Hypertension, hyperekplexia, and pyramidal paresis due to vascular compression of the medulla.

MRI showed impingement of the vertebral artery on the left lateral medulla in two patients with arterial hypertension, exaggerated startle reflexes (hyperekplexia), and progressive spastic paresis. One patient underwent microvascular decompression with normalization of arterial hypertension, disappearance of hyperekplexia, and improvement of spastic paresis. The combination of arterial hypertension, hyperekplexia, and progressive spastic paresis should arouse suspicion of neurovascular compression of the lateral medulla.

Oncocytic meningioma.

Six cases of meningioma showing oncocytic changes are described. The lesions were composed mostly of sheets, nests, and cords of large polygonal cells with finely granular eosinophilic cytoplasm rich in mitochondria. Neoplastic cells showed nuclear pleomorphism with prominent nucleoli. Necrosis and high mitotic rate were present in the majority of cases. Oncocytic differentiation was demonstrated by conventional histology, immunocytochemistry, electron microscopy, and Western-blot analysis. Oncocytic meningiomas showed an aggressive behavior; recurrences were observed in three cases, and invasion of brain cortex was evident in other two cases.

In vitro and in vivo MRS study of human glioma metabolites.

In this study an in vitro multinuclear magnetic resonance spectroscopy (MRS) characterization of water soluble metabolites and of the lipid fraction obtained from 19 human gliomas (12 glioblastomas, 3 anaplastic astrocytomas, 2 anaplastic oligodendrogliomas and 2 oligodendrogliomas) with a total of 27 surgical specimens, is reported. Moreover, some in vivo H-1 MRS results are shown. The regional metabolic heterogeneity of glioblastomas, according to their morphological heterogeneity, is documented. For glioblastomas a specific in vitro H-1 MRS metabolite profile cannot be defined. Low and high grade oligodendrogliomas showed characteristic choline/creatine and alanine/creatine metabolite ratios. The spectroscopical characterization of histopathological factors concerning malignant gliomas is shown.

In vivo single voxel 1H MRS of glial brain tumors: correlation with tissue histology and in vitro MRS.

Eighteen patients affected by glial brain tumors were examined by localized single voxel magnetic resonance spectroscopy (1H MRS). For all patients histological diagnosis was obtained after stereotactic biopsy or surgical removal of the tumor. Histological nuclear atypia, tumor cellularity, necrosis, vascular proliferation and percentage of Ki-67 positive cells in the total cell populations were evaluated. It was found that some of the in vivo 1H MRS parameters were related to the histological features of the tumor. In particular, the Choi/Cri metabolite ratio obtained from the neoplastic tissues paralleled the Ki-67 cell proliferation index. For some of the patients 1H and 13C in vitro high resolution magnetic spectroscopy of surgical specimens was also carried out. 1H MRS of aqueous extracts from different biopsy specimens of the same tumor furnished fine biochemical information on intratumor heterogeneity. The degree of histological vascular proliferation of the tumor was correlated to the presence of cholesteryl esters in the 13C spectrum of the lipid fraction of surgical specimens.

Cytogenetic investigation of chordomas of the skull.

We report the first cytogenetic investigation of cranial chordoma. Three cranial chordomas were examined, two of which could be further histopathologically classified as chondroid chordomas. In addition, we have included a case of chordoma of a cervical vertebra to compare the cytogenetic abnormalities. Diagnosis was made at histological and immunohistochemical levels. The three cases of cranial chordoma showed a normal karyotype, while one vertebra showed 46,XY,t(6;11)(q12;q23). Chordomas, particularly those containing cartilage, have to be distinguished from chondrosarcomas of the skull base. Such a distinction is normally based on expression of epithelial markers which usually are lacking in chondrosarcoma. Cytogenetic investigation may eventually prove to be useful in the distinction of the two lesions, if chromosome anomalies are consistently absent in chordoma, although some chondrosarcomas may also present a normal karyotype. Such a distinction has clinical implications because chondroid chordomas show better survival, whereas chondrosarcomas show a propensity to infiltrate the surrounding tissues.

Early ictal speech and motor inhibition in fronto-mesial epileptic seizures: a polygraphic study in one patient.

