✓ The authors report a case of chondrosarcoma arising in the cerebellum 16 years after treatment of a cerebellar malignant astrocytoma by subtotal resection and irradiation. It is thought that the chondrosarcoma arising within the intracranial cavity was a probable consequence of previous ionizing radiation.
Mark Bernstein, Richard G. Perrin, Marjorie E. Platts and William J. Simpson
Mark Bernstein, Robert A. Hegele, Fred Gentili, Michael Brothers, Richard Holgate, William C. Sturtridge and John Deck
✓ The authors report a case of pituitary apoplexy resulting in bilateral internal carotid artery occlusion, with marked depression of consciousness and hemiplegia. After transsphenoidal tumor decompression, restoration of flow in both carotid arteries was documented angiographically and the patient made an excellent clinical recovery. The unique aspect of this case is that the pituitary apoplexy was apparently precipitated by neuroendocrine manipulation, performed as a preoperative test of pituitary function.
Long-term follow-up and treatment guidelines
Mark Bernstein, Harold J. Hoffman, William C. Halliday, E. Bruce Hendrick and Robin P. Humphreys
✓ The authors review the cases of 60 children (aged 5 months to 18 years) with thalamic tumors who were seen at The Hospital for Sick Children between 1951 and 1983. The diagnosis of thalamic tumor was based on neuroradiological studies; 33 children were first seen in the pre-computerized tomography (CT) era and 27 since the advent of CT scanning. Histological diagnosis was obtained in 37 patients at the time of operation. A further four tumors were verified histologically at autopsy. Thirty-six patients required cerebrospinal fluid shunt placement. Sixteen patients had no surgery on their tumor, three underwent needle biopsy, 20 underwent open biopsy, and 21 had partial resection. Forty-four patients were irradiated. All 20 patients with malignant tumors died, with a mean survival time of 1.1 years. Of 19 patients with benign tumors, 11 died, with a mean survival time of 5.3 years, and eight are still alive after a mean period of 7.2 years since diagnosis. Based on this series, the authors recommend open biopsy of thalamic tumors (and resection if deemed safe), followed by radiation in selected cases.
Pierre Lasjaunias, Ming Chiu, Karel Ter Brugge, Atul Tolia, Michel Hurth and Mark Bernstein
✓ The authors describe their experience with four cases of dural arteriovenous malformation (AVM) which led them to analyze the clinical aspects of these lesions in an attempt to understand their pathophysiology. An additional 191 previously reported cases of dural AVM's were reviewed with special attention to the mechanism of intradural, central, and peripheral nervous system manifestations. Apart from the peripheral cranial nerve symptoms, which are most likely due to arterial steal, the central nervous system (CNS) symptoms appear to be related to passive venous hypertension and/or congestion. Generalized CNS symptoms can be related to cerebrospinal fluid malabsorption due either to increased pressure in the superior sagittal sinus, to venous sinus thrombosis, or to meningeal reaction resulting from minimal subarachnoid hemorrhages. These phenomena are not related to the anatomical type of venous drainage. On the other hand, focal CNS symptoms are specifically indicative of cortical venous drainage. Seizures, transient ischemic attacks, motor weakness, and brain-stem and cerebellar symptoms can be encountered depending on the territory of the draining vein or veins. Therefore, the localizing value of focal CNS symptomatology relates to the venous territory and not to the nidus or to the arterial supply characteristics of dural AVM's. Furthermore, the venous patterns of various dural AVM's at the base of the skull are expressed by differences in their clinical presentation. Dural AVM's of the floor of the anterior cranial fossa and of the tentorium are almost always drained by the cortical veins and, therefore, have a high risk of intradural bleeding.
The remarkable similarities in the manifestations of dural and brain AVM's and the differences in the manifestations of dural and spinal dural AVM's are pointed out. High-quality angiograms and a multidisciplinary approach to the study of dural AVM's will provide the best understanding of their symptoms and, therefore, the most appropriate treatment strategy.
