams were obtained in normal subjects and in patients with intracranial tumors and cerebral vascular disease, using a multicrystal positron camera. The radiopharmaceuticals were 68Ga complexed with adenosine triphosphate (68Ga-ATP), 13N-ammonia (13NH3), and 15O2. Six clinical cases are described to illustrate the different cerebral distributions of intravenously administered 68Ga-ATP, 13NH3, and inhaled 15O2. The possible value of these agents in the study of cerebral metabolism and in differential diagnosis of intracranial disease is discussed.

Giant cystic arachnoid granulations: a rare cause of lytic skull lesions.

Arachnoid granulations provide the pathway for drainage of cerebrospinal fluid from the subarachnoid space into the dural venous sinus system. They frequently produce small, well-defined indentations on the inner table of the calvarium that are easily recognized on radiographic studies and gross examination of the skull. We report a series of four giant cystic arachnoid granulations presenting as large "destructive" osteolytic lesions that required surgical exploration. The cysts were filled with cerebrospinal fluid and were delineated by a thin fibrous wall that contained peripheral clusters of arachnoid cells. We discuss the pathologic and radiographic differential diagnosis of osteolytic skull lesions and present a hypothesis regarding the formation of giant cystic arachnoid granulations.

Combined approach to "dumbbell" intrathoracic and intraspinal neurogenic tumors.

The unexpected finding of an extension of a neurogenic tumor from the thorax through the spinal foramen into the neural canal complicates its removal. Serious neurological complications may result from a two-stage approach, whether done first through the thorax or neural canal. Vertebral tomography or computed tomographic scanning reveals enlargement of a spinal foramen in advance of operation. Myelography confirms the probable presence of an intraspinal component. Four patients have been operated on using an approach designed to allow wide posterolateral thoracotomy and concomitant laminectomy for single-stage removal of the entire tumor. In 3 patients the diagnosis was schwannoma and in 1, neurofibroma. All had good results.

Retrosigmoid approach to acoustic neuroma (vestibular schwannoma).

The retrosigmoid approach for the microsurgical removal of an acoustic neuroma (vestibular schwannoma) is described, and perioperative medical management of the patient is discussed. The techniques for monitoring facial and cochlear nerve function are presented. The supine-oblique position, skin incision, bone removal, dural opening, and initial exposure are outlined. Important points in the technique for removing acoustic neuromas and preserving hearing, when possible, are described and illustrated.

Bilateral acoustic neuromas: clinical aspects, pathogenesis, and treatment.

Fifteen patients with bilateral acoustic neuromas are presented. Ten patients became symptomatic before age 21, and 9 of these patients developed additional central nervous system tumors. Cutaneous manifestations of neurofibromatosis and a positive family history of this disorder were absent in many of the patients. Most patients required multiple surgical procedures, and the timing, techniques, and results of these operations are reported. The pathogenesis of these tumors, the genetic aspects of this disorder, and potential treatment alternatives are discussed.

Intramedullary cavernous angiomas of the spinal cord: clinical presentation, pathological features, and surgical management.

Cavernous angiomas of the spinal cord are rare lesions that can cause severe neurological symptoms. We add a series of 6 patients with intramedullary cavernous angiomas of the spinal cord to 30 patients with 31 histologically verified lesions already described in the literature. Four types of clinical presentation were seen in the 36 patients: 1) discrete episodes of neurological deterioration with varying degrees of recovery between episodes (13 patients); 2) slow progression of neurological decline (12 patients); 3) acute onset of symptoms with rapid decline (8 patients); and 4) acute onset of mild symptoms with subsequent gradual decline lasting weeks to months (3 patients). Of the 36 patients, 25 (69%) were women and 11 (31%) were men. The peak age of presentation was in the fourth decade. The thoracic spinal cord was affected in more than half the patients, with the cervical cord being the next most common location. Histological examination demonstrated the closely apposed vascular channels characteristic of cavernous angiomas. While most vascular channels were thickened and hyalinized, three angiomas had foci of small vessels resembling a capillary angioma. The available data suggest that surgical management of intramedullary angiomas should attempt complete extirpation. The lesions are often well circumscribed with a glial plane between the lesion and normal cord. However, spinal cord angiomas, unlike cranial lesions, have little room for enlargement before devastating symptoms occur. We achieved complete excision in all of our patients, who are all ambulatory and have improved symptoms. Little, if any, increase in neurological deficit was caused by myelotomy and lesion excision. These spinal lesions should be considered for surgery early, before repeated hemorrhage or enlargement can occur.

