Stroke due to vertebral artery injury.

Minimal neck injury produced vertebral artery damage in two women and three men, 20 to 57 years of age. Serious neurologic deterioration occurred in two patients when positioned for roentgenographic procedures. Dissection or thrombosis of one or both vertebral arteries while in the transverse foramina occurred in three, one at the site of an osteophyte. The arteriogram of one patient showed a traumatic aneurysm at C-7, and a second patient had segmental arterial stenosis with bilateral traumatic aneurysms at C-5. The location of the arterial injury is not restricted to the occipitoatlantoaxial level but includes the course through the transverse foramina. The use of anticoagulation appears to be important in the immediate management of this process.

Xenon-enhanced computed tomography in brain death.

The absence of cerebral blood flow is a valuable adjunct confirming clinical criteria of brain death. However, current methods to confirm absent cerebral blood flow have problems that limit their clinical use. We reviewed cerebral blood flow data obtained with xenon-enhanced computed tomography in nine patients who were being evaluated for brain death. In eight patients who met clinical criteria for brain death, mean cerebral blood flow was 1.6 +/- 2.0 mL X 100 g X min. This value was within the range of error inherent in the method, and therefore represented absent flow. In a patient with persistent respiratory efforts, flow values compatible with absent flow were obtained in the supratentorial compartment, while mean flows as high as 24 mL X 100 g X min were measured in selected regions of interest in the infratentorial compartment, correlating with the clinical evidence of residual function of the brain stem. Xenon-enhanced computed tomography may be a useful test to confirm the absence of cerebral blood flow in patients being evaluated for brain death.

Superficial temporal to middle cerebral artery anastomosis. Clinical outcome in patients with ischemia of infarction in internal carotid artery distribution.

The clinical outcome of our first 40 patients (six with transient ischemic attacks, 22 with mild ischemic infarctions, and 12 with moderate ischemic infarctions) treated with a superficial temporal artery-middle cerebral artery anastomosis was analyzed. All cerebral ischemias or infarctions occurred in the internal carotid artery distribution. An independent neurologist observer recorded the patients preoperative and postoperative medical and neurological histories and objective neurological findings. There was no operative mortality. During the period of observation (up to 36 months), four patients died of probable myocardial infarction. No patient suffered from recurrent cerebral infarction. Three patients experienced a single ischemic event postoperatively. Neurological deficits were either unchanged (21 patients) or improved (19 patients). Postoperative angiograms showed patency in 97% of the anastomoses.

Childhood oligodendrogliomas presenting with seizures and low-density lesions on computed tomography.

Three children with cerebral oligodendrogliomas causing partial complex or generalized seizures presented with completely normal neurologic examinations. CT showed low-density, nonenhancing surface lesions. Although these CT features are usually associated with infarcts or cysts, neoplasm was suspected because of irregularity of the margins and erosion of the adjacent inner table of the skull. Oligodendrogliomas often enlarge slowly and may cause seizures years before they produce focal neurologic signs. CT of all children with seizures not responsive to anticonvulsant medication and focal clinical or EEG abnormalities will hasten diagnosis of slowly growing intracranial mass lesions.

Advances in the genetics of cerebrovascular disease and stroke.

MEDLINE searches identified epidemiologic, experimental, and clinical studies on the genetics of cerebrovascular disease and stroke, including the following topics: genetic epidemiology of stroke; genetics of systemic disorders that cause ischemic stroke, including coagulation disorders, connective tissue disorders, vasculopathies, metabolic disorders, and disorders of unknown etiology; and genetics of systemic disorders that cause hemorrhagic stroke. Recent discoveries in stroke genetics involve the genetic basis of monogenic disorders such as cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy and sickle cell disease. Reproducing similar advances in other forms of cerebrovascular disease and stroke will be more difficult because their inheritance is complex, multigenic, and heterogeneous. However, the future is promising with the application of molecular genetic approaches such as linkage analysis, allele-sharing methods, association studies, and polygenic analysis of experimental crosses as well as the transmission/disequilibrium test--a statistical method for detection of linkage between a marker and a disease-susceptibility locus.

Intracerebral hemorrhage in the full-term neonatal infant.

