Rasmussen encephalitis associated with Parry-Romberg syndrome.

Parry-Romberg syndrome is a rare disorder associated with unilateral facial atrophy involving skin, subcutaneous tissue, skeletal muscle, and bone. Occasionally, there is CNS involvement with epilepsy being the most common CNS manifestation. The authors report a child with Parry-Romberg syndrome with a course strongly suggestive of Rasmussen encephalitis. The boy underwent hemispherectomy, and pathology showed the typical findings of Rasmussen encephalitis, suggesting that these two conditions may share common etiologic factors.

The role of the carboxyl-terminal fragments of amyloid precursor protein in Alzheimer's disease.

Two major pathological hallmarks of Alzheimer's disease (AD) are the senile plaques that are primarily composed of amyloid beta-peptide (Abeta) and neurofibrillary tangles consisting of tau aggregates. Abeta is generated proteolytically from a family of Abeta-containing precursor proteins (APP; 695-770 amino acid) by secretase enzymes to different specific carboxyl-terminal fragments (CTFs). Herein we examined APP and its products in autopsied brain sections from 10 AD and 10 non-AD control subjects immunochemically using an antibody that was raised against APP751-770 residue (O443). The O443 antibody was initially characterized by Western blot analysis and immunoprecipitation. In this study, we used this antibody for immunohistochemical analysis to determine the distribution of APP and its CTF species. In 10 brain regions showing different levels of plaques and tangles, antibody O443 stained the perinuclear region of the nucleus, plaques, and neurites. Tangle-bearing neurons also appeared to stain with the antibody, suggesting that these dysfunctional neurons continue to synthesize APP/CTF. Alternatively, the normally short-lived APP/CTF can be stabilized and persist in these neurons. Taken together, these results suggest that, in addition to the widely believed role of Abeta, CTFs may play a key role in the pathogenesis of AD. Studying their localization and biogenesis may reveal the biological activities of CTFs of APP. The present study may pave the way for possible antiamyloidogenic therapy in the treatment of AD.

Volumetric MRI, pathological, and neuropsychological progression in hippocampal sclerosis.

OBJECTIVE: To examine the relationships between age at onset and duration of seizure disorder with severity of hippocampal sclerosis (HS) and cognitive functioning in patients with HS and unilateral temporal lobe epilepsy. METHODS: Twenty-six subjects had left temporal lobe seizure onset; 20 had right temporal onset. Measures were age at seizure onset, duration of seizure disorder divided by age (seizure duration), history of febrile convulsion (FC), ratio of the smaller hippocampal volume to the larger (HF) as determined by volumetric MRI, and pathologic HS grade. RESULTS: Results showed that pathologic HS grade and HF were positively related to seizure duration, and negatively related to seizure onset. When subjects were divided into onset prior to age 10 versus later, subjects with earlier onset had higher mean pathologic HS grade and smaller (more asymmetric) mean HF. When subjects were divided into seizure duration <0.5 (i.e., less than half current lifetime) vs greater, subjects with seizure duration > or =0.5 had higher mean pathologic HS grade and lower mean HF. There was also evidence for earlier age at seizure onset and longer seizure duration being associated with worse performance on neuropsychological measures. FC was not related to either seizure duration or age at seizure onset, but patients with a history of FC showed higher pathologic HS grade and lower HF. A history of FC was not related to cognitive functioning. CONCLUSIONS: Unilateral HS patients with earlier seizure onset and longer duration of epilepsy have more severe HS and greater hippocampal volume asymmetry. This suggests that HS may be a progressive disorder with risk for cognitive dysfunction.

Spinal cord sarcoidosis: a diagnostic dilemma.

Sarcoidosis is a chronic disorder of unknown etiology characterized by the development of non-caseating granulomas with derangement of the normal tissue architecture. Compromise of the spinal cord is one of the rarest neurologic manifestations of the disease, which may be clinically and radiologically indistinguishable from a spinal cord malignant tumor. However, neurosarcoidosis can be treated with steroids. This study reviews the clinical, radiological, and pathological features of the sarcoid compromise of the spinal cord, emphasizing the difficulties commonly encountered in making a diagnosis.

