[Lombosacral epidural capillary hemangioma mimicking a dumbbell-shaped neurinoma: A case report and review of the literature]. / Hémangiome capillaire épidural lombosacré mimant un neurinome en sablier : à propos d'un cas et revue de la littérature.

INTRODUCTION: Capillary hemangiomas are benign endothelial cell neoplasms that are believed to be hamartomatous proliferations of vascular endothelial cells. The occurrence of spinal epidural capillary hemangiomas is exceedingly rare. Only 8 epidurally located cases of capillary hemangiomas in the spinal canal have been reported in the literature. CASE REPORT: We report for the first time, to our knowledge, a case of lumbosacral epidural capillary hemangioma revealed by S1 back pain and radicular pain in a 60-year-old patient, caused by an L5-S1 epidural capillary hemangioma. The neurological symptoms of the patient improved after surgery. CONCLUSION: Spinal epidural capillary hemangioma is exceedingly rare. These lesions are benign and can mimic dumbbell-shaped neurinoma. Total removal by surgery is curative.

[Cilioretinal artery occlusion and central retinal vein occlusion complicating hyperhomocysteinemia: a case report]. / Occlusion mixte : de l'artère ciliorétinienne et de la veine centrale de la rétine par hyperhomocystéinémie (à propos d'un cas).

INTRODUCTION: Hyperhomocysteinemia is known to be a risk factor in both retinal artery and retinal vein occlusions. We report the case of a young patient with combined occlusion of the cilioretinal artery and the central retinal vein due to hyperhomocysteinemia. PATIENTS AND METHODS: A 23-year-old patient without significant medical history, presented for sudden, painless visual loss in the right eye. Ophthalmologic examination revealed best-corrected visual acuity of the right eye 8/10 P2, and 10/10 P2 on the left. Anterior segment exam was normal in both eyes, while the right fundus revealed white, ischemic edema, centered around a cilioretinal artery, sparing the fovea, with some hemorrhagic spots and disc edema. Fluorescein angiography confirmed delayed filling of the right cilioretinal artery and revealed a normal disc on the left. Two weeks later, the clinical picture had evolved into a right ischemic CRVO, confirmed by a second angiogram, with a decrease in visual acuity to 3/10. RESULTS: A work-up was performed, including: a full lipid profile, serum electrolytes, ESR, CRP, a complete blood count (leukocytes, platelets, hemoglobin were normal), a coagulation work-up (PT, PTT, protein C, protein S, antithrombin III, factor V Leiden were normal), ANCA, antiphospholipid antibodies and antinuclear antibodies were negative, and finally cardiology studies (cardiac echo, carotid Doppler) and neurology (brain MRI) were ordered and came back normal. Otherwise, plasma homocysteine was moderately high on two samples, at 18.3 µmol/L and 17.78 µmol/L. Thyroid and renal work-ups were ordered. Urgent PRP was performed, and vitamin therapy (vitB12, vitB6, folic acid) was instituted. The subsequent course was remarkable for recovery of visual acuity to 10/10, P2 with persistence of an inferior altitudinal central scotoma. MTHFR C677T polymorphism was negative. DISCUSSION: Retinal vascular occlusions (RVO) are serious events, which require investigation for underlying systemic disease, which can be life-threatening. The clinical picture is variable depending on the location of the occlusion, the extent of the ischemic area and the degree of macular involvement. The etiologies of RVO are varied, requiring a thorough biological assessment in young subjects. The association between hyperhomocysteinemia and RVO is proven, while this association with the MTHFR C677T polymorphism was not found. Vitamin therapy reduces plasma levels of homocysteine by 25% but its role in the treatment and prevention of RVO remains to be demonstrated. CONCLUSION: Several cases of occlusion of the central retinal vein or one of its branches have been published. Combined occlusion of the central retinal vein and cilioretinal artery secondary to hyperhomocysteinemia does not appear to have been published, which would make our case unique.

