François Proust | Université de Strasbourg (original) (raw)
Papers by François Proust
Stereotactic and Functional Neurosurgery
In our stereotactic procedure, intraoperative radiological documentation of electrode localizatio... more In our stereotactic procedure, intraoperative radiological documentation of electrode localization is performed using the Stereoplan. The aim of the study was to evaluate its accuracy. Data of 20 consecutive patients, treated for Parkinson's disease by implantation of deep brain stimulators into the subthalamic nucleus, were collected prospectively. During surgery, Stereoplan coordinates of the tip of the central macroelectrodes were compared with intended coordinates along the central trajectory at 4 levels: (1) 10 mm above the anatomical target, (2) in the target, (3) in the substantia nigra pars reticulata, and (4) at the depth of contact 1. Before the frame's removal, Stereoplan coordinates of the contacts of the definitive electrode were compared with postoperative MRI coordinates. The mean of the differences was calculated in the x-, y-, and z-axis. Clinical results at 6 months were recorded. The mean of the differences between Stereoplan coordinates and intended coord...
Annales de pathologie, 2014
We report on a 51-year-old woman who presented with a cervical spinal cord tumor clinically suspe... more We report on a 51-year-old woman who presented with a cervical spinal cord tumor clinically suspected to be a metastasis. Histological examination revealed an anaplastic meningioma containing epithelial nests arranged in a gland-like pattern suggestive of adenocarcinoma. This component strongly expressed cytokeratins whereas the meningothelial component was vimentin--epithelial membrane antigen--and progesterone receptor-immunoreactive, suggesting either anaplastic meningioma with adenocarcinoma-like metaplasia, or adenocarcinoma metastasis in a meningioma, but the search for a primitive neoplasia including thoracic-abdominal-pelvic computed tomography and mammography was negative. Anaplastic meningiomas with adenocarcinoma-like metaplasia are uncommon lesions, 4 cases having been reported in the literature so far. Their immunohistochemical and chromosomal characteristics are similar to those observed in secretory meningiomas. When available, fluorescence in situ hybridization detec...
Journal of neuroradiology. Journal de neuroradiologie, 2012
This report is of a case of gemistocytic astrocytoma investigated by perfusion-weighted magnetic ... more This report is of a case of gemistocytic astrocytoma investigated by perfusion-weighted magnetic resonance (MR) imaging associated with proton MR spectroscopy. The conventional MR imaging presentation of gemistocytic astrocytoma is similar to that of HGG, metastasis or lymphoma. Nevertheless, a precise diagnosis is crucial as the prognosis and therapy of these diseases are different. Quantitative MR imaging sequences, which provide metabolic and vascular information, could be useful in the prevention of misdiagnosis.
Journal of Neurosurgery: Pediatrics, 2012
Intracranial bleeding following stereoelectroencephalography (sEEG) is rare and commonly occurs e... more Intracranial bleeding following stereoelectroencephalography (sEEG) is rare and commonly occurs early after electrode implantation. The authors report the case of a delayed intracranial hematoma following sEEG. This 10-year-old boy was referred to the authors' department to undergo an sEEG study for intractable epilepsy, with the hypothesis of a single localized epileptic zone in the left precentral region. To perform the exploration, 14 depth electrodes were implanted under stereotactic conditions. The results of a postoperative CT scan performed routinely at the end of the surgical procedure were normal. Eight days later, following an epileptic seizure, the child's condition worsened. The neurological examination revealed a left hemiparesis, agitation, and coma due to a right subdural hematoma with intraparenchymal bleeding. Despite a surgical evacuation followed by a decompressive craniectomy, the curative treatments were stopped 1 week later due to severe diffuse ischemic lesions found on MRI studies. This is the first observation of a delayed hematoma following an sEEG procedure. The mechanism underlying this complication remains unclear, but the rupture of a growing pseudoaneurysm caused by the electrode's implantation or the tearing of a neighboring vessel by an electrode were suspected. In consequence, physicians must remain vigilant during the entire sEEG recording period and probably also several days after electrode removal.
La Presse Médicale, 2007
ABSTRACT L'incidence de l'hémorragie sous-arachnoïdienne anévrismale est 10,5 cas... more ABSTRACT L'incidence de l'hémorragie sous-arachnoïdienne anévrismale est 10,5 cas/100 000 sujets/an. Il faut exclure précocement le sac anévrismal pour éviter le resaignement toujours dramatique. Une présentation sur dossier en staff de neurovasculaire pour décider de la meilleure procédure d'exclusion anévrismale est souhaitable. La morbidité procédurale est de 8 % après coïling endovasculaire, 10 % après clipping microchirurgical. L'exclusion du sac est complète chez 60 % des patients après coïling endovasculaire et chez 95 % après clipping microchirurgical. Les mesures préventives contre l'ischémie cérébrale comportent remplissage vasculaire et utilisation d'un inhibiteur calcique. L'hospitalisation en milieu spécialisé, des mesures prophylactiques contre la maladie thromboembolique, la douleur et l'épilepsie sont indispensables. Il faut mettre en place de mesures curatives vis-à-vis d'un hématome, d'une hydrocéphalie ou d'une ischémie retardée.