Myopathy with muscle spindle excess: A new congenital neuromuscular syndrome?

An infant presented with congenital weakness, hypotonia, arthrogryposis, atrial tachycardia, and a left intra-abdominal neuroblastoma. Muscle biopsy revealed marked excess of muscle spindles with atrophy of extrafusal fibers. The patient expired at age 14 months from progressive cardiorespiratory failure. Postmortem examination demonstrated muscle-spindle excess in other muscles, along with hypertrophic obstructive cardiomyopathy and organomegaly. Muscle spindle excess has previously been reported in two patients with Noonan syndrome and progressive hypertrophic cardiomyopathy. Muscle spindle excess with hypertrophic cardiomyopathy, organomegaly, and, possibly, congenital neuroblastoma suggests a syndromic association and may represent an unusual form of congenital myopathy.

Intravascular papillary endothelial hyperplasia arising within a posteroinferior cerebellar artery aneurysm: case report and review of the literature.

OBJECTIVE AND IMPORTANCE: Intravascular papillary endothelial hyperplasia (IPEH) is an atypical proliferation of endothelium that results in abnormal organization for thrombus formation. Intracranial IPEH is a rare entity and has not been reported to arise from within an intracranial aneurysm. Furthermore, the elapsed time during which acquired intracranial IPEH develops has not been previously documented. CLINICAL PRESENTATION: In this case report, a patient with facial and neck pain was noted to have an enhancing mass lesion lateral to the medulla in magnetic resonance imaging scans. Angiography revealed a vascular mass adjacent to the posteroinferior cerebellar artery. Normal magnetic resonance imaging and magnetic resonance angiographic findings had been obtained for the patient 29 months earlier. INTERVENTION: During surgery, a thrombosed, 2.5-cm, posteroinferior cerebellar artery aneurysm was resected and noted to contain florid IPEH. There has been no evidence of recurrence in 1 year of follow-up monitoring. A literature search revealed 13 cases of intracranial IPEH, in which recurrence was observed for incompletely resected lesions. CONCLUSION: IPEH can develop in a relatively short time, can present as a hypervascular mass lesion or within an intracranial aneurysm, and should be completely resected to prevent recurrence.

Magnesium sulfate protection of fetal rat brain from severe maternal hypoxia.

OBJECTIVE: To determine whether severe maternal hypoxia affects fetal rat physical characteristics and causes neuronal damage, and whether magnesium sulfate can decrease these effects. METHODS: At 17 days gestation, rats were randomly assigned to one of four groups that received saline injections and room air (n = 6), magnesium sulfate and room air (n = 5), saline and hypoxia (n = 5) or magnesium sulfate and hypoxia (n =5). Maternal magnesium sulfate or saline injections were given for 4 hours. In groups 3 and 4 this was followed by a hypoxia chamber protocol that included a gas mixture of 9% oxygen and 3% carbon dioxide for 2 hours. After 72 hours of recovery, fetuses were delivered abdominally, perfused transcardially, and brains removed intact. Fetal body and brain weight and size were measured. Brains were embedded in paraffin, sectioned, and stained. A neuropathologist masked to the protocol performed histologic grading of brain regions. RESULTS: Exposure to the hypoxia chamber resulted in decreased maternal oxygen tension and pH (from 82.8 +/- 20.0 to 49.2 +/- 14.4 mmHg, and from 7.37 +/- 0.05 to 7.20 +/- 0.04, respectively; P <.005). Magnesium sulfate administration resulted in higher magnesium levels in blood (from 1. 52 +/- 0.2 to 3.77 +/- 0.7 mg/dL; P <.001). The hypoxia protocol resulted in a significant decrease in fetal body and brain size, but not weight. Hypoxia also caused an increase in the proportion of fetal rats that had brain injury, including shrinkage of cells and karyorrhexis in the hippocampus and thalamus (from 0% to 38.1% and 38.9%, respectively; P <.05). Magnesium sulfate reduced these effects on fetal brain histopathology and size. CONCLUSION: Severe maternal rat hypoxia resulted in significant fetal neuronal damage and decreased fetal body and brain size. Maternal magnesium sulfate administration reduced the effect of hypoxia on fetal brain histopathology and size without affecting body size.

