Pseudotumor cerebri in a case of ulcerative colitis with sagittal sinus thrombosis

Thromboembolic events are a known complication of Inflammatory Bowel Disease [IBD] especially during disease relapse, more commonly in deep veins of extremities and lung, and rarely as Cerebral Sinovenous Thrombosis [CSVT]. We describe an 11 year old male patient with 3 months history of Ulcerative Colitis [UC] who presented as pseudotumor cerebri due to superior sagittal sinus thrombosis during an acute exacerbation of his colitis, that was successfully treated with heparin and then warfarin. In any known cases of UC presenting as acute severe headache, consider CSVT and request brain MRI and MRV to facilitate the diagnosis and early treatment

case of megalencephalic leukoencephalopathy with subcortical cysts in an iranian consanguineous family

Megalencephalic leukoencephalopathy with subcortical cysts [MLC] is an autosomal recessive disorder characterized by macrocephaly, and slowly progressive clinical course marked by ataxia, spasticity and mental decline. MLC is caused by mutations in the gene MLC1 which encodes a novel protein, MLC1. A 4-year-old girl with macrocephaly, spasticity, ataxia and abnormal cerebral white matter and subcortical cysts in brain MRI diagnosed with MLC. This is the first report of MLC in an Iranian family. MLC1 should be considered in children with macrocephaly and slowly progressive psychomotor decline. This disease can be prenatally diagnosed and genetic counseling offered for future pregnancies

Evaluation of humoral immune function in patients with bronchiectasis

Bronchiectasis is a chronic debilitating condition characterized by abnormal dilated thickwalled bronchi. To investigate humoral immune function in bronchiectatic patients, this study was performed. Forty patients with established diagnosis of bronchiectasis, who were referred from two tertiary care pulmonology centers in Tehran, were investigated in this study. Immunoglobulin isotypes concentrations and IgG-subclasses were measured by nephelometry and enzymelinked immunosorbent assay [ELISA] methods, respectively. All patients received unconjugated pneumococcal vaccine, and blood samples were taken before and 21 days after vaccination. Specific antibodies against whole pneumococcal antigens were measured using the ELISA method. Fifteen [37.5%] out of 40 patients were diagnosed to have defects in antibody mediated immunity including 5 [12.5%] patients with immunoglobulin class deficiency [2 with common variable immunodeficiency and