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Biomedical subjects

J J Moreau

Publications and source records attributed to J J Moreau.

At least 19 recordsLinked to original sources
PubMed2006

A report of a desmoplastic ganglioglioma in a 12-year-old girl with review of the literature.

Desmoplastic infantile ganglioglioma is a supratentorial tumor that typically occurs in infants below the age of 24 months. Rare tumors with the same radiological and histological characteristics have been described in older subjects. We report a case of desmoplastic ganglioglioma in a 12-year-old girl with a 13 years follow-up. The patient presented with an inaugural generalized seizure. CT scan demonstrated a large superficial parieto-occipital mass, attached to the dura with solid and cystic components. Surgical resection was macroscopically complete. No adjuvant treatment was given. Thirteen years after surgery, the patient is symptom free. Histological examination revealed a pleomorphic tumor involving the meningeal space and the cortex. Meningeal portion was made of neoplastic astrocytes enmeshed in a dense network of connective tissue. The cortical component showed abnormal neurons, tumoral astrocytes and small foci of poorly differentiated cells with rare mitoses. Our observation and the seven others found in the literature indicate that desmoplastic gangliogliomas can occur in children and even in young adults. Despite some worrisome radiological and histological features, these tumors should not be misdiagnosed as malignant glioma. Like infantile cases, non-infantile desmoplastic gangliogliomas seem to have a favorable prognosis without additional therapy, if a total surgical resection can be performed.

Age Factors↗
PubMed2004

Mutations within the programmed cell death 10 gene cause cerebral cavernous malformations.

Cerebral cavernous malformations (CCMs) are hamartomatous vascular malformations characterized by abnormally enlarged capillary cavities without intervening brain parenchyma. They cause seizures and cerebral hemorrhages, which can result in focal neurological deficits. Three CCM loci have been mapped, and loss-of-function mutations were identified in the KRIT1 (CCM1) and MGC4607 (CCM2) genes. We report herein the identification of PDCD10 (programmed cell death 10) as the CCM3 gene. The CCM3 locus has been previously mapped to 3q26-27 within a 22-cM interval that is bracketed by D3S1763 and D3S1262. We hypothesized that genomic deletions might occur at the CCM3 locus, as reported previously to occur at the CCM2 locus. Through high-density microsatellite genotyping of 20 families, we identified, in one family, null alleles that resulted from a deletion within a 4-Mb interval flanked by markers D3S3668 and D3S1614. This de novo deletion encompassed D3S1763, which strongly suggests that the CCM3 gene lies within a 970-kb region bracketed by D3S1763 and D3S1614. Six additional distinct deleterious mutations within PDCD10, one of the five known genes mapped within this interval, were identified in seven families. Three of these mutations were nonsense mutations, and two led to an aberrant splicing of exon 9, with a frameshift and a longer open reading frame within exon 10. The last of the six mutations led to an aberrant splicing of exon 5, without frameshift. Three of these mutations occurred de novo. All of them cosegregated with the disease in the families and were not observed in 200 control chromosomes. PDCD10, also called "TFAR15," had been initially identified through a screening for genes differentially expressed during the induction of apoptosis in the TF-1 premyeloid cell line. It is highly conserved in both vertebrates and invertebrates. Its implication in cerebral cavernous malformations strongly suggests that it is a new player in vascular morphogenesis and/or remodeling.

Apoptosis Regulatory Proteins↗
PubMed2004

Mutations within the MGC4607 gene cause cerebral cavernous malformations.

Cerebral cavernous malformations (CCM) are hamartomatous vascular malformations characterized by abnormally enlarged capillary cavities without intervening brain parenchyma. They cause seizures and focal neurological deficits due to cerebral hemorrhages. CCM loci have already been assigned to chromosomes 7q (CCM1), 7p (CCM2), and 3q (CCM3) and have been identified in 40%, 20%, and 40%, respectively, of families with CCM. Loss-of-function mutations have been identified in CCM1/KRIT1, the sole CCM gene identified to date. We report here the identification of MGC4607 as the CCM2 gene. We first reduced the size of the CCM2 interval from 22 cM to 7.5 cM by genetic linkage analysis. We then hypothesized that large deletions might be involved in the disorder, as already reported in other hamartomatous conditions, such as tuberous sclerosis or neurofibromatosis. We performed a high-density microsatellite genotyping of this 7.5-cM interval to search for putative null alleles in 30 unrelated families, and we identified, in 2 unrelated families, null alleles that were the result of deletions within a 350-kb interval flanked by markers D7S478 and D7S621. Additional microsatellite and single-nucleotide polymorphism genotyping showed that these two distinct deletions overlapped and that both of the two deleted the first exon of MGC4607, a known gene of unknown function. In both families, one of the two MGC4607 transcripts was not detected. We then identified eight additional point mutations within MGC4607 in eight of the remaining families. One of them led to the alteration of the initiation codon and five of them to a premature termination codon, including one nonsense, one frameshift, and three splice-site mutations. All these mutations cosegregated with the disease in the families and were not observed in 192 control chromosomes. MGC4607 is so far unrelated to any known gene family. Its implication in CCMs strongly suggests that it is a new player in vascular morphogenesis.