OBJECTIVE: To investigate ictal motor inhibition occurring during seizures in a patient with a tumor located in the left fronto-mesial pre-central cortex. METHODS: Awake and sleep video-polygraphic monitoring, recording scalp EEG and EMG activities from several cranial, trunk and limbs muscles, was performed in a patient with drug-resistant recurrent focal motor seizures before surgical treatment. Speech/motor tasks were repeatedly administered to the patient during the recording sessions in order to evaluate the occurrence of early ictal motor inhibition. RESULTS: Thirty-four seizures were recorded during wakefulness showing a stereotyped pattern of inhibition of speech and voluntary movements followed by sequential activation of upper limb-trunk-lower limb muscles contralateral to the tumor. Polygraphic recordings showed that: (1) initial speech and motor arrest were associated with the EMG evidence of progressive muscle tone suppression in cranial and right distal upper limb muscles; (2) tonic contraction of right deltoid, biceps brachii, intercostalis and paraspinalis muscles appeared after motor inhibition; (3) tonic-clonic activity in the right tibialis anterior muscle occurred at the end of seizures. Eleven subclinical seizures were recorded during sleep showing mild focal tonic EMG activity in right side trunk muscles. CONCLUSIONS: Our findings evidenced early and somatotopically organized inhibition of voluntary movement at the beginning of epileptic seizures with fronto-mesial onset. The demonstration that speech and motor arrest were associated with progressive EMG suppression in cranial and limb muscles supports the hypothesis of motor inhibitory seizures originating in the mesial aspect of pre-motor frontal cortex.

Granulomatous hypophysitis caused by a ruptured intrasellar Rathke's cleft cyst: report of a case and review of the literature.

OBJECTIVE AND IMPORTANCE: Ruptured Rathke's cleft cyst is a rare cause of giant cell granulomatous hypophysitis. Chronic inflammatory reaction is caused by extravased cyst content into the adjacent gland. We provide a demonstration that mucins produced by cells lining the cyst wall caused the granulomatous giant cell reaction. CLINICAL PRESENTATION: A 37-year-old nonpregnant woman presented with a 3-year-history of headache and amenorrhea. She had experienced normal sexual maturation, and her medical history was unremarkable. Radiologically, the lesion appeared as an intrasellar mass with a cystic component indistinguishable from a pituitary adenoma with cystic degeneration. TECHNIQUE: The patient underwent a transsphenoidal approach. Because no demarcation between normal and affected tissue was evident at surgery, the lesion and residual pituitary were radically removed. Tissue was studied using routine hematoxylin and eosin and histochemical stainings for mucins and immunocytochemical techniques. CONCLUSION: This study demonstrates that mucins that had spilled out from the cyst caused the granulomatous reaction. Using computed tomography, magnetic resonance imaging, and gross inspection, distinction between granulomatous hypophysitis and pituitary adenoma was virtually impossible. Nevertheless, a granulomatous reaction of the pituitary gland should be suspected in a case of a sellar mass having a cystic area. In such cases, intraoperatory diagnosis on frozen sections is mandatory because adoption of a conservative treatment allows preservation of the gland.

Magnetic resonance spectroscopy study of low grade extra and intracerebral human neoplasms.

In this study we report an in vitro 1H magnetic resonance spectroscopy (MRS) characterization of water soluble metabolites obtained from 17 low grade extra and intracerebral human tumors (8 meningiomas, 5 oligodendrogliomas, 2 subependimomas and 2 ependimomas). In addition, the in vivo localized 1H MRS results for 4 of the meningiomas are reported. The main metabolic features characterizing low grade tumors were investigated. Meningiomas are characterized by high Cho/Cr and Ala/Cr ratios; in many cases both in the in vitro and the in vivo spectra these ratios cannot be evaluated, due to the absence of the Cr metabolite. Low grade oligodendrogliomas are characterized by low Cho/Cr ratios. High amounts of myo-inositol are found in the spectra of ependimomas and subependimomas which are distinguished by different Cho/Cr ratio values and by a different Ala content.

Sialorrhea from fourth ventricle hydrocephalus. Case report.

The case of a 50-year-old man with tetraventricular hypertensive hydrocephalus is presented, remarkable for fourth ventricle dilatation. This patient showed a significant sialorrhea as the main symptom, which is quite unusual. This condition was successfully treated by cerebrospinal fluid diversion. The uncommon features of this case are summarized.

Expanding lacunae causing triventricular hydrocephalus. Report of two cases.

Two patients are reported in whom the presence of triventricular hydrocephalus and aqueductal obstruction or stenosis due to multiple expanding lacunae in the mesencephalothalamic region possibly corresponds to abnormally dilated perivascular spaces. Placement of a ventriculoperitoneal cerebrospinal fluid (CSF) shunt in one patient and the performance of a third ventricle cisternotomy in the other reversed the hydrocephalic syndrome, but did not modify the complex neuroophthalmological disturbance and rubral tremor presumably related to the compressive effects of the lacunae on adjacent parenchyma. In one patient the number and size of the lacunae were increased 4 years after CSF shunt placement. A review of the literature revealed two cases in which magnetic resonance imaging demonstrated a similar, poorly understood pathological condition.

