Recent advances on cutaneous lymphoma epidemiology.

BACKGROUND: Primary cutaneous lymphomas are a group of T- (CTCL) and B-cell (CBCL) malignancies. These diseases have different clinical presentations and prognosis. Our knowledge on their epidemiology is limited. Aim of this review was to summarize recent findings on the incidence of CTCL and CBCL, how they change over time, and to describe possible causes and consequences. We found that although there are important differences in the epidemiology of cutaneous lymphomas in different countries, the relative frequency of certain, especially rare lymphomas remains stable. Several studies described growing incidences of both CTCL and CBCL. The emergence of new diagnostic criteria, a more precise definition of the entities and new biomarkers enable a better classification of cases.

Olive Leaf Extracts Toxicity to the Migratory Locust, Locusta migratoria: Histopathological Effects on the Alimentary Canal and Acetylcholinesterase and Glutathione S-Transferases Activity.

The migratory locust, Locusta migratoria (Linnaeus), is the most widespread locust species. Frequent applications of insecticides have inevitably resulted in environmental pollution and development of resistance in some natural populations of the locust. To find a new and safe alternative to conventional insecticides, experiments were conducted to assess the effect of olive leaf extracts on L. migratoria fifth instar larvae. The methanolic extracts were prepared from the leaves sampled during four phenological growth stages of olive tree which are as follows: Cluster formation (Cf), Swelling inflorescence buds (Sib), Full flowering (Ff), and Endocarp hardening (Eh). The most relevant result was noted with the extract prepared from the leaves collected at the Sib-stage. Results showed that treatment of newly emerged larvae resulted in a significant mortality with a dose-response relationship. The olive leaf extracts toxicity was also demonstrated by histopathological changes in the alimentary canal resulting in a considerable disorganization and serious damage of the midgut, ceca, and proventriculus structure. Epithelial cells alterations, less dense and degraded striated border, disintegrated regeneration crypts, vacuolarized cells, extrusion of cytoplasmic contents, and rupture of muscular layer were evident in the midgut and ceca of treated larvae. Data of biochemical analyzes showed that olive leaf extracts induced a significant decrease of the hemolymph metabolites (proteins, carbohydrates, and lipids). In a second series of experiments, we showed that the olive leaf extracts reduced the activity of acetylcholinesterase and induced the glutathione S-transferases with a dose-response relationship.

[Multiple arterial thrombosis in Behçet's disease]. / Thromboses artérielles multiples au cours de la maladie de Behçet.

Behcet's disease (BD) is a multisystemic vasculitis. Its etiopathogeny remains unknown. Vascular involvement in BD is frequent and venous thrombosis is the most common manifestation (30% of cases). Arterial involvement is rare (2.7 to 7%). The latter is often severe and considered as a life threatening complication. Pathogenesis of thrombosis occurring in BD remains unclear. We report a 45-year-old man, from south of Tunisia, who presented a BD with a bifocal arterial involvement: right internal carotid thrombosis and bilateral proximal thrombosis of the two pulmonary arteries. Therapeutic strategies to address this multiple arterial involvement and the pathogenesis of thrombosis raise many questions.

Tunisian hereditary spastic paraplegias: clinical variability supported by genetic heterogeneity.

Hereditary spastic paraplegias (HSP) constitute a clinically and genetically heterogeneous group of neurodegenerative disorders characterized by slowly progressive spasticity of the lower extremities. We performed the first clinical, epidemiological and genetic study of HSP in Southern Tunisia. We investigated 88 patients belonging to 38 unrelated Tunisian HSP families. We could establish the minimal prevalence of HSP in the district of Sfax at 5.75/100,000. Thirty-one percent of the families had a pure HSP, whereas 69% had a complicated form. The mode of inheritance was almost exclusively compatible with an autosomal recessive trait (97%, 37/38). Taking into account previously published results and new data generated in this work, genetic studies revealed significant or putative linkage to known HSP loci in 13 families (34.2%) to either SPG11 (7/38, 18.4%), SPG15 (4/38, 10.5%) or to SPG4 and SPG5 in one family each. The linkage results could be validated through the identification of two recurrent truncating mutations (R2034X and M245VfsX246) in the SPG11 gene, three different mutations (Q493X, F683LfsX685 and the novel S2004T/r.?) in the SPG15 gene, the recurrent R499C mutation in the SPG4 gene as well as the new R112X mutation in the SPG5 gene. SPG11 and SPG15 are the major responsible HSP genes in Tunisia.

[Cardiac involvement in Steinert myotonic dystrophy]. / Les anomalies cardiaques au cours de la dystrophie myotonique de Steinert.