Supratentorial intracerebral hemorrhage was diagnosed in 18 full-term neonates, including six with primarily intraparenchymal hemorrhage and 12 with primarily intraventricular hemorrhage. Precipitating or associated factors were hypoxic-ischemic injury in five patients, polycythemia in two, and cranial birth trauma in two. Nine other infants had no identifiable medical risk events. The pathogenesis of intraparenchymal hemorrhage was probably related to hemorrhagic infarction, but the pathogenesis of intraventricular hemorrhage was often unknown. All 17 survivors returned for neurologic and developmental examinations between 1 and 7 years of age. Follow-up assessments were normal in nine children and abnormal in eight. Two had perceptual difficulties, three had moderate-to-severe cognitive deficiencies (two of the three had hemiplegia), and three had severe mental and neurologic handicaps. Eight of nine children with known or suspected hypoxic-ischemic or traumatic insults suffered moderate-to-severe disabilities whereas eight of nine children with no known precipitating cause for their hemorrhage developed normally.

Long-term outcome of Hurler syndrome following bone marrow transplantation.

Previous reports suggested a therapeutic response of lysosomal storage diseases such as Hurler syndrome following bone marrow transplantation. However, a clearer understanding of outcome has awaited long-term follow-up. We evaluated prospectively 11 consecutive patients with Hurler syndrome receiving marrow from an HLA-identical sib donor between September 1983-October 1988. Follow-up evaluations included assessment of donor engraftment by restriction fragment polymorphism analysis, determination of leukocyte alpha-L-iduronidase level, measurement of lumbar cerebrospinal fluid (CSF) pressure, computerized tomography (CT) of the brain, and psychometric testing. In this series there was a survival rate of 9/11 (82%) with all survivors showing complete (7 patients) or partial (2 patients) donor engraftment. Prospective longitudinal evaluation of the 9 surviving children, now 3.8-8.9 years posttransplantation (median 5.5) demonstrated persistence of previously deficient leukocyte alpha-L-iduronidase at levels reflecting the donor genotype and degree of donor engraftment. Urinary glycosaminoglycan excretion declined to near-normal within 5 months of donor engraftment. Prior to treatment, 7 of 8 children studied were found to have occult intracranial hypertension (lumbar CSF pressure > 20 cm CSF); however, all surviving children attained normal or near-normal pressure within 18 months of donor engraftment. Longterm follow-up CT imaging of the brain did not show progressive volume loss (cerebral atrophy) after donor engraftment. Of 9 survivors, 4 children having a developmental quotient (DQ, Mental Development Index on Bayley Scales of Infant Development) above 80 prior to transplantation subsequently maintained IQ scores above this level. However, 5 patients with lower pretransplant DQ scores now have significant cognitive deficits and attention deficit hyperactivity disorder. Progressive brain damage resulting from communicating hydrocephalus may be prevented by successful engraftment. Early transplantation of children with Hurler syndrome who have normal intelligence is likely to have the clearest benefit because long-term intellectual outcome will be limited by brain damage which has occurred prior to treatment.

The use of nonionic contrast agents in neuroangiography. A review of the literature and recommendations for clinical use.

