[A case of cystic craniofacial fibrous dysplasia]. / Un cas de dysplasie fibreuse kystique craniofaciale.

INTRODUCTION: Fibrous dysplasia is a benign, idiopathic, fibro-osseous disease. CLINICAL CASE: A 17-year-old girl had presented with left proptosis for the previous two years, associated to homolateral hemicranial pain. Clinical examination was normal except for non-reducible axile exophthalmia. Computed tomography and magnetic resonance imaging of the head revealed an extensive cystic tumor of the left fronto-ethmoido-sphenoidal region, with compression of the left frontal lobe and medial orbital wall. The radiological appearance suggested a mucocele but histological examination, after surgery, proved a fibrous dysplasia. DISCUSSION: Fibrous dysplasia with fronto-sphenoido-ethmoidal localization may be misdiagnosed as mucocele. Histology proves the diagnosis.

Sphenoid sinus aspergillosis simulating pituitary tumor in immunocompetent patient.

Aspergillosis of the sphenoid sinus is rare in immunocompetent patients. It may be mistaken for a sellar region tumor. A 65-year-old, human immunodeficiency virus-negative man presented with a 3-week history of cranial nerve III paresis and visual deterioration. The patient had a long-term history of tobacco snuff abuse. CT scans and MRI demonstrated a space-occupying lesion of the sellar and sphenoid sinus region. Presumptive diagnosis of pituitary macroadenoma was made and the patient was operated on via a transnasal-transsphenoidal approach. After the sphenoid sinus was opened, a yellow-brownish gluey material with crumbly debris extruded and was aspirated. The dura was intact. Histopathology revealed numerous Aspergillus hyphae without tissue invasion. Postoperatively, the cranial nerve III paresis resolved in a few days and visual acuity improved. Sphenoid sinus aspergillosis should be included in the differential diagnosis of sellar region processes, even in immunocompetent patients. Early diagnosis and transsphenoidal removal provides good results without the need for systemic antifungal therapy in non-invasive aspergillosis.

[Os odontoideum: clinical and radiological aspects]. / Os odontoïdeum : aspects cliniques et radiologiques.

Few reports of os odontoideum have been made. We report two cases where this affection was revealed by cervical pain and hemiparesis in one case and acute tetraparesis in the other. Patients with os odontoideum usually present with neurological signs, but some have only cervical pain and some others remain asymptomatic. Radiological exams, including radiograms, cervical scanner and MRI lead to the diagnosis. Different surgical treatment can be proposed to symptomatic patients. Prophylactic surgical treatment is not indicated.

Disruption of mouse polymerase zeta (Rev3) leads to embryonic lethality and impairs blastocyst development in vitro.

Multiple DNA polymerases exist in eukaryotes. Polymerases alpha, delta and epsilon are mainly responsible for chromosomal DNA replication in the nucleus and are required for proliferation. In contrast, the repair polymerases beta and eta are not essential for cellular proliferation in yeast or mice, but a lack of either polymerase can lead, respectively, to defects in base excision repair or the ability to replicate past lesions induced by ultraviolet (UV) radiation [1-3]. Here, we have focused on polymerase zeta. This was first described as a non-essential product of the yeast REV3/REV7 genes involved in UV-induced mutagenesis, and was later implicated in trans-lesion synthesis [4,5]. Unlike in yeast, the mouse homologue (mRev3) was found to be essential for life. Homozygous mutant mice died in utero. Mutant embryos were considerably reduced in size at day 10.5 of development and usually aborted around day 12.5. It is likely that this block reflects a need for mRev3 in proliferative clonal expansion (rather than in the production of a particular cell type) as mutant blastocysts showed greatly diminished expansion of the inner cell mass in culture. Thus, mRev3 could be required to repair a form of externally induced DNA damage that otherwise accumulates during clonal expansion or, consistent with the high homology shared between its Rev7 partner and the mitotic checkpoint gene product Mad2 [6], mRev3 might play a role in cell proliferation and genomic stability even in the absence of environmentally induced damage.

The effect of intron sequences on expression levels of Ig cDNAs.

Several cDNA expression vectors were constructed and tested by stable transfection into a murine lymphoid cell line in order to compare secretion rates of a human immunoglobulin (Ig) light chain (LC). When the cDNA was under transcriptional control of the SV40 promoter and enhancer and preceded by the SV40 19S late mRNA intron, a weak LC production was detected. Secretion rate was not improved by replacing the SV40 promoter and enhancer by a combination of a murine Ig heavy chain (HC) gene promoter and enhancer even with insertion of additional Ig enhancers. In contrast, replacement of the 19S intron by a large intron derived from a human Ig HC gene and containing the intronic enhancer dramatically increased the secretion rate. High-level production was also obtained with the same enhancer-containing intron placed downstream from the LC cDNA. Stable transfectants were obtained that secreted the human LC in amounts comparable to those obtained with Ig genes. Our results suggest that the SV40 19S late mRNA intron used in several expression vectors is not appropriate when the purpose is to produce large amounts of antibody molecules. By providing transcriptional, splicing and polyadenylation signals, the presently described vectors will be useful for easy cloning and high-level expression in lymphoid cells of cDNAs or PCR products encoding antibody molecules.

