It is also known as hemophagocytic syndrome, defined as pathology group characterised in common by the proliferation and the accumulation of macrophages in the bone marrow and the lymphoid system, leading to an abnormal phagocytosis of the blood cells and the production of proinflammatory cytokines. It has a non specific clinical picture as well as a perturbed laboratory findings. We differentiate the hereditary primary froms and the secondary related to several etiologies. Its pathophysiology is not yet clear and the treatment remains poorly codified. It is a serious life-threatening disease but recent advances in the comprehension of its physiopathologic mechanisms will allow for a better adapted treatment and improve the survivial rate of these patients.

It is the most frequent location of the primary digestive tract lymphoma , originally from the lymphoid tissue of the digestive tract or MALT. We distinguishe two types: the marginal zone lymphoma of malt and diffuse large B cell lymphoma .The clinical symptoms are not specific and the diagnosis depend on the biopsy results. The disease staging is systematic looking for remote associated nodal or visceral involvement. The role of the Helicobacter pylori recently implied in the genesis of these malignant lymphomas reopened all the debates including the therapeutic. The discovery in the latter years of anatomopathological, immunohistochemical and molecular diagnosis tools and also new efficient therapeutic strategies contributed to improve the prognosis. The treatment is based on the helicobacter pylori eradication. The marginal zone lymphoma treatment is consensual and well codified, chemotherapy and /or radiotherapy can be proposed if no improvement. For the diffuse large B cell lymphoma the chemotherapy is the standard treatment associated or not with surgery or radiotherapy. The addition of the CD 20 antibody can improve the results.

Linfection a cryptocoque est une complication redoutable chez les patients traites par immunosuppresseurs et dont levolution peut etre rapidement fatal en cas de retard diagnostic. Nous rapportons le cas dune patiente agee de 70 ans, ayant des antecedents de pemphigus vulgaire traite par prednisone et azathioprime et admise dans le service de medecine Interne pour des nodules sous cutanes atypiques. Le diagnostic retenu etait celui dune cryptococcose disseminee. Levolution etait rapidement fatale malgre le traitement antifongique.

Familial hypercholesterolaemia (FH) is an autosomal dominant lipoprotein disorder characterized by significant elevation of low-density lipoprotein cholesterol (LDL-C) and markedly increased risk of premature cardiovascular disease (CVD). Because of the very high coronary artery disease risk associated with this condition, the prevalence of FH among patients admitted for CVD outmatches many times the prevalence in the general population. Awareness of this disease is crucial for recognizing FH in the aftermath of a hospitalization of a patient with CVD, and also represents a unique opportunity to identify relatives of the index patient, who are unaware they have FH. This article aims to describe a feasible strategy to facilitate the detection and management of FH among patients hospitalized for CVD.A multidisciplinary national panel of lipidologists, cardiologists, endocrinologists and cardio-geneticists developed a three-step diagnostic algorithm, each step including three key aspects of diagnosis, treatment and family care.A sequence of tasks was generated, starting with the process of suspecting FH amongst affected patients admitted for CVD, treating them to LDL-C target, finally culminating in extensive cascade-screening for FH in their family. Conceptually, the pathway is broken down into 3 phases to provide the treating physicians with a time-efficient chain of priorities.We emphasize the need for optimal collaboration between the various actors, starting with a "vigilant doctor" who actively develops the capability or framework to recognize potential FH patients, continuing with an "FH specialist", and finally involving the patient himself as ”FH ambassador” to approach his/her family and facilitate cascade screening and subsequent treatment of relatives.

Mesenteric venous thrombosis causing small-bowel infarction is an extremely rare cause of acute abdomen and is often difficult to diagnose. Both congenital and acquired causes are responsible. Protein C deficiency is a rare genetic abnormality that predisposes the patient to thrombophilia and leads to thrombosis, often at unusual sites.

Kaposi ‘s sarcoma is the common neoplasm in patients with acquired immune deficieny syndrome (Aids). The purpose of this retrospective study is to determine the epidemiological, clinical, therapeutic features and the evolution of Kaposi’s sarcoma as an inaugurating event of Aids. Between january 2002 and decembre 2008, three cases were hospitalised in our departement. There were two men and one woman with an average age of 39 years. The diagnosis of Kaposi sarcoma established by clinical aspect and histological study. The lrsions were found in the skin and in the lymphatic nodes in all patients, moucous membranes in two patients (70%), in the gastrointestinal tract in one case and in the lung in one case. The average CD4 cells count was 200/mm3 Chemotherapy associated with Highly Active AntiRetroviral Treatment (HAART) was instituated in all patients. The evolution has been favourable in two cases and one patient died. He had a pulmonary Kaposi’s sarcoma. Our study demonstrates the severity and the disseminated charactere of Aids-associated Kaposi’s sarcoma,whose diagnosis is done at the advanced stages of HIV infection. Chemotherapy is expansive and induces serious adverse effects with difficult managements. An early screening would allow an early diagnosis of HIV infection and of a located Kaposi’s sarcom in which case Highly Active AntiRetroviral Treatment (HAART) is generally sufficient.

Infection by human immunodeficiency virus (HIV) is characterized by a myriad of clinical manifestations affecting almost every organ system in the body. During this infection, the immune system becomes dysfunctional because of the existence of immunodeficiency and immune hyperactivity, and a disregulated production or activity of cytokines. Some of these mechanisms explain the development of rheumatic manifestations associated with HIV infection. Highly active antiretroviral therapy has changed the course of HIV infection and the spectrum of the HIV-associated rheumatic manifestations.