Female↗
PubMed2003

Endoscopic endonasal management of invasive skull base mucocele: report of two cases.

Two cases of massive erosive skull base sphenoid mucocele are reported. Even if intra-sphenoid mucoceles are an ENT pathology, when extensive they become of neurosurgical interest. An endoscopic minimally invasive procedure was performed in both cases with an excellent outcome. Patient 1 presented with several years of retro-orbital bilateral pain and several months of purulent pharyngeal leak. CT scan and MRI imaging showed an extensive erosive intra-sphenoid mass, with peripheral contrast enhancement. Patient 2 was referred because of several months of frontal headache and decreased left visual acuity. A wide intra-sphenoid mass was evident at CT scan and MRI. This mass included an anterior compartment, hyperintense on both T (1)- and T (2)-weighted images, and a posterior part, hypointense on T (1)- and hyperintense on T (2)-weighted images. A pure endoscopic endonasal one nostril pure procedure was performed in both cases. Endoscopic technique has been shown to be an easy and minimally invasive procedure. It should be the treatment of choice in case of invasive skull base mucocele in order to avoid blind damage of vasculo-nervous structures which are not protected by the bone, extensively eroded by the lesion.

Adult↗
PubMed2002

[Direct transnasal approach. Description and feasibility in 60 patients].

BACKGROUND AND PURPOSE: The direct transnasal approach is a less invasive approach to the sella turcica and represents an alternative to the sublabial transsphenoidal approach with minor oro-nasal complications. In this study, we describe this approach with emphasis on post-operative course. METHODS: We studied retrospectively 60 patients operated on consecutively by a transnasal approach between January 1996 and December 2000. RESULTS: There were 47 macroadenomas, 9 microadenomas and 4 non-adenomatous pathologies. Despite its narrowness, the transnasal approach was possible in all cases and allowed tumor removal as satisfactory as with the sublabial approach. Eight patients had complications not directly related to the approach: 1 death (meningitis with brain stem ischemia), 3 pituitary fossa hematomas, 4 rhinorrheas and 2 meningitis. Seven patients had complications related to the approach: 2 nasal obstructions, 1 nasal dryness, 2 sinusitises and 2 hyposmias. All these local complications were transitory and disappeared within 3 months. CONCLUSIONS: In our experience, the direct transnasal approach is safe, quick and less invasive than the sublabial approach.

Adenoma↗
PubMed2002

[Image-guided neuroendoscopy].

BACKGROUND AND PURPOSE: By definition, image-guided neuroendoscopy is a connection between a neuroendoscope and a neuronavigational system creating a computer-guided instrument. Our objective was to adapt our rigid endoscope with our neuronavigation system to perform computer-guided endoscopy. METHODS AND RESULTS: The rigid neuroendoscope, equipped with light emitting diodes, was connected to the work station. We report our early results with this device in 17 patients: 8 ventriculocisternostomies, 5 ventriculocisternostomies with biopsies of tumors of the posterior part of the third ventricule, 4 biopsies or tumor removal like colloid cysts. No technical complications were encountered. CONCLUSIONS: The development of image-guided neuroendoscopy has modified our approach to neuroendoscopy because the surgical procedure is facilitated. The use of fluoroscopy becomes unnecessary. The surgeon can choose, independent of the preestablish surgical technique, the entry point, the target and, of course, the optimal trajectory. The technique is adaptable to the individual anatomy of each patient, the location and the nature of the target. It is an undeniably useful teaching tool and represents a real progress in minimally invasive neurosurgery.

Adult↗
PubMed2001

Trans-sinusal frontal approach for olfactory groove meningiomas.