Use of the RTOG recursive partitioning analysis to validate the benefit of iodine-125 implants in the primary treatment of malignant gliomas.

PURPOSE: To date, numerous retrospective studies have suggested that the addition of brachytherapy to the conventional treatment of malignant gliomas (MG) (surgical resection followed by radiotherapy +/- chemotherapy) leads to improvements in survival. Two randomized trials have suggested either a positive or no survival benefit with implants. Critics of retrospective reports have suggested that the improvement in patient survival is due to selection bias. A recursive analysis by the RTOG of MG trials has stratified MG patients into 6 prognostically significant classes. We used the RTOG criteria to analyze the implant data at Wayne State University to determine the impact of selection bias. METHODS AND MATERIALS: Between July 1991 and January 1998, 75 patients were treated with a combination of surgery, radiotherapy, and stereotactic I-125 implant as primary MG management. Forty-one (54.7%) were male; 34 (45.3%) female. Median age was 52 years (range 4-79). Twenty-two (29.3%) had anaplastic astrocytoma (AA); 53 (70.7%), glioblastoma multiforme (GBM). Seventy-two patients had data making them eligible for stratification into the 6 RTOG prognostic classes (I-VI). Median Karnofsky performance status (KPS) was 90 (range 50-100). There were 14, 0, 14, 31, 12, and 1 patients in Classes I to VI, respectively. Median follow-up time for AA, GBM, and any surviving patient was 29, 12.5, and 35 months, respectively. RESULTS: At analysis, 29 (40.3%) patients were alive; 43 (59.7%), dead. For AA and GBM patients, 2-year and median survivals were: 58% and 40%; 38 and 17 months, respectively. For analysis purposes, Classes I and II, V and VI were merged. By class, the 2-year survival for implanted patients compared to the RTOG data base was: III--68% vs. I--76%; III--74% vs. 35%; IV--34% vs. 15%; V/VI--29% vs. V--6%. For implant patients, median survival by class was (in months): I/II--37; III--31; IV--16; V/VI--11. CONCLUSION: When applied to MG patients receiving permanent I-125 implant, the criteria of the RTOG recursive partitioning analysis are a valid tool to define prognostically distinct survival groups. As reflected in the RTOG study, a downward survival trend for the implant patients is seen from "best to worse" class patients. Compared to the RTOG database, median survival achieved by the addition of implant is improved most demonstrably for the poorer prognostic classes. This would suggest that selection bias alone does not account for the survival benefit seen with I-125 implant and would contradict the notion that the patients most eligible for implant are those gaining the most benefit from the treatment. In light of the contradictory results from two randomized studies and given the present results, further randomized studies with effective stratification are required since the evidence for a survival benefit with brachytherapy (as seen in retrospective studies) is substantial.

Fetal rat brain damage caused by maternal seizure activity: prevention by magnesium sulfate.

