[Neurological manifestations of Behcet's disease: 22 cases among 170 patients]. / Etude de 22 observations de neuroBehçet dans une série de 170 maladies de Behçet.

OBJECTIVES: To present neurological involvement in Behçet's disease, its prognosis and treatment. METHODS: Our study was retrospective and was done at Hotel-Dieu Hospital of Beirut between 1980 and 2005. All these patients fulfilled the International Study Group criteria for diagnosis of Behçet's disease. RESULTS: Neurological involvement was observed in 13% (22/170) of our patients and was more frequent in men (sex-ratio: 1,75). The mean age of onset for Behçet's disease and NeuroBehçet's syndrome was 26+/-6 and 30+/-8 years respectively. Central nervous system involvement was found in 21 patients and peripheral nervous system involvement in one. Meningoencephalitis and/or transverse myelitis were found in 57% (12/21) of cases (in association with brainstem syndrome in 2 of these cases), brainstem syndrome without meningoencephalitis in 5 cases, tumor-like syndrome in 2 cases, repetitive ischemic attacks in 1 case and cerebral venous thrombosis in one. Focal deficits were the major presenting signs (16 cases) and external oculomotor nerve paralysis was observed in 4 patients. In meningoencephalitis, the cerebrospinal fluid findings were lymphocytic pleocytosis and elevated protein level. CT Scan, performed in 6 patients, was normal in 33% of cases. MRI, performed in 9 patients, was abnormal in 6 and showed abnormal signals distributed over the brainstem and the thalamus in 4, a tumor-like lesion and thrombosis of the left lateral sinus one each. Corticosteroids were usually efficacious but, when used alone, relapse was observed in 31% of patients. One patient who had brainstem syndrome died within 18 months because of a delayed corticosteroid treatment. CONCLUSION: Within central neurological involvement in Behçet's disease, we can individualize 4 clinical aspects: meningoencephalitis (and/or myelitis), brainstem syndrome, tumor-like features and cerebral venous thrombosis. Abnormalities, observed on CT Scan and MRI, by their brainstem localization and their multiplicity, should evoke the diagnosis. Corticosteroids, when prescribed early, are useful and are associated with better prognosis; their association to immunosuppressant agents should be considered in the parenchymatous forms.

[Giant mediastinal cystic lymphangioma: clinical case and review of the literature]. / Lymphangiome kystique médiastinal géant. Un cas clinique et revue de la littérature.

Benign cystic lymphangioma is a relatively rare tumor of the human body. It's more frequent in young patients. It is usually localized in the neck but may have mediastinal or intra-abdominal localization. It's usually asymptomatic with good prognosis after total excision. We report in this article the case of a young man with a huge mediastinal cystic lymphangioma which was successfully operated and a review of the literature.

[Multisystemic amyloidosis. Clinical study of 39 patients in Lebanon]. / Amyloses multisystémiques. Etude clinique de 39 patients au Liban.

OBJECTIVE: Clinical analysis of multisystem amyloidosis in Lebanon, by histological type. METHOD: Retrospective study of 39 cases of multisystem amyloidosis diagnosed histologically in a university hospital center between 1991 and 2002. It analyzed the following clinical data: age, gender, type of presentation, time from symptom onset to diagnosis, clinical features, concomitant diseases, family history of amyloidosis, biopsy sites, presence of urinary or serum monoclonal gammopathy, immunohistochemical type, prognosis and treatment. RESULTS: Median age at diagnosis was 56+/-18 years. The overall ratio of men to woman was 1.4. AL amyloidosis (amyloid light chain) accounted for 54% (21/39) of the cases, AA (amyloid-associated) amyloidosis 36% (14/39), while 10% (4/39) were not typed. Among the 21 cases of AL amyloidosis, 12 were idiopathic (57%) and 9 (43%) were associated with multiple myeloma; among the 14 cases of AA amyloidosis, 7 were associated with familial Mediterranean fever and 5 with chronic disorders. Proteinuria was often the first symptom. The initial manifestations in AL amyloidosis patients with myeloma were more often related to amyloidosis than to myeloma. Renal involvement was seen in 95% (37/39) of all cases (95% of AL versus 93% of AA), proteinuria in 87% of cases and renal failure in 72%. Cardiac amyloidosis (57% of AL versus 7% of AA; p>0.05), infiltration of the tongue (19% of AL versus 0% of AA; p>0.05) and neurological manifestations (24% of AL versus 7% of AA; p>0.05) were more frequent in AL amyloidosis. The 7 patients who died (18%) had AL amyloidosis (5 of them had myeloma). Heart failure was the most frequent cause of death related to amyloid. CONCLUSION: Multisystem amyloidosis is frequent in Lebanon and familial Mediterranean fever is still frequently associated with the secondary type. Accurate diagnosis and classification are essential for the prognosis and treatment of the disease. Poor prognosis was associated with the AL type, especially when accompanied by myeloma, and with cardiac amyloidosis.

[Clinico-pathological correlation--Lebanese experience]. / Biopsie renale : corrélation clinico-pathologique Expérience Libanaise.