INTRODUCTION: Myotonic dystrophy type 1 (DM1) is an autosomal dominant multisystemic disease with frequent cardiac involvement that may cause sudden death. This study was performed to determine the various cardiac manifestations in DM1, their frequency and the relevance of cardiac electrophysiological study in this disease. METHODS: Ten patients with DM1, five men and five women, mean age 44.3+/-7.8 years underwent neurological and cardiac assessments. RESULTS: The most frequent electrocardiographic findings were conduction abnormalities, essentially by intraventricular conduction defects (eight out of ten cases) such as bundle branch or fascicular blocks. Echocardiography showed alterations in systolic left ventricular function in two cases. Invasive electrophysiology testing showed sub-hisien block in three patients, requiring cardiac pacemaker implantation. These three patients had normal duration of PR interval and normal width of QRS complex. CONCLUSIONS: We recommend that all patients with DM1 should undergo cardiac investigation to detect subclinical cardiac involvement.

[Relevance of plasma exchange in the treatment of myasthenia gravis: study of 11 cases]. / Apport des échanges plasmatiques dans le traitement de la myasthénie: étude de 11 cas.

BACKGROUND: The use of plasma exchange (PE) constituted an advance in the treatment of myasthenia. The objective of our study was to determine the relevance of PE in the treatment of myasthenia and to study the different complications which can be observed during PE. PATIENTS AND METHODS: We studied retrospectively 11 patients who have generalized myasthenia and underwent PE. We used an intermittent flow cell separator and we performed PE three times a week. Biological assessment was performed before and after PE for all patients. The exchange volume was calculated according to the patient weight, gender and the value of hematocrit. RESULTS: Our series included six women and five men. The mean age at onset of the disease was 41.4+/-14.1 years (range: 18 to 68). Indication of PE was myasthenia crisis (eight cases), resistance to classic treatment (two cases) and exacerbation after thymectomy (one case). An improvement was observed rapidly in five cases and delayed in three cases. The remaining three patients did not improve. The most frequent side effects of PE were hypotension (four cases), heart arrhythmia (two cases) and hypoglycemia (one case). Three patients dead in the seven days after the first PE. CONCLUSION: PE represents an interesting tool to treat severe forms of myasthenia and improve prognosis. High incidence of complications in our series can be explained by the initial disease severity, the used method of PE, the existence of associated illness, and a long stay in intensive care unit.

[Familial spastic paraplegia with severe amyotrophy of the hands. (Silver syndrome?)]. / Paraplégie spastique familiale avec amyotrophie sévère des mains (syndrome de Silver?).

Familial spastic paraplegia (FSP) with severe muscular atrophy of hands and feet is exceptional. Autosomal dominant forms were initially described by Silver in 1966. We report two cases, from the same Tunisian family, presenting FSP with severe amyotrophy of the hands. A brother and his sister, aged respectively 37 and 36 years old, presented practically the same clinical picture. Their parents were cousins. The female patient was hospitalized. Both patients developed gait disorders around the age of three years. Muscular atrophy of the hands arose much later, around the age of 20 years. The neurological examination disclosed a spastic gait with distal amyotrophy, severe in the hands and moderate in the feet. Sensitivity was preserved and there was no fasciculation. The spinal cord and cerebral MRI was normal. Electromyography (EMG) showed a neurogenic pattern in the distal muscles. Stimulation of the median, ulnar and sciatica nerves was ineffective. The somatosensory evoked potentials (EP) were delayed (upper limb) or desynchronised (lower limb). The auditory and visual EP were normal. The cerebrospinal fluid contained 1 mononuclear cell/mm3 and 10 mg protein/100 ml. Abnormalities of the cranio-vertebral junction, Arnold-Chiari malformation, syringomyelia and familial juvenile amyotrophic lateral sclerosis (ALS) were excluded and the diagnosis of Silver's syndrome was evoked.

Polysaccharide storage myopathy--case report and literature review.

Polysaccharide myopathy is a rare form of storage muscular disorder. The clinical picture of this particular form of myopathy is unspecific. We report a 62-year-old woman with late-onset progressive weakness and wasting, affecting proximal muscles of the four limbs and the girdles. No myalgia, dysphagia nor symptoms of cardiac failure were observed. Muscle biopsy revealed a vacuolar myopathy with accumulation of amylopectin-like polysaccharide. This material was strongly PAS-positive and diastase-resistant. At electron microscopy, the deposits were composed of non-membrane-bound filamentous and granular material surrounded by numerous mitochondria. No enzyme deficiency was found. Clinical presentation of our patient was similar to the 16 cases reported in the literature. She did not have myocardiopathy and her survival is much longer. Hypothetic mechanisms of polysaccharide accumulation are reviewed.

