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Object: To determine principal prognostic factors and the effect of timing of radiotherapy (RT) on disease-specific survival (DSS) and progression-free survival (PFS) in WHO Grade II astrocytomas. Methods: Histologic slides of 166 consecutive patients with the original tissue diagnosis of low-grade, non-pilocytic astrocytoma were reviewed. One-hundred and six were selected where two additional certified neuropathologist agreed on the grading of WHO Grade II astrocytoma. In 97 out of 106 cases follow-up informations were available. Early postoperative RT was given to 36 out of 97 patients (37%). The two groups of patients ( early vs. delayed RT) were well balanced in respect to extent of surgery and other main clinical prognostic factors. Median follow-up of surviving patients was 79 months. The 5- and 10-year PFS was 52.2% and 30.7% with early RT and 39.5% and 12.4% with delayed RT (p = 0.0388). In respect to DSS, there was no significant difference in the 5- and 10-year actuarial survival rate according to the timing of RT (60.5% and 26.5% vs. 66.6% and 23.7%; p = 0.7545). Age ( p = 0.0145) and extent of surgery ( p = 0.0473) were significant prognostic variables in respect to DSS. Subdividing the irradiated group based on the extent of surgery, early RT in the subtotal group significantly improved 5- year PFS ( 60.0% vs. 12.4%; p = 0.0036) and DSS ( 66.7% vs. 49.8%; p = 0.0389). However, postoperative RT had no influence on PFS ( p = 0.6812) and DSS ( p = 0.3987) in the group with extensive resection. Conclusion: Early postoperative RT in subtotally resected, Grade II astrocytomas significantly improves both progression-free and disease-specific survival. Early RT does not benefit patients with extensive resection, RT should be withheld in these patients until progression.

The activities of adenylosuccinate synthetase, adenylosuccinate lyase, and adenosine monophosphate deaminase were measured in muscle from patients suffering from fatigue and cramps following exercise. Results denote the existence of secondary deficiencies of adenylosuccinate synthetase and/or adenylosuccinate lyase in subjects with congenital or acquired myopathies. They also suggest that searches are warranted for primary deficiencies of adenylosuccinate synthetase as a cause of exercise intolerance.

Angiolipomas (ALs) are hamartomas composed of abnormally differentiated vessels and mature adipose tissue. Although they are most commonly found in peripheral tissues, ALs sometimes grow in the spinal epidural space. Intracranial ALs (ICALs) are rare: only seven cases have been reported in the literature. The authors describe the case of a 70-year-old woman who presented with ocular symptoms from a clinically and radiologically progressing parasellar ICAL. The radiological as well as the microsurgical findings are illustrated and compared with the seven previously published cases. The most frequent location of ALs is the skull base, especially the parasellar region. Other ICALs were diagnosed as components of cerebral arteriovenous malformations and were not symptomatic by themselves. Neuroradiological studies of ICALs usually demonstrate the characteristics of both adipose and vascular tissues. However, a review of the literature shows that the diagnosis had not been suspected preoperatively in any of the cases. Operative descriptions emphasize that most neurosurgeons were caught off guard by the profuse bleeding and the unusual relationship of this unexpected lesion to the cavernous sinus, so that removal was rarely complete. The authors conclude that preoperative diagnosis of ICALs is achievable based on magnetic resonance analysis, which should help optimize the microsurgical management of these lesions.

Angiolipomas (ALs) are hamartomas composed of abnormally differentiated vessels and mature adipose tissue. Although they are most commonly found in peripheral tissues, ALs sometimes grow in the spinal epidural space. Intracranial ALs (ICALs) are rare: only seven cases have been reported in the literature. The authors describe the case of a 70-year-old woman who presented with ocular symptoms from a clinically and radiologically progressing parasellar ICAL. The radiological as well as the microsurgical findings are illustrated and compared with the seven previously published cases. The most frequent location of ALs is the skull base, especially the parasellar region. Other ICALs were diagnosed as components of cerebral arteriovenous malformations and were not symptomatic by themselves. Neuroradiological studies of ICALs usually demonstrate the characteristics of both adipose and vascular tissues. However, a review of the literature shows that the diagnosis had not been suspected preoperatively in any of the cases. Operative descriptions emphasize that most neurosurgeons were caught off guard by the profuse bleeding and the unusual relationship of this unexpected lesion to the cavernous sinus, so that removal was rarely complete. The authors conclude that preoperative diagnosis of ICALs is achievable based on magnetic resonance analysis, which should help optimize the microsurgical management of these lesions., SCOPUS: ar.j, info:eu-repo/semantics/published