OBJECTIVE: Our purpose was to determine whether maternal rat seizure activity was associated with fetal histopathologic brain changes and whether magnesium sulfate reduced these changes. STUDY DESIGN: Electrodes were stereotaxically implanted into the hippocampus of nonpregnant rats 1 week before breeding. Pregnant rats were randomly assigned to 1 of 4 groups: (1) sodium chloride solution and no seizure (n = 2), (2) magnesium sulfate and no seizure (n = 2), (3) sodium chloride solution and seizure (n = 5), and (4) magnesium sulfate and seizure (n = 5). On gestational days 9, 11, 13, 15, 17, and 19, subcutaneous doses of sodium chloride solution or magnesium sulfate were administered to all rats every 20 minutes for 4 hours (loading-maintenance-loading), followed by seizure induction. On gestational day 20, the rats were perfused with formalin and fetuses were delivered via cesarean. Fetuses were perfused with formalin, brains were obtained and embedded in paraffin, and the forebrain and hindbrain were sectioned in the coronal plane and stained with hematoxylin and eosin. A neuropathologist masked to the protocol performed histopathologic grading of each section, including extent and nature of cellular damage. Eleven brain regions were examined in each section. Scores were expressed as mean +/- SD. Kruskal-Wallis analysis of variance was used, and P <.05 was considered significant. RESULTS: We evaluated 26 fetal brains in group 1, 9 in group 2, 72 in group 3, and 45 in group 4. Fetuses in the sodium chloride solution-and-seizure group (group 3) presented significantly higher grades of neuronal damage in the hippocampus (group 1, 0.50 +/- 0. 88; group 2, 0.22 +/- 0.66; group 3, 1.01 +/- 1.17; and group 4, 0. 48 +/- 0.72) and in the tegmentum region (group 1, 1.0 +/- 1.0; group 2, 0.8 +/- 1.0; group 3, 1.7 +/- 0.7; and group 4, 1.5 +/- 0. 8) (P <.05, group 3 compared with others). Isolated and patchy neuronal injury with shrinkage of cells, nuclear pyknosis, and karyorrhexis were the main histologic findings. CONCLUSIONS: Maternal rat seizure activity was associated with histologic brain injury in the fetus. Maternal administration of magnesium sulfate before seizure prevented or significantly decreased this effect.

Altered in vitro and in vivo flumazenil binding in human epileptogenic neocortex.

In vitro and in vivo parameters of flumazenil (FMZ) binding were measured in spiking and nonspiking neocortex identified by intraoperative electrocorticography in epileptic patients who underwent cortical resection for seizure control. In vitro measures of receptor affinity (K(D)), number (Bmax) and laminar distribution for [3H]-FMZ binding in the epileptic focus (n = 38) were compared to nonspiking cortex from a subgroup of the patients (n = 12) and to tissue obtained from trauma patients (n = 5). The in vitro binding parameters were compared to in vivo [11C]-FMZ binding measured with positron emission tomography (PET) (n = 19). The Bmax was higher in the 38 spiking tissues as compared to the 12 nonspiking tissues (P = .012). Paired comparison of spiking versus nonspiking binding in the 12 patients from whom nonspiking tissue was available showed increases in both K(D) (P = .037) and Bmax (P = .0047) in spiking cortex. A positive correlation was found between K(D) and Bmax values for 38 patients (r = 0.55, P < .0001), the magnitude of the K(D) increase being twice that of the Bmax increase. In addition, there was a significant correlation between the asymmetry indices of the in vivo FMZ binding on PET and in vitro K(D) of spiking cortex (n = 19, r = 0.52, P = .02). The laminar distribution of [3H]-FMZ showed increased FMZ binding in cortical layers V-VI in spiking cortex compared to nonspiking and control cortex. The increased receptor number in spiking cortical layers V-VI may be a compensatory mechanism to decreased GABAergic input. The increased Bmax in spiking cortex was accompanied by a larger decrease in the affinity of FMZ for the receptor suggesting that decreased FMZ binding in the epileptic focus measured with PET is due to a decrease in the affinity of the tracer for the receptor.

Ontogeny of recurrent hydrocephalus: presentation in three fetuses in one consanguineous family.

A consanguineous couple had 3 pregnancies in which prenatally diagnosed hydrocephalus was observed (in 1 female and 2 male fetuses). This case appears to represent an autosomal recessive form of hydrocephalus, given the consanguinity, affected sibs of both genders, and no evidence for intrauterine infection, chromosome abnormality, or neural tube defect.

Evaluation of expanded polytetrafluoroethylene (ePTFE) versus polydioxanone (PDS) for the repair of dura mater defects.