A modified transfacial approach to the clivus.

Anterior approaches to the clivus must provide excellent visualization of the lesion, give adequate access for dural repair, and be cosmetically acceptable. Most current approaches enter through the nasopharynx or oropharynx, with either palatal, maxillary, or mandibular splitting for greater exposure. We have modified the transfacial approach described by others, which provides excellent access to the clivus along its rostrocaudal extent. A lateral rhinotomy incision is used and carried along the base of the right alae nasi and columella. The nasal bones are osteotomized bilaterally, and the nose is rotated on a pedicle flap, thus opening the entire nasal cavity to view. The septum and medial maxillary walls are removed. This provides excellent visualization of the ethmoid, sphenoid, posterior nasopharynx, and upper oropharynx. At the conclusion of the procedure, the nasal incision is closed, with good cosmesis. A case of recurrent chordoma of the middle and lower clivus is presented to exemplify this technique. The approach has since been used to approach clivus tumors and midline aneurysms of the vertebrobasilar system.

Cerebral vasospasm with ruptured saccular aneurysm--the clinical manifestations.

Fifty cases of verified intracranial ruptured saccular aneurysm were analyzed to investigate the relationship of the development of a delayed cerebral ischemic deficit to the presence of cerebral vasospasm visualized on angiography. Twenty-five patients developed a delayed ischemic deficit (DID), and all showed Grade 3+ or 4+ vasospasm. Nineteen patients had Grade 0, 1+, or 2+ vasospasm, and none developed a DID. It was concluded that in this study vasospasm accounted for all DID's and that in the absence of vasospasm DID did not occur. The DID occurred most often on Day 8 (7 of 25 cases).

Superior temporal gyrus approach to middle cerebral artery aneurysms: technique and results.

Aneurysms of the bifurcation of the middle cerebral artery (MCA) can be approached through a small incision in the anterior portion of the superior temporal gyrus. The pterion and the lateral aspect of the lesser wing of the sphenoid bone are removed. The aneurysm is approached, using microsurgical techniques, by following the main divisions of the MCA to the parent trunk and the base of the aneurysm. Once the parent vessel and the origin of the major divisions are clearly identified, it is usually preferable to dissect and mobilize the entire aneurysmal complex to elucidate the anatomy and prepare the neck for clipping. This approach offers the advantages of minimal brain retraction and minimal manipulation of the main trunk and perforators of the MCA. In addition, it allows a more complete exposure of the aneurysmal complex and facilitates dissection behind the aneurysm, which is more difficult when the aneurysm is approached from the front by opening the sylvian fissure medially to laterally. A potential disadvantage of this method is that proximal control is not obtained until the base of the aneurysm is reached, but this has not been a problem in our experience. Other disadvantages are the need for a slightly larger bone flap and the potentially increased risk of epilepsy. This approach is not suitable when the main trunk of the MCA is short and the aneurysm is in front of the insula. It is also not recommended for the rare cases in which the aneurysm points back over the insula. During a 6-year period, this approach was used in 49 of 58 cases of MCA aneurysm. The only deaths in this group occurred in patients who were in deep coma before operation. Two patients were made worse by operative complications, and 2 more worsened as a result of postoperative vasospasm. There was a significant incidence of thrombophlebitis and pulmonary embolism in this series.

Specific estradiol binding in schwannomas, meningiomas, and neurofibromas.