This is a retrospective and descriptive study done at an academic hospital center. It collects data about 143 patients admitted during 10 years at the hospital, between January 1989 and December 2000, to have renal biopsy for suspicion of glomerulopathy. The glomerulonephritides most frequently present were: mesangial proliferative glomerulonephritis (15%), membrano-proliferative glomerulonephritis (13%), chronic glomerulonephritis (13%), membranous glomerulonephritis (8%), proliferative focal and segmental glomerulonephritis (8%) and scleronodular focal glomerulonephritis (8%). Patients with arterial hypertension represented 89% of patients with chronic glomerulonephritis and 54% of patients with scleronodular focal glomerulonephritis. Those with heavy proteinuria represented 82% of patients with primarily membranous glomerulonephritis, 66% of patients with minimal change disease and 44% of patients with membrano-proliferative glomerulonephritis. Those with active urinary sediment have had mostly membrano-proliferative glomerulonephritis, rapidly progressive glomerulonephritis, postinfectious glomerulonephritis and proliferative mesangial glomerulonephritis. High rates of evolution to end-stage renal disease, renal transplantation or dialysis were mostly depicted between patients with scleronodular focal glomerulonephritis (91% of cases), chronic (89% of cases) and membrano-proliferative (72% of cases) glomerulonephritis.

Vascular manifestations of Behçet's disease. Eighteen cases among 140 patients.

OBJECTIVES: To describe the features, prognosis, and treatment of vascular involvement in Behçet's disease (BD). PATIENTS: Among 140 patients with BD seen at the Hôtel-Dieu Hospital in Beirut between 1980 and 2000, 18 (13%) had vascular involvement and were included in this retrospective study. All these patients fulfilled International Study Group criteria for BD. RESULTS: Men with BD were more likely to have vascular involvement (13/77, 17%) than women (5/63, 8%) (P = 0.12) and were younger at diagnosis of vascular disease (32 +/- 7 vs. 36 +/- 7.5 years; P < 0.01). Many patients had vascular disease at more than one site: 17 had thrombophlebitis, 10 had arterial thromboses, and one had an aneurysm. Thrombophlebitis was more common in men (82% vs. 18%; P < 0.03) and arterial occlusion in women (70% vs. 30%; P > 0.05). Caval thrombosis and arterial occlusions were the most serious complications. Combined treatment with glucocorticoids, anticoagulants, and immunosuppressants was effective in superior vena cava syndrome and extracranial arterial occlusion. CONCLUSION: Vascular manifestations of BD are common in Lebanon, particularly venous lesions. Aneurysms are seen less often than arterial occlusions. Medical treatment may be sufficient in superior vena cava syndrome and arterial occlusion.

[Typhoid fever. Retrospective study of 52 cases in Lebanon]. / Fièvre typhoïde. Etude rétrospective de 52 cas observés au Liban.

OBJECTIVE: Present the epidemiology and clinical characteristics of typhoid fever in the Lebanon. METHODS: Retrospective study of 52 patients admitted to a university hospital center between 1995 and 1999. The criteria for inclusion were a positive Salmonella typhi or paratyphi hemoculture and/or Widal serodiagnosis > 1/160 for O agglutinin, in the presence of evocative symptoms. RESULTS: The patients were aged a mean of 31 +/- 24 years. The mean duration between the onset of fever and diagnosis was of 10 +/- 8 days. Fever was observed in 96% of cases and the other predominant symptoms were diarrhea (37%), abdominal pain (31%) and headache (29%). Feverish gastroenteritis is a frequent manifestation in children (61% of cases). Complications were noted in 33% of cases and were predominantly digestive. Leukopenia is not a good diagnostic marker. S. typhi was the cause in 83% of cases. Resistance to ampicillin was noted in 13% of cases, to cotrimoxazole and to chloramphenicol in 10% and to ofloxacine in 3% of cases. One death was reported (2%) of an immunodepressed patient. CONCLUSION: Typhoid fever is still an endemic disease in the Lebanon and should be systematically evoked in the case of prolonged fever, feverish gastroenteritis and/or headache. The appearance of bacteria resistant to antibiotics makes ceftriaxone or ciprofloxacine the empirical treatment of choice.

[Acute deafness after carbon monoxide poisoning. Case report and review of the literature]. / Surdité aiguë par intoxication au monoxyde de carbone. A propos d'un cas et revue de la littérature.

A 61-year-old man developed an extrapyramidal syndrome 15 days after an episode of carbon monoxide poisoning. Two months later he developed severe deafness. Brain imaging revealed ischemic lesions in the region of the basal ganglia. Audiometric studies disclosed neurosensorial deafness. The patient died nine months later due to respiratory complications.

[Peritoneal pseudomyxoma: two case reports and review of the literature]. / Pseudomyxome péritonéal: a propos de deux cas et revue de la littérature.

In this article, we present two cases of peritoneal pseudomyxoma suspected on abdominal CT Scan and then confirmed on pathologic examination. A mucinus carcinoma was the primary lesion in the two cases, appendicular in the first case and with an undeterminate origin in the second. The prognosis was bad in these cases despite the debulking surgery and the systemic and intraperitoneal chemotherapy.