Study of the involvement of pancreastatin in the physiopathology of diabetes mellitus associated with nonsecreting pituitary adenomas.

Pancreastatin, derived from chromogranin A, inhibits insulin and stimulates glucagon secretion in rodents. Immunohistochemistry localised pancreastatin in human pancreatic islet cells and gonadotroph pituitary cells. Nonsecreting pituitary adenomas, frequently associated with diabetes mellitus, arise quasi-constantly from gonadotroph cells. We evaluated the possible involvement of pancreastatin in the physiopathology of diabetes mellitus associated with nonsecreting pituitary adenomas. Plasma pancreastatin levels were measured by radioimmunoassay in 5 groups of subjects: 10 patients with nonsecreting pituitary adenomas associated with diabetes mellitus (group I), 10 patients with nonsecreting pituitary adenomas without diabetes (Group II), 10 patients with ACTH or GH-secreting pituitary adenomas and diabetes mellitus (Group III), 10 diabetic patients without pituitary adenomas (Group IV), and 10 healthy controls (Group V). Kidney and liver functions were normal in all of them and no patient was treated with a proton pump inhibitor. All pituitary adenomas were trans-sphenoidally removed. Immunohistochemistry against pancreastatin was performed in 5 patients of each of the 3 groups of pituitary adenomas. Plasma pancreastatin levels were not different between the different groups: 182±46 pg/ml (Group I), 195±57 pg/ml (Group II), 239±42 pg/ml (Group III), 134±31 pg/ml, (Group IV), and 122±29 pg/ml (Group V). In contrast, they were significantly (p<0.05) higher before (391±65 pg/ml) than after trans-sphenoidal surgery (149±18 pg/ml) without post-surgical change in diabetes. An immunostaining against pancreastatin was found in a majority of pituitary adenomas, associated or not with diabetes mellitus. These results argue against a role of pancreastatin in the pathogenesis of diabetes mellitus associated with nonsecreting pituitary adenomas.

Simultaneous suprasellar and pineal germ cell tumors in five late stage adolescents: endocrinological studies and prolonged follow-up.

Primary germ cell tumors (PGCT) of the central nervous system usually develop in the third ventricle area, and most frequently in the pineal region. The suprasellar region is the second preferential site for development of these tumors which are rarely simultaneously present in these two sites. We report five new cases of PGCT with pineal and suprasellar localizations, which appeared in late puberty in four boys and one girl aged 17-19 years. The clinical picture associated signs of intracranial hypertension, convergence and verticality palsies, diabetes insipidus and pituitary deficiency. Encephalic MRI revealed a double localization. Endocrine tests revealed a particular pattern associating central diabetes insipidus and a hypothalamic-pituitary disconnection syndrome. Following identification of the pathological type of lesions via a neurosurgical approach, treatment was based on a combined method using chemotherapy, radiotherapy and hormone replacement. Based on this treatment, prolonged remissions were obtained with a good quality of life.

[Radiation-induced cavernoma of the central nervous system]. / Cavernome radio-induit du système nerveux central. A propos d'un cas.

Central nervous system radiation-induced cavernoma (RIC) is a rare entity. We report one case with a review of the literature. This case illustrates the following features: mean age of 11.7 years at time of radiation and mean latency period of nine years for these RIC, which are often numerous (38%), and located in the field of the craniospinal radiation therapy. This nosological entity belongs to the spectrum of radiation-induced lesions, and requires a long-term MRI follow up in patients who underwent cranial radiation therapy.

[Pineal cyst: usefulness of endoscopic treatment]. / Kyste épithélial de la région pinéale: intérêt de la fenestration endoscopique.

Glial cysts of the pineal gland are usually benign and asymptomatic. They develop from the pineal parenchyma and contain liquid. The diagnosis is made by magnetic resonance imaging. In contrast large cysts can be symptomatic due to compression of the aqueduct of Sylvius, compression of the midbrain tectum or mass effect in the posterior fossa. We report the case of a symptomatic cyst treated by an endoscopic procedure.

[Spontaneous supratentorial cerebral hemorrhage: role of surgical treatment]. / Hémorragie cérébrale supratentorielle spontanée: quelle place pour un traitement chirurgical?

