Systemic lupus erythematosus complicated with cerebral venous sinus thrombosis: a report of two cases

A rare manifestation of systemic lupus erythematosus (SLE) is cerebral venous sinus thrombosis (CVST), in which early diagnosis and aggressive therapy are of prime importance for favorable outcome. The pathogenesis of CVST is largely unknown, but it is thought to be caused by cerebral vasculitis, antiphospholipid antibodies or other conditions associated with enhanced coagulability. We describe two cases of SLE with CVST which were not associated with antiphospholipid antibodies. Both cases were treated with immunosuppressants (intravenous methylprednisolone and cyclophosphamide pulse therapy) and anticoagulant drugs (heparin and subsequent maintenance therapy with warfarin). There was a marked improvement of neurologic symptoms with the disappearance of thrombus in a follow-up MRI. The possibility of CVST should be considered in any patients with SLE who show neuropsychiatric manifestations.

Clinical manifestations of amanita subjunquillea poisoning / 대한내과학회지

BACKGROUND: To the best of our knowledge, the report on Amanita subjunquillea poisoning has not been found in the medical literature. We investigated the clinical aspects of Amanita subjunquillea poisoning. METHODS: Sixteen subjects who had ingested the mushroom (A. subjunquillea) were examined for clinical features, laboratory and radiologic findings prospectively. RESULTS: The mean incubation period was 11.5 hours(range: 3 to 17 hours). The initial presentations were gastrointestinal symptoms which persisted for 2 to 4 days. The transaminase levels were elevated in all subjects and peaked on day 3 after ingestion of the mushrooms (mean AST/ALT levels : 3241 IU/L and 3741 IU/L, respectively). Biochemical evidence of pancreatitis and disseminated intravascular coagulation were frequent (83.3% and 62.5%, respectively). Liver ultrasonography and scintigraphy revealed abnormalities in most cases. Massive hepatic necrosis was confirmed by liver biopsy in one subject. The overall mortality was 12.5%. CONCLUSION: Clinical manifestations of A. subjunquillea poisoning were similar to those of other poisonous Amanitaceae intoxication. However, the mortality rate was lower in A. subjunquillea poisoning. Our data showed strong evidence that A. subjunquillea should be classified in the group of poisonous mushrooms.

Amatoxins Poisonings in Korea / 대한간학회지

BACKGROUND/AIMS: From the review of Korean literature most fatal mushroom poisonings have been due to amatoxins with high mortality. So far there have never been investigations on the amatoxins poisonings such as annual incidence, mortality, common causal species, and endemic areas. This study was carried out to develop some basic statistics as part of studies for an effective management of amatoxins intoxications. METHOD: For the year 1999 authors collected cases of mushroom poisonings which had been mainly gathered from hospitals nation-wide. All of the cases with suggestive amatoxins poisonings were screened by symptomatology and laboratory findings. The causal species of mushrooms were identified grossly and microscopically. RESULTS: A total of 54 victims with mushroom poisonings were evaluated. The causal mushroom toxins were diagnosed or strongly suggested as amatoxins in 43 of 54 victims. Eleven of 54 victims did not conform to the category of amatoxins intoxication due to absent or minimal elevation of aminotransferase. Mean age of the victims was 44.3 23.3 (range: 7-78) with male predominance (1.2 : 1). The causal species were confirmed, or strongly suggested, as Amanita virosa in 25 victims, Amanita subjunquillea in 14, and unknown species in 4. Thirty-five out of a total of 43 were regarded as moderate to severe intoxication (AST or ALT >1,000 IU/L) with 20% mortality. Most fatal victims showed marked thrombocytopenia (40,000 19,000/mm3) compared to non-fatal victims (109,066 42,245/mm3). A total of 88.4% of victims was developed in the Kangwon and Kyungpuk provinces. Both are west of the Taebaek Mountains (38/43). CONCLUSIONS: Although the common causal species for amatoxins poisonings in Korea are different from European countries and North America, the mortality is similar to that of those areas. In order to further reduce the mortality, bedside diagnostic methods using biological fluids and more effective therapy for liver failure should be established.

Rheumatoid arthritis associated with myelodysplastic syndrome: a case report

Myelodysplastic syndromes (MDS) are a group of refractory anemias resulting from a clonal stem cell disorder often associated with cytogenetic abnormalities. There is increasing recognition of immunological abnormalities in patients with MDS, including defective B- and T-cell function, hyper- or hypogammaglobulinemia and monoclonal gammopathy. MDS have been associated with Sjogren's syndrome, polymyalgia rheumatica, relapsing polychondritis and systemic lupus erythematosus. Although there may be various rheumatologic features, including acute arthritis in MDS, chronic inflammatory arthritis is uncommonly combined. There have been a few reports that described cases of rheumatoid arthritis (RA) concurrent with MDS, but advanced rheumatoid arthritis with typical joint deformities has rarely been reported. We report a case of rheumatoid arthritis with atlantoaxial subluxation combined with refractory anemia in a 31-year-old woman.

A Case of Behcet's Disease Complicated with Deep Vein Thrombosis and Cerebral Venous Sinus Thrombosis / 대한류마티스학회지

Behcet's disease is a multisystemic disease, in which vascular involvement occurs in 7. 7-60% of patients. There are three forms of vascular involvement such as venous occlusion, arterial occlusion and arterial aneurysm. Venous lesions are more common than arterial lesions and the common sites of venous thrombosis are inferior vena cava, superior vena cava, and superficial or deep veins of extremities. Thrombosis of cerebral venous sinus is an infrequent complication of Behcet's disease and it seems to be rare that both deep vein thrombosis and cerebral venous sinus thrombosis occured at the same time. We experienced a case of Behcet's disease complicated with thromboses of left common femoral vein and right transverse sinus. A 29-year-old man presented with persistent dull nature headache, nausea, vomiting, blurred vision and left lower leg swelling. Duplex ultrasonography of lower extremity showed thrombus in the left common femoral vein. Magnetic resonance imaging of brain showed isodense signal intensity lesions in right transverse sinus on Tl weighted image. On magnetic resonance angiography, the right transverse, sigmoid sinus and right internal jugular vein were not visualized. The patient showed substantial improvement after treatment with steroid, cyclosporine A and anticoagulants.