A comparative animal experimental study was performed to test the potential application of expanded polytetrafluoroethylene (ePTFE) vs. polydioxanone (PDS) as dural substitutes. Sixty male Sprague-Dawley rats underwent a right frontoparietal craniotomy, opening of the dura mater, and a small cortical lesion. The dural defect was covered with a piece of ePTFE or PDS. Animals were sacrificed at 30 days or 90 days. Following decalcification, heads including scalp, skull, and underlying brain were sectioned, stained with hematoxylin-eosin, and histologically analyzed. Dural defects repaired with ePTFE, showed minimal reactive changes and no adhesions to the brain surface. No foreign body type giant cell reaction was seen, and the graft became enclosed in a thin sheet of connective tissue. Dural defects repaired with PDS, showed some giant cell infiltration and ingrowth of collagen fibers. Both substitutes provided satisfactory biological function and biocompatibility. Expanded PTFE advantages included relative suppression of tissue ingrowth, ensheathment by connective tissue, and a high tearing strength. Although both materials show promise for use in dural grafting, further clinical studies are necessary to determine their potential applications as a human dural substitute.

Muscle and nerve biopsy in the evaluation of neuromuscular disorders: the surgeon's perspective.

BACKGROUND/PURPOSE: A muscle biopsy frequently is requested by the neurologist evaluating a child with neuromuscular symptoms. However, there are no reports discussing the preoperative evaluation for, and diagnostic yield of, this procedure. The authors reviewed our experience over a 10-year period to obtain these data. METHODS: The records of 153 patients who underwent muscle biopsy were reviewed with particular attention to the cardiology evaluation, the pathology report, and any resultant change in diagnosis and treatment of the child. RESULTS: All 153 specimens contained adequate tissue for complete histological analysis. Preoperative cardiology consults were obtained in 82% of the children, with abnormalities found in 9%. Severe cardiac dysfunction was found in three children, all of whom had a previously diagnosed cardiomyopathy or dysrhythmia. No pathological abnormality was found in 41% of the muscle biopsy specimens, and nonspecific pathological findings were described in 23%. A specific diagnosis was made in 36%. Only 19% of the children had their treatment changed by the results of the muscle biopsy. CONCLUSIONS: Muscle biopsies can be performed safely without routine preoperative cardiac evaluation. A specific diagnosis, however, may be made in less than half of the patients with a change in therapy available for even fewer.

Magnetic resonance imaging of intercranial plasmocytic granuloma.

The objective of this study is to determine characteristic magnetic resonance imaging (MRI) features of intracranial plasmocytic granulomas. Pathological confirmation of three patients with intracranial pathologically confirmed plasmocytic granuloma are presented. Clinical records as well pre- and postgadolinium-enhanced images from each patient are reviewed. The location of the abnormalities is compared with previous reported cases of plasmocytic granulomas, to determine if there is a characteristic finding in this disense. The predominance of this abnormality in the pediatric and young adult patient was striking. On T(1)-weighted MRI, plasmocytic granulomas appear as hypointense lesions, with isointense appearance on T(2) images, and significant, variable patterns of enhancement after the infusion of gadolinium. Typically, the lesion is infiltrating, and causes little mass effect. A dural based lesion, as well as a sellar region abnormality and an infiltrating cortical lesion with little mass effect in the pediatric or young adult age group may lead the observer to suspect the diagnosis of plasmocytic granuloma.

Arteritis and fatal subarachnoid hemorrhage complicating occult Candida meningitis: unusual presentation in pediatric acquired immunodeficiency syndrome.

We report the case of an 11-month-old child with acquired immunodeficiency syndrome, who despite treatment for systemic candidiasis developed undetected Candida meningitis. This uncommon manifestation of candidiasis was accompanied by basilar granulomatous inflammation and fibrosis of meninges with arteritis, vascular invasion by fungi, and terminal subarachold hemorrhage. To our knowledge, this constellation of findings has not been reported previously in pediatric acquired immunodeficiency syndrome.

Neuropathologic findings in a case of OFDS type VI (Váradi syndrome).

Oral-facial-digital syndrome type VI (OFDS VI) or Váradi syndrome is a rare autosomal-recessive disorder distinguished from other oral-facial-digital syndromes by metacarpal abnormalities with central polydactyly and by cerebellar abnormalities. Histopathologic characterization of the cerebellar abnormalities has not been described previously. We describe the neuropathologic findings in a stillborn, 21-week estimated gestational age (EGA) male fetus diagnosed antenatally with signs of OFDS VI. Autopsy findings included: facial abnormalities, postaxial central polydactyly of the right hand, bilateral bifid toes, and absence of cerebellar vermis with hypoplasia of the hemispheric cortex. Microscopic analysis of the cerebellum demonstrated absence of the subpial granular cell layer and disruption or dysgenesis of the glial architecture. These histopathologic findings suggest that a primary neuronal or glial cell defect, rather than an associated Dandy-Walker malformation, may account for the cerebellar abnormalities in this form of oral-facial-digital syndrome.

