Pascal Rousseaux

Randomized, double-blind, placebo-controlled, pilot trial of high-dose methylprednisolone in aneurysmal subarachnoid hemorrhage

OBJECT The object of this study was to determine the efficacy of methylprednisolone in reducing symptomatic vasospasm and poor outcomes after subarachnoid hemorrhage (SAH). METHODS Ninety-five patients with proven SAH were recruited into a double-blind, placebo-controlled, randomized trial. Starting within 6 hours after angiographic diagnosis of aneurysm rupture, placebo or methylprednisolone, 16 mg/kg, was administered intravenously every day for 3 days to 46 and 49 patients, respectively. Deterioration, defined as development of a focal sign or decrease of more than 1 point on the Glasgow Coma Scale for more than 6 hours, was investigated by using clinical criteria and transcranial Doppler ultrasonography, cerebral angiography, or CT when appropriate. The end points were incidence of symptomatic vasospasm (delayed ischemic neurological deficits associated with angiographic arterial narrowing or accelerated flow on Doppler ultrasonography, or both) and outcome 1 year after entry into the study according to a simplified Rankin scale (Functional Outcome Scale [FOS]) in living patients and the Glasgow Outcome Scale in all patients included. RESULTS All episodes of deterioration and all living patients with a 1-year outcome were assessed by a review committee. In patients treated with methylprednisolone, the incidence of symptomatic vasospasm was 26.5% compared with 26.0% in those given placebo. Poor outcomes according to FOS were significantly reduced in the Methylprednisolone Group at 1 year of follow-up; the risk difference was 19.3% (95% CI 0.5-37.9%). The outcome was poor in 15% (6/40) of patients in the Methylprednisolone Group versus 34% (13/38) in the Placebo Group. CONCLUSIONS A safe and simple treatment with methylprednisolone did not reduce the incidence of symptomatic vasospasm but improved ability and functional outcome at 1 year after SAH.

Incidence of basioccipital hypoplasia in Chiari malformation type I: comparative morphometric study of the posterior cranial fossa. Clinical article.

OBJECT The chronic tonsillar herniation defining Chiari malformation Type I (CMI) is thought to result from overcrowding of a normally developing hindbrain within a congenitally small posterior cranial fossa (PCF) due to occipital hypoplasia. The goals in the present study were to authenticate the cranioencephalic disproportion in a group of patients with CMI and to discuss new developmental aspects according to which part of the occipital bone was underdeveloped. METHODS The authors retrospectively examined a group of 17 patients with CMI. Measurements of osteotentorial and neural structures of the PCF were made on MR images of the brain. The results were compared with findings in 30 healthy controls by using the Mann-Whitney U-test. RESULTS Dimensions of the neural structures did not differ between the 2 groups of patients. The mean length of the basiocciput was significantly shorter in the CMI group (19.4 mm) compared with the control group (25.7 mm; p = 0.0003). The mean diameter of the foramen magnum was larger in the CMI group, but this difference was not statistically significant. The dimensions of the supraocciput and the mean angle of the cerebellar tentorium were identical in the 2 groups. CONCLUSIONS Data in this study support the idea that occipital hypoplasia is the main cause of overcrowding within the PCF. Basioccipital shortness is a cardinal feature of the resultant shallow PCF and could proceed from a congenital disorder of the cephalic mesoderm of the parachordal plate or occur later in the infancy because of premature stenosis of the sphenooccipital synchondrosis.