Technology and neurosurgery in developing countries: experience and present situation in Morocco.

OBJECTIVE: The high cost of technology is considered to be the determining factor slowing the expansion of modern neurosurgery in many developing countries. The literature dedicated to this topic rarely proposes internal solutions whereby affected countries can overcome this economic impediment. Certain articles cite inevitable obstacles, and the neurosurgeons of these countries can become disheartened when these articles conclude with calls for foreign help as the only approach to the development of neurosurgery. METHODS: Morocco is presented as an example of a developing country in which neurosurgery has become well established in the past 30 years, using a program based on four guidelines, as follows: 1) encouraging the local training of young neurosurgeons, 2) organizing and promoting neurosurgery, 3) integrating the development of neurosurgery into the health care pyramid system, and 4) stimulating research on local pathological conditions. RESULTS: Because of the internal planning efforts stimulated by the first national neurosurgeons, Morocco has progressed from 2 underequipped neurosurgical services and 5 neurosurgeons in 1968 to 12 well-equipped services and 80 neurosurgeons in 1998. The main benefits of this progress are discussed. CONCLUSION: Neurosurgery in developing countries can be promoted if the first working neurosurgeons take up their responsibilities as pioneers. This role requires that they initiate the training of young neurosurgeons as soon as possible and that they find in the local conditions the necessary factors to promote neurosurgery and to integrate it into the health care development of their country.

African neurosurgery: current situation, priorities, and needs.

OBJECTIVE: The beginning of a new millennium causes us to reflect on the state of neurosurgery in the African countries and to affirm that we are eager to provide quality health care for our people. This study is important because its main purpose is to assess progress toward achievements and to identify potential problems, so that remedial action can be taken. METHODS: To attain these objectives, the following steps were performed: planning, surveying, analyzing feedback, and reporting. We first formed the World Health Organization African Subcommittee. In a meeting held in Dakar on May 25, 1998, we agreed on a strategy and devised a statistical form to adopt. After the drafts were approved, they were distributed to 51 African countries. The survey was performed by representatives who spent 6 months analyzing and collecting data. RESULTS: The results were alarming. After data analysis, we classified the countries into four main groups with respect to the number of neurosurgeons, the equipment potential, and the training capacity. One of the main observations is the need for organization and structured training. Another weakness is the lack of medicosurgical equipment. Poor funding and a lack of resources are responsible for this situation. CONCLUSION: Overcoming these problems will require that African neurosurgeons target a number of priorities, i.e., devising local training programs and obtaining the necessary equipment. We appeal to national and international institutions to focus on these two points to ensure long-term results, including greater involvement of African countries in local training and greater cooperation in terms of technical support and funding.

Pattern of cerebral aneurysms in Morocco: review of the concept of their rarity in developing countries: report of 200 cases.

OBJECTIVE: Many neurosurgeons consider cerebral aneurysms to be rare in Africa and the Middle East. In this report, we describe the pattern of cerebral aneurysms in Morocco and call into question the idea of their rarity in developing countries. Our objective is to urge neurosurgeons in these areas to track them and to treat them under better conditions. METHODS: We report a retrospective study of 200 patients with cerebral aneurysms admitted to our department between 1983 and 1999. The results of this study are supported by pertinent epidemiological surveys, anatomic studies on the incidence of cerebral aneurysms in Morocco, and analysis of the literature related to the epidemiology of aneurysms in developing countries. RESULTS: The patients in our series ranged in age from 7 to 70 years (mean age, 52 yr), with a slight female predominance (52%). They presented with subarachnoid hemorrhage (173 patients), cranial nerve palsy (18 patients), or mass symptoms (9 patients). The delay between subarachnoid hemorrhage and admission ranged from 1 to 30 days (mean, 14 d). The aneurysm was located in the internal carotid artery in 42%, in the anterior communicating and anterior cerebral arteries in 28%, in the middle cerebral artery in 19%, and in the vertebrobasilar artery in 10%. Multiple aneurysms were encountered in 9% and giant aneurysms in 15.5%. Seventeen patients died before surgery (with vasospasm in 13 cases and rebleeding in 4 cases), and 19 died after surgery. Follow-up, ranging between 1 and 10 years, revealed good outcomes with complete recovery in 64.5% and recovery with major sequelae in 7%. Pre- and postoperative mortality represented 18%; there was no operative treatment and no follow-up in 11.5%. CONCLUSION: Some data in this study (the delay between subarachnoid hemorrhage and admission, the high incidence of urban patients [80%], and the high rate of giant aneurysms) explain why many cases of ruptured aneurysms are not diagnosed. The analysis of our clinical series and the results of the epidemiological surveys show that the incidence has doubled every 5 years. These findings confirm that cerebral aneurysms are not rare in Morocco. A critical reading of the published articles claiming a low incidence of cerebral aneurysms in Africa, the Middle East, and Asia shows that this conclusion is not based on accurate and reliable statistical studies. Neurosurgeons in these regions should abandon this idea of rarity, and they should search for arterial cerebral aneurysms and develop the optimum conditions for the treatment of patients with aneurysms.