Clonal analysis of gliomas.

In malignant gliomas, the characteristically heterogeneous features and frequent diffuse spread within the brain have raised the question of whether malignant gliomas arise monoclonally from a single precursor cell or polyclonally from multiple transformed cells forming confluent clones. Although monoclonality has been shown in surgically resected tissues, these may not include the full spectrum of patterns seen on autopsy material. Little is known about the clonality of low-grade gliomas from which malignant gliomas may sometimes arise. We sought to investigate the clonality of low-grade and malignant gliomas by using and comparing surgical and autopsy material with a Polymerase chain reaction (PCR)-based assay for nonrandom X chromosome inactivation. For that, purpose, archival surgical and autopsy material from 15 female patients (group A) (age 4 to 73 years; median, 45) with malignant gliomas (12 glioblastomas, one gliosarcoma, one anaplastic oligoastrocytoma, one gliomatosis cerebri), surgical material only from 21 female patients (group S) (age 6 to 78 years; median, 60) with low-grade and malignant gliomas (four low-grade astrocytomas, three oligoastrocytomas, two anaplastic astrocytomas, one gemistocytic astrocytoma, four oligodendrogliomas, seven glioblastomas) were analyzed. In group A, representative areas (mean = 5/patient; median = 7) were microdissected from tissue sections and assayed by PCR amplification of a highly polymorphic microsatellite marker locus of the human androgen receptor gene (HUMARA) in the presence of alpha32P with and without predigestion with a methylation-sensitive restriction enzyme (HhaI). Products were resolved by denaturing gel electrophoresis and autoradiographed. In group S, selected tumor areas were used for the assay. Each patient's normal brain tissue was used for control. The band intensity of alleles were measured by densitometric scanning. In group A, 13 of 15 cases were informative (heterozygous). The same pattern of nonrandom X chromosome inactivation was present in all areas of solid dense and moderate tumor infiltration in eight including all components of the gliosarcoma. Two of eight also showed focal loss of heterozygosity (LOH). One of 13 presented global LOH. Two of 13 showed microsatellite instability, one of which in a patient with Turcot syndrome, the other in gliomatosis cerebri. Opposite skewing patterns were seen in distant areas of gliomatosis cerebri consistent with oligoclonal derivation. Clonality remained indeterminate in one glioblastoma and in the anaplastic oligoastrocytoma because of skewed lyonization in the normal control. In group S, 19 of 21 cases were informative. Fifteen of 19 were monoclonal (four low-grade astrocytomas, one anaplastic astrocytoma, one gemistocytic astrocytoma, two oligodendrogliomas, one oligoastrocytoma, six glioblastomas). Four of 19 were indeterminate. We conclude that (1) Low-grade and malignant gliomas are usually monoclonal tumors, and extensively infiltrating tumors must result from migration of tumor cells (2) Gliomatosis cerebri may initiate as an oligoclonal process or result from collision gliomas (3) Biphasic gliomas likely arise from a single precursor cell. (4) LOH at the HUMARA locus is probably related to partial or complete deletion of an X-chromosome, which occurs in malignant gliomas during clonal evolution.

Fetal rat brain injury: effect of transient maternal hypoxemia.