The aim of the present study was to describe a case of Behçet's disease revealed by a recurrent meningitis and to review literature on these two conditions. We describe the case of a 25-year-old man who presented four episodes of recurrent meningitis without any locoregional cause and developed oral and genital ulcerations few months later. Behçet's disease is a chronic, multisystemic disorder with variable prevalence in different geographical areas. Its neurological manifestations are well recognized. Both central and peripheral nervous systems can be involved. Recurrent meningitis in Behçet's disease is exceptional. To our knowledge, only two cases reported recurrent meningitis as initial manifestation of Behçet's disease. This case report underscores another facet of neurological manifestations of Behçet's disease.

La paralysie faciale périphérique a frigore ou idiopathique, dite encore de paralysie de Charles Bell chez les anglo-saxons, représente plus de la moitié des causes de paralysies faciales périphériques de l’adulte. S’il s’agit d’une pathologie dite bénigne, son retentissement fonctionnel et psychologique peut être important. L’hypothèse physiopathologique principale est celle d’une réactivation du virus HSV-1 dans le ganglion géniculé à l’origine d’un œdème du nerf facial et sa compression dans l’aqueduc de Fallope, canal osseux inextensible. La symptomatologie, un déficit moteur d’intensité variable intéressant les muscles faciaux ipsilatéraux des territoires supérieur et inférieur, cesse de progresser le plus souvent en moins de 24 heures et toujours en moins de trois jours. Fréquemment, elle est précédée ou accompagnée de douleurs rétro-auriculaires et/ou de sensation d’engourdissement de l’hémiface concernée. Le diagnostic est clinique et s’appliquera à éliminer les diagnostics différentiels représentés par les paralysies faciales de cause centrale ou périphérique secondaire (infections, néoplasies, pathologies auto-immunes). Dans les trois-quarts des cas, l’évolution est spontanément satisfaisante avec une récupération complète. Une corticothérapie orale de courte durée instaurée précocement (dans les 72 heures) améliore encore les taux de récupération complète, alors que l’intérêt de l’associer à un traitement antiviral n’est pas démontré. Des spasmes hémifaciaux (contractions involontaires des muscles de l’hémiface) ou des syncinésies (contractions involontaires accompagnant une contraction volontaire en lien avec un phénomène de réinnervation aberrante) peuvent émailler son évolution. L’électroneuromyographie peut montrer le bloc de conduction, la perte axonale, puis la réinnervation, et aider au diagnostic des complications.Idiopathic peripheral facial palsy, also named Bell's palsy, is the most common cause of peripheral facial palsy in adults. Although it is considered as a benign condition, its social and psychological impact can be dramatic, especially in the case of incomplete recovery. The main pathophysiological hypothesis is the reactivation of HSV 1 virus in the geniculate ganglia, leading to nerve edema and its compression through the petrosal bone. Patients experience an acute (less than 24 hours) motor deficit involving ipsilateral muscles of the upper and lower face and reaching its peak within the first three days. Frequently, symptoms are preceded or accompanied by retro-auricular pain and/or ipsilateral face numbness. Diagnosis is usually clinical but one should look for negative signs to eliminate central facial palsy or peripheral facial palsy secondary to infectious, neoplastic or autoimmune diseases. About 75% of the patients will experience spontaneous full recovery, this rate can be improved with oral corticotherapy when introduced within the first 72 hours. To date, no benefit has been demonstrated by adding an antiviral treatment. Hemifacial spasms (involuntary muscles contractions of the hemiface) or syncinesia (involuntary muscles contractions elicited by voluntary ones, due to aberrant reinnervation) may complicate the disease's course. Electroneuromyography can be useful at different stages: it can first reveal the early conduction bloc, then estimate the axonal loss, then bring evidence of the reinnervation process and, lastly, help for the diagnosis of complications.

Le syndrome hypereosinophilique, defini par une hypereosinophilie prolongee sans cause connue, est une pathologie multisystemique rare de l'homme d'âge moyen. Il se manifeste par des atteintes souvent multiviscerales (poumons, systeme nerveux, foie, reins, peau) et dont l'atteinte cardiaque reste la plus frequente et la plus deletere. Nous rapportons 3 cas de syndrome hypereosinophilique colliges dans le service de medecine interne du CHU de Rabat.

Nous rapportons 162 cas de maladie de Behcet colliges dans un service de Medecine Interne de l'Hopital Ibn Sina de Rabat, entre janvier 1983 et juin 1996. Cette serie a comporte 124 hommes et 38 femmes, marocains, dont l'âge moyen au moment de la premiere hospitalisation etait de 32 ans, et l'âge moyen au moment du premier signe de la maladie de 26 ans. Le diagnostic a ete retenu d'apres les criteres de Mason et Barnes et/ou du Groupe International d'Etude sur la Maladie de Behcet. Ainsi, nous avons retrouve une atteinte cutaneo-muqueuse dans 100 % des cas, oculaire dans 50 % des cas, articulaire dans 45 % des cas, neurologique chez 43,2 % de nos malades, vasculaire dans 62,34 % des cas, mediastino-pulmonaire dans 13 % des cas, digestive dans 8 cas, cardiaque chez 5 patients, une fievre au long cours chez 5 malades et un cas d'amylose. Nous avons revu nos resultats a la lumiere des donnees de la litterature, et nous constatons que notre serie se singularise par une grande frequence de l'atteinte neurologique, en particulier de l'hypertension intracrânienne benigne et des thromboses veineuses profondes. Nous remarquons par ailleurs la rarete des atteintes digestives.