Cytotoxic effect of essential oil of thyme (Thymus broussonettii) on the IGR-OV1 tumor cells resistant to chemotherapy

The anti-tumor effect of the Moroccan endemic thyme (Thymus broussonettii) essential oil (EOT) was investigated in vitro using the human ovarian adenocarcinoma IGR-OV1 parental cell line OV1/P and its chemoresistant counterparts OV1/adriamycin (OV1/ADR), OV1/vincristine (OV1/VCR), and OV1/cisplatin (OV1/CDDP). All of these cell lines elicited various degrees of sensitivity to the cytotoxic effect of EOT. The IC50 values (mean ± SEM, v/v) were 0.40 ± 0.02, 0.39 ± 0.02, 0.94 ± 0.05, and 0.65 ± 0.03 percent for OV1/P, OV1/ADR, OV1/VCR, and OV1/CDDP, respectively. Using the DBA-2/P815 (H2d) mouse model, tumors were developed by subcutaneous grafting of tumor fragments of similar size obtained from P815 (murin mastocytoma cell line) injected in donor mouse. Interestingly, intra-tumoral injection of EOT significantly reduced solid tumor development. Indeed, by the 30th day of repeated EOT treatment, the tumor volumes of the animals were 2.00 ± 0.27, 1.35 ± 0.20, and 0.85 ± 0.18 cm³ after injection with 10, 30, or 50 æL per 72 h (six times), respectively, as opposed to 3.88 ± 0.50 cm³ for the control animals. This tumoricidal effect was associated with a marked decrease of mouse mortality. In fact, in these groups of mice, the recorded mortality by the 30th day of treatment was 30 ± 4, 18 ± 4, and 8 ± 3 percent, respectively, while the control animals showed 75 ± 10 percent of mortality. These data indicate that the EOT which contains carvacrol as the major component has an important in vitro cytotoxic activity against tumor cells resistant to chemotherapy as well as a significant antitumor effect in mice. However, our data do not distinguish between carvacrol and the other components of EOT as the active factor.

Cytotoxic effect of essential oil of thyme (Thymus broussonettii) on the IGR-OV1 tumor cells resistant to chemotherapy.

The anti-tumor effect of the Moroccan endemic thyme (Thymus broussonettii) essential oil (EOT) was investigated in vitro using the human ovarian adenocarcinoma IGR-OV1 parental cell line OV1/P and its chemoresistant counterparts OV1/adriamycin (OV1/ADR), OV1/vincristine (OV1/VCR), and OV1/cisplatin (OV1/CDDP). All of these cell lines elicited various degrees of sensitivity to the cytotoxic effect of EOT. The IC50 values (mean +/- SEM, v/v) were 0.40 +/- 0.02, 0.39 +/- 0.02, 0.94 +/- 0.05, and 0.65 +/- 0.03% for OV1/P, OV1/ADR, OV1/VCR, and OV1/CDDP, respectively. Using the DBA-2/P815 (H2d) mouse model, tumors were developed by subcutaneous grafting of tumor fragments of similar size obtained from P815 (murin mastocytoma cell line) injected in donor mouse. Interestingly, intra-tumoral injection of EOT significantly reduced solid tumor development. Indeed, by the 30th day of repeated EOT treatment, the tumor volumes of the animals were 2.00 +/- 0.27, 1.35 +/- 0.20, and 0.85 +/- 0.18 cm(3) after injection with 10, 30, or 50 microL per 72 h (six times), respectively, as opposed to 3.88 +/- 0.50 cm(3) for the control animals. This tumoricidal effect was associated with a marked decrease of mouse mortality. In fact, in these groups of mice, the recorded mortality by the 30th day of treatment was 30 +/- 4, 18 +/- 4, and 8 +/- 3%, respectively, while the control animals showed 75 +/- 10% of mortality. These data indicate that the EOT which contains carvacrol as the major component has an important in vitro cytotoxic activity against tumor cells resistant to chemotherapy as well as a significant antitumor effect in mice. However, our data do not distinguish between carvacrol and the other components of EOT as the active factor.

[Sexual dysfunction among 78 Moroccan male hemodialysis patients: clinical and endocrine study]. / Troubles sexuels chez 78 hémodialysés chroniques marocains de sexe masculin: étude clinique et endocrinienne.