Trends in Neurovascular Interventions, 2014
Various mini-invasive approaches have been developed over the last decade to expose the suprasell... more Various mini-invasive approaches have been developed over the last decade to expose the suprasellar area. The supraorbital approach takes a predominant place in exposing the suprasellar area and the Sylvian fissure. Based on our surgical experience, the technique of supraorbital subfrontal approach is described in detail in this article. After an eyebrow incision, a small frontal craniotomy was performed. Indications, advantages and limitations: This mini-invasive approach was indicated for patients with unruptured aneurysm, in patients with aneurysmal SAH without intracranial hypertension, and especially in elderly patients. This minicraniotomy (1) gave quick and direct access to the aneurysm; (2) provided less trauma of the temporal muscle and improved the cosmetic resuilts; and (3) reduced the risk of postoperative epidural hematoma thanks to the small detachment of the dura mater from the vault. We concluded that this limited supraorbital approach gave adequate visualization and allows surgical manipulation within eloquent structures and can be specifically applied in absence of intracranial hypertension.
World Neurosurgery, 2012
Inflammatory pseudotumors are ubiquitous lesions characterized by a polymorphous inflammatory inf... more Inflammatory pseudotumors are ubiquitous lesions characterized by a polymorphous inflammatory infiltrate containing plasma cells and lymphocytes. In the central nervous system, this pathological condition is rare and the association with Crohn's disease has never been described. A 31-year-old woman with a history of Crohn's disease was referred to our department for progressive headaches and nausea. Neurological examination was normal. Magnetic resonance imaging showed an irregular heterogeneous enhanced mass infiltrating the left cerebellar hemisphere. Total resection was performed and pathological examination led to the conclusion of an inflammatory pseudotumor. To our knowledge, this case is the first describing an intra-cerebral inflammatory pseudotumor associated with an inflammatory bowel disease. The diagnosis of an extradigestive location of Crohn's disease was excluded by pathological examination. Although the precise cause of this association remains unknown, it could be hypothesized that the intra-cranial lesion could be the result of the immunosuppressive therapy given for Crohn's disease, or, more likely, could be a part of a systemic dysimmune process.
Surgical Neurology, 2004
The authors describe 2 cases of dysplasic gangliocytoma of the cerebellum or Lhermitte-Duclos dis... more The authors describe 2 cases of dysplasic gangliocytoma of the cerebellum or Lhermitte-Duclos disease revealing Cowden disease or multiple hamartoma neoplasia syndrome. Cowden disease is a rare autosomal dominant disorder, now considered as a phakomatosis. Nevertheless, relationships between both conditions still remain unclear, since Lhermitte-Duclos disease can also be sporadic. Two patients, 25 and 27 years old, were admitted to the emergency department for an acute intracranial hypertension. In both cases, magnetic resonance imaging (MRI) scan showed a mass in the cerebellar hemisphere. Pathological examination of surgical resection specimens concluded Lhermitte-Duclos disease. Because of the patients' previous personal and familial medical history, Cowden disease was suspected and confirmed by mutational analysis of the phosphatase and tensin homolog (PTEN) gene. In the first case, a nonsense punctual mutation in exon 8 was found. In the second one, a mutation was revealed in the exon 5, a mutational hot spot encoding the phosphatase catalytic core motif. Lhermitte-Duclos disease and Cowden disease can be associated. Germline mutations of PTEN gene are known to be implicated in Cowden disease. This gene, located at chromosome 10q23-3, is a tumor suppressor gene that encodes a protein with phosphatase activity. To date, more than 80 mutations have been reported in Cowden disease. When the diagnosis of either one of these two disorders is established, it is imperative to search for the other one to detect early malignant lesions that occur in Cowden disease. Finally, a long-term follow up of the patient is required and a thorough familial screening is necessary.