Douglas Kondziolka, Mark Bernstein, Lothar Resch, Charles H. Tator, J. F. Ross Fleming, R. G. Vanderlinden and Hart Schutz
✓ A retrospective clinical and pathological review of 905 consecutive brain tumor cases (excluding pituitary adenoma and recurrent tumor) was conducted to identify cases in which intratumoral hemorrhage was confirmed grossly and/or pathologically. There were 132 cases so identified, for an overall tumor hemorrhage rate of 14.6%; of these, 5.4% were classified as macroscopic and 9.2% as microscopic. The presence of hemorrhage was correlated with the neurological presentation. The highest hemorrhage rate (70.0%) was found in patients with prior neurological history who experienced apoplectic deterioration (acute-on-chronic presentation). Only 57.1% of patients with acute deterioration in the absence of prior neurological symptoms had hemorrhages. The highest hemorrhage rate for primary brain tumors was 29.2% for mixed oligodendroglioma/astrocytoma, while the highest hemorrhage rate for any tumor type was 50% for metastatic melanoma. The clinical relevance of tumor hemorrhage is discussed.
Douglas Kondziolka, Mark Bernstein, S. M. Spiegel and Karel ter Brugge
✓ The authors describe three cases of clinical cerebral ischemia associated with angiographic evidence of cerebral arterial luminal narrowing presenting 7, 14, and 52 weeks after subarachnoid hemorrhage (SAH) and aneurysm clipping. Delayed vasospasm, in its usual time setting 1 or 2 weeks after hemorrhage, did not occur symptomatically in these patients. No evidence for aneurysm clip migration or rebleed was present. All patients responded favorably to volume expansion and elevation of blood pressure. This unusual occurrence of a very delayed vasospasm may further the understanding of the vasospastic process. The symptomatic onset of arterial luminal narrowing months after SAH may suggest that a proliferative vasculopathy more accurately explains the observed vessel narrowing, rather than conventional active constriction of vascular smooth muscle.
Fred S. C. Kim, James T. Rutka, Mark Bernstein, Lothar Resch, Ellen Warner and Dominic Pantalony
✓ Granulocytic sarcoma usually occurs in the setting of leukemia and myeloproliferative disorders. Rarely, it can occur in isolation at various anatomical sites without hematological evidence of leukemia. The unique case of an elderly man presenting with right L2–3 radiculopathy is described. Intradural granulocytic sarcoma of the L-2 and L-3 nerve roots with extradural extension was found at surgery and he was treated with incomplete resection and antileukemic chemotherapy. Local recurrence at 3 months was treated with irradiation. Granulocytic sarcoma is frequently misdiagnosed and invariably progresses to acute leukemia. The chloroacetic acid esterase, granulocytic immunohistochemical markers, and electron microscopy appearance can aid in diagnosis. The prognosis is improved with initial aggressive antileukemic chemotherapy and local irradiation.
Thomas J. Zwimpfer and Mark Bernstein
✓ The hallmark of concussion injuries of the nervous system is the rapid and complete resolution of neurological deficits. Cerebral concussion has been well studied, both clinically and experimentally. In comparison, spinal cord concussion (SCC) is poorly understood. The clinical and radiological features of 19 SCC injuries in the general population are presented. Spinal cord injuries were classified as concussions if they met three criteria: 1) spinal trauma immediately preceded the onset of neurological deficits; 2) neurological deficits were consistent with spinal cord involvement at the level of injury; and 3) complete neurological recovery occurred within 72 hours after injury.
Most cases involved young males, injured during athletics or due to falls. Concussion occurred at the two most unstable spinal regions, 16 involving the cervical spinal and three the thoracolumbar junction. Fifteen cases presented with combined sensorimotor deficits, while four exhibited only sensory disturbances. Many patients showed signs of recovery with the first few hours after injury and most had completely recovered within 24 hours. Only one case involved an unstable spinal injury. There was no evidence of ligamentous instability, spinal stenosis, or canal encroachment in the remaining 18 cases. Two patients, both children, suffered recurrent SCC injuries. No delayed deterioration or permanent cord injuries occurred.
Spinal abnormalities that would predispose the spinal cord to a compressive injury were present in only one of the 19 cases. This suggests that, as opposed to direct cord compression, SCC may be the result of an indirect cord injury. Possible mechanisms are discussed.