The authors report on their experience with the trans-sinusal frontal approach in removing olfactory groove meningiomas. Six tumors were operated on by the trans-sinusal frontal approach, using a bicoronal incision; two tumors developed on one side, and there were four bilateral olfactosellar tumors. Osteotomy of the anterior wall of the frontal sinus was performed with an oscillating saw without any burr hole. The posterior wall of the sinus was resected and the tumor was attacked through a real subfrontal route along the plane of the anterior skull base. Ethmoidal blood supply was controlled at the initial stages of the operation, allowing avascular tumor debulking. Olfactory nerves, invaded by the tumor, were removed along with the tumor. Tumor extensions toward the sella and the optic canals were removed without brain retraction, opening of the Sylvian fissure, or dissection of the carotid arteries. All patients made a good neurologic recovery; intellectual impairment disappeared within 1 month, and visual acuity normalized within 2 weeks. Olfactory nets were preserved on the contralateral side in unilateral tumors. The trans-sinusal frontal approach is technically easy and safe to achieve. Osteotomy and replacement of the anterior wall of the frontal sinus are rapidly performed. When the frontal sinus is small, imageguided surgery allows precise deliniation of its limits and the free bone flap, including the calvarial outer layer, tangentially cut from one supraorbital canal to the other. The cosmetic result is perfect, as the normal contour of the forehead is maintained without any scar or visible burr hole. The trans-sinusal frontal approach gives access to the orbital roofs and to the central anterior skull base from the crista galli to the tuberculum sellae and the anterior clinoid processes. The trans-sinusal frontal approach represents an alternative to conventional craniotomies for tumors developed in the central anterior skull base, especially for olfactory groove meningiomas, whatever their size.

Journal Article↗
PubMed2001

Intrathecal baclofen after traumatic brain injury: early treatment using a new technique to prevent spasticity.

BACKGROUND: In the early course of severe head trauma, the clinical value of intrathecal administration of baclofen to reduce autonomic disorders and spasticity has not been established. METHODS: We studied four patients (Glasgow Coma Scale score 3 or 4) with autonomic disorders and spasticity who failed to respond to conventional treatment during the early course of head injury. Baclofen (25 microg/mL) was infused continuously through an intrathecal catheter inserted at patient bedside and subcutaneously tunneled. When this treatment was successful, the spinal catheter was removed and surgically replaced by another catheter connected to a subcutaneous pump. Clinical follow-up was obtained at 6 months after the head injury. RESULTS: Mean delay for the initiation of intrathecal baclofen was 25 days (range, 21 to 31 days), and optimal dose was 385 +/- 185 microg/day. In all patients, the Ashworth score was consistently reduced (3.5 +/- 0.5 vs. 4.5 +/- 0.5 for upper limbs and 2 +/- 0.5 vs. 4.5 +/- 0.5 for lower limbs), as were both the frequency and intensity of autonomic disorders. The spinal catheters were used during a mean period of 9.5 +/- 1.7 days without complications. All three survivors were equipped with a programmable pump and had a lower Ashworth score at 6 months. Autonomic disorders had disappeared in two patients and remained modest in the remaining patient. CONCLUSION: Continuous administration of baclofen via the intrathecal route using this new technique seems to reduce autonomic disorders and spasticity during the early course of severe traumatic head injury.

Adult↗
PubMed2001

[Epidural non-Hodgkin's lymphoma presenting as a spinal cord compression].

OBJECTIVE AND METHODS: epidural localization is a rare presenting sign of non Hodgkin's lymphoma. In this study, we describe the clinical, histological and immunohistochemical data in 13 cases (9 men and 4 women) of non-Hodgkin's lymphoma with epidural involvement. RESULTS: the median age was 63 years (range 36-76 years). Clinically, most patients complained of back pain (median duration, 3.4 months) followed by acute neurological deterioration. In 10 out of 13 tumors, a thoracic localization was observed and a decompressive laminectomy was performed in all cases. Histology and immunohistochemistry showed all tumors to be B-cell lymphomas which were classified as: lymphocytic lymphoma (3 cases), prolymphocytic lymphoma (1 case), follicular lymphoma (2 cases), diffuse large cell lymphoma (6 cases) and AIDS associated Burkitt lymphoma (1 case). Post-operative staging revealed bone and/or paravertebral involvement in 11 cases and disseminated disease in 5 cases. Median survival following complementary therapy, radiotherapy and chemotherapy in most cases, was 20 months. CONCLUSION: histological features of epidural lymphomas are similar to other extranodal lymphomas and their prognosis is better than other epidural neoplasms, especially when the tumor is localized.

Adult↗
PubMed2001

[Adenoid cystic carcinomas invading the skull base. Apropos of 4 cases and review of the literature].