A hazard of craniotomy in the sitting position: the posterior compartment syndrome of the thigh. Case report.

A bilateral posterior compartment syndrome of the thigh with a sciatic neuropathy in a patient following a craniotomy in the sitting position is described. The pathophysiology of the sciatic nerve dysfunction is discussed and the diagnostic value of computerized tomography is emphasized. Prompt decompression of the nerve is suggested.

Osseous metaplasia in a growth hormone-secreting pituitary adenoma.

A case of growth hormone-secreting adenoma of the pituitary gland showing osseous metaplasia is described in a 56-year-old acromegalic female. The tumor was composed of nests of densely granulated cells separated by and intermixed with calcifications, trabeculae of mature bone and fat. Calcifications are seldom found in pituitary adenomas. In rare instances, calcium deposits can be prominent enough to lead to the formation of pituitary stones and bone which replace the entire tumor mass. Analogously with metaplastic meningiomas, we propose using the term metaplastic adenoma to define cases with osseous metaplasia in order to distinguish between lesions containing bone from the more frequently seen calcified adenomas.

Metastases of the spinal cord from remote neoplasms. Study of five cases.

Five patients with acute or subacute myelopathy are reported in whom neuroradiologic imaging or open surgery showed an intrinsic spinal cord tumor. Histologic diagnosis was obtained either through direct spinal cord biopsy or after biopsy or removal of a former or concurrent intracranial metastasis. Carcinoma of "possible" gastrointestinal origin, oat-cell carcinoma, melanoma, and bronchogenic adenocarcinoma in two cases were demonstrated. The clinical course showed a devastating and irreversible deterioration of the neurological condition in all patients but one, who exhibited a mildly symptomatic disease. Our uncertainty with cases of intramedullary spinal cord metastasis resulted in a nonstandardized approach to these patients. The role of magnetic resonance imaging, the radiotherapeutic option, and the pertinent literature are briefly discussed.

Delayed angiographic appearance of a large basilar aneurysm.

A technically adequate four-vessel cerebral angiogram was normal on the first day after a typical episode of subarachnoid hemorrhage. The study was repeated 40 days later showing a large aneurysm of the basilar bifurcation. Hypothetically, the first attempt to visualize the aneurysm was negative due to early transitory thrombosis of the aneurysmal sac. In some cases of subarachnoid hemorrhage it is beneficial to repeat previously normal angiographic studies.

[A case of cerebral coenurosis]. / Su di un caso di cenurosi cerebrale.

In this article the authors describe a case of cerebral coenurosis, a rare infection caused by the larval stage of the tapeworm Taenia multiceps, encountered during their professional practice. The specific epidemiological elements, linked to the parasitic lifecycle in dogs, will also be covered. The authors consider the diagnostic, pathologic and clinical elements that allow differential diagnosis with respect to neurocysticercosis, a parasitic infection caused by the larval form of other Cestoda, discussing possible medical therapeutic approaches (albendazole or praziquantel) and neurosurgical intervention. They emphasize the need to obtain a correct hystopathological diagnosis in order to achieve a differential diagnosis versus the other larval parasitosis. According to current public health regulations this diagnosis must be reported to the Health Official to allow the necessary epidemiological interventions to be planned.

[Metastasis in the inner auditory canal]. / Metastasi nel condotto uditivo interno.

Metastasis in the inner auditory canal (IAC), or more precisely in the pontocerebellar angle (PCA) is extremely rare. Indeed acoustic neurinoma (AN), meningioma and other types of benign neoformations, in decreasing order, account for nearly all expansive pathologies in this anatomical region. The present paper reports a clinical case of a patient with the sudden onset of an hearing loss in the right ear. This hearing loss was promptly followed by paralysis of the homolateral facial nerve. The function of both cranial nerves worsened progressively and rapidly. Cerebral radiology, performed with MRI and the administration of gadolinium, showed a AN-compatible neoformation fundamentally affecting the IAC. However, histological tests performed after surgery indicated an adenocarcinoma. Post-operative instrumental tests identified the primary neoplasm in the lower lobe of the right lung. The purpose of this work was to highlight, in view of the literature on the topic, the main clinical aspects to consider when a malignant expansion is suspected in the PCA. Naturally there is greater basis for such suspicion when a primary malignant neoplasm has previously been diagnosed. Generally AN progresses quite gradually, in line with an extremely slow growth rate. Therefore it is normally seen in a progressive worsening of hearing, tinnitus and equilibrium disorders. Appearance of a facial nerve palsy is strictly limited to relatively large neoplasms and is encountered at a later stage. This is why a rapid progression of hearing damage accompanied, or promptly followed, by complete facial nerve paralysis must alert one to the possibility of a PCA malignancy. Imaging does not permit easy pre-operative diagnosis since the radiological signs and morphologies are often quite similar to those produced by AN. Therefore, post-operative histological examination can hold some surprises.