RATIONALE AND OBJECTIVES: Several large studies have demonstrated the improved safety record of nonionic versus ionic contrast agents for intravenous administration. However, nonionic agents are much more expensive than ionic agents. The author addresses whether, given this large cost differential, nonionic contrast agents should always be used in neuroangiography (cerebral and spinal cord angiography and intravascular neurointerventional procedures). The answer could come from a closer examination of the effects of contrast agents on the brain. METHODS: There have been a number of animal experiments and clinical trials performed using a variety of available intravascular contrast agents. In an attempt to arrive at some reasonable conclusions regarding the use of contrast agents today, the author reviews several of these studies. In the human studies, three areas were analyzed: 1) cerebral angiography, 2) spinal cord angiography, and 3) intravascular neurointervention. The author explains why demonstrating the effect of a contrast agent on the brain or spinal cord in the clinical setting is more difficult than studying the effect of this agent on the liver, heart, or kidney. For example, obtaining objective measurements of altered cerebral physiology following intravascular injection of a contrast agent may itself alter the physiology. In lieu of objective measurements, investigators must rely on apparent changes in behavior, mentation, or the production of a focal neurologic deficit. However, it is extremely difficult, if not impossible, to separate the effect of the contrast agent from the effects of the arteriographic procedure, or from the disease process being evaluated. RESULTS: The neuronal environment is protected by the blood-brain barrier. A number of animal experiments have demonstrated that nonionic agents produce breakage of the blood-brain barrier less frequently than do ionic agents. In these studies, nonionic agents also produced fewer neurologic effects than did ionic agents. The human studies showed no statistically significant differences in neurologic effects when ionic and nonionic agents were compared. Cerebral: minor changes in heart rate were more common with ionic than with nonionic agents; there were no significant electroencephalogram changes in any of the patients studied. Spinal cord: the effects of intravascular injections of contrast material into the spinal cord of experimental animals have been rarely evaluated; direct comparisons of contrast agents in human spinal cord angiography have not been performed. Neurointervention: there have been no comparative studies of different contrast agents used during intravascular neurointerventional procedures in humans. CONCLUSIONS: Extensive animal data demonstrate that nonionic contrast agents are safer than ionic for cerebral angiography. Animals in the cited studies show less blood-brain barrier disruption, fewer direct neuronal effects, and fewer neurobehavioral deficits. However, the overwhelming conclusion from the human studies is that, while there is evidence in the experimental animal that nonionic agents produce fewer neurologic effects than do ionic agents, no study to date has been able to translate these findings into an apparent clinical difference in humans, mainly because it's so difficult to detect and measure neurologic changes in human trials. In addition, differences in neurologic effects between contrast agents used in human studies may be relatively small. Thus, one must make an educated guess as to the appropriate use of contrast agents in the context of their apparent clinical safety and cost-benefit ratio.

Lumbar myelography with iohexol and metrizamide. A comparative multicenter prospective study.

Diagnostic quality and adverse reactions associated with metrizamide and iohexol as contrast agents for lumbar myelography were compared in a prospective randomized double-blind study in 350 patients at seven centers. Both contrast media were administered in comparable volumes at a concentration of 180 mg I/ml. Overall quality of radiographic visualization was graded as "good" or "excellent" in 95% of 175 metrizamide studies and in 98% of 175 iohexol myelograms. Ninety-three patients examined with metrizamide (53%) and 130 patients studied with iohexol (74%) experienced no discomfort during or after myelography. The incidence of postmyelographic headache was 38% with metrizamide and 21% with iohexol. Nausea and vomiting were also more common with metrizamide. Five patients examined with metrizamide (3%) experienced transient confusion and disorientation after lumbar myelography. No such reactions were observed after iohexol myelography.

Normal pituitary gland: 2. Microscopic anatomy-CT correlation.

Pituitary microadenomas may present with subtle or no mass effect. They may appear lucent, dense, or heterogeneous on computed tomographic (CT) images. The normal pituitary gland may also have a nonhomogeneous CT appearance with intermingled lucent and dense areas. This heterogeneity is related in part to microscopic variation within the anterior and posterior lobes. Thirteen autopsy specimens of the pituitary gland were examined by coronal CT and histologically sectioned in similar coronal planes. The CT-lucent and CT-dense areas of the anterior and posterior lobes were correlated with corresponding areas on microscopic examination. To determine the effects of contrast infusion during CT, density measurements of lucent and dense areas in the anterior lobes of 26 normal contrast-enhanced pituitary glands in vivo were compared with density measurements of adjacent vascular structures at the time of scanning. Microscopic features corresponding to increased CT density in the anterior lobe included increased tissue compactness and increased cellular granularity, both of which usually occurred together in areas composed of tightly compacted and heavily granulated acidophilic cells. Conversely, microscopic features corresponding to decreased CT density in the anterior lobe included decreased tissue compactness and decreased cellular granularity, both of which frequently occurred together in areas containing chromophobic and/or mildly to moderately granulated basophilic cells. The degree of contrast enhancement within the anterior lobe appeared primarily to depend on vascularity, which in turn often depended on the degree of tissue compactness. In the posterior lobe, CT-lucent areas appeared to correspond to less compact and/or less vascular neurohypophyseal tissue.