Central nervous system tumors in Morocco. Retrospective analysis of 2374 cases.

BACKGROUND: No epidemiological data about central nervous system tumors in Morroco have been published. The objective of the present study is to assess topographic and demographic patterns of a large series of histologically confirmed tumors of central nervous system (CNS), skull and vertebral column, examined in the main neuropathology laboratory of the country. METHODS: No. 2363 tumors have been collected in the department of Neuropathology in Hôpital des spécialités oto-neuro-ophtalmiques, Rabat, between 1988 and 1997. Data concerning age, sex, tumor location and histological type were retrieved from the laboratory files. Histological typing was based on the World Health Organization (WHO) classification of central nervous system tumors and grading on the Kernohan and Mayo Clinic-St. Anne systems. RESULTS: No. 1454 intraparenchymatous and 904 extraparenchymatous tumors were studied. The topographic repartition of these tumors presented no differences when compared to the literature data. The main differences were noted for malignant astrocytomas and glioblastomas which were less frequent than in Western countries and occurred in younger people. The young age of Morrocan population probably explains these findings. Childhood tumors repartition was similar to Western series. CONCLUSIONS: These results emphazise the need for a population based registry in order to verify our findings and adapt efficient health intervention.

Thoracic epidural hemangiopericytoma. Case report.

Hemangiopericytoma is an uncommon mesenchymal neoplasm that rarely affects the spinal canal. We report a case of thoracic epidural hemangiopericytoma in a 39-year-old man with symptoms of spinal cord compression studied by myelo-computed tomography (CT) and magnetic resonance imaging (MRI). Total resection was followed by complete recovery. Postoperative radiotherapy was performed. The clinical, radiological, and histological findings of epidural spinal hemangiopericytoma are reviewed in the light of relevant literature

African neurosurgery part II: current state and future prospects.

A survey conducted among African neurosurgeons shows that there are now 500 neurosurgeons in Africa; that is, one neurosurgeon for 1,350,000 inhabitants, and 70,000 km2. The distribution of these neurosurgeons shows a striking regional disparity: North Africa has 354 neurosurgeons for 119 million inhabitants; that is, one neurosurgeon for 338,000 inhabitants; and South Africa has 65 neurosurgeons for 40 million inhabitants; that is, one neurosurgeon for 620,000 inhabitants. Between these two areas where neurosurgery is developing quite well, we have the majority of African countries with a scant density of neurosurgeons (81 neurosurgeons for 515 million inhabitants; that is, one neurosurgeon for 6,368,000 inhabitants). The Panafrican Association of Neurological Sciences (PAANS) brings together African neurosurgeons. This continental African association represents African neurosurgeons in the World Federation of Neurosurgical Societies (WFNS). In addition to this continental association, there are national societies of neurosciences. However, there are only six societies of neurosurgery. Two systems of training exist in Africa: (1) local training, and (2) training abroad. These two systems have unequal quality and specific difficulties that are pointed out. Among the optimistic elements that make us believe in the development of neurosurgery in Africa, are the existence of a quite good level of neurosurgery at the two extremities of the continent (North Africa and South Africa), the development of neurosciences in African universities, and the increasing interest that the international community bears to Africa in the last years. However, the real factor of optimism is the African neurosurgeons who should promote neurosurgery in their continent, at the level of their own countries by developing information and health education, setting their specialty in the education syllabus and health planning, and settling into active and performing societies. At the continental and international level, African neurosurgeons should institutionalize inter-African cooperation, expedite their continental association (PAANS), and further exchanges with the other continents through the WFNS. The latter, together with other associations such as the European Association of Neurosurgical Societies (EANS) could provide help to the development of neurosurgery in Africa as far as training, exchanges, research, and organization are concerned.