Surgical Neurology, 2006
Primary diffuse leptomeningeal gliomatosis is an exceptional neoplasm, and only 30 cases have bee... more Primary diffuse leptomeningeal gliomatosis is an exceptional neoplasm, and only 30 cases have been reported in the literature. We report a recent case and compare data with previously published observations. A 50-year-old man was admitted to the neurosurgery department for a previous 4-month history of headache, associated with nonspecific neurological signs. Biologic data and cerebrospinal fluid examination suggested an inflammatory process. The patient was given an antituberculous therapy. Magnetic resonance imaging revealed a multinodular enhancement of spinal nerve roots. A biopsy of sacral rootlets was performed. Histological examination revealed an anaplastic astrocytoma. Patient's status worsened, and death occurred 7 months later. Complete neuraxis postmortem examination revealed no intraparenchymatous glioma and was conclusive for the diagnosis of primary leptomeningeal gliomatosis (astrocytic, World Health Organization grade III), with a multinodular pattern in the spinal cord, the brainstem, and the brain base with diffuse extension into the cerebellar subarachnoid spaces. Our case illustrates the diagnostic difficulties in making the premortem diagnosis. The review of the literature indicates that there are no specific clinical or biologic signs. Magnetic resonance imaging using T1-weighted images with gadolinium enhancement and biopsy material may be useful diagnostic tools. In most cases, autopsy evaluation alone permits definitive primary diffuse leptomeningeal gliomatosis diagnosis. Whatever the histological characteristics of proliferating cells are, the prognosis remains poor. No prognostic factors have been shown to be correlated with survival time. Unfortunately, no routine treatment has been yet proposed.
Seminars in Neurology, 1998
Subarachnoid hemorrhage is a formidable and common health care problem. Early diagnosis and manag... more Subarachnoid hemorrhage is a formidable and common health care problem. Early diagnosis and management are crucial to reduce the morbidity form this complex and multifaceted disease. Open surgery and endovascular techniques both aim at eliminating the source of hemorrhage. The choice of therapy can be made rationally based on an understanding of the merits, risks, and limitations of each therapy. The care of pregnant patients with subarachnoid hemorrhage and patients who harbor both aneurysms and AVMs can be approached rationally with an understanding of the complex pathophysiology behind these clinical scenarios. Familiarity with the signs of mild SAH, and advances in familial screening, noninvasive imaging, and therapies for vasospasm will continue to lessen the toll of this dramatic illness on the public well-being.
Parkinsonism & Related Disorders, 2011
Journal of Neurosurgery, 2009
For anterior communicating artery (ACoA) aneurysms, endovascular coil embolization constitutes a ... more For anterior communicating artery (ACoA) aneurysms, endovascular coil embolization constitutes a safe alternative therapeutic procedure to microsurgical clip occlusion. The authors' aim in this study was to evaluate the quality of life (QOL), cognitive function, and brain structure damage after the treatment of ruptured ACoA aneurysms in a group of patients who underwent microsurgical clipping (36 patients) compared with a reference group who underwent endovascular coiling (14 patients). At 14 months posttreatment all patients underwent evaluations by independent observers. These observers evaluated global efficacy, executive functions using a frontal assessment battery of tests (Trail making test, Stroop tasks, dual task of Baddeley, verbal fluency, and Wisconsin Card Sorting test), behavior dysexecutive syndrome (the Inventaire du Syndrome Dysexécutif Comportemental questionnaire [ISDC]), and QOL by using the Reintegration To Normal Living Index. Brain damage was analyzed using MR imaging. In the microsurgical clipping and endovascular coiling groups, the distribution on the modified Rankin Scale (p = 0.19) and mean QOL score (85.4 vs 83.4, respectively) were similar. Moreover, the proportion of executive dysfunctions (19.4 vs 28.6%, respectively) and the mean score on the ISDC questionnaire (8.9 vs 8.5, respectively) were not significant, but verbal memory was more altered in the microsurgical clipping group (p = 0.055). Magnetic resonance imaging revealed that the incidence of local encephalomalacia and the median number of lesions per patient increased significantly in the microsurgical clipping group (p = 0.003). In the 2 groups, no significant difference was observed regarding QOL, executive functions, and behavior. Despite the significant decrease in verbal memory after microsurgical clipping, the interdisciplinary approach remains a safe and useful strategy.
Journal of Neurosurgery, 2002
Cavernous internal carotid artery (ICA)-anterior cerebral artery (ACA) anastomoses are unusual an... more Cavernous internal carotid artery (ICA)-anterior cerebral artery (ACA) anastomoses are unusual anomalies in which a duplicated A, segment of the ACA arises from the infraoptic ICA. The authors report on a 30-year-old woman who presented with subarachnoid hemorrhage from an anterior communicating artery (ACoA) aneurysm associated with an extremely rare variant of this anastomosis. The extra A, segment emerged from the ICA within the cavernous sinus rather than at or above the level of the ophthalmic artery. The presence of the anomalous vessel provided a straightforward endovascular approach to the ACoA and allowed the use of coil placement rather than surgical clipping to treat the aneurysm successfully.