Hemodynamic parameters such as total cerebral blood volume (total CBV), cerebral parenchymal blood volume (CBV), cerebral blood flow (CBF) and cerebral blood velocity index were measured in rats 6, 12 and 18 months after single exposures of brain to 5, 10, 15 and 20 Gy X rays for total CBV, CBF and blood velocity index, and only 20 Gy for CBV. Total CBV and blood velocity index were determined by a noninvasive blood dilution method using [99mTc]pertechnetate and CBF by [131I]iodoantipyrine brain extraction. The CBV was obtained from both parenchymal plasma and erythrocyte volumes measured in isolated brain by 125I-labeled serum albumin and 51Cr-labeled erythrocytes, respectively. Neither the dose nor the time after irradiation influenced total CBV. Nevertheless, CBV decreased slightly while CBF decreased strongly at 12 and 18 months after 20 Gy. In contrast, the blood velocity index increased progressively at 12 and 18 months after 15 Gy and at all times after 20 Gy. According to the coexistence in irradiated brains of a remodeling with microvascular occlusions and dilated abnormal vessels, this lowered CBF can be explained by the smaller number of open capillaries and a "steal phenomenon" through low-resistance channels developed in the parenchymal and extraparenchymal vasculatures. Such a "steal phenomenon" is also supported by the response of the blood velocity index, which appears to be the earliest sensitive index for the detection of hemodynamic changes with respect to time (6 months) and dose of radiation (15 Gy).

Several recent studies have attributed the occurrence of acute myopathy in intensive care unit patients to the combination of corticosteroids and neuromuscular junction blocking agents (NMBAs) used for mechanical ventilation. We present 4 patients who developed acute myopathy after administration of high doses of glucocorticoids during sedation with propofol without any NMBAs. All patients had elevated creatine kinase levels. Electrophysiological studies indicated normal motor and sensory nerve conduction velocities but reduced motor nerve response amplitudes. Needle electromyography identified abnormal spontaneous activity; motor unit potentials were polyphasic of low amplitude and short duration, characteristic of a myopathic process. Muscle biopsy demonstrated a prominent acute necrotizing myopathy in all 4 patients with a loss of thick filaments. Our observations support glucocorticoids rather than NMBAs as the main offending drug in acute corticosteroid myopathy. The predisposing factor should be the hypersensitivity of paralyzed muscles to corticosteroids regardless of the drug inducing paralysis: NMBAs or propofol.

Gliomas are regionally heterogeneous tumors. The local relationship between histologic features and radiotracer uptake evaluated by PET should therefore influence analysis and interpretation of PET results on gliomas, This study explored this local relationship as a result of PET guidance of stereotactic biopsies. Methods: Local histology was confronted to the regional uptake of F-18-2-fluoro-2-deoxy-D-glucose (F-18-FDG) and C-11-methionine (C-11-MEF) in 14 patients with high-grade glioma diagnosed during a procedure of PET-guided stereotactic biopsies, We analyzed the uptake of both tracers in regions of interest centered on the stereotactic coordinates of 93 biopsy samples. Results: A semiquantitative analysis revealed a significant regional correlation between C-11-MET and F-18-FDG uptakes. Uptake of both tracers was significantly higher on the site of tumor samples showing anaplastic changes than in the rest of the tumor. Presence of necrosis in anaplastic areas of the tumor significantly reduced the uptake of C-11-MET, Conclusion: PET with C-11-MET and F-18-FDG may help to evaluate, in vivo, the metabolic heterogeneity of human gliomas, Anaplasia is a factor of increased uptake of both tracers, but microscopic necrosis in anaplastic areas influences their uptake differently, This finding probably relates to the differences in tracer uptake by non-neoplastic components of necrotic tumors. These results underline the complementary role oF F-18-FDG and C-11-MET for the study of brain tumors and favors their use for stereotactic PET guidance of diagnostic or therapeutic procedures.