Pattern of care and effectiveness of treatment for glioblastoma patients in the real world: Results from a prospective population-based registry. Could survival differ in a high-volume center?

BACKGROUND: As yet, no population-based prospective studies have been conducted to investigate the incidence and clinical outcome of glioblastoma (GBM) or the diffusion and impact of the current standard therapeutic approach in newly diagnosed patients younger than aged 70 years. METHODS: Data on all new cases of primary brain tumors observed from January 1, 2009, to December 31, 2010, in adults residing within the Emilia-Romagna region were recorded in a prospective registry in the Project of Emilia Romagna on Neuro-Oncology (PERNO). Based on the data from this registry, a prospective evaluation was made of the treatment efficacy and outcome in GBM patients. RESULTS: Two hundred sixty-seven GBM patients (median age, 64 y; range, 29-84 y) were enrolled. The median overall survival (OS) was 10.7 months (95% CI, 9.2-12.4). The 139 patients ≤aged 70 years who were given standard temozolomide treatment concomitant with and adjuvant to radiotherapy had a median OS of 16.4 months (95% CI, 14.0-18.5). With multivariate analysis, OS correlated significantly with KPS (HR = 0.458; 95% CI, 0.248-0.847; P = .0127), MGMT methylation status (HR = 0.612; 95% CI, 0.388-0.966; P = .0350), and treatment received in a high versus low-volume center (HR = 0.56; 95% CI, 0.328-0.986; P = .0446). CONCLUSIONS: The median OS following standard temozolomide treatment concurrent with and adjuvant to radiotherapy given to (72.8% of) patients aged ≤70 years is consistent with findings reported from randomized phase III trials. The volume and expertise of the treatment center should be further investigated as a prognostic factor.

Focal epilepsies associated with glioneuronal tumors: review article.

Glioneuronal tumors (GNTs) are an increasingly recognized cause of focal epilepsies, particularly in children and young adults. GNTs consist of a mixture of glial and neuronal elements and most commonly arise in the temporal lobe, particularly in the temporo-anterior-basal mesial site. They are often associated with cortical dysplasia or other neuronal migration abnormalities. Epilepsy associated with GNT is poorly controlled by antiepileptic drugs in many cases; but, it is extremely responsive to surgical treatment. However, the best management strategy of tumor-related focal epilepsies remains controversial and still remain one of the contemporary issues in epilepsy surgery. Temporo-mesial GNT are associated with a widespread epileptic network, defining, therefore, a distinct anatomo-clinico-pathological group with complex epileptogenic mechanisms. By using an epilepsy surgery oriented strategy GNT associated with focal epilepsies may have an excellent seizure outcome and, therefore, surgical treatment can be offered early to avoid both the consequences of uncontrolled seizures as well as the side effects of prolonged pharmacological therapy and the rare risk of tumor growth or malignant transformation.

Revascularized giant aneurysm of the anterior communicating artery after surgery and embolization, occluded by placement of a Leo+Baby intracranial stent. A case report.

Balt (Montmorency, France) recently manufactured the Leo+Baby dedicated intracranial stent for arteries with a calibre between 1.5 and 3.10 mm. We describe a patient with a partially thrombosed giant sacciform aneurysm of the anterior communicating artery treated without success by surgery and coil embolization subsequently occluded by placement of a Leo+Baby stent (Balt, Montmorency, France). A 56-year-old man presented with a giant aneurysm in the anterior communicating artery region. Following successive surgical intervention and embolization procedures the patient was referred to us with a revascularized aneurysm measuring 15×9×8 cm. To stabilize the endovascular occlusion a combined treatment was scheduled with coil embolization and stent deployment after dual antiplatelet therapy started five days before the interventional procedure. Treatment was undertaken two weeks later under general anaesthesia and total heparinization. A microcatheter was inserted into the aneurysmal sac and four metal coils were released for a total of 61cm obtaining almost complete occlusion of the aneurysm from the circulation. A Leo+Baby stent (2.5×18 mm) was subsequently deployed across the aneurysm neck. At follow-up angiography two months later the aneurysm appeared substantially excluded from the arterial circulation except for a small portion in the neck. Six months later, four months after suspending antiplatelet therapy, follow-up angiography disclosed the complete exclusion of the aneurysm from the circulation. Deployment of the new ministent through the same microcatheter used to release the coils made the interventional procedure simpler and faster.