PURPOSE: To investigate the correlation between biochemical and endocrine variables with sexual disorders in 78 male patients on chronic hemodialysis at the Ibn Rochd University Hospital (Casablanca-Morocco). METHODS: Seventy-eight male hemodialysis patients with chronic renal failure were evaluated with regard to their sexual function. All patients answered a personal questionnaire on their sexual activity. Hormone (FSH, LH, prolactin, testosterone, and parathyroid hormone) and zinc and ferritin assays were also performed. RESULTS: The men reported erection (44.9%), libido (44.9%), ejaculation (26.8%), and orgasm (21.8%) disorders. Gynecomastia was observed in 17.9% of the patients. There was no correlation with weight nor the nature of the causal nephropathy nor with duration of dialysis. Levels of gonadotropins (FSH, LH), prolactin, and parathyroid hormone were elevated. Testosterone levels were low. Ferritinemia was elevated but there was no significant variation in zincemia. There was a negative curvilinear relationship between serum testosterone and sexual disorders, and between gynecomastia and ferritinemia. LH and prolactin levels were positively correlated with gynecomastia. CONCLUSION: Abnormal hormonal and iron overload could be important factors involved in the complex pathogenesis of sexual dysfunction in chronic renal failure patients undergoning hemodialysis.

[Cerebellar primary germinoma. Case report]. / Le germinome cérébelleux primitif.

Germinoma cell tumors account for 1% of all primary cerebral tumors. They occur in pineal and in supra-sellar regions, and rarely, in basal ganglia and thalami. Germinoma cell tumors originating in the posterior fossa are very rare. We report a case of primary germinoma found in the cerebellar hemisphere in a 45-year-old immunocompetent man presented with intracranial hypertension and cerebellar ataxia. The CT scan revealed a heterogeneous mass in the cerebellar right hemisphere with a cystic component. Intense and heterogeneous enhancement after contrast infusion was observed. Two other nodules were identified in the frontal and occipital lobe. The pineal region was normal. Total resection was performed. The histological finding were a germinoma. Extensive systemic examination indicated that the intracranial lesion had not metastasized from a primary extracranial tumor.

[Metastatic and hemorrhagic brain arteriovenous fistulae due to a choriocarcinoma. Case report]. / Fistule artérioveineuse cérébrale métastatique hémorragique d'origine choriocarcinomateuse.

Brain metastasis of choriocarinoma is uncommon. These tumors develop in women of childbearing age and commonly produce signs and symptoms of subarachnoid hemorrhage, intracerebral hemorrhage, or brain tumor. Diagnosis can be established by histologic study of operative swabs and bioassay of the patient's blood, urine and cerebrospinal fluid for chorionic gonadotropin. This condition is highly chemo- and radiosensitive. We report the case of a 36-year-old woman with intracranial neoplastic fistulae. Rupture occurred 3 days after spontaneous abortion at 3 months of pregnancy. The patient presented with hemiplegia, aphasia and unclear consciousness. Left fronto-parietal hematoma was diagnosed on the CT scan, and cerebral angiography showed an arteriovenous intracranial fistulae. The hematoma and angioma were surgically removed successfully. The histological examination showed a metastatic choriocarcinoma. Surgery was followed by chemotherapy and radiotherapy. After 6 years of follow-up, complete remission has been obtained.

Evidence that simulated microgravity may alter the vascular nonreceptor tyrosine kinase second messenger pathway.

Simulated microgravity (hind limb unweighting; HU) reduces maximal contractile capacity to norepinephrine (NE) but not 5-hydroxytryptamine (5-HT) in the rat abdominal aorta of male Wistar rats. Our earlier study showed that voltage-operated calcium channels, the MAPK pathway [1], and vasoconstrictive prostaglandins contribute to the NE-induced contraction of control (C) but not HU, aorta rings. Genistein, a general tyrosine kinase inhibitor, caused a significant reduction in vascular contractility in C but not HU arteries. The present study explored the role of protein kinase C (PKC) and extracellular receptor-activated kinase 1 and 2 (ERK1/2) in the HU-induced vascular hyporesponsiveness to NE. Microgravity was simulated in Wistar rats by 20 day HU. The abdominal aorta was removed from control and HU rats, cut into 3 mm rings, and mounted in tissue baths to measure isometric contraction. Protein levels were determined using Western blot analysis. PD98059, a selective MAPKK inhibitor, caused a marked inhibition of NE-induced contraction in both C and HU arteries. Calphostin C, a PKC inhibitor, completely abolished the contractile response to NE in both C and HU tissues. Phosphorylated (activated) ERK1/2 protein mass was greater in C, compared to HU, aortas, and was reduced by genistein only in C tissues. MAPK total protein levels in the rat aorta were increased in the HU-treated, compared to C, animals. These results indicate that PKC represents an early transduction step in the contractile response to NE in the rat abdominal aorta. That inhibition of the step immediately before activation of MAPK reduced contraction in both C and HU tissues, while general tyrosine kinase inhibition with genistein blocked only the control responses, suggests that a nonreceptor tyrosine kinase may be involved in HU-induced vascular hyporesponsiveness to NE.