Atherosclerosis, 2010
Because an underlying arteriopathy might contribute to the development of intracranial aneurysms ... more Because an underlying arteriopathy might contribute to the development of intracranial aneurysms (IAs), we assessed the elastic properties of proximal conduit arteries in patients with IA. In 27 patients with previous ruptured IA and 27 control subjects matched for age, gender and BMI, we determined arterial pressure, internal diameter, intima-media thickness (IMT), circumferential wall stress (CWS) and elastic modulus (wall stiffness) in common carotid arteries using applanation tonometry and echotracking. Moreover, carotid augmentation index (AIx, arterial wave reflections) and carotid-to-femoral pulse wave velocity (PWV, aortic stiffness) were assessed. Compared with controls, patients with IA exhibited higher brachial and carotid systolic and diastolic blood pressures, with similar brachial but higher carotid artery pulse pressure (35 + or - 6mm Hg vs. 41 + or - 8mm Hg, P=0.014). Moreover, patients have higher PWV (7.8 + or - 1.2ms(-1) vs. 8.3 + or - 1.1ms(-1), P=0.048) and AIx (15.8 + or - 10.8% vs. 21.1 + or - 8.5%, P<0.001) which contributes to increase carotid blood pressures. Furthermore, carotid IMT was higher in patients (546 + or - 64 microm vs. 642 + or - 70 microm, P<0.001) without difference in diameter suggesting an adaptive hypertrophy. However, patients display a lower CWS (61.6 + or - 9.2 kPa vs. 56.9 + or - 10.3 kPa, P=0.007) and no correlation between IMT and pulse pressure (r=0.152, P=NS) in contrast to controls (r=0.539, P<0.001) showing the contribution of a pressure-independent process. Finally, despite this lesser CWS, elastic modulus was increased in patients (310 + or - 105 kPa vs. 383 + or - 174 kPa, P=0.026). This study demonstrates that patients with IA display a particular carotid artery phenotype with an exaggerated hypertrophic remodeling and altered elastic properties. Thus, a systemic arteriopathy might contribute, together with the arterial wall fatiguing effect of the increased pulsatile stress, to the pathogenesis of IA.
Annales Françaises d'Anesthésie et de Réanimation, 2007
Acta Obstetricia et Gynecologica Scandinavica, 2004
Cerebral aneurysmal complications rarely occur during pregnancy. Telling the difference between e... more Cerebral aneurysmal complications rarely occur during pregnancy. Telling the difference between eclampsia and cerebral hemorrhage due to aneurysmal rupture can prove to be difficult. Aneurysmal management should be performed in an emergency but fetal prognosis should be considered. We report a series of eight pregnant women presenting aneurysmal complications and we have assessed their management and outcome. Both maternal and perinatal mortality rates were correlated with the maternal clinical score. We stress the role of combined care by both neurosurgeons and obstetricians. An emergency cesarean section followed by aneurysmal treatment appears to be a widely accepted strategy in pregnant women with cerebral aneurysmal complications.
Surgical Neurology, 2008
Prevalence of cerebral cavernomas in the general population is close to 0.5%. In contrast, SCCs a... more Prevalence of cerebral cavernomas in the general population is close to 0.5%. In contrast, SCCs are rare. The aim of this study was to determine the outcome of SCC in a large sample of patients. Clinical and neuroradiologic findings were retrospectively collected in a multicentric study. Diagnosis was based on pathologic criteria or MR findings. Fifty-three patients were included (26 males, 27 females). Mean age at onset of symptoms was 40.2 years (11-80 years). Initial symptoms were progressive (32) or acute myelopathy (20). One case was asymptomatic. Triggering factors were found in 14 of the patients (26%). Clinical symptoms were related to spinal cord compression (27) and hemorrhage (22). Spinal cord cavernoma was thoracic in 41 cases and cervical in 12. Mean size of the lesions was 16.3 mm (3-54 mm). In the 40 surgical patients, long-term follow-up was available in 37 cases for a mean time of 7.3 years (0.4-50 years). During the follow-up period, 20 patients improved, 6 remained on their preoperative baseline, and 11 got worse. Surgical improvement was more often found in posterior rather than anterior location. Using McCormick classification, 22 patients were autonomous (grades 1-2), 12 handicapped (grade 3), and 3 bedridden (grade 4) at the end of the follow-up. This study has defined clinical and MR patterns of spinal cavernomas. Surgery lastingly improved more than half of the patients.