[Extra-adrenal pheochromocytoma: report of 4 cases]. / Les localisations extrasurrénaliennes du phéochromocytome: à propos de quatre cas.

Extra-adrenal pheochromocytoma is rare. The diagnosis must be evoked in signs of pheochromocytoma with absence of tumor of the adrenal. We report four cases of ectopic pheochromocytoma while specifying their clinical particularities and diagnosis procedure.

[Therapeutic aspects of renal abscess. Report of 50 cases]. / Aspects thérapeutiques des abcès du rein. A propos de 50 cas.

INTRODUCTION: Renal abscess often raises therapeutic problems. Based on their experience and a review of the literature, the authors propose a treatment plant for renal abscess. MATERIAL AND METHODS: The authors report 50 cases of renal abscess treated between January 1988 and September 1999 in the Department of Urology of Charles Nicolle Hospital in Tunis. The diameter of the abscess was less than or equal to 4 cm in 19 cases, between 4 and 10 cm in 29 cases and greater than 10 cm in 2 cases. Renal ultrasound and intravenous urography are an integral part of the initial morphological assessment. All patients were treated by intravenous antibiotics for a mean duration of 28 days. Complementary percutaneous drainage of the abscess was indicated in 25 patients. Surgical treatment was indicated in 13 patients in the presence of a large perirenal collection or severe sepsis, or following failure of percutaneous drainage. RESULTS: Antibiotics alone were proposed in only 17 patients with an abscess less than 4 cm in diameter and 90% of these patients were cured. Percutaneous treatment was performed in 25 patients with an 80% success rate; the other patients underwent open surgery. One patient died after surgery in a context of septic shock despite salvage nephrectomy. The other patients has a favourable outcome. CONCLUSION: The treatment of renal abscess is based on antibiotics alone or combined with a percutaneous or surgical drainage procedure depending on the size of the abscess and the clinical course. The authors describe their decisional flow-chart.

Sacral meningeal arteriovenous fistula supplied by branches of the hypogastric artery revealed by conus medullaris infarction.

OBJECTIVE: Spinal dural arteriovenous fistulas (DAVF), the most common vascular malformations of the spine, are usually supplied by branches of the intercostal or lumbar arteries. Rarely, the DAVF are fed by branches of the hypogastric artery. Only 12 such cases have been reported. CASE REPORT: A 28 year-old man presented with a 2-month history of micturition dysfunction and progressive weakness of the legs. Physical examination showed motor deficit of the lower limbs with brisk knee jerks, absent ankle reflexes and normal plantar reflexes. Cremasteric reflexes were absent. We noted hypoesthesia of the lower limbs with complete anesthesia of the perineum. MRI of the lumbo-sacral spine demonstrated an enlargement of the conus medullaris with high T2 signal intramedullary lesion. It showed also large intradural serpentine vessels. A left iliac angiogram disclosed a nidus of arteriovenous malformation (AVM) supplied by a lateral sacral artery and draining by two enlarged ascending perimedullary veins. No clinical improvement was observed after surgical removal of the AVM. CONCLUSION: The screening examination of choice for spinal DAVF remains MRI. When selective spinal arteriography is normal, we have to search for an unusual arterial supply particularly from the hypogastric artery.

[Cystic cervicofacial lymphangioma]. / Lymphangiomes kystiques cervico-faciaux.

A review of fifteen new cases of cystic cervicofacial lymphangiomas is presented. While discussing the different localizations of these malformations, the authors emphasize the relationships existing between this type of malformation and both the facial nerve and the vasculonervous axis of the neck. They recommend surgical management. 1-to-10 years' follow-up revealed one case of local recurrence associated with incomplete excision.

[Spondyloepiphyseal dysplasia tarda with progressive arthropathy. Apropos of 3 cases]. / Dysplasie spondylo-épiphysaire tardive avec arthropathies progressives. A propos de trois observations.

Two brothers and a sister with spondyloepiphyseal dysplasia tarda who subsequently developed severe generalised peripheral arthropathy are described. The condition appeared to be transmitted as an autosomal recessive trait.

[Genetic study of eight cases of spondylo-epiphyseal dysplasia tarda and its autosomal recessive transmission]. / Etude génétique de huit observations de dysplasie spondylo-épiphysaire d'apparition tardive et de transmission autosomique récessive.

Spondylo-epiphyseal dysplasia tarda is an hereditary disease of bone first described by Maroteaux in 1957. The disease affects only boys, then is transmitted on X-linked recessive pattern. The authors describe two tunisians families with 8 patients presenting clinical and radiologic symptoms resembling spondylo-epiphyseal dysplasia tarda but affecting both sexes. Autosomal recessive inheritance is probable in these families. Conclusion is that genetic heterogeneity of the disease must be precise.