FRACTIONATED STEREOTACTIC RADIOTHERAPY TREATMENT OF CAVERNOUS SINUS MENINGIOMAS: A STUDY OF 100 CASES

PURPOSE We discuss our experiences with fractionated stereotactic radiotherapy (FSR) in the treatment of cavernous sinus meningiomas. METHODS AND MATERIALS From 1995 to 2006, we monitored 100 patients diagnosed with cavernous sinus meningiomas; 84 female and 16 male patients were included. The mean patient age was 56 years. The most common symptoms were a reduction in visual acuity (57%), diplopia (50%), exophthalmy (30%), and trigeminal neuralgia (34%). Surgery was initially performed on 26 patients. All patients were treated with FSR. A total of 45 Gy was administered to the lesion, with 5 fractions of 1.8 Gy completed each week. Patient treatment was performed using a Varian Clinac linear accelerator used for cranial treatments and a micro-multileaf collimator. RESULTS No side effects were reported. Mean follow-up period was 33 months, with 20% of patients undergoing follow-up evaluation of more than 4 years later. The tumor control rate at 3 years was 94%. Three patients required microsurgical intervention because FSR proved ineffective. In terms of functional symptoms, an 81% improvement was observed in patients suffering from exophthalmy, with 46% of these patients being restored to full health. A 52% improvement was observed in diplopia, together with a 67% improvement in visual acuity and a 50% improvement in type V neuropathy. CONCLUSIONS FSR facilitates tumor control, either as an initial treatment option or in combination with microsurgery. In addition to being a safe procedure with few side effects, FSR offers the significant benefit of superior functional outcomes.

Fractionated stereotactic radiotherapy for acoustic neuromas: A prospective monocenter study of about 158 cases

PURPOSE To evaluate long-term outcomes and efficacy of fractionated stereotactic radiotherapy in the treatment of acoustic neuromas. MATERIAL AND METHODS Between January 1996 and December 2009, 158 acoustic neuromas were treated by FSR in 155 patients. They received a dose of 50.4 Gy, with a safety margin of 1-2mm with a median tumor volume at 2.45 mL (range: 0.17-12.5 mL) and a median follow-up duration at 60 months (range: 24-192). RESULTS FSR was well tolerated in all patients with mild sequelae consisting in radiation-induced trigeminal nerve impairments (3.2%), Grade 2 facial neuropathies (2.5%), new or aggravated tinnitus (2.1%) and VP shunting (2.5%). The treatment failed in four patients (2.5%) who had subsequent surgery respectively at 20, 38, 45 and 84 months post-FSR. The local tumor control rates were respectively 99.3%, 97.5% and 95.2% at 3, 5 and >7-year of follow-up. For initial Gardner-Robertson Grade 1 and 2 ANs, the preservation of useful hearing was possible in 54% of the cases; only Grade 1 ANs had stabilized during the course of the follow-up with 71% >7 years. However, hearing preservation was not correlated to the initial Koos Stage and to the radiation dose delivered to the cochlea. Tinnitus (70%), vertigo (59%), imbalance (46%) and ear mastoid pain (43%) had greatly improved post-FRS in most patients. Tumor control, hearing preservation and FRS toxicity were quite similar in patients with NF2, cystic acoustic neuroma, prior surgical resection and Koos Stage 4 AN. No secondary tumors were observed. CONCLUSION FSR is a safe and effective therapeutic for acoustic neuromas and could be an alternative to microsurgery. Compared to radiosurgery, there are no contraindications for fractioned doses of stereotactic radiotherapy especially for Stage-4 tumors and patients at high risk of hearing loss.

Transcranial resection of a large sinonasal juvenile psammomatoid ossifying fibroma.

IntroductionJuvenile psammomatoid ossifying fibroma (JPOF) is a benign but potentially locally aggressive fibroosseous lesion predominantly arising in the paranasal sinuses in children and young adults. Intracranial extension is rare but occurs sometimes. In such cases, tumor resection may often require the combination of neurosurgical and facial approaches. Histological diagnosis remains a challenge because the lesion can be easily mistaken for another fibroosseous lesion or for a meningioma.Case reportWe report the case of a 12-year-old boy with a JPOF arising from the right paranasal sinuses and extension towards the anterior skull base and the orbit. Despite the tumor had eroded through nasal septum, medial orbit wall, and right maxilla, it could be entirely removed performing an extended frontobasal approach via a bifrontoorbital craniotomy, obviating the need for a transfacial approach.ConclusionRadiologically and histologically, the lesion could be mistaken either for a meningioma or another type of ossifying fibroma. Histological aspects and alternative surgical approaches to these rare entities are discussed.