Stereotactic and Functional Neurosurgery
In our stereotactic procedure, intraoperative radiological documentation of electrode localizatio... more In our stereotactic procedure, intraoperative radiological documentation of electrode localization is performed using the Stereoplan. The aim of the study was to evaluate its accuracy. Data of 20 consecutive patients, treated for Parkinson's disease by implantation of deep brain stimulators into the subthalamic nucleus, were collected prospectively. During surgery, Stereoplan coordinates of the tip of the central macroelectrodes were compared with intended coordinates along the central trajectory at 4 levels: (1) 10 mm above the anatomical target, (2) in the target, (3) in the substantia nigra pars reticulata, and (4) at the depth of contact 1. Before the frame's removal, Stereoplan coordinates of the contacts of the definitive electrode were compared with postoperative MRI coordinates. The mean of the differences was calculated in the x-, y-, and z-axis. Clinical results at 6 months were recorded. The mean of the differences between Stereoplan coordinates and intended coord...
Annales de pathologie, 2014
We report on a 51-year-old woman who presented with a cervical spinal cord tumor clinically suspe... more We report on a 51-year-old woman who presented with a cervical spinal cord tumor clinically suspected to be a metastasis. Histological examination revealed an anaplastic meningioma containing epithelial nests arranged in a gland-like pattern suggestive of adenocarcinoma. This component strongly expressed cytokeratins whereas the meningothelial component was vimentin--epithelial membrane antigen--and progesterone receptor-immunoreactive, suggesting either anaplastic meningioma with adenocarcinoma-like metaplasia, or adenocarcinoma metastasis in a meningioma, but the search for a primitive neoplasia including thoracic-abdominal-pelvic computed tomography and mammography was negative. Anaplastic meningiomas with adenocarcinoma-like metaplasia are uncommon lesions, 4 cases having been reported in the literature so far. Their immunohistochemical and chromosomal characteristics are similar to those observed in secretory meningiomas. When available, fluorescence in situ hybridization detec...
Journal of neuroradiology. Journal de neuroradiologie, 2012
This report is of a case of gemistocytic astrocytoma investigated by perfusion-weighted magnetic ... more This report is of a case of gemistocytic astrocytoma investigated by perfusion-weighted magnetic resonance (MR) imaging associated with proton MR spectroscopy. The conventional MR imaging presentation of gemistocytic astrocytoma is similar to that of HGG, metastasis or lymphoma. Nevertheless, a precise diagnosis is crucial as the prognosis and therapy of these diseases are different. Quantitative MR imaging sequences, which provide metabolic and vascular information, could be useful in the prevention of misdiagnosis.
Journal of Neurosurgery: Pediatrics, 2012
Intracranial bleeding following stereoelectroencephalography (sEEG) is rare and commonly occurs e... more Intracranial bleeding following stereoelectroencephalography (sEEG) is rare and commonly occurs early after electrode implantation. The authors report the case of a delayed intracranial hematoma following sEEG. This 10-year-old boy was referred to the authors' department to undergo an sEEG study for intractable epilepsy, with the hypothesis of a single localized epileptic zone in the left precentral region. To perform the exploration, 14 depth electrodes were implanted under stereotactic conditions. The results of a postoperative CT scan performed routinely at the end of the surgical procedure were normal. Eight days later, following an epileptic seizure, the child's condition worsened. The neurological examination revealed a left hemiparesis, agitation, and coma due to a right subdural hematoma with intraparenchymal bleeding. Despite a surgical evacuation followed by a decompressive craniectomy, the curative treatments were stopped 1 week later due to severe diffuse ischemic lesions found on MRI studies. This is the first observation of a delayed hematoma following an sEEG procedure. The mechanism underlying this complication remains unclear, but the rupture of a growing pseudoaneurysm caused by the electrode's implantation or the tearing of a neighboring vessel by an electrode were suspected. In consequence, physicians must remain vigilant during the entire sEEG recording period and probably also several days after electrode removal.
La Presse Médicale, 2007
ABSTRACT L'incidence de l'hémorragie sous-arachnoïdienne anévrismale est 10,5 cas... more ABSTRACT L'incidence de l'hémorragie sous-arachnoïdienne anévrismale est 10,5 cas/100 000 sujets/an. Il faut exclure précocement le sac anévrismal pour éviter le resaignement toujours dramatique. Une présentation sur dossier en staff de neurovasculaire pour décider de la meilleure procédure d'exclusion anévrismale est souhaitable. La morbidité procédurale est de 8 % après coïling endovasculaire, 10 % après clipping microchirurgical. L'exclusion du sac est complète chez 60 % des patients après coïling endovasculaire et chez 95 % après clipping microchirurgical. Les mesures préventives contre l'ischémie cérébrale comportent remplissage vasculaire et utilisation d'un inhibiteur calcique. L'hospitalisation en milieu spécialisé, des mesures prophylactiques contre la maladie thromboembolique, la douleur et l'épilepsie sont indispensables. Il faut mettre en place de mesures curatives vis-à-vis d'un hématome, d'une hydrocéphalie ou d'une ischémie retardée.