The cytoplasmic fractions of schwannomas (acoustic neuromas), meningiomas, and neurofibromas were assayed for the presence of estrogen receptors. Specific estradiol binding was detected in 7 of 16 schwannomas, 7 of 10 meningiomas, and 1 of 6 neurofibromas. A nontumorous vestibular nerve was also studied and showed no estradiol binding. In the tumors, the concentration of the estradiol binding sites as estimated by saturation binding analysis covered a wide range of values (21 to 2430 fmol/g of tumor) but, overall, meningiomas contained the highest amount of estradiol binder. A Scatchard plot analysis of one of the schwannoma specimens demonstrated high affinity estradiol binding (Ka = 1.695 X 10(10) M-1). Although there were more females than males in each tumor category, the overall incidence of estradiol binding was similar in males (5 of 11, 45%) and in females (10 of 21, 48%). In 5 cases, progestin binding was also measured and was detected in two meningiomas (both from female patients); one meningioma and two neurofibromas showed no progestin binding. A discussion is presented of the possible role of estradiol in the pathogenesis or modulation of meningeal and Schwann cell tumors as well as in the genetic disorder neurofibromatosis.

Risks of surgical management for cavernous malformations of the nervous system.

OBJECTIVE: As more information evolves regarding the natural history of cavernous malformations (CMs), the risks of operative intervention must be balanced against nonoperative management. In an attempt to better delineate the surgical risks for operable CMs, we undertook a retrospective analysis of 94 patients with 97 CMs surgically excised at the Massachusetts General Hospital. METHODS: Data regarding surgical complications and outcome measures, including neurological status and seizure outcome, were analyzed. RESULTS: The incidence of transient neurological morbidity was 20.6%, but only 4 of the 97 operations (4.1%) resulted in persistent disabling neurological complications and 2 (2.1 %) in nondisabling deficits. There was no operative mortality. Brain stem lesions (n=14) were associated with the highest incidence of neurological complications, both transient and persistent (odds ratio, 4.8; 95% confidence interval, 1.5-15.7). The overall neurological outcome was excellent or good in 89.7% of all lesions: 96.8% of lobar CMs (n=63), 64.2% of brain stem CMs (n=14), 87.5% of cerebellar CMs (n=8), 100% of cranial nerve CMs (n=4), and 75% of spinal cord CMs (n=8). Patients with brain stem and spinal cord CMs were in poorer preoperative neurological condition than were patients with CMs in other locations and therefore had a significantly reduced level of function after surgery (P < 0.01). There was improvement in 35.7% of the patients with brain stem lesions and 62.5% of the patients with spinal cord lesions after surgery. In the 38 patients presenting with seizures, 97% were seizure-free after surgery. CONCLUSION: The risks of operative management of CMs varies based on location. When evaluating patients with operable CMs for surgery, the incidence of complications as well as final neurological outcome should be carefully weighed against the existing knowledge of the natural history of lesions managed expectantly.

Large and giant paraclinoid aneurysms: surgical techniques, complications, and results.

Twenty-five patients with giant (greater than 25 mm in diameter) and 9 patients with large (15 to 25 mm in diameter) aneurysms of the internal carotid artery in the ophthalmic or paraophthalmic region are reviewed. In 23 of these patients the aneurysm was clipped directly. There was 1 death in this group, and none of the survivors had disabling neurological complications outside the visual system. The other 11 patients were treated by a trapping procedure or by either common carotid ligation or internal carotid ligation in the neck. Of the 5 patients treated by internal carotid ligation preceded by an extracranial to intracranial bypass graft, 3 developed embolic complications, which in 1 patient resulted in death. One of the 4 patients treated by ligation of the common carotid artery died 1 year later from a recurrent subarachnoid hemorrhage. Of the total group, 18 patients had visual loss preoperatively as a result of aneurysmal compression; in 10 the vision was improved by operation, in 3 it was made worse, and in 2 it was unchanged. In another patient the vision continued to deteriorate slowly after common carotid occlusion, and the other 2 patients died postoperatively before vision could be assessed. The complications in the patients are described and analyzed in detail. Maneuvers found to be of value in the direct approach to these lesions are described. Of these, exposure of the internal carotid artery in the neck for temporary occlusion during clipping and thorough drilling of the anterior clinoid process and unroofing of the optic canal were particularly helpful. The literature on indirect methods of treatment by carotid occlusion with and without bypass graft is reviewed with special reference to the complications and effectiveness of each alternative. Based on this review of the literature and our experience, a treatment scheme is suggested for these aneurysms depending on their mode of presentation.

Giant intracranial aneurysms of the anterior circulation: clinical characteristics and diagnosis by computed tomography.