We report a 25-year old immunocompetent woman with a high grade primary non-Hodgkin's lymphoma of the central nervous system (PNHL-CNS) in whom the administration of dexamethasone alone during three months produced a complete clinical and radiological response lasting over four years. If complete remission of PNHL-CNS induced by glucocorticoids are well known, the opportunity to observe glucocorticoid-induced remission for a long period of time without radio- and chemotherapy is rare. Only nine other cases of PNHL-CNS with complete remission induced by glucocorticoids lasting from 6 to 60 months, were found in the literature and are summarized here. Duration of glucocorticoids therapeutic effect in PNHL-CNS is probably underestimated. Glucocorticoids cannot be recommended as sole initial treatment for PNHL-CNS. However, we suggest standard therapies to be delayed in those patients responding completely to glucocorticoids where radio- and chemotherapy should be contraindicated (kidney, liver, bone marrow failure, pregnancy).

Gliomas are regionally heterogeneous tumors. The local relationship between histologic features and radiotracer uptake evaluated by PET should therefore influence analysis and interpretation of PET results on gliomas. This study explored this local relationship as a result of PET guidance of stereotactic biopsies. METHODS: Local histology was confronted to the regional uptake of 18F-2-fluoro-2-deoxy-D-glucose (18F-FDG) and 11C-methionine (11C-MET) in 14 patients with high-grade glioma diagnosed during a procedure of PET-guided stereotactic biopsies. We analyzed the uptake of both tracers in regions of interest centered on the stereotactic coordinates of 93 biopsy samples. RESULTS: A semiquantitative analysis revealed a significant regional correlation between 11C-MET and 18F-FDG uptakes. Uptake of both tracers was significantly higher on the site of tumor samples showing anaplastic changes than in the rest of the tumor. Presence of necrosis in anaplastic areas of the tumor significantly reduced the uptake of 11C-MET. CONCLUSION: PET with 11C-MET and 18F-FDG may help to evaluate, in vivo, the metabolic heterogeneity of human gliomas. Anaplasia is a factor of increased uptake of both tracers, but microscopic necrosis in anaplastic areas influences their uptake differently. This finding probably relates to the differences in tracer uptake by non-neoplastic components of necrotic tumors. These results underline the complementary role of 18F-FDG and 11C-MET for the study of brain tumors and favors their use for stereotactic PET guidance of diagnostic or therapeutic procedures., Comparative Study, Journal Article, Research Support, Non-U.S. Gov't, info:eu-repo/semantics/published

info:eu-repo/semantics/nonPublished

A 60 year-old woman complaining of diplopia presents an ocular motility disturbance mimicking internuclear ophthalmoplegia. Idiopathic dermatomyositis is diagnosed by the help of clinical, biological, electrophysiological and histological data. The outcome is favorable under corticotherapy. Ocular muscle involvement is rare in dermatomyositis. An overlap syndrome with another auto-immune disorder like myasthenia should be excluded in this kind of manifestation., Case Reports, English Abstract, Journal Article, SCOPUS: ar.j, info:eu-repo/semantics/published

Nerve rhabdomyomas are exceedingly rare benign tumors of the peripheral nerves consisting of well-differentiated striated muscle fibers admired with parental nerve fibers. Only one case of intracranial nerve rhabdomyoma has been described, which affected the trigeminal nerve. This report presents the detailed neuropathological description of a nerve rhabdomyoma arising in the schwannian portion of the facial nerve root in a 41-year-old Caucasian man. The nerve fibers were arranged chaotically as in a traumatic neuroma. Because of the intimate intermingling of this slow-growing tumor with the parental nerve fibers, complete excision should be avoided.