Trends in Neurovascular Interventions, 2014
Various mini-invasive approaches have been developed over the last decade to expose the suprasell... more Various mini-invasive approaches have been developed over the last decade to expose the suprasellar area. The supraorbital approach takes a predominant place in exposing the suprasellar area and the Sylvian fissure. Based on our surgical experience, the technique of supraorbital subfrontal approach is described in detail in this article. After an eyebrow incision, a small frontal craniotomy was performed. Indications, advantages and limitations: This mini-invasive approach was indicated for patients with unruptured aneurysm, in patients with aneurysmal SAH without intracranial hypertension, and especially in elderly patients. This minicraniotomy (1) gave quick and direct access to the aneurysm; (2) provided less trauma of the temporal muscle and improved the cosmetic resuilts; and (3) reduced the risk of postoperative epidural hematoma thanks to the small detachment of the dura mater from the vault. We concluded that this limited supraorbital approach gave adequate visualization and allows surgical manipulation within eloquent structures and can be specifically applied in absence of intracranial hypertension.
World Neurosurgery, 2012
Inflammatory pseudotumors are ubiquitous lesions characterized by a polymorphous inflammatory inf... more Inflammatory pseudotumors are ubiquitous lesions characterized by a polymorphous inflammatory infiltrate containing plasma cells and lymphocytes. In the central nervous system, this pathological condition is rare and the association with Crohn's disease has never been described. A 31-year-old woman with a history of Crohn's disease was referred to our department for progressive headaches and nausea. Neurological examination was normal. Magnetic resonance imaging showed an irregular heterogeneous enhanced mass infiltrating the left cerebellar hemisphere. Total resection was performed and pathological examination led to the conclusion of an inflammatory pseudotumor. To our knowledge, this case is the first describing an intra-cerebral inflammatory pseudotumor associated with an inflammatory bowel disease. The diagnosis of an extradigestive location of Crohn's disease was excluded by pathological examination. Although the precise cause of this association remains unknown, it could be hypothesized that the intra-cranial lesion could be the result of the immunosuppressive therapy given for Crohn's disease, or, more likely, could be a part of a systemic dysimmune process.
Surgical Neurology, 2004
The authors describe 2 cases of dysplasic gangliocytoma of the cerebellum or Lhermitte-Duclos dis... more The authors describe 2 cases of dysplasic gangliocytoma of the cerebellum or Lhermitte-Duclos disease revealing Cowden disease or multiple hamartoma neoplasia syndrome. Cowden disease is a rare autosomal dominant disorder, now considered as a phakomatosis. Nevertheless, relationships between both conditions still remain unclear, since Lhermitte-Duclos disease can also be sporadic. Two patients, 25 and 27 years old, were admitted to the emergency department for an acute intracranial hypertension. In both cases, magnetic resonance imaging (MRI) scan showed a mass in the cerebellar hemisphere. Pathological examination of surgical resection specimens concluded Lhermitte-Duclos disease. Because of the patients' previous personal and familial medical history, Cowden disease was suspected and confirmed by mutational analysis of the phosphatase and tensin homolog (PTEN) gene. In the first case, a nonsense punctual mutation in exon 8 was found. In the second one, a mutation was revealed in the exon 5, a mutational hot spot encoding the phosphatase catalytic core motif. Lhermitte-Duclos disease and Cowden disease can be associated. Germline mutations of PTEN gene are known to be implicated in Cowden disease. This gene, located at chromosome 10q23-3, is a tumor suppressor gene that encodes a protein with phosphatase activity. To date, more than 80 mutations have been reported in Cowden disease. When the diagnosis of either one of these two disorders is established, it is imperative to search for the other one to detect early malignant lesions that occur in Cowden disease. Finally, a long-term follow up of the patient is required and a thorough familial screening is necessary.
