M. Adnaoui

Les troubles neurologiques par carence en vitamine B12 : étude rétrospective de 26 cas

Purpose. – Neurological symptoms of B12 vitamin deficiency are polymorph. Causes are dominated by Biermers disease and B12 vitamin non dissociation that is frequent in the elderly. Methods. – We realized a retrospective study during 11 years. Patients with neurological symptoms associated to megaloblastosis were included. Treatment with B12 vitamin lead to haematological manifestation regression. Results. – 26 cases were analyzed. Mean age was 50 ± 14.5 years and there were 11 women and 15 men. Neurological signs included combined medullar sclerosis (N = 10), peripheral neuropathy (N = 10), isolated paresthesia (N = 5) and inferior limb pyramidal syndrome (N = 1). Neurological signs revealed vitamin B12 deficiency in 4 cases. Mean haemoglobin rate was 6.2 ± 2.6 g/dl, mean MCV was 109 ± 56 fl. Eight patients had macrocytic anaemia, nine bicytopenia and eight deep pancytopenia. Electromyography (N = 8) confirmed neuropathy and medullar MRI (N = 2) showed antero-posterior cordonal demyelisation of cervico-dorsal medulla. Causes of B12 vitamin deficiency were Biermers disease (N = 11), non dissociation of B12 vitamins syndrome (N = 8) and partial gastrectomy (N = 1). In 6 patients, no aetiology was found. Treatment with parenteral vitamin B12 induced neurological symptoms regression in 14 cases. Patient with neurological disorder had significantly higher platelet count and haemoglobin level as compared with patients without neurological disorder. Reticulocyte crisis was more precocious in patients with neurological disorders. Conclusion. – Neurological symptoms in vitamin B12 deficiency are frequent. We insist on isolated forms, inaugural forms and on the interest of medullar MRI for early diagnosis.

Contribution of spinal MRI for unsuspected cobalamin deficiency in isolated sub-acute combined degeneration

Sub-acute combined degeneration (SCD) is a rare cause of demyelination of the dorsal and lateral columns of the spinal cord and is a neurological complication due to vitamin B12 deficiency. Revealing forms of SCD without anemia are rare. We report a case of SCD of the spinal cord in a 33-year-old woman without anemia but with a 10-month history of paresthesis and urine imperiosity. Magnetic resonance imaging (MRI) of the spine showed intramedullary hyperintensity seen on T2-weighted images in the posterior column of the cervico-dorsal spinal cord, extending from C1 to D1. A diagnosis of SCD of the spinal cord was considered and confirmed by a low serum cobalamin. The patient was treated with vitamin B12 supplements and showed gradual improvement in her clinical symptoms.

Association anévrysme de l'artère pulmonaire, thromboses intracardiaques et anticorps antiphospholipides au cours de la maladie de Behçet. À propos d'un cas

INTRODUCTION Cardiac thromboses are unusual in the course of Behçets disease and are frequently associated with endomyocardial fibrosis of the right heart. Vascular pulmonary involvement with either pulmonary aneurysm or parenchyma alterations is also often observed. However, pathogenesis of thromboses occurring in the course of Behçets disease is still unclear. CASE REPORT The authors report the case of a 28-year-old man who presented with Behçets disease accompanied by pulmonary aneurysm, multiple thrombi of the right heart and antiphospholipid antibodies. CONCLUSION The choice of therapeutical strategies for curative treatment of this association as well as the interpretation of the role of antiphospholipid antibodies in this polyvascular disease raises questions.

Article originalLes troubles neurologiques par carence en vitamine B12 : étude rétrospective de 26 casNeurological manifestations of vitamin B12 deficiency: a retrospective study of 26 cases

Purpose. – Neurological symptoms of B12 vitamin deficiency are polymorph. Causes are dominated by Biermers disease and B12 vitamin non dissociation that is frequent in the elderly. Methods. – We realized a retrospective study during 11 years. Patients with neurological symptoms associated to megaloblastosis were included. Treatment with B12 vitamin lead to haematological manifestation regression. Results. – 26 cases were analyzed. Mean age was 50 ± 14.5 years and there were 11 women and 15 men. Neurological signs included combined medullar sclerosis (N = 10), peripheral neuropathy (N = 10), isolated paresthesia (N = 5) and inferior limb pyramidal syndrome (N = 1). Neurological signs revealed vitamin B12 deficiency in 4 cases. Mean haemoglobin rate was 6.2 ± 2.6 g/dl, mean MCV was 109 ± 56 fl. Eight patients had macrocytic anaemia, nine bicytopenia and eight deep pancytopenia. Electromyography (N = 8) confirmed neuropathy and medullar MRI (N = 2) showed antero-posterior cordonal demyelisation of cervico-dorsal medulla. Causes of B12 vitamin deficiency were Biermers disease (N = 11), non dissociation of B12 vitamins syndrome (N = 8) and partial gastrectomy (N = 1). In 6 patients, no aetiology was found. Treatment with parenteral vitamin B12 induced neurological symptoms regression in 14 cases. Patient with neurological disorder had significantly higher platelet count and haemoglobin level as compared with patients without neurological disorder. Reticulocyte crisis was more precocious in patients with neurological disorders. Conclusion. – Neurological symptoms in vitamin B12 deficiency are frequent. We insist on isolated forms, inaugural forms and on the interest of medullar MRI for early diagnosis.