OBJECTIVE: To determine whether transient acute maternal hypoxemia during the end of pregnancy may cause neuronal damage in fetal rat brains. STUDY DESIGN: Nine pregnant rats (4 study and 5 controls) at 16-17 gestational days were studied. The study rats were placed in a chamber and breathed a gas mixture of 11.8% oxygen, 4.95% CO2, and nitrogen for either 1 or 2 h, while the control animals breathed room air. Tail venous blood was collected and gases were evaluated at the beginning and conclusion of the exposure periods. After 72 h of recovery, at 19-20 days' gestation, the fetal cardiovascular systems were perfused with saline and formalin. The brains were embedded in paraffin, sectioned in coronal plane, and stained with hematoxylin and eosin Histologic assessment of sections was performed by a neuropathologist blinded to the protocol. Statistical analysis of the data was performed using analysis of variance and the chi-square test. RESULTS: Exposure to the gas mixture resulted in decreased maternal pO2 from 39.9 +/- 7.6 mm Hg in the control group to 28.8 +/- 2.0 mm Hg in the 2-hour hypoxia group (p < 0.05). No significant changes in maternal pH or pCO2 status have been noted. A total of 34 fetal rat brains served as controls and 26 brains as the hypoxia study group There was a significant increase in isolated neuronal damage, including necrosis and shrinkage of cells, with karyorrhexis (fragmentation and breakage of the nucleus) in the hippocampus, basal ganglia, thalamus, and hypothalamus in the hypoxemia rats as compared with controls. CONCLUSION: Transient maternal hypoxemia may cause neuronal necrosis in vulnerable regions of the fetal rat brain, including the hippocampus, basal ganglia, thalamus, and hypothalamus.

Pathophysiological classification of human spinal cord ischemia.

Diagnosis of myelopathies of vascular origin is difficult and they are probably underdiagnosed at this time because of the lack of diagnostic tools. A recent report of a 58 year old patient who developed ASAS after an episode of cardiac arrest pointed out the importance of MRI and somatosensory evoked potentials (SEP) to support the diagnosis. MRI with T2 weighted imaging demonstrated diffuse signal abnormalities in both gray matter and surrounding white matter below T7. Furthermore, SEP latencies showed a delay between T6 and T7. Therefore, new technologies including MRI and SEP may improve the diagnosis of spinal cord ischemic injuries. A brief discussion of the normal blood supply of the human spinal cord is presented in this review followed by new, pathophysiologically based classifications of the clinical syndromes of vascular myelopathies. A complete description of the clinical syndromes related to vascular myelopathies is included. Vascular myelopathies were divided into acute and chronic syndromes depending on the time at which the pathophysiological events take place. Subsequently, the two major groups of vascular myelopathies were divided depending on the type of vascular damage, e.g., arterial, venous and/or mixed origin. Posttraumatic spinal cord ischemia is included in the present classification because it is generally considered to be a significant factor contributing to secondary damage following blunt trauma. Since several new diagnostic techniques are now available to characterize the pathology of spinal cord injury, physicians involved in the diagnosis and treatment of vascular myelopathies may find the new classification useful in correlating clinical presentation with subjacent pathology. Identification of the correct pathology should result in more accurate treatment approaches.

Leptomeningeal dissemination of malignant glioma simulating cerebral vasculitis. Case report with angiographic and pathological studies.

BACKGROUND: The complex clinical and radiological picture of leptomeningeal spread of tumor is well recognized as a problem of systemic cancer but is less frequent in primary cerebral glioma, particularly as a presenting picture. While brain ischemia and infarction may occur in patients with subarachnoid tumor, the mechanism for these complications remains unclear. Angiographic and pathological demonstrations of direct vascular involvement by disseminated glioma are particularly sparse. We report a patient presenting with multiple infarctlike lesions with postmortem evidence of direct vascular involvement by glioma. CASE DESCRIPTION: A 54-year-old woman presenting with seizures, headache, and changes in mental status was found to have vascular narrowing in cerebral blood vessels and ischemic lesions on neuroimaging studies of the brain, interpreted as cerebral vasculitis. A brain biopsy showed leptomeningeal glioma. Postmortem examination demonstrated a glioblastoma arising around the right sylvian fissure with extensive subarachnoid dissemination of tumor. The leptomeningeal tumor caused vascular narrowing by encasement, direct vascular wall invasion, and thrombosis and was associated with underlying infarctlike foci of parenchymal necrosis. CONCLUSIONS: This case demonstrates an unusual presentation of glioblastoma clinically and radiographically mimicking cerebral vasculitis, and it illustrates a variety of mechanisms for tumor-produced vascular compromise.