Surgical Neurology, 2006
Primary diffuse leptomeningeal gliomatosis is an exceptional neoplasm, and only 30 cases have bee... more Primary diffuse leptomeningeal gliomatosis is an exceptional neoplasm, and only 30 cases have been reported in the literature. We report a recent case and compare data with previously published observations. A 50-year-old man was admitted to the neurosurgery department for a previous 4-month history of headache, associated with nonspecific neurological signs. Biologic data and cerebrospinal fluid examination suggested an inflammatory process. The patient was given an antituberculous therapy. Magnetic resonance imaging revealed a multinodular enhancement of spinal nerve roots. A biopsy of sacral rootlets was performed. Histological examination revealed an anaplastic astrocytoma. Patient's status worsened, and death occurred 7 months later. Complete neuraxis postmortem examination revealed no intraparenchymatous glioma and was conclusive for the diagnosis of primary leptomeningeal gliomatosis (astrocytic, World Health Organization grade III), with a multinodular pattern in the spinal cord, the brainstem, and the brain base with diffuse extension into the cerebellar subarachnoid spaces. Our case illustrates the diagnostic difficulties in making the premortem diagnosis. The review of the literature indicates that there are no specific clinical or biologic signs. Magnetic resonance imaging using T1-weighted images with gadolinium enhancement and biopsy material may be useful diagnostic tools. In most cases, autopsy evaluation alone permits definitive primary diffuse leptomeningeal gliomatosis diagnosis. Whatever the histological characteristics of proliferating cells are, the prognosis remains poor. No prognostic factors have been shown to be correlated with survival time. Unfortunately, no routine treatment has been yet proposed.
Seminars in Neurology, 1998
Subarachnoid hemorrhage is a formidable and common health care problem. Early diagnosis and manag... more Subarachnoid hemorrhage is a formidable and common health care problem. Early diagnosis and management are crucial to reduce the morbidity form this complex and multifaceted disease. Open surgery and endovascular techniques both aim at eliminating the source of hemorrhage. The choice of therapy can be made rationally based on an understanding of the merits, risks, and limitations of each therapy. The care of pregnant patients with subarachnoid hemorrhage and patients who harbor both aneurysms and AVMs can be approached rationally with an understanding of the complex pathophysiology behind these clinical scenarios. Familiarity with the signs of mild SAH, and advances in familial screening, noninvasive imaging, and therapies for vasospasm will continue to lessen the toll of this dramatic illness on the public well-being.
Parkinsonism & Related Disorders, 2011
Journal of Neurosurgery, 2009
For anterior communicating artery (ACoA) aneurysms, endovascular coil embolization constitutes a ... more For anterior communicating artery (ACoA) aneurysms, endovascular coil embolization constitutes a safe alternative therapeutic procedure to microsurgical clip occlusion. The authors' aim in this study was to evaluate the quality of life (QOL), cognitive function, and brain structure damage after the treatment of ruptured ACoA aneurysms in a group of patients who underwent microsurgical clipping (36 patients) compared with a reference group who underwent endovascular coiling (14 patients). At 14 months posttreatment all patients underwent evaluations by independent observers. These observers evaluated global efficacy, executive functions using a frontal assessment battery of tests (Trail making test, Stroop tasks, dual task of Baddeley, verbal fluency, and Wisconsin Card Sorting test), behavior dysexecutive syndrome (the Inventaire du Syndrome Dysexécutif Comportemental questionnaire [ISDC]), and QOL by using the Reintegration To Normal Living Index. Brain damage was analyzed using MR imaging. In the microsurgical clipping and endovascular coiling groups, the distribution on the modified Rankin Scale (p = 0.19) and mean QOL score (85.4 vs 83.4, respectively) were similar. Moreover, the proportion of executive dysfunctions (19.4 vs 28.6%, respectively) and the mean score on the ISDC questionnaire (8.9 vs 8.5, respectively) were not significant, but verbal memory was more altered in the microsurgical clipping group (p = 0.055). Magnetic resonance imaging revealed that the incidence of local encephalomalacia and the median number of lesions per patient increased significantly in the microsurgical clipping group (p = 0.003). In the 2 groups, no significant difference was observed regarding QOL, executive functions, and behavior. Despite the significant decrease in verbal memory after microsurgical clipping, the interdisciplinary approach remains a safe and useful strategy.
Journal of Neurosurgery, 2002
Cavernous internal carotid artery (ICA)-anterior cerebral artery (ACA) anastomoses are unusual an... more Cavernous internal carotid artery (ICA)-anterior cerebral artery (ACA) anastomoses are unusual anomalies in which a duplicated A, segment of the ACA arises from the infraoptic ICA. The authors report on a 30-year-old woman who presented with subarachnoid hemorrhage from an anterior communicating artery (ACoA) aneurysm associated with an extremely rare variant of this anastomosis. The extra A, segment emerged from the ICA within the cavernous sinus rather than at or above the level of the ophthalmic artery. The presence of the anomalous vessel provided a straightforward endovascular approach to the ACoA and allowed the use of coil placement rather than surgical clipping to treat the aneurysm successfully.