Atteinte des artères des membres et maladie de Horton : à propos de cinq cas

INTRODUCTION Giant cell arteritis of the limbs is rarely reported. It may be underestimated because it is usually asymptomatic. The aim of this study was to describe the distinctive features of this involvement. METHODS Retrospective study of 50 patients with giant cell arteritis diagnosed from January 1985 to December 2007, satisfying the American college of rheumatology (ACR) classification criteria. All five patients had typical lesion at examination of temporal artery biopsy. Among these patients, those with ischemic manifestations of the limbs were selected. All patients disclosed elevated acute phase reactants and typical radiological findings. Temporal artery histopathology was made necessary to select patients. RESULTS Five female (mean age: 66.8 years) out of 50 patients (10%) with temporal arteritis presented with symptomatic limb arteriopathy. Limb arteritis was the presenting feature in four patients. Clinical presentation was similar to other non-inflammatory arteritis. One patient presented with involvement of her four limbs. Typical arteriographic abnormalities were presents in all patients (occlusion or moniliform stenosis without atheromatous lesions). Despite therapy with corticosteroids associated to anticoagulants or antiplatelet drugs, four amputations were necessary. CONCLUSION Specific limb involvement may be the presenting feature of temporal arteritis. It can mimic arteriosclerosis vascular disease, particularly prevalent in this age group.

Les complications oculaires au cours de l’infection par le VIH : expérience du pôle d’excellence Nord du Maroc☆

INTRODUCTION HIV infection is associated with a wide variety of ophthalmic manifestations. The objective of this study was to identify the ocular complications of HIV/AIDS in Morocco. MATERIAL AND METHODS A retrospective study conducted in the internal medicine department of the Rabat Teaching Hospital between 1998 and 2008. All HIV-positive patients were retained for the study. Each patient had an exhaustive ocular examination. RESULTS Of 115 ophthalmologic examinations, 28 were abnormal. Twenty-seven patients had stage C HIV infection and one patient was in stage A. The ocular manifestations were: 21 cases of ocular HIV-related anomalies (microangiopathies), four cases of cytomegalovirus (CMV) retinitis, one case of toxoplasmosis chorioretinitis, one case of CMV retinitis associated with toxoplasmosis chorioretinitis, two cases of Cryptococcus neoformans infection, two cases of varicella-zoster virus (VZV) retinitis, and one case of conjunctival Kaposi sarcoma. The CD4 count average was of 86±91/mm(3). Twenty-six patients had a CD4 count lower than 200/mm(3). Progression was favorable in 85 % of the cases, with three cases of blindness: bilateral in a case of VZV retinitis and unilateral in two cases of CMV retinitis and toxoplasmosis chorioretinitis. CONCLUSION CMV infection is the main ocular opportunistic infection in our series. However, Highly Active Antiretroviral Treatment (HAART) may be the cause for the decline in the prevalence of ocular diseases and visual impairment in HIV/AIDS.

Kikuchi-Fujimoto disease: report of 4 cases and review of the literature.

INTRODUCTION Kikuchi-Fujimoto disease or histiocytic necrotizing lymphadenitis is a benign disease of unknown origin predominantly affecting young women and presenting in the form of cervical lymphadenopathy and/or prolonged fever. MATERIAL AND METHODS The authors report 4 cases of Kikuchi-Fujimoto disease diagnosed in the Internal Medicine Department of Ibn Sina university hospital in Rabat between 2009 and 2010. RESULTS These 4 women with a mean age of 27±8.6years [16-37] were admitted with febrile syndrome and cervical lymphadenopathy. The diagnosis was based on histological examination of a lymph node biopsy. The disease was associated with systemic lupus erythematosus in one case and actinomycosis in another case. A favourable course was observed in response to corticosteroid therapy in two patients, antibiotic therapy in one patient and antipyretic treatment alone in the fourth patient. CONCLUSION In the light of these four cases, the authors discuss the diagnostic difficulties, the modalities of treatment of Kikuchi-Fujimoto disease and its clinical course.