In order to take advantage of the metabolic information provided by positron emission tomography (PET) in cases of brain tumour, we have developed a technique to integrate PET images routinely in the planning of stereotactic brain biopsy. We used stereotactic PET with [F-18]-labelled fluorodeoxyglucose (PET-FDG) in 38 patients undergoing brain biopsy. To evaluate the contribution of PET-FDG in guiding brain biopsy, we analyzed the diagnosis provided by the 78 stereotactic trajectories obtained in these patients. We found that stereotactic PET-FDG seemed to provide more information in cases of anaplastic astrocytomas and glioblastomas than in low-grade gliomas. Our results also show that biopsy trajectories performed in areas where increased FDG uptake is found within the lesion boundaries always provide interpretable specimens; this was not the case for trajectories guided by CT only. Therefore, the routine integration PET-FDG in the planning of stereotactic brain biopsy may lead to a reduction in sampling. Recently, we also tested consecutive stereotactic PET with [C-11]-labelled methionine (PET-Met) and PET-FDG. This technique allowed us to compare accurately the tumoural glucose metabolism and protein synthesis. Our results suggest that stereotactic PET may increase the diagnostic yield of brain biopsy and may improve the understanding of PET in neuro-oncology.

SCOPUS: le.j, info:eu-repo/semantics/published

We have studied a girl with multiple congenital anomalies, growth and mental deficiency, characteristic facial anomalies, cataracts, cerebellar atrophy, and severe hypocholesterolemia. Death occurred at age 7 years. After excluding several syndromes, i.e. peroxisomal disorders, mevalonic acidaemia, and Marinesco-Sjögren syndrome, it is concluded that this girl had severe Smith-Lemli-Opitz Syndrome (SLOS) with exceptionally long survival. This diagnosis was confirmed through assay of 7-dehydrocholesterol in cultured fibroblasts., Case Reports, Journal Article, info:eu-repo/semantics/published

SCOPUS: cp.j, info:eu-repo/semantics/published

The aim of the present study was to determine whether routine integration of positron emission tomography (PET) with 18F-labeled fluorodeoxyglucose (FDG) in the planning of stereotactic brain biopsy increases the technique's diagnostic yield. Forty-three patients underwent combined FDG-PET- and computerized tomography (CT)-guided stereotactic biopsy of intracranial lesions according to a previously described technique. In 36 patients, an area of abnormal FDG uptake was used to guide at least one stereotactic biopsy trajectory. A total of 90 stereotactic trajectories were performed; among them, 55 were based on FDG-PET-defined targets and 35 were based on CT-defined targets. Histological diagnosis was obtained in all patients, but six of the 90 trajectories were nondiagnostic; all six were based on targets defined by CT only. Differences between the diagnostic yield of trajectories based on FDG-PET-defined targets and those based on CT-defined targets were statistically significant in patients with contrast-enhanced lesions, but not in patients with nonenhancing lesions. These results support the view the FDG-PET may contribute to the successful management of brain tumor patients requiring stereotactic biopsy. Because no significant increase in discomfort or morbidity related to the technique was found, it is suggested that the development of similar techniques integrating PET data in the planning of stereotactic biopsy should be considered by centers performing stereotactic surgery and having access to PET technology., Clinical Trial, Comparative Study, Journal Article, Research Support, Non-U.S. Gov't, info:eu-repo/semantics/published

We describe a family with an autosomal dominant form of retinal-cerebellar atrophy. There is an extreme variability in age of onset and severity of the clinical symptoms: some patients remain nearly asymptomatic throughout their entire life; others develop severe retinal and cerebellar symptoms after the age of 35 years; others suffer from a severe disorder with onset in adolescence and death during the third decade of life; in others the onset is in early childhood with prevalence of cerebellar symptoms. There is neither dementia nor epilepsy in any of the patients. Four out of five autopsies showed a severe retinal atrophy, and all five autopsies were also characterized by (1) a cerebellar atrophy affecting the spinocerebellar and olivocerebellar tracts, the cerebellar cortex and the efferent cerebellar pathways, (2) an involvement of the pyramidal pathways and of the motor neurons of brain stem and spinal cord, and (3) an atrophy of the subthalamic nucleus and to a much lesser extent of the pallidum, with also some damage to the substantia nigra. The posterior columns are much less affected except in one patient. In this family, we have excluded linkage with the two loci for spinocerebellar ataxia, i.e. SCA1 on chromosome 6p and SCA2 on chromosome 12q as well as with the locus for Machado-Joseph disease (MJD) on chromosome 14q. A genome-wide search is currently being performed to detect the disease locus responsible., Journal Article, Research Support, Non-U.S. Gov't, SCOPUS: ar.j, info:eu-repo/semantics/published