Atherosclerosis, 2010
Because an underlying arteriopathy might contribute to the development of intracranial aneurysms ... more Because an underlying arteriopathy might contribute to the development of intracranial aneurysms (IAs), we assessed the elastic properties of proximal conduit arteries in patients with IA. In 27 patients with previous ruptured IA and 27 control subjects matched for age, gender and BMI, we determined arterial pressure, internal diameter, intima-media thickness (IMT), circumferential wall stress (CWS) and elastic modulus (wall stiffness) in common carotid arteries using applanation tonometry and echotracking. Moreover, carotid augmentation index (AIx, arterial wave reflections) and carotid-to-femoral pulse wave velocity (PWV, aortic stiffness) were assessed. Compared with controls, patients with IA exhibited higher brachial and carotid systolic and diastolic blood pressures, with similar brachial but higher carotid artery pulse pressure (35 + or - 6mm Hg vs. 41 + or - 8mm Hg, P=0.014). Moreover, patients have higher PWV (7.8 + or - 1.2ms(-1) vs. 8.3 + or - 1.1ms(-1), P=0.048) and AIx (15.8 + or - 10.8% vs. 21.1 + or - 8.5%, P<0.001) which contributes to increase carotid blood pressures. Furthermore, carotid IMT was higher in patients (546 + or - 64 microm vs. 642 + or - 70 microm, P<0.001) without difference in diameter suggesting an adaptive hypertrophy. However, patients display a lower CWS (61.6 + or - 9.2 kPa vs. 56.9 + or - 10.3 kPa, P=0.007) and no correlation between IMT and pulse pressure (r=0.152, P=NS) in contrast to controls (r=0.539, P<0.001) showing the contribution of a pressure-independent process. Finally, despite this lesser CWS, elastic modulus was increased in patients (310 + or - 105 kPa vs. 383 + or - 174 kPa, P=0.026). This study demonstrates that patients with IA display a particular carotid artery phenotype with an exaggerated hypertrophic remodeling and altered elastic properties. Thus, a systemic arteriopathy might contribute, together with the arterial wall fatiguing effect of the increased pulsatile stress, to the pathogenesis of IA.
Annales Françaises d'Anesthésie et de Réanimation, 2007
Acta Obstetricia et Gynecologica Scandinavica, 2004
Cerebral aneurysmal complications rarely occur during pregnancy. Telling the difference between e... more Cerebral aneurysmal complications rarely occur during pregnancy. Telling the difference between eclampsia and cerebral hemorrhage due to aneurysmal rupture can prove to be difficult. Aneurysmal management should be performed in an emergency but fetal prognosis should be considered. We report a series of eight pregnant women presenting aneurysmal complications and we have assessed their management and outcome. Both maternal and perinatal mortality rates were correlated with the maternal clinical score. We stress the role of combined care by both neurosurgeons and obstetricians. An emergency cesarean section followed by aneurysmal treatment appears to be a widely accepted strategy in pregnant women with cerebral aneurysmal complications.
Surgical Neurology, 2008
Prevalence of cerebral cavernomas in the general population is close to 0.5%. In contrast, SCCs a... more Prevalence of cerebral cavernomas in the general population is close to 0.5%. In contrast, SCCs are rare. The aim of this study was to determine the outcome of SCC in a large sample of patients. Clinical and neuroradiologic findings were retrospectively collected in a multicentric study. Diagnosis was based on pathologic criteria or MR findings. Fifty-three patients were included (26 males, 27 females). Mean age at onset of symptoms was 40.2 years (11-80 years). Initial symptoms were progressive (32) or acute myelopathy (20). One case was asymptomatic. Triggering factors were found in 14 of the patients (26%). Clinical symptoms were related to spinal cord compression (27) and hemorrhage (22). Spinal cord cavernoma was thoracic in 41 cases and cervical in 12. Mean size of the lesions was 16.3 mm (3-54 mm). In the 40 surgical patients, long-term follow-up was available in 37 cases for a mean time of 7.3 years (0.4-50 years). During the follow-up period, 20 patients improved, 6 remained on their preoperative baseline, and 11 got worse. Surgical improvement was more often found in posterior rather than anterior location. Using McCormick classification, 22 patients were autonomous (grades 1-2), 12 handicapped (grade 3), and 3 bedridden (grade 4) at the end of the follow-up. This study has defined clinical and MR patterns of spinal cavernomas. Surgery lastingly improved more than half of the patients.