Head and neck adenoid cystic carcinomas may invade the adjacent skull base by bone lysis and/or by perinervous and perivascular spread within the skull base foramina. Neurosurgical decision making is not well defined regarding the extent of intracranial tumor component removal, as neurosurgical expertise is limited for this peculiar type of tumors. The issue is to decide whether a radical supposedly locally curative surgery should be attempted, or if a large non disfigurating surgery is mandatory, keeping in mind the frequency of local recurrences and of distant metastases. Over a 13-year period, four adenoid cystic carcinomas invading the skull base were operated on at our institution: two tumors originated in the parotid gland, one in the sphenoid sinus, and one in the ethmoid sinus. Surgical removal was total in one case, subtotal in three cases. Post-operative irradiation was delivered in the four patients (two neutron irradiation, two conventional). One patient with advanced metastatic disease was submitted to chemotherapy. Three patients died from local tumor progression and distant metastases within three years after the intracranial tumor extension has been diagnosed. The patient with an ethmoid tumor is still alive seven years after surgery without any evidence of local tumor progression nor distant metastases. Surgery remains the gold standard treatment for adenoid cystic carcinomas invading the skull base. However, in our opinion a large tumor removal, without or with bone osteotomies, but without sacrifice of cranial nerves, cavernous sinus, internal carotid artery, and of the orbit allows patient survival with an acceptable comfort and absence of psychological distress due to disfigurating surgery nor surgically induced neurological functional deficit. Post-operative irradiation may sometimes stabilize locally the lesions. The place of chemotherapy has, yet, to be determined.

Adult↗
PubMed2000

Immobilization of rhodium complexes in chiral organic-inorganic hybrid materials.

Two new alkoxysilylated derivatives of (-)-(1R,2R)-1, 2-diaminocyclohexane: M = N-[(triethoxysilyl)propyl]-(-)-(1R,2R)-1, 2-diaminocyclohexane and B = N, N'-bis[(triethoxysilyl)propyl]-(-)-(1R,2R)-1,2-diaminocyclohexane have been synthesized. Their complexation with [Rh(cod)Cl]2 in the presence of TEOS = Si(OEt)4, followed by sol-gel hydrolysis-condensation, afforded new catalytic chiral hybrid materials. Evidence for the presence of the organic moieties complexed by rhodium in these solids was obtained by UV-visible spectroscopy, FT-IR studies, solid state 13C and 29Si CP-MAS NMR analysis, energy-dispersive X-ray (EDX) techniques, and elemental analysis. The nitrogen sorption studies and BET analyses ranged these solid gels from nonporous to highly porous materials. The catalytic activities and selectivities of the solid materials have been studied in the asymmetric hydrogen-transfer reduction of prochiral ketones and compared to that of the homogeneous rhodium complexes of the ligands M and B. The hybrid materials appeared interesting supports for enantioselective heterogeneous catalysis leading to chiral alcohols with ee up to 58% in the reduction of acetophenone and up to 98% in the case of the more hindered related ketones. The catalytic properties as a function of the nature of chiral hybrid solid are discussed.

Catalysis↗
PubMed1999

[Postero-lateral surgical approach to soft cervical herniated discs. Technical note].

Most ruptured cervical discs are operated on by an anterolateral approach. The posterior approach is an older procedure, nowadays underused because it was associated with a high morbidity, especially in terms of medullary complications. However, posterior approach has evolved in posterolateral route, which is not so devastating and has a very low morbidity rate. It gives excellent functional results when surgery is dedicated to monoradiculopathy from soft posterolateral cervical hernias. It seems appropriate to consider this surgical route as an alternative to anterolateral surgery in these very selected cases. The authors describe and comment the technique.

Cervical Vertebrae↗
PubMed1999

[Frontal sinus approach to olfactory groove meningiomas].

We report on our experience of the frontal sinus approach for removing olfactory groove meningiomas. Five tumors were operated on, one unilateral, four bilateral. Osteotomy of the anterior wall of the frontal sinus was performed with an oscillating saw without any burr hole. The posterior wall of the sinus was resected and the tumor was attacked along the plane of the anterior skull base. Ethmoidal blood supply was controlled at the initial stage of the operation, allowing avascular tumor debulking. Olfactory nerves, invaded by the tumor, usually cannot be spared. Tumor extensions towards the sella and the optic canals were removed without any brain retraction, nor opening of the sylvian fissure, nor dissection of the carotid arteries. The frontal sinus approach is technically easy to achieve. Osteotomy and reconstruction of the anterior wall of the frontal sinus are rapidly performed. When the frontal sinus is small, image guided surgery allows to delineate precisely its limits and the flap includes the calvarial outer layer, tangenitally cut from one supra-orbital canal to the other. Cosmetic result is perfect. The frontal sinus approach gives access to the medial part of the orbital roofs and to the central anterior skull base from the crista galli to the tuberculum sellae and the anterior clinoids. The frontal sinus approach represents an alternative to conventional craniotomies for tumors developed in the central anterior skull base, especially for olfactory groove meningiomas.