Until very recently, no specific therapies have been demonstrated to improve outcome after spontaneous intracerebral haemorrhage (ICH). The STICH (surgical treatment for intracerebral haemorrhage) study showed no overall benefit from early surgery when compared with initial conservative treatment. In contrast, the stereotactic aspiration technique can be safely performed and in a uniform manner. Despite the reduction of ICH volume, no improvement in mortality and functional result was obtained. Endoscopy is a new therapeutic option for ICH with good results for hematoma removal. Based on these feasibility studies, a randomized control trial regarding this procedure would be required to assess the efficacy of this procedure. Due to the lack of benefit observed in the recent STICH trial, emergency surgical evacuation should be reserved for patients with large lobar haemorrhage, mass effect and rapidly deteriorating clinical condition.

Usefulness of multidetector-row computerized tomographic angiography for the surgical planning in stereoelectroencephalography.

PURPOSE: Surgical planning of depth electrode implantation in stereo-electro-encephalography (SEEG) routinely uses magnetic resonance imaging (MRI) alone. Accurate visualization of arteries and veins in the vicinity of the electrode is essential to plan a safe trajectory to presumably reduce the risk of intracranial bleeding. The goal of this study was to compare multidetector row computerized tomographic angiography (MDCTA) with MRI for the visualization of vessels along each planned trajectory in patients who undergo SEEG. MATERIALS AND METHODS: Ten consecutive patients who were scheduled to undergo SEEG procedure were included. T1-weighted gadolinium-chelate enhanced MR sequence, stereotactic MDCT and MDCTA were performed after fixation of Leksell's frame. For each of the 106 planned stereotactic trajectories, the number of vessels in a 4.0mm diameter circle around the trajectory from the dura mater to the target that were visible on MDCTA were compared to that of visible vessels in the same areas on MRI. RESULTS: Ten vessels (10/106; 9.4%) were seen on MRI and 66 (66/106; 62.3%) on MDCTA (P<0.0001). All vessels visible on MRI were visible on MDCTA. The difference in number of visible vessels between the two techniques remained significant for the different lobes (i.e., frontal lobe, temporal lobe and parieto-occipital lobe). CONCLUSION: MDCTA enabled visualization of more vessels than MRI based SEEG. MDCTA may help neurosurgeons better define the trajectory of the electrode and reduce the risk of intracranial bleeding.

[Unruptured intracranial aneurysm: possible therapeutic strategies?]. / Anévrismes intracrâniens non rompus: que proposer?

Intracranial unruptured aneurysm (ICUA) has become a common condition for patient consultation. The mortality rate after fissuration is estimated to be between 52% and 85.7%. The final therapeutic decision results from a balance between the risk of rupture and risks related to the aneurysmal exclusion. Analysis of the risk of rupture risk enables a classification of risk factors. Depending on the circumstances of diagnosis, we considered the ICUA at high risk of rupture for incidental ICUA larger than 7 mm and in the event of associated aneurysms. Classifying by morphologic features, high-risk ICUA were located in the vertebrobasilar system (RR: 4.4; 95%CI: 2.7-6.8), those with a size between 7 and 12 mm (RR: 3.3; 95%CO: 1.3-8.2), larger than 12 mm (RR: 17; 95%CI: 8-36.1), those that were multilobular or a larger size and those ones with a index P/L superior to 3.4 (risk x20). Familial ICUA would expose to a major rupture risk (2 to 7 times sporadic ICUA). Some systemic factors were related to ICUA rupture: arterial hypertension (RR: 1.46; 95%CI: 1.01-2.11) and smoking addiction (RR: 3.04; 95%CI: 1.21-7.66). After microsurgical exclusion, the morbidity and mortality rates were 10% and 2% respectively. Some microsurgical morbidity factors were identified: age (32%>65 years), size (14%>15 mm), vertebrobasilar location and temporary occlusion. The rupture incidence after microsurgical exclusion was estimated 0.26%/year. After endovascular exclusion, the morbidity and mortality rates were 8% and 1% respectively. The complete exclusion rate varied between 47% and 67%. The rupture risk was estimated at 0.9%/year. Treatment recommendations were classified into 3 categories.

Impact of deep brain stimulation on pharyngo-esophageal motility: a randomized cross-over study.