Tumor-like tuberculosis of the sacrum.

Isolated tuberculosis of the sacrum in a 43-year-old woman manifested as functional impairment of the right lower limb. Sacral tuberculosis is rare in patients with no history of tuberculosis. Another unusual feature was the tumor-like aspect of the lesion, with diffuse, ill-defined osteolysis of a large part of the sacrum and extension to the presacral soft tissues responsible for rectal displacement.

[Behcet's disease with multiple intracranial arterial aneurysms. Report of a case]. / Maladie de Behçet avec anévrismes artériels intracrâniens multiples. A propos d'un cas.

Behçet disease is a rare condition in central Europe but more common in Morocco. A case of multiple intracranial arterial aneurysms occurring in a 44 year-old Moroccan patient with 2-years history of Behçet's disease is reported. CT-scan showed an infarction in the right middle cerebral artery territory. Panangiography showed sacciform aneurysms of the bifurcation of the right and left middle cerebral arteries. The draining veins and sinuses were normal. The two aneurysms were successfully clipped by two microsurgical frontotemporal approach in one surgical time. There have been only eight reports of intracranial arterial aneurysms associated to Behçet disease in the literature.

Spinal neurenteric cyst. A report of a case.

A case of intradural extramedullary neurenteric cyst is reported. The embryogenesis and surgical treatment of this lesion are discussed.

Disorganization in mice and humans and its relation to sporadic birth defects.

Disorganization (Ds) is a mouse mutant best known for producing an exceptional variety of unusual developmental anomalies, such as mirror-limb duplications and hamartomatous skin papillae. So great is the range of malformations that no two affected mice are identical. Several patients with a similar variety of exceptional anomalies have been reported, raising the possibility of the existence of a human homologue of Ds. However, although these human cases represent the most striking findings seen in Ds mice, they do not represent the full range of defects. Most affected mice have only a single malformation, and most of these malformations are similar to both common (neural tube defects, orofacial clefting, gastroschisis, limb reductions) and rare (anophthalmia, duplicated rectum) human birth defects. It is therefore possible that the full spectrum of the human homologue of Ds includes not only patients with the unusual combination of anomalies but also common sporadic birth defects. We suggest that the low penetrance (approximately 0-30%) and highly variable expression of Ds make it a paradigm for understanding the genetic basis for many seemingly sporadic birth defects.

X chromosome inactivation in 30 girls with Rett syndrome: analysis using the probe.

Rett syndrome (RS) is a neurologic disorder with an exclusive incidence in females. A nonrandom X-inactivation could provide insight into the understanding of this disease. We performed molecular analysis based on the differential methylation of the active and inactive X with probe M27 beta, taking into account the parental origin of the two Xs, in 30 control girls, 8 sisters, and 30 RS girls. In 27 control an 31 RS mothers, the inactivation status of the X transmitted to their daughters was also analyzed. The results showed a significantly increased frequency of partial paternal X inactivation (> 65%) in lymphocytes from 16/30 RS compared with 4/30 controls (P = 0.001). These results do not support the hypothesis of a monogenic X-linked mutation but should be taken into account when researching the etiology of this disease.

[Double localization of intracranial germinoma. Apropos of a case]. / Double localisation d'un germinome intracrânien. A propos d'un cas.

The authors report a case of a double localization of an intracranial germinoma in the pineal and suprasellar regions, which represents only 2 to 7% of all germinomas. Reviewing the literature, they discuss the pathogenesis, histopathology, biology and treatment of these tumours.

Additional case of female monozygotic twins discordant for the clinical manifestations of Duchenne muscular dystrophy due to opposite X-chromosome inactivation.

A pair of female monozygotic (MZ) twins, heterozygous carriers for a deletion in the DMD gene and discordant for the clinical manifestations of Duchenne muscular dystrophy, were analyzed by molecular studies, in situ hybridization, and methylation pattern of X chromosomes to search for opposite X inactivation as an explanation of their clinical discordance. Results in lymphocytes and skin fibroblast cell lines suggest a partial mirror inactivation with the normal X chromosome preferentially active in the unaffected twin, and the maternal deleted X chromosome preferentially active in the affected twin. A review shows that MZ female twins discordant for X-linked diseases are not uncommon. Twinning and X inactivation may be interrelated and could explain the female twins discordant for X-linked traits.