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Emicrania emiplegica sporadica in una paziente con sindrome poliendocrina
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A 46-year-old woman with a long history of headache came to our observation for suspected TIA. The patient was a homozygote carrier of mutation in MTHFR gene, she also suffered from polyendocrine syndrome. The temporal reconstruction of the events, the neurological examination and the instrumental tests allowed us to make the diagnosis of "sporadic hemiplegic migraine”. A malignancy of the kidney was diagnosed during the admission
MACROPHAGE ACTIVATION SYNDROME AS A PRESENTING FEATURE OF SYSTEMIC LUPUS ERYTHEMATOSUS
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ENDOCARDITIS AND LYMPHOMA: WHAT IS THE THERAPY ?
A 68 year old man presented with fever, anemia (6.8gr%), pancytopenia and weight loss. The fever, of 2 months duration, was characterized by daily intermittent peaks, accompanied by chills which did not respond to antimicrobial treatment. Chest X-ray was normal. Viral and serological investigations (Vidal-Writh and Weil-Felix, tests for leishmania and malaria) were negative. Blood cultures were positive for Staphilococcus Epidermidis. A transesophageal echocardiography revealed valvular vegetations. Abdominal ultrasonography and total body CT scan demonstrated splenomegaly and enlarged lymph nodes. A bone marrow biopsy was performed showing a non-Hodgkin large B cells lymphoma (HL) grade IV. Additionally the patient was HBV positive. The Immunologist was consulted and he prescribed combined treatment with Gentamicin, Rifampicin and Vancomicin for 6 weeks and once echocardiography resulted normal, to start antineoplastic treatment. Instead, the haematologist recomended only steroid treatment followed by one infusion of Vincristin and once healed endocarditis, to give four cycles of R-CHOP, combined with Lamuvidin. From a review of the literature, we decided to begin therapy with antibiotics, steroids, Vincristine and Rituximab in four doses and prophylaxis with Lamivudine [1,2]. The patient continued chemotherapy with a complete response after six months
Macrophage Activation Syndrome as Onset of Systemic Lupus Erythematosus: A Case Report and a Review of the Literature
Macrophage activation syndrome (MAS) is a potentially fatal condition. It belongs to the hemophagocytic lymphohistiocytosis group of diseases. In adults, MAS is rarely associated with systemic lupus erythematosus, but it also arises as complication of several systemic autoimmune disorders, like ankylosing spondylitis, rheumatoid arthritis, and adult-onset Still’s disease. Several treatment options for MAS have been reported in the literature, including a therapeutic regimen of etoposide, dexamethasone, and cyclosporine. Here we report a case of 42-year-old woman in whom MAS occurred as onset of systemic lupus erythematosus
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A 49- year-old woman had a clinical history that began at age 35, characterized by the presence of anemia, increased C-reactive protein (CRP) and Erythrocyte sedimentation rate (ESR), hypergammaglobulinemia, symmetric polyarthritis involving the small joints in the hands and wrists, treated which resulted with improvement of symptoms. She was hospitalized in March because of fever and dyspnea. The chest CT scanning revealed interstitial pneumonia and pleural effusion, she was treated with antibiotic therapy for about 10 days. A medical examination showed fever, chest pain and cardipalmo and she was again hospitalized. At the clinical examination, she appeared unwell, febrile at 39 oC, with a widespread vasculitic rash was present and most prominent on the legs. At the physical exam of the chest: dullness to percussion at the right base, with increased tactile fremitus and bilateral basal crackles. No evidence of lymphadenopathy was observed and the rest of the examination was unremarkable. During the hospitalization she presented pancitopenica, serum C3 level was decreased and antinuclear antibody, anti-dsDNA antibody, anti-SS-A, anti-SS-B, anti-Sm and anti-RNP antibody were positive. Futhermore she showed hyperferritinemia, hypofibrinogenemia, liver dysfunction with hypertriglyceridemia and raised liver enzymes, splenomegaly and bone marrow aspirate showed hemophagocytosis. Repeated urine and blood cultures were negative. Sputum was negative for bacterial and fungal cultures. A thorough infection screen, including a viral panel for Herpes Simplex, Herpes Zoster, Epstein-Barr, Cytomegalovirus, Hepatitis B, Hepatitis C, HIV, Echo and Coxsackie viruses was negative. She was diagnosed having Systemic lupus erythematosus (SLE) associated with Macrophage activation syndrome (MAS).
She was treated only with corticosteroids (metilprednisolone) with improvement of symptoms
A case of tension-type headache in fibromyalgia.
Ref.: Ms. No. TJHP-D-10-00029R1
A 57 years-old-woman was admitted to our ward for a daily tension-type headache, non responsive to usual pharmacological treatment. Five years ago she underwent a hysterectomy. Since then, she referred muscular rigor of the neck and the shoulder girdle, intense constrictive pain localized in the occipital spine. She also reported weakness of the upper and lower limbs, tingling, tremors and difficulties in walking and climbing. She referred widespread pain, unusually severe, above all at joints and muscles, without any sign of inflammation at clinical examination. The diagnosis of a connective tissue was excluded, remaining the diagnosis of tension-type headache in Fibromyalgia the most probable one. The patient has been treated with antidepressants, anxiolytics, and antiepileptic drugs with improvement of the symptoms
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