BACKGROUND: Bilateral subthalamic nucleus (STN) stimulation is used to alleviate Parkinson's disease (PD) motor symptoms. Recently, it has been shown that this therapeutic also increased gut cholinergic contractions. We therefore investigated the effect of STN stimulation on esophageal motility in an interventional randomized study. METHODS: Sixteen humans PD patients (4 women, 12 men; age: 62.4 ± 9.3-years old) who underwent STN stimulation for at least 6 months were randomly evaluated with either stimulator turned OFF then ON, or inversely. Esophageal high resolution manometry was performed at the end of each ON and OFF period, with a 5 min resting period followed by ten swallows of 5 mL. KEY RESULTS: During the ON, an increase in the distal contractility index was found (OFF: 1750 ± 629 vs ON: 2171 ± 755 mmHg/cm/s; p = 0.03), with no difference in the distal front velocity. A decrease in the integrative relaxation pressure of the lower esophageal sphincter (LES) was noted (OFF: 11.1 ± 1.8 mmHg vs ON: 7.2 ± 1.8 mmHg; p < 0.05) in ON. The LES resting pressure remained unchanged during the two periods. This resulted in a decrease in the intrabolus pressure (p = 0.03). No difference was observed for the upper esophageal sphincter, nor the pharyngeal contraction amplitude and velocity. CONCLUSIONS & INFERENCES: In conclusion, STN stimulation in PD patients increased esophageal body contractions and enhanced the LES opening. This suggests that the nigrostriatal-striatonigral loop is involved in the control of esophageal motility.

Tentorial edge traumatic aneurysm of the superior cerebellar artery. Case report.

The authors report an unusual case of a traumatic aneurysm of the right superior cerebellar artery (SCA). A 22-year-old woman presented with continuous headaches that appeared 15 days after she experienced closed head trauma as a result of a cycling accident. Computerized tomography scanning performed 3 months later showed a nodular lesion on the free edge of the tentorium, which mimicked a meningioma. The aneurysm was identified on magnetic resonance angiography, which showed the SCA as the parent vessel. The parent vessel was trapped, and the aneurysm sac was excised via right temporal craniotomy. Pathological examination of the sac revealed a false aneurysm. The patient's outcome was excellent. The pathophysiology of traumatic aneurysm at such a location suggests that surgery may be the treatment of choice.

Pediatric cerebral aneurysms.

OBJECT: The exceptional pediatric aneurysm can be distinguished from its adult counterpart by its location and size; however, patient outcomes remain difficult to evaluate based on the published literature. METHODS: Twenty-two children, all consecutively treated in three neurosurgery departments, were included in this study. Each patient's preoperative status was determined according to the Hunt and Hess classification. Routine computerized tomography scanning and angiography were performed in all children on the 10th postoperative day. Each patient's clinical status was evaluated 2 to 10 years postoperatively by applying the Glasgow Outcome Scale (GOS). Twenty-one children presented with a subarachnoid hemorrhage (SAH) and one child harbored an asymptomatic giant aneurysm. Thirteen patients were in good preoperative grade (Hunt and Hess Grades I to III) and eight in poor preoperative grade (Hunt and Hess Grade IV or V). The symptomatic aneurysms were located on the internal carotid artery bifurcation (36.4%); middle cerebral artery (36.4%), half of which were found on the distal portion; anterior communicating artery (18.2%); and within the vertebrobasilar system (9.1%). A giant aneurysm was observed in 14% of patients. Overall outcome was favorable (GOS Score 5) in 14 children (63.6%) and death occurred in five (22.7%). Causes of unfavorable outcome included the initial SAH in four children, a complication in procedure in three children, and edema in one child. CONCLUSIONS: Pediatric aneurysms have a specific distribution unlike that of aneurysms in the adult population. The incidence of giant aneurysms and outcomes were similar to those in the adult population. The major cause of poor outcome was the initial SAH, in particular, the high proportion of rebleeding possibly due to a delay in diagnosis.

[Primary carcinoma of the pituitary gland with Cushing's syndrome and metastases. Apropos of a case with review of the literature]. / Carcinome primitif de l'hypophyse avec syndrome de Cushing et métastases. A propos d'un cas avec revue de la littérature.

We report the case of a 25 year old man with pituitary carcinoma with Cushing's syndrome. Though the diagnosis was initially proposed, it was only confirmed by the appearance of meningeal and lymphatic metastases. These were secretory, as confirmed by immunocytochemistry and electron microscopy. These tumours are rare, whether they secrete or not, and can be confirmed only by the existence of metastases, most often in the brain or the liver.