Intracranial teratomas in children: the role and timing of surgical removal

OBJECT In this study, the authors report their experience with the surgical treatment of intracranial teratomas with an emphasis on the indications for delayed resection after oncological treatment. METHODS The authors retrospectively reviewed the cases of 14 children with intracranial teratomas. The mean age at diagnosis was 10.5 years (range 2 days-18 years), and 11 patients were male. The final histological analysis revealed pure mature teratoma in 5 cases, mixed teratoma with germinoma in 3 cases, and nongerminomatous malignant germ cell tumor in 6 cases. Thirteen patients underwent tumor resection, and these patients were divided into 2 subgroups according to the timing of surgery. In Group A, 10 patients underwent resection as the primary treatment because no tumor markers were detected in 4 patients, a teratomatous component was revealed on biopsy sampling in 3 patients, and a large tumor volume in 3 patients. In Group B, 3 patients underwent removal of residual pure mature teratoma after oncological treatment. RESULTS Seven of the 8 patients (87.5%) with pure mature teratomas or with mixed teratoma and germinoma are currently alive (mean follow-up of 9 years); the eighth patient died of postoperative meningitis. Two of the 6 patients (33%) with mixed nongerminomatous malignant germ cell tumors died of tumor progression regardless of the timing of surgery. CONCLUSIONS The results of this study support the belief that microsurgical removal is the only effective treatment for intracranial teratomas. Surgery may be performed as the primary therapy when there is evidence of a noninvasive teratoma, and as a secondary therapy if there is only a partial response to neoadjuvant therapy or if progression is observed in mixed malignant germ cell tumors.

An Unusual Case of Ectopic Adrenocorticotropin Secretion

A 55-yr-old man presented with clinical features suggesting slowly evolving Cushing’s syndrome that was confirmed by an elevated level of 24 h urinary free cortisol: 341 g (N 50 g) and impaired plasma cortisol suppression (166 nmol/liter, 6 g/dl) after overnight 1 mg dexamethasone. Pituitary dependent Cushing’s disease was suggested by unsuppressed plasma ACTH levels with an obvious increase (6.1 to 12.2 pmol/liter, 30.5 to 61 pg/ml) after iv CRH injection, and in addition, significant response of cortisol and ACTH (5.6 to 20.5 pmol/liter, 28 to 102.5 pg/ml) after desmopressin injection. Magnetic resonance imaging (MRI) of the pituitary region showed a normal pituitary gland (Fig. 1A). On the right side of the sphenoidal sinus, an apparent 6-mm septal “polyp” was seen (Fig. 1). Inferior petrosal sinus sampling revealed a 4.4-central/peripheral ACTH gradient 2 min after CRH injection. Therefore, an occult pituitary ACTH-secreting microadenoma was suspected (1). Neurosurgery was performed by the transnasal and transeptal approach, the mucosal polyp was first removed, then the intact sellar floor was opened, but no adenoma was identified despite extensive pituitary dissection (2). Microscopical examination of sections of the “small sphenoidal polyp” revealed an endocrine tumor with sheets and nests of adenoma cells with basophilic cytoplasm and round nucleus. Immunostaining demonstrated an ACTH cell adenoma (Fig. 2) and normal tissue in the pituitary biopsies (3). Immediate postoperative corticotropic insufficiency was observed. One year later the patient had normal urinary free cortisol and circadian rhythm of plasma cortisol with a negative desmopressin test demonstrating remission of Cushing’s disease. Ectopic pituitary adenoma can develop from Rathke’s pouch remnants (4), more often in sphenoidal localization (5). This case demonstrates that a small ectopic pituitary adenoma, frankly separated from pituitary, can present the same hormonal regulation than pituitary corticotropic adenoma with even results of inferior petrosal sinus sampling consistent with a pituitary source of ACTH, a finding compatible with the venous drainage of sphenoidal sinus mucosa. Numerous publications have emphasized the pitfalls of pituitary MRI in ACTH-dependant