The computed tomography (CT) characteristics and clinical features of giant (globoid) aneurysms of the anterior circulation are reviewed. These lesions appear on the CT scan as smoothly encapsulated ovoid masses, within which a partially patent lumen is seen after the infusion of iodinated contrast material. With careful analysis of the CT scan it may be possible to differentiate giant internal carotid artery, anterior cerebral artery, and middle cerebral artery aneurysms from other parasellar and hemispheric lesions.

Temporal clustering of hemorrhages from untreated cavernous malformations of the central nervous system.

OBJECTIVE: Hemorrhages from cerebral cavernous malformations (CMs) sometimes seem to occur in closely spaced "clusters" interspersed with long hemorrhage-free intervals. Clustering of hemorrhages could affect retrospective assessments of radiosurgery efficacy in prevention of CM rehemorrhage. However, this empirical observation had not been tested quantitatively. To test whether CM hemorrhages tend to cluster, we reviewed pretreatment rebleeding rates after a first symptomatic hemorrhage in CM patients who later underwent surgery or radiosurgery. METHODS: We performed a retrospective review of 141 patients with CMs who presented with clinically overt hemorrhage, and who subsequently underwent surgery or proton beam radiosurgery during an 18-year period. Statistical models were used to analyze all events per person and identify potential variation in rebleeding risk with time after a previous hemorrhage. RESULTS: Sixty-three of 141 patients experienced a second hemorrhage before treatment; 16 had additional hemorrhages. Five hundred thirty-eight patient years elapsed between first hemorrhages and treatment. The cumulative incidence of a second hemorrhage after the first CM hemorrhage was 14% after 1 year and 56% after 5 years. During the first 2.5 years after a hemorrhage, the monthly rehemorrhage hazard was 2%. The risk then decreased spontaneously to less than 1% per month, which represents a 2.4-fold decline (P < 0.001). Rehemorrhage rates were higher in younger patients (P < 0.01), but not in females or in patients with deep lesions. Shorter intervals between successive hemorrhages did not predict higher subsequent rehemorrhage risk. CONCLUSION: The rehemorrhage rate from untreated CMs is high initially, and it decreases 2 to 3 years after a previous hemorrhage. This hazard pattern generates the observed temporal clustering of hemorrhages from untreated CMs.

The tradition of Harvey Cushing commemorated by a stamp in the Great American stamp series. The 1987 AANS (American Association of Neurological Surgeons) presidential address.

With the announcement that Harvey Cushing is to be honored by a United States postage stamp in the Great American stamp series, the qualities that this remarkable man possessed are reviewed--artist, author, bibliophile, scientist, soldier, physician, and teacher. The events that led to Cushing becoming a neurosurgeon are summarized. The recognition by the United States Postal Service of physicians and others who have appeared on stamps that had some relationship to Cushing's activities is discussed. Based on the tradition of Harvey Cushing, eight guidelines are presented.

Basal rupture of saccular aneurysm. A pathological case report.

In a case of subarachnoid hemorrhage, the arteries of the circle of Willis were left undisturbed and undissected at postmortem examination. A block of frontal lobe tissue with the attached vessels was serially sectioned disclosing in its entirety a saccular aneurysm that had ruptured at the base rather than the dome. The histology of the hemostatic process was clearly depicted.

Posterior fossa craniotomy for lesions of the cerebellopontine angle. Technical note.

A safe technique is described for performing a lateral posterior fossa craniotomy to gain access to the cerebellopontine angle. The method makes use of currently available high-speed air drills. Thus, it is possible to replace the removed bone at the conclusion of the procedure and to re-establish normal tissue planes while providing rigid protection to the posterior fossa.

Neurotrauma care and the neurosurgeon: a statement from the Joint Section on Trauma of the AANS and CNS.

The AANS and CNS support the concept of organized neurosurgical trauma care consisting of the appropriate combination of prepared communities and institutions and of adequate numbers of committed neurosurgeons. Furthermore, we support the guidelines of the ACS regarding institutions designated to receive trauma patients, and support the concept of prehospital triage of trauma victims based upon well-trained EMS personnel guided by criteria selected by the local neurosurgical community. We support audit systems that include neurosurgeons to assess and improve the quality of neurotrauma care. By using the principles outlined above, neurosurgeons can plan, support, and evaluate whatever system their community or region requires for the optimal care of the neurotrauma patient.