[A case of primitive meningeal rhabdomyosarcoma. Histological, immunohistochemical and ultrastructural study]. / Un cas de rhabdomyosarcome primitivement méningé. Etude histologique, immunohistochimique et ultrastructurale.

A 64 year-old patient, complained of headache and neurological disorders. CT scan found a voluminous solitary tumor of the posterior part of the left cavernous sinus. Removal of tumor was followed by a rapid recurrence and by the patient's death. Histologic study found a malignant undifferentiated tumoral proliferation, with strap-like cells. Immunohistochemical stains were positive for conjunctival and muscular differentiation. Ultrastructural study revealed intracytoplasmic filamentous striated structure. The primary meningeal rhabdomyosarcoma is an exceptional tumor, generally affecting young patients. Its prognosis is poor and its histogenesis remains unclear.

[Isolated cerebral metastases disclosing rectal adenocarcinoma]. / Métastases cérébrales isolées révélatrices d'un adénocarcinome rectal.

Colorectal cancer is usually revealed by modifications of bowel habit and/or signs of haemorrhage. Hepatic and lung metastases are the common sites of metastatic involvement of this cancer. Brain metastasis are rare, especially when they are isolated. We report the case of a 37-year-old patient presenting with isolated brain metastasis revealing a rectal cancer. The patient was initially treated by surgical excision of the symptomatic brain metastasis, followed several days later by anterior resection of the rectum and whole-brain radiotherapy. This unusual strategy, due to the lack of preoperative diagnosis, did not improve the poor prognosis of brain metastasis.

[Optochiasmatic tuberculoma and MRI. 2 cases]. / Tuberculomes opto-chiasmatiques et IRM. Deux observations.

Two cases are reported of optochiasmatic tuberculoma, an exceptional complication difficult to diagnose in the absence of any tuberculous context. Although diagnosis of the optochiasmatic occupying process is simple by scan or NMR imaging, it is generally difficult to identify the chiasma in a process of this type. It is therefore impossible to recognize the two forms of these tuberculomas: intra- and peri-chiasmatic histologic types, and yet these have an incidence on therapy and prognosis. Surgical exploration is justified when the diagnosis remains in doubt or when the functional prognosis is implicated.

[Vasospasm and ischemia in a series of 60 aneurysms treated quickly surgically]. / Le vasospasme et l'ischémie dans une série de 60 anévrismes opérés tôt.

Sixty patients with ruptured cerebral aneurysms were operated during the first 3 days after the onset of bleeding. In all cases the following investigations were performed: before surgery and 10 days after surgery angiography and CTScan, two months later a control CTScan. The first CTScans have been classified according to the staging defined by Fisher. Vasospasms (VS) were assessed comparing the arteriographies before and after surgery and classified as narrow or moderate; ischemia were assessed on post surgical CTScans. VS were found in any of the Fisher grades but were more frequent in grade 4; they occurred in 50% of cases (30 cases). Narrow VS were found 17 times in any Fisher grade. Ischemia occurred in 7 cases (1 death); following narrow VS in 6 cases. The high incidence of VS demonstration is emphasized: it is probably due to the lag time between bleeding and angiography and to the fact that angiographic controls were systematically performed. On the other hand ischemia occurred in 7 patients: the respective role of normalized intracranial pressure, hypervolemia, drug use and surgical procedure are discussed as candidate factors of this low incidence.

[Treatment of colloid cysts of the third ventricle by cerebrospinal fluid shunt]. / Traitement des kystes colloïdes du 3e ventricule par dérivation ventriculaire.

The authors report 6 cases of colloid cyst of the third ventricle treated with unilateral ventriculo-atrial shunt and followed up 14 to 3 years. Today the diagnostic of these cysts is safer with C.T.-Scan and M.R.I.; in the 6 cases hydrocephalus was reduced and the volume of the cysts did not increase on C.T.-Scan controls. Nowadays the stereotactic approach seems to be safer than direct surgery but is not always a radical treatment and can be insufficient to treat hydrocephalus. We think that ventricular shunt can be a good alternative as a first and definitive treatment.

[Bilateral chronic subdural hematoma: spontaneous intracranial hypotension?]. / Hématome sous-dural chronique bilatéral: une hypotension intracrânienne spontanée?