Reformatted imaging to define the intercommissural line for CT-guided stereotaxic functional neurosurgery.

Functional stereotaxic neurosurgery has traditionally required definition of the line between the anterior and posterior commissures as the basis for defining the target site for a procedure such as stereotaxic thalamotomy. While axial computed tomographic (CT) imaging precisely defines the third ventricle and the commissures, the planes of the axial images are not necessarily parallel to the important intercommissural line. A technique is described in which coronal oblique reformations are obtained, with the reformations passing through both the anterior and posterior commissures. Stereotaxic coordinates of the target obtained from this reformatted image are directly transferable to the CT-compatible Leksell frame. Both imaging and surgery are performed on the scanner, which is located in an operating room especially designed for CT-guided surgical procedures.

Embolization of intramedullary arteriovenous malformations of the spinal cord.

Intramedullary spinal cord arteriovenous malformation (AVM) can be a devastating disease. It is not usually amenable to surgery without producing severe neurologic deficit. Previously, the risk of cord ischemia was considered a contraindication to therapeutic embolization. Three patients with intramedullary spinal cord AVMs were examined and treated using polyvinyl alcohol foam, mixed with Gelfoam in two cases. In all three cases there was marked improvement, in two to complete normalcy and in the other to near-normalcy. Successful therapy involves the appropriate angiographic mapping, choice of embolus size and composition, and challenge of function by either test occlusion or long perfusion with contrast material.

Computed tomography and myelography of the postoperative lumbar spine.

Postoperative myelographic changes in the thecal sac, epidural tissues, and bony canal, as well as nerve roots, may be difficult to interpret. A series of 32 postoperative patients, all of whom had a metrizamide myelogram and subsequent lumbar computed tomogram, was reviewed to examine the ability of computed tomography to recognize abnormalities when the myelogram is equivocal or uninterpretable. Criteria to distinguish recurrent herniated disk from postoperative changes are presented, including the demonstration of mass densities similar to and in continuity with the intervertebral disk. In 12 reoperated cases, five recurrent herniated disks and two new herniated disks were diagnosed and confirmed. In 20 nonreoperated cases, no recurrent herniated disks were identified, although two new herniated disks were found at levels not believed clinically significant. Computed tomography after metrizamide myelography appears to be a reliable technique for distinguishing abnormalities in the postoperative spine.

Neuroblastoma presenting as central nervous system disease.

Neuroblastoma may be extremely difficult to recognize, particularly when the tumor presents as a primary central nervous system disease. Central nervous system involvement may be considered as primary intracerebral neuroblastoma, metastases to the cranium from an occult primary, primary intraorbital neuroblastoma originating in the ciliary ganglion, metastatic intraorbital neuroblastoma from an occult primary, primary intraspinal neuroblastoma originating in dorsal root ganglia, intraspinal metastatic disease, and distant effects such as myoclonic encephalopathy. Primary neuroblastoma within the ciliary ganglion and primary intraspinal neuroblastoma are extremely rare entities. Illustrative cases the demonstrate the broad spectrum of neurologic presentations are offered. The second known report of neuroblastoma in association with primary pulmonary hypoventilation (Ondine curse) is included.

Iohexol for cervical myelography via C1-C2 puncture: study of efficacy and adverse reactions.

Cervical myelography with iohexol via C1-C2 puncture was performed in 30 patients in two medical centers using a concentration of 240 mg l/ml. The study demonstrated iohexol to be a safe contrast medium without significant changes in neurologic and physical examination, vital signs, electrocardiogram, or hematologic or blood chemistry parameters. Fifteen patients had electroencephalograms (EEGs); two were abnormal. In one patient the baseline EEG demonstrated nonspecific slow waves in the temporal regions bilaterally that remained unchanged after myelography. In the second patient, transient changes in the left hemisphere during either hyperventilation or photic stimulation on postmyelographic EEG had not been present on the baseline recording. The relation of these changes to the drug remains unclear. Iohexol was found to be an efficacious myelographic contrast agent, with good to excellent myelograms in 93% of cases. Headache occurred in 13% and nausea in 3%.