We have recently described a technique allowing routine integration of PET scan data in the planning of stereotactic brain biopsy [Levivier et al: Neurosurgery, 1992; 31:792-797]. We now report our results in a consecutive series of 38 patients that underwent combined FDG-PET and CT-guided stereotactic biopsy between June 1991 and January 1993. Stereotactic procedures were performed according to a standard protocol in which two biopsy trajectories were used whenever possible. This yielded to a total of 78 trajectories. In 31 patients, at least 1 trajectory was defined using an abnormality visualized on PET. The histological diagnosis was obtained in all cases. The diagnostic yield of each trajectory according to the FDG uptake of PET and contrast enhancement on CT showed that most low-grade tumors were found in hypometabolic/hypodense areas, glioblastomas were all diagnosed in areas with increased FDG uptake and contrast enhancement on CT while data for anaplastic astrocytomas were heterogeneously distributed. In the present series, 6 targets defined on CT were nondiagnostic; this never occurred when targets were defined on FDG-PET. In one case of anaplastic astrocytoma, the diagnosis was only made using a target defined on PET in a normal CT area; the second trajectory was made in a contrast-enhanced area on CT and was nondiagnostic. Because only structural images such as CT or MRI are available preoperatively, we analyzed separately the benefit of FDG-PET-guided biopsy in patients who have either an enhanced- or a nonenhanced lesion on CT. CT-enhanced lesions were found in 27 patients.(ABSTRACT TRUNCATED AT 250 WORDS), Journal Article, Research Support, Non-U.S. Gov't, info:eu-repo/semantics/published

OBJECTIVE: We tested adjuvant chemotherapy combining dibromodulcitol (DBD) and bischloroethylnitrosourea (BCNU) given postoperatively to adults with newly diagnosed supratentorial malignant gliomas. METHODS: We enrolled 269 patients, 255 of whom were eligible. After surgery, we treated all patients with radiation therapy, using a median dose of 60 Gy given in 30 fractions. After randomization, patients in the chemotherapy group also received (1) six weekly courses, administered during irradiation, of DBD 700 mg/m2 and (2) one to nine (median, four) courses, administered during the first year following radiation therapy, of DBD 1,000 mg/m2 on day 1 and BCNU 150 mg/m2 on day 2, with the course being repeated every 6 weeks. RESULTS: Patients treated with radiation therapy along with DBD plus BCNU (group 2) had significantly longer survival time (p = 0.044) and time to progression (p = 0.003) than did those treated with radiation therapy alone (group 1). The median survival time was 13.0 months for group 2 and 10.4 months for group 1; the median time to progression was 8.1 months for group 2 and 6.7 months for group 1. The percentage of patients alive at 18 and 24 months was 34% and 21% in group 2 compared with 21% and 12% in group 1. CONCLUSION: DBD plus BCNU is an effective adjuvant therapy for malignant glioma., Clinical Trial, Journal Article, Multicenter Study, Randomized Controlled Trial, SCOPUS: ar.j, info:eu-repo/semantics/published

A female neonate with pyruvate dehydrogenase (PDH) deficiency is presented with clinical, radiologic, biochemical, neuropathologic, and molecular genetic data. She was dysmorphic, with a high forehead, lowset ears, thin upper lip, upturned nose, and rhizomelic limbs. Cranial MRI revealed severe cortical atrophy, ventricular dilatation, and corpus callosum agenesis. Pyruvate and lactate levels were increased in CSF and blood. Urinary organic acid profile was compatible with PDH deficiency. PDH activity was normal in fibroblasts, lymphocytes, and muscle. The PDH E1-α gene was sequenced and a single base mutation was found within the regulatory phosphorylation site in exon 10. It is postulated that this mutation causes a cerebral form of PDH deficiency. Tissue-specific expression of the disease could be explained by differential X chromosome inactivation because the PDH E1-α gene is located on this chromosome. Dysmorphism with severe cerebral malformations in female patients merits a metabolic evaluation, including determination of lactate and pyruvate levels in CSF. © 1993., SCOPUS: ar.j, info:eu-repo/semantics/published