PS8:156 Hemophagocytic syndrome in systemic lupus erythematosus. a monocentric study of 20 cases

Objectives We reviewed the medical records of adult patients with SLE for a recent 8 years period and identified patients who had developed HS. The diagnosis of SLE was made using ACR criteria (4 or more criteria) and HS has been diagnosing dusing Hunter criteria (5 or more). We conducted statistical analyses to identify the characteristics of those patients in comparison with SLE patients without HS. Results Among 208 consecutive lupus patients, 20 patients (19 women) was identified having HS. The mean age of patients was 35.4+11.4 years (21–68). HS revealed lupus in 7 patients; in the others the delay between diagnosis of SLE and HS was 33 months (1 months – 108). Fever, pericarditis and splenomegaly were found in 95%, 70% and 50% of patients at presentation of HS. Bone marrow aspiration indicated hemophagocytosis in all patients. Cutaneo-mucous and arthritis were present in 95% and 70% of patients at presentation of HS. Bicytopenia or pancytopenia, high C-reactive protein level (mean 74 mg/L) and hyperferritinemia (mean 8687 ng/ml), hypertriglyceridemia (mean 4.35 g/L) were present in all patients. All patients had anti-nuclear and. Anti-double-stranded DNA antibodies were present in all patients. Serum complement C3 was low in 17 patients. HS was associated with a lupus flare in 10 patients. Infections was diagnosed in 11 patients. The most commonly used therapy was corticosteroids, which were initially administered in all patients. Immunosuppressant therapy was used with corticosteroids in 9 patients. Intravenous immunoglobulin was given in 4 cases and Rituximab in one patients. Anti-tuberculosis treatment was the first line treatment in 5 patients. All patients had a good outcome without any mortality with a mean follow-up of 12 months. Compared with SLE patients without HS, those with HS was significantly older and showed more oral ulcerations, a higher serum C-reactive protein level, a higher ferritinemia, higher SLEDAI and H score and, splenomegaly. Conclusion HS was observed in 9,26% Moroccan patients with SLE. Recognition of the cause of HS was particularly challenging because it may mimic the clinical features of the under lying disease or be confused with an infectious complication.

238 Hemophagocytic syndrome in systemic lupus erythematosus : a monocentric review of 13 cases

Background and aims In recent years hemophagocytic syndrome (HS) has been increasingly reported in patients with systemic lupus erythematosus (SLE). Methods We reviewed the medical records of adult patients with SLE and HS for a recent 6 years period (2010–2015). The diagnosis of SLE was made using ACR criteria and of HS using Hunter criteria. Results Among 110 consecutive patients, 13 (12 women) was identified having HS. The mean age was 37.69+/-11.4 years (21-68). HS revealed lupus in 3 patients. Fever, pericarditis and splenomegaly were found in 100%, 54% and 46% at presentation of HS. Bone marrow aspiration indicated hemophagocytosis in all patients. Laboratory features were bicytopenia or pancytopenia, high C-reactive protein level (mean 93 mg/L) hyperferritinemia (mean 11.082 ng/ml), hypertriglyceridemia (mean 4.2 g/L) in all patients. All patients had anti-nuclear antibodies when the HS occurred. Serum complement C3 was low in 10 patients. HS was associated with a lupus flare in 8 patients. Infections was diagnosed in 11 patients. Both conditions was considered present in 6 patients. Corticosteroids were initially administered in all patients. Immunosuppressant therapy was used together with corticosteroids in 7 patients. Intravenous immunoglobulin was given in 3 cases. Anti-tuberculosis treatment was used also as first line treatment in 4 patients with life threatening presentation. All patients had a good outcome with a mean follow-up of 25 months. Conclusions The occurrence of HS was most frequently associated with the SLE disease activity and bacterial infection. Profound cytopenia, high SLEDAI score are the characteristics of SLE patients with HS in our series.

[Biological disturbances during the lupus-associated pancreatitis: case report].

We report in this paper the case of female patient, hypertriglyceridemia associated with milky serum and hyperglycemia have been the alarm signal of a lupus-associated pancreatitis, the confirmation of this entity was done with elevated rate of serum lipase activity. It is about a 33 years age female. She has as unique antecedent a lupus diagnosed on January of the same. The patient was admitted on august 2013 for another episode of lupus associated to the lower lamb edema with a rate of C3 at 0.4 g/L (0.82-1,93) and C4 at 0.05 g/L (0.15-0.57). One day after the beginning of the corticotherapy, the patient presented hyperthermia, ataxis and behavior troubles, epigastric and articular pains and vomiting. Biochemical tests found hyperglycemia at 38.9 mmol/L (3.9-6.1), dyslipidemia with hypertriglyceridemia at 15.7 mmol/L (0.3-1.7) and total cholesterol rate at 5.2 mmol/L (<5.2) associated with milky serum. Haematological tests objective normocytic normochromic anemia with 81 g/L of hemoglobin, lymphopenia at 0.88 G/L and normal platelet rate. Lupus associated pancreatitis was suggested and confirmed biologically with an hyperlipasemia at 180 UI/L (8-78) and radiologicaly with the image of focal hepatic steatosis. We conclude that on the presence of lupus, gastrointestinal and/or biological signs must motivate the measurement of the serum lipase activity as quickly as possible to assess the diagnosis of lupus-associated pancreatitis.