Iohexol vs. metrizamide: study of efficacy and morbidity in cervical myelography.

A double-blind study was conducted in 60 patients undergoing either cervical or more complete myelography via C1-C2 puncture. Patients received either iohexol or metrizamide at a 300 mg l/ml concentration. The contrast media were equally efficacious in the production of high-quality radiographs and CT scans. However, the incidence of adverse reactions differed markedly. Of patients receiving metrizamide, 68% had some type of adverse reaction, whereas only 26% receiving iohexol had symptoms. The incidence of headache (metrizamide, 34%; iohexol, 26%) was not statistically different, but the quality of the headache differed: half of the metrizamide headaches were moderate or severe, whereas all iohexol headaches were mild. Nausea (31%) and vomiting (28%) were common with metrizamide but unusual (3% nausea) with iohexol. Of the metrizamide patients, 21% had overt psychologic changes that did not occur in the iohexol group.

Anatomy of the brainstem: correlation of in vitro MR images with histologic sections.

MR imaging was performed on three formaldehyde-fixed brainstem specimens that were sectioned in the axial plane and myelin stained. The histologic sections were used to identify and label the structures demonstrated on short TR/TE axial MR images. Fiber tracts and nuclei that cannot be resolved on in vivo scans were well delineated by in vitro MR. Improved gray-white differentiation may be due to much greater T1 shortening of gray matter relative to white matter after fixation. The excellent anatomic detail provided by these scans should facilitate comparison of clinical scans with histologic sections.

Neuroradiologic aspects of pediatric orthotopic liver transplantation.

To investigate the occurrence of neurologic symptomatology in pediatric orthotopic liver transplantation patients and to evaluate the utility of CT in uncovering the origin of their symptoms, we reviewed the medical records and head scans (when performed) of 71 patients. Neurologic problems occurred in 48%; the majority involved seizures, mental status changes, or coma. Patients who only had seizures generally had negative CT scans, except for two patients with minimal subarachnoid hemorrhages. Three-quarters of the comatose patients, however, had significant intracerebral hemorrhages defined by CT. Prominent sulci and ventricles were found in approximately one-third of the patients scanned, but did not correlate with symptomatology or steroid dose.

Cerebellar venous angioma: "benign" entity?

Four patients with bleeding venous angiomas in the cerebellar hemispheres are reported. All of the hemorrhages were subacute; three were recurrent. A literature review substantiated venous angioma as a recognized source of spontaneous cerebellar hemorrhage. Cerebellar venous angiomas may be more prone to bleed than venous angiomas in other locations. Their natural history seems to be similar to that of arteriovenous malformations. Though a minority bleed, the hemorrhagic event can be acute or subacute, recurrent, or catastrophic. Thus the clinical course is not always innocuous.

Xe/CT cerebral blood flow evaluation of delayed symptomatic cerebral ischemia after subarachnoid hemorrhage.

PURPOSE: We examined the xenon/CT method of measuring cerebral blood flow in assessing the location, pattern of onset, and severity of delayed cerebral ischemia. PATIENTS AND METHODS: Fourteen patients with delayed neurologic deficits due to ischemia were selected from a group of 66 patients with subarachnoid hemorrhage. All blood flow studies were performed within 12 hours of deterioration and at regular intervals during medical management. RESULTS: In 10 of the 14 patients, noncontrast CT did not identify a cause for deterioration, whereas the blood flow study revealed diminished flow values. Location of blood flow reduction was variable. In five of the 14 patients, blood flow reduction was closely related anatomically to the vessel of aneurysm origin. In another three, blood flow reduction was anatomically remote to the vessel of origin. The remaining six experienced local and remote cerebral blood flow reduction. Six of 14 patients suffered sudden, devastating deterioration, refractory to therapy and associated with blood flow of 15 cc/100 g.min or less, resulting in local or widespread infarction. The remaining eight had less severe blood flow reduction and did not infarct those territories. CONCLUSIONS: Vasospasm can affect remote vessels as severely as local vessels and can affect remote vessels alone. Diminished cerebral blood flow correlated closely with clinical vasospasm in this group of patients. Xenon/CT cerebral blood flow studies can identify tissue at risk of infarction when CT is normal.