The serum and CSF of two women with a severe subacute pancerebellar syndrome contained high titers of antibodies directed against antigens present in the perikaryon of Purkinje cells. In Western blots performed on human Purkinje cells extracts these antibodies reacted with two groups of proteins the molecular weights of which were estimated to 34-38 and 62 kilodaltons (anti-Yo antibodies). Complementary investigations revealed a tubar adenocarcinoma in the first case, an ovarian carcinoma in the second. The tubar tumoral cells also contained the protein of the highest molecular weight. Compared to the serum, the CSF contained a higher proportion of anti-Yo antibodies per mg IgG, and a fraction of the latter was likely synthesized intrathecally. These antibodies may be involved in the almost total disappearance of the Purkinje cells, as observed at autopsy of both cases.

The present paper is based on stereotactic biopsies (SB) of 870 patients during the last 20 years. Approximately 700 patients suffered with brain tumour. Dementias and various diseases such as vasculopathies, leucoencephalopathies and inflammatory lesions were diagnosed in the other cases. Three groups of problems can be diagnosed with SB: 1) deeply located brain lesions; 2) early stages of lesions and diffuse or multiple lesions detected with imaging techniques; 3) some progressive dementing or degenerative diseases. Multiple (generally 4 to 6) SB should be taken in the vicinity and various parts of the lesion; they should be numbered, described separately and located exactly on an image of the brain. Handling the SB with care and the quality of the processing are important. Previous contact between neurosurgeon and neuropathologist can improve the accuracy of the diagnosis, which is reliable in the majority of cases, without producing undesirable brain damage.

A female neonate with pyruvate dehydrogenase (PDH) deficiency is presented with clinical, radiologic, biochemical, neuropathologic, and molecular genetic data. She was dysmorphic, with a high forehead, low-set ears, thin upper lip, upturned nose, and rhizomelic limbs. Cranial MRI revealed severe cortical atrophy, ventricular dilatation, and corpus callosum agenesis. Pyruvate and lactate levels were increased in CSF and blood. Urinary organic acid profile was compatible with PDH deficiency. PDH activity was normal in fibroblasts, lymphocytes, and muscle. The PDH E1-alpha gene was sequenced and a single base mutation was found within the regulatory phosphorylation site in exon 10. It is postulated that this mutation causes a cerebral form of PDH deficiency. Tissue-specific expression of the disease could be explained by differential X chromosome inactivation because the PDH E1-alpha gene is located on this chromosome. Dysmorphism with severe cerebral malformations in female patients merits a metabolic evaluation, including determination of lactate and pyruvate levels in CSF.

Neuropathological findings are reported of a 6-month-old female child with a ''cerebral'' lactic acidosis. A mutation in the pyruvate dehydrogenase (PDH) E1alpha gene was found. Gross examination of the brain revealed a severe thinning of the cerebral parenchyma marked hydrocephalus sparing the aqueduct and fourth ventricle, agenesis of the corpus callosum and heterotopic noduli of gray matter in subependymal regions. Microscopical examination showed heterotopic inferior olives, absent pyramids and focal neuroglial overgrowth into meninges. In addition some heterotopia of Purkinje cells and dysplasia of the dentate nuclei were observed. There was a marked vascular proliferation with many thin-walled, congestive vessels in the cerebral and cerebellar white matter. and to a lesser extent in the striatum. To our knowledge these cerebellar and vascular abnormalities have not been reported before in patients with ''cerebral'' lactic acidosis.The combination of these neuropathological findings might be characteristic for PDH deficiency and more specifically for its E1alpha subtype. Neuropathological examination could lead to the retrospective diagnosis of PDH E1alpha deficiency in those cases where biochemical investigations were not or incompletely performed. This may have potential implications for genetic counseling.