[MR imaging in the diagnosis of intradural extramedullary tuberculoma. Report of a case and review of the literature]. / Apport de L'IRM dans le diagnostic du tuberculome intradural extramédullaire. A propos d'un cas et revue de la littérature.

Intradural extramedullary tuberculoma without any bony involvement is exceptional. Nineteen cases were found in the literature. We report the case of 5-year-old boy who presented acute paraplegia 8 months after a tuberculous meningitis under antituberculous treatment. Dorsal gadolinium enhanced MRI revealed an intradural extramedullary bunch-shaped enhancing mass at T5-T6 level. The tuberculous nature was confirmed by histology after surgical removal of the lesion followed by a good recovery. To our knowledge, this is the first intradural extramedullary tuberculoma detected by MRI. We underline the role of MRI in the diagnosis of this particular localisation of tuberculosis.

[Primary intradural extramedullary hydatidosis. Case report and review of the literature]. / L'hydatidose intradurale extramédullaire primitive. A propos d'un cas and revue de la littérature.

The authors report a case of cauda equina compression by intradural hydatid cyst. An 18-year-old man presented with paraparesis and sphincter dysfunction. MRI showed an intradural cystic lesion extending from L1 to L2 with low signal intensity on T1 and high signal intensity on T2. The cyst was removed after laminectomy and opening of the dural sac. Histological and parasitic examinations confirmed a diagnosis of hydatid cyst. The patient improved progressively after surgery. The similar 22 cases of intradural extramedullary hydatid disease reported in the literature were reviewed. All spinal areas were involved, with a predilection for the thoracic region. Neurological complications were usual with rapid spinal cord compression in this rare form of hydatid disease. The treatment was by surgery with a favourable outcome compared to the classic hydatid cyst of the spine.

[Orbital meningoencephalocele: two case studies]. / La méningo-encéphalocèle orbitaire. A propos de 2 cas.

Primitive orbital meningocele is a rare congenital malformation. It is defined as a herniation of meninges into the orbit through a congenital defect in the orbital bones. In meningoencephalocele, there is atrophic brain tissue in the herniated meningeal sac. We report the cases of two babies who presented with a medial orbital tumoral syndrome. Computed tomography showed a cystic tumor close to a bony defect in the anterior frontoethmoidal junction. A transcranial approach confirmed the diagnosis of meningoencephalocele; it was resected and the bony and dural defects were closed. The outcome was uneventful. Orbital meningoencephalocele is probably linked to an abnormal closure of the rostral neuropore. It generally presents in the first months of life with very suggestive radiological and clinical features. Surgical treatment using a transcranial approach is indicated in all cases and provides definitive recovery.

[Inflammatory pseudotumor of the lacrimal gland. Apropos of 2 cases]. / Les pseudo-tumeurs inflammatoires de la glande lacrymale. A propos de 2 cas.

BACKGROUND: Inflammatory pseudotumors of the orbit are relatively common accounting for 12 to 15% of all orbital diseases. Lacrimal gland location is exceptional. CASE REPORTS: We report 2 patients aged 30 and 25 years who underwent surgery for an isodense tumor of the lacrimal gland. Immunohistochemistry revealed an inflammatory pseudotumoral process. Both patients are recurrence-free 2 years follow-up. DISCUSSION: Inflammatory pseudotumors of the orbit, particularly those located in the lacrimal gland still raise unresolved questions concerning the pathogenesis, diagnosis and treatment. CONCLUSION: Lacrimal gland localizations of inflammatory pseudotumors must be recognized due to difficulties in diagnosis and therapeutic management.

[Isolated lumbar intradural hydatid cyst]. / Kyste hydatique intradural lombaire isolé.

Intradural hydatidosis is a very uncommon form of vertebrospinal hydatidosis. We report a intradural lumbar localization, presenting the magnetic resonance imaging findings. We emphasize MRI for diagnosis and follow-up in search for residual or recurrent lesions.

[Orbital lymphangioma. A case report]. / Lymphangiome orbitaire. A propos d'un cas.

The diffuse form of orbital lymphangioma is well known for its difficult surgical treatment. A diffuse orbital lymphangioma was diagnosed in a 6-year-old girl, revealed by unilateral recurrent proptosis. The imaging procedure discovered a mass presumed to be vascular in nature. The initial incompleted surgical removal was followed by 3 recurrences motivating 3 reoperations. Finally, the tumor was removed incompletely, with an acceptable reduction of the proptosis. The pathologic analyses indicated lymphangioma. Some vascular orbital tumors such as lymphangioma, may be very difficult to manage because of local spreading and frequent recurrence.