M. Maamar

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.

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.

Systemic lupus erythematosus associated with sickle-cell disease: a case report and literature review

IntroductionThe occurrence of systemic lupus erythematosus has been only rarely reported in patients with sickle-cell disease.Case presentationWe describe the case of a 23-year-old North-African woman with sickle-cell disease and systemic lupus erythematosus, and discuss the pointers to the diagnosis of this combination of conditions and also present a review of literature. The diagnosis of systemic lupus erythematosus was delayed because our patient’s symptoms were initially attributed to sickle-cell disease.ConclusionsPhysicians should be alerted to the possible association of sickle-cell disease and systemic lupus erythematosus so as not to delay correct diagnosis and initiation of appropriate treatment.

Gayet–Wernicke encephalopathy: A rare and serious complication of gastrointestinal involvement in HIV infection

A patient 58 years of age was admitted to our institution with vomiting, dysphagia and chronic diarrhea of 8 months’ duration. On clinical examination, the patient was cachectic with buccal candidiasis. Laboratory tests revealed hypocalcemia (70 mg/L), hypoprotidemia (54 g/L), hypocholesterolemia (0.6 g/L), hemoglobin at 8 g/dL, ferritin at 10 mg/L and lymphocyte levels of 200 cells/mm. Human immunodeficiency virus (HIV) serology was positive, with a CD4 count of 30/mm and viral load of 120,000 copies/mL. Digestive explorations revealed cytomegalovirus (CMV) colitis. Treatment with fluconazole and ganciclovir with antiviral triple therapy was started. The outcome was favorable. However, after 1 month, the patient presented with severe horizontal nystagmus and confusion. A cerebral computed tomography (CT) scan was normal. However, brain magnetic resonance imaging (MRI) revealed the characteristic findings of Gayet–Wernicke encephalopathy (GWE): namely, symmetrical high-signal intensities in the

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.

Extensive calcinosis cutis in a patient with systemic lupus erythematosus: An exceptional complication

Figure 1 Numerous small and extensive soft-tissue calcifications (A, B, C, D, E); on the scalp (C), on the upper limbs (E), and on the both thighs (D). Pathology (F) of the skin and subcutaneous tissue reveal patches of black-colored staining, indicating tissue calcification under microscopy (hematoxylin-eosin stain, 40 ) Calcinosis cutis is a disorder defined by skin and subcutaneous calcifications. It has been described in association with autoimmune diseases, particularly with dermatomyositis and systemic sclerosis. The association with systemic lupus erythematosus (SLE) is rare, only a few cases have been reported [1,2]. We reported a new case of SLE associated with extensive calcinosis cutis. A 38-year-old Moroccan woman with a history of SLE presented with subcutaneous nodules. She had been treated with corticosteroids and Mycophenolate Mofetil for 4 years for her skin (disseminated discoid systemic lupus), articular and renal (Class IV nephritis) disease. Physical examination revealed cicatricial and atrophic discoid lupus lesions, with subcutaneous nodules skin on the head (figure 1C), the anterior, posterior and lateral side of both thighs, upper and lower limbs (figure 1D and E). The nodules were firm, yellow-to-white. There were no signs of disease and the patient was pain-free. Plain radiographs showed an increase in density of the softtissues of the lower and upper limbs (figure 1A and B) consistent with diffuse calcinosis cutis. The calcinosis did not rely on the vascular frame and there were no signs of muscular or visceral involvement. Laboratory tests showed no signs of SLE activity. White blood cell, red blood cell counts, erythrocyte sedimentation rate, were normal. The ANA by indirect immunofluorescence 1:160, antiribonucleoprotein antibodies, SS-A (Ro), SS-B (La), Sm, Scl-70 and Jo-1 were negative, anti-DNA double-chained antibodies by means of Crithidia luciliae negative, anti-cardiolipins (ACL) IgM and IgG, b 2-glycoprotein I, IgM and IgG and lupoid inhibitor negative, anti-cytoplasmic antibodies by immunofluorescence negative and total complement and C3 and C4 fractions were normal. There was no abnormal level of calcium, phosphorus, parathyroid hormone, 25-hydroxyvitamin D or muscle enzyme. The urine levels of calcium, phosphorus were also normal. There

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.

Immunological and Clinical Characteristics of Systemic Lupus Erythematosus: A Series from Morocco

Systemic Lupus Erythematosus (SLE) is a complex autoimmune disease with a high female predominance. To date, studies about SLE in Morocco are few. This retrospective study describes the clinical and immunological features in a series of 50 SLE Moroccan patients in University Hospital Center of Rabat, Morocco, between December 2011 and December 2013. All patients were screened for antinuclear antibodies (ANA) and anti-DNA antibodies by indirect immunofluorescence, followed by identification of anti-extractable nuclear antigen antibodies by ELISA. The female to male ratio was 6.1:1. Mean age was 31.72 years. The main clinical manifestations were arthritis (82%), mucocutaneous manifestations (80%), renal manifestations (50%), and hematological features (46%). Of the mucocutaneous features, the highest frequencies were observed in the malar rash (68%) and photosensitivity (60%). Of the hematological features, lymphopenia was most frequently observed in 30% of patients, followed by hemolytic anemia in 16% and leucopenia and thrombocytopenia in 8%. Central nervous system was involved in 10%. ANA were found in 88%, anti-DNA antibodies in 56%, and anti-Sm antibodies in 50%. Anti-SSA, anti-SSB, anti-Sm/RNP, and anti-Scl70 antibodies were detected in 38%, 10%, 48%, and 8%, respectively. Our data show that, in our patients, the main clinical and immunological features of SLE remain comparable to patients from other Arab countries.

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.