A 48-year-old woman was referred from Zaire with suspected rabies encephalitis. Survival was 34 days after the onset of symptoms and the diagnosis of rabies was ultimately proven after brain autopsy. Serial neurophysiological investigations, including EEG and multimodality evoked potentials and radiological examinations (cerebral magnetic resonance imaging and computed tomodensitometry) were performed. Whereas the clinical findings and EEG were compatible with brain death at the end of the course, this diagnosis could be definitely ruled out by multimodality evoked potentials, magnetic resonance imaging and autopsy. The validity of multimodality evoked potentials is discussed in this particular setting.

Neurological complications of myeloma are multiple and various. Cranial and intracranial locations have been rarely reported. They can be classified into three clinical groups: (1) cranial nerve palsies secondary to single or multiple lesions in the base of the skull, (2) intraorbital tumors, (3) intracranial tumors, either cranial myeloma extending intracranially or pure intracranial tumor (dural and/or cerebral). In our case, macroscopic and microscopic examination of the brain showed dural and cerebral involvement, confirmed by immunohistochemical studies. The radiological features are discussed (CT scan, MRI, angiography). To our knowledge, a single report of intracranial plasmacytoma documented by MRI has been reported in the literature.

Neurological complications of myeloma are multiple and various. Cranial and intracranial locations have been rarely reported. They can be classified into three clinical groups : (1) cranial nerve palsies se to single or multiple lesions in the base of the skull, (2) intraorbital tumors, (3) intracranial tumors, either cranial myeloma extending intracranially or pure intracranial tumor (dural and/or cerebral). In our case, macroscopic and microscopic examination of the brain showed dural and cerebral involvement, confirmed by immunohistochemical studies. The radiological features are discussed (CT scan, MRI, angiography) To our knowledge, a single report of intracranial plasmacytoma documented by MRI has been reported in the literature.

Transgenic mice were generated using a construct that encodes mouse polyoma virus large T antigen, one of three oncogenic products of the "early region" of the polyoma viral genome. Of 16 transgenic families developed, 1 was characterized by a neurologic disorder consisting of constant tremor and recurrent seizures. Morphologic analysis of the central nervous system (CNS) of affected transgenic mice included: classical light and electron microscopic examination; immunohistochemical assessment of the presence and localization of myelin-specific proteins, of the astrocyte marker glial fibrillary acidic protein, of the oligodendrocyte marker galaetosyl cerebro-side, and of large T; double immunolabeling of glial fibrillary acidic protein or galactosyl cerebro-side and large T to identify the CNS cell type in which large T is expressed; and in situ hybridization to study myelin basic protein gene expression. Our results suggest that polyoma large T is expressed in astrocytes, possibly resulting in altered glial-glial interactions causing impaired oligodendroglial development and secondary dysmyelination. Transgenic oligodendrocytes exhibit features of immaturity, failing to myelinate axons properly and producing morphologic phenotypes of early stages of myelination, such as numerous mesaxonal profiles. Myelin proteins are markedly reduced in transgenic CNS, and myelin basic protein transcripts, while present, are generally decreased. We believe that expression of large T in astrocytes could influence the complex and dynamic interactions between astrocytes and oligodendrocytes, perhaps with regard to the molecular (trophic) signals in the local CNS environment, bringing about arrested oligodendroglial maturation and hypomyelination. This raises intriguing questions concerning the importance of glial-glial interactions in the CNS and the complex levels of control involved in biological expression of genetic information in glial cells.

Two cases of tuberous sclerosis are presented. Extensive superficial occipital calcifications were found as classically described in Sturge-Weber syndrome. Other radiologic signs of tuberous sclerosis, such as subependymal calcifications in both patients and surgically proved giant cell astrocytoma in one patient, were present. At pathologic examination, the calcifications appeared to be located in extensive cortical tubers.

190s, info:eu-repo/semantics/published, American Academy of Neurology. 44th annual meeting. San Diego, California, May 3-9, 1992