Frontal Sinus↗
PubMed1998

Renal lesions in Von Hippel-Lindau disease: the benign, the malignant, the unknown.

OBJECTIVES: To describe the features and treatment of renal lesions in von Hippel-Lindau disease (VHL) from a series of patients, to highlight important issues in the management of these lesions. MATERIALS AND METHODS: We performed a retrospective study in 7 patients with VHL who underwent surgery for renal lesions between January 1990 and July 1996. The initial evaluation consisted of an abdominal CT scan and renal arteriography. RESULTS: The mean age of patients at the time of discovery was 38 (+/- 12.01 years). All cases were of type 1. Radiology assessment underestimated the gravity of the lesions in 5 patients. In all patients, unilateral surgery was performed with the kidney being conserved in 4 cases. Of the 44 lesions removed, 23 had signs of progressiveness (atypical or malignant cyst, renal cell carcinoma), most of the cancers were of low grade. 84% of the lesions did not exceed 3 cm and 48% were </=1 cm. During follow-up (42 months), 2 patients relapsed. CONCLUSION: The clinical and pathological features of renal lesions in VHL require strict and early follow-up (from the age of 15). Only under these circumstances should primary conservative surgery be performed, however, recurrences are frequent. This conservative approach remains to be validated by multicentric prospective studies.

Adult↗
PubMed1997

[Adenocarcinoma of the ethmoid sinus in woodworkers. Retrospective study of 25 cases].

A retrospective oncological study was performed in 25 woodworkers, in whom an adenocarcinoma of the ethmoid sinuses was discovered between March 1985 and December 1993. All patients were males with a mean age of 57 years, and a mean duration of wood dust exposure of 24 years. Signs of nasal obstruction, drainage, and discomfort were present in all cases. Ophthalmological findings were a poor prognosis indicator. It was possible to precisely evaluate treatment and outcome in 23 cases. The majority of tumors were classified as T3 or T4 (72%), with extension beyond the ethmoid sinuses; all were in contact with the roof of the ethmoidal sinuses. Extension was predominantly into the orbital and intracranial cavities as compared with extension posteriorly or into the maxillary sinuses. Treatment was identical in the 25 patients: a) combined surgery including a paranasal and a neurosurgical approach, b) postoperative radiotherapy. Results were expressed in terms of morbidity related to surgery and the oncologic outcome. Operative morbidity and mortality were substantially reduced with reconstruction of the roof of the ethmoidal sinuses. Meticulous excision, in addition to postoperative radiotherapy, resulted in a decreased rate of local recurrence (26%). On the other hand, metastasis were encountered more frequently (30%). Radiotherapy was insufficient when macroscopic excision was incomplete. Chemotherapy was used as palliative treatment in the event of a recurrence and/or metastases. Survival rate was 68% at 3 years, and 48% at 5 years. Most complications and recurrences arose within the first two years. Exophthalmos, intracranial extension, incompleteremoval, and extensive class T4 tumors were associated with a poor prognosis. Optimal therapy for malignant tumors of the ethmoid sinuses requires combined transfacial and neurosurgical approaches that allow precise assessment of tumor extension and adequate excision, yielding an improved oncologic outcome. Followed by radiotherapy, this association can result in a remission. Patient prognosis depends essentially on management of the initial lesion.

Adenocarcinoma↗
PubMed1996

Synovial cyst at the C1-C2 junction and spondyloarthropathy.

A spinal synovial cyst is a rare extradural benign tumor generally located at the lumbar spine, arising at the facet joint capsule, and usually associated with degenerative changes. We describe a 64-year-old woman with a synovial cyst involving the quadrate ligament of the odontoid process, which caused neurologic signs. Her family history was positive for spondyloarthropathy. The radiologic investigations, preoperative differential diagnosis, and association of spondyloarthropathy with this rare benign foramen magnum tumor are of particular interest.

Cervical Vertebrae↗