We report a case of bilateral chronic subdural hematoma (SDH) in a 48-year-old man, who presented with postural headaches, tinnitus and progressive confusion without intoxication, head trauma or abnormal hemostasis. Magnetic resonance imaging revealed cerebellar tonsillar herniation in the foramen magnum and a deformation of the brainstem. Outcome was normal after surgery. We discuss about the rare causes of SDH in young adults.

[Aneurysms with severe clinical manifestations (Hunt and Hess grade IV and V). A series of 66 cases]. / Les anévrismes à expression clinique grave d'emblée (grades IV et V de Hunt et Hess). Une série de 66 cas.

Among 250 patients consecutively admitted in our center with a ruptured intracranial aneurysm, 66 patients (24.4%) were initially classified in Hunt and Hess clinical grade IV (37 cases) or grade V (29 cases). All patients were studied as following: --The severity of subarachnoid haemorrhage was evaluated on the pre-operative C.T. scan using Fisher's criteria. Quantification of the intracerebral haematoma or the intraventricular associated haemorrhage or a subdural haematoma was estimated as well. --The arterial diameter, aneurysm size and location, and the eventual presence of intra-arterial embolus were noted on the pre-operative angiography. Aneurysm location was: anterior artery 27 cases, internal carotid artery 9 cases, middle cerebral artery 27 cases, posterior cerebral artery 2 cases, and 1 case on the basilar artery. In this series, a poor clinical condition on admission was related to the direct effect of the initial haemorrhage in 84.9% of the patients. Early seizures (7.6%) acute hydrocephalus (1.5%), multiple emboli (3%) and apparently early diffuse vasospasm (1.5%) were the other documented causes explaining the initial poor clinical condition. One case remained completely unexplained. Sixteen patients admitted with bilateral fixed dilated pupils or a major intracerebral haematoma from a ruptured anterior artery aneurysm were not operated on and subsequently died. Operative treatment (aneurysm clipping in all cases, and haematoma evacuation on demand) was performed in the remaining 50 cases within 12 hours after their admission. Thus, these patients underwent surgery on Day 0 in 31 cases, on Day 1 in 11 cases, and on Day 2 in 8 cases. A post-operative C.T. scan was performed in 46 cases. Post-operative angiographic control was only performed in 29 cases. Changes on the post-operative C.T. scan or the angiographic control were strictly compared to the neuroradiological information previously available. Final outcome was assessed at least two months after the onset. According to the Glasgow Outcome Score, the results were: good recovery 12 patients (18.2%); moderate disability 1 patient; severe disability 4 patients (6%); vegetative state 4 patients; death 45 patients (68.2%). Excluding the patients admitted in poor clinical grade but presenting with early seizures or minor initial haemorrhage, the mortality rate was 74.2%. According to the initial clinical grade, the initial C.T. scan findings, the eventual post-operative angiographic presence of an arterial thrombosis or vasospasm, it was obvious that the final bad outcome was mainly related to the severity of the initial haemorrhage. However, in 7 patients, post-operative disability or death can be explained by other complications, principally an arterial thrombosis.(ABSTRACT TRUNCATED AT 250 WORDS)

[Ewing's sarcoma of the ethmoid bone. Case report]. / Sarcome d'Ewing de l'ethmoïde. A propos d'un cas.

Ewing's sarcoma is rarely located at the base of the skull. We report a case of ethmoid Ewing's sarcoma. Diagnosis was established on microscopic examination. A complete surgical resection was performed, followed by cranial base reconstruction. During the postoperative period, a combined course of chemotherapy and radiotherapy was performed. We also present a review of the literature.

[Primary cauda equina epidermoid cyst. A case report]. / Kyste épidermoïde congenital de la queue de cheval.

A primary epidermoid cyst of the cauda equina was diagnosed in an eleven-year-old boy. The patient had a previous two months history of lumbar pain with unilateral L 4 radicular pain. The diagnosis of an epidermoid cyst of the cauda equina, suspected by MRI, was confirmed by histology following surgery. Wide tumor resection, as complete as possible, was performed with minimum trauma. Presence of an epidermoid cyst in the filum terminale is a rare finding, few cases have been reported in the literature. However, surgical treatment and favorable post-operative course are similar to that observed in intraspinal localizations. MRI is helpful for the preoperative and differential diagnosis and for post-operative follow-up.