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We describe a rare case of giant intracranial chordoma, emphasizing the patient's long survival and his excellent response to radiotherapy that led to a progressive regression of neurological symptomatology up to disappearance, in the absence of cerebral white matter damages.

Background: Oligodendroglial tumors are rare and chemosensitive diseases; but the overall results with current chemotherapy regimens cannot be considered satisfactory and other active treatments are necessary. We decided to determine the efficacy and toxicity profile of the carboplatin and etoposide (CE) regimen in this setting., Methods: In this phase II trial we evaluated the response rate of first or second line CE regimen (Carboplatin AUC 5 on day 1 and Etoposide 120 mg/m2 on days 1-3 every 28 days) in patients with recurrent/progressive oligodendroglial tumors., Results: Thirty-two patients were enrolled. Median age was 42 years (range 22-66); median ECOG PS was 0 (range 0-2); 9 patients had oligodendroglioma, 3 patients had oligoastrocytoma, 11 patients had anaplastic oligodendroglioma, 9 patients had anaplastic oligoastrocytoma. CE regimen showed a response rate of 46.9% with 5 complete responses (15.6%) and 10 partial responses (31.3%). Eleven patients (34.4%) had stable disease. Median time to progression was 8 months, progression-free survivals at 6 and 12 months were 80% and 46.9%, respectively. Toxicity was mainly hematological, with grade 3-4 neutropenia in 5 (15.6%) patients., Conclusions: In this trial CE regimen has shown relevant activity with a favourable safety profile.

Neuroblastoma (NB) is characterised by the secretion of catecholamines in approximately 95% of patients. Tyrosine hydroxylase is the rate-limiting enzyme in catecholamine biosynthesis pathway. Expression of the tyrosine hydroxylase gene (TH) is regulated in a tissue-specific manner during neonatal development and differentiation, therefore TH mRNA expression is a specific tumour marker for NB. Here we present a real-time reverse transcriptase polymerase chain reaction (RT-PCR) assay using TaqMan technology for detection and quantification of TH mRNA in bone marrow (BM) NB patients. The degree of TH expression was derived from the ratio of the mRNAs of this gene and the reference gene, beta-actin. A ratio greater than 3x10(-2) was considered as positive for TH mRNA presence. Samples were also examined for TH mRNA by first and nested RT-PCR. Seventeen BM samples from 4 patients with disseminated NB (3 stage IV and 1 stage IVs) were evaluated at diagnosis and during treatment. We found a variable degree of TH expression ranging from 0.0344 to 26.3370 in 12/17 positive samples, while no TH mRNA (value lower than 3x10(-2)) was detected in 5/17 samples obtained after consolidation therapy. Our results show a moderate concordance between different qualitative RT-PCR methods and real-time RT-PCR. The real-time RT-PCR results seem to fit better with the natural short-term clinical follow-up of the evaluated patients, with respect to qualitative methods. Real-time TH RT-PCR could therefore be of clinical value for the assessment of a patient's prognosis by monitoring minimal residual disease (MRD).

The present study explored the frequency of neuromorphological, neurological and neuropsychological abnormalities in 13 patients with an ICD-8/9 diagnosis of simple schizophrenia, also fulfilling DSM-IV criteria for 'simple deteriorative disorder', and in 13 matched patients with an ICD-8/9 diagnosis of a subtype of schizophrenia other than simple schizophrenia, fulfilling DSM-IV criteria for schizophrenia. The frequency of neuromorphological abnormalities in the two patient groups was also compared with that observed in 13 neurological control subjects. Both patients with simple schizophrenia and those with other schizophrenia subtypes showed a higher frequency of brain developmental abnormalities and greater ventricular and subarachnoid space volumes than controls. There was no significant difference between the two groups of patients with respect to neuromorphological variables. Two patients with simple schizophrenia (vs. none of those with other schizophrenia subtypes) had gross brain abnormalities; they were the most deteriorated subjects in the whole sample. Patients with simple schizophrenia, as compared to those with other schizophrenia subtypes, presented a higher frequency of soft neurological signs and a greater impairment of social relationships.

Objective: To ascertain the diagnostic ability of a computerized fetal heart rate (FHR) analysis system in the identification of patients at risk of fetal distress in labor., Study Design: Five-hundred and seventy-seven healthy term pregnancies were enrolled in a prospective, cross-sectional study and subdivided into two groups, with (n = 90) or without (n = 487) fetal distress in labor. Computerized FHR recordings were performed periodically and regression analysis was performed in a univariate and multivariate way to assess the ability of the last FHR recordings in the prediction of subsequent fetal distress., Results: The two groups showed a significant difference in almost all of the FHR parameters studied. The multivariate analysis showed that only the FHR baseline and the percentage of small acceleration were independently and significantly related to the labor outcome. The combination of these two parameters reaches a sensitivity of 45%, specificity of 91%, positive predictive value of 48% and negative predictive value of 90%, with an overall accuracy of 84%., Conclusions: The increase in FHR baseline and in small FHR accelerations can be major factors in the prediction of subsequent fetal distress in healthy term fetuses.

Aneurysmal bone cysts (ABC) are relatively uncommon benign expansile osteolytic lesions characterized by multiple cavities with serum-blood levels and delimited by a thin periosteal external border. The differential diagnosis is difficult to make with conventional radiography, while CT and MRI are elective techniques. Ten patients with ABC (7 central and 3 eccentric lesions) were examined with CT and MRI. Four cysts were localized at the proximal femur, 2 in calcaneal, 2 in vertebral (cervical and dorsal), 1 in tibial and 1 in iliac sites. Diagnostic criteria were the presence of fluid-fluid levels and a thin hyperdense peripheral border at CT, while hyperintense cavities on T2-weighted sequences, fluid-fluid levels, pseudodiverticular features and a low-signal border were found at MRI. Intralesional levels were detected in 9 patients at CT and in 10 at MRI; the 3 peripheral cysts exhibited a hyperdense extraosseous border at CT, corresponding to the periosteal shell, considered a benignity sign. To conclude, CT and MRI, thanks to their high resolution, clearly depict the anatomopathologic features of ABC, thus allowing this type of lesion to be differentiated from other benign and malignant osteolytic lesions.

Pulsed Doppler duplex sonography was performed longitudinally on 11 uncomplicated singleton pregnancies from 20 to 41 week's gestation (87 measurements). Blood flow velocities were recorded at 3 locations: abdominal insertion site (O), in the free floating part (C), placental insertion site (PL). The objective of the study was to determine whether the C site S/D ratio, the quickest and easiest to perform, relates to the other sites. The S/D ratio measurements fell from the near abdomen (O) to the free floating part (C) to the placental insertion site (PL) between 25-35 week's gestation. Statistical analysis, including analysis of variance for repeated measures and student t test, showed no significant difference in S/D ratio among the locations before 25 and after 35 week's gestation. The S/D ratio at the O site was significantly higher than at the PL site between 25-35 week's gestation; the C site was not significantly different from the PL site.

Eighteen cases of cerebral cavernous angiomas are reported, and their histopathological, clinical and radiological features are reviewed. MR complements TC in characterizing cavernomas and in distinguishing them from similar-appearing lesions. However, when such lesions are seen with atypical features, discrimination from hemorrhagic tumors remains problematic. More malignant lesions present, on MRI, a much larger area of abnormality and make themselves obvious in a shorter period. Gadolinium administration may allow a more specific interpretation of small lesions.

Twenty-one patients (9 female and 12 male) aged from 13 to 22 years were observed for lumbago and lumbo-sciatica caused by a discopathy which was subsequently confirmed by radiology and surgery. None of these patients had a history of trauma. It must be underlined that the painful symptoms exhibited and the young age of the patients suggest such pathologies as infections, tumours or malformations, but the possibility of discopathy must always be considered. It is also interesting to note the differences observed in symptomatology between adults and adolescents and to consider the various pathogenetic factors which might cause discopathy in adolescents while it was until now believed to be reserved to adults.

A case of low dorsal spinal cord compression, due to ossifying arachnoiditis, is reported. Its relationship with a venous malformation overlying the dorsal surface of the spinal cord is mentioned. The literature is reviewed and possible etiopathogenetic factors favouring this rare condition are discussed in the light of few similar reports. The value of surgical treatment is stressed.

The Authors report three cases of non tumoral aqueductal stenosis associated with von Recklinghausen disease in children. Moreover, 16 similar cases collected from the literature are illustrated. The clinical features are evaluated in light of literature's data. Among all 19 cases, the median age was 19 years (range 6-46 years) and 9 patients were under the age of 13 years. In this latter group, the most represented clinical symptoms were headache and gait disturbances. As regards the neuroradiological procedures, CT scan represented the best tool in the evaluation of the ventricular size. Our own three cases underwent to ventriculo-atrial shunt procedures, followed by clinical recovery (follow-up: 3 months-5 years). In conclusion it is felt that, among all the pathological events usually described in patients affected by neurofibromatosis, aqueductal stenosis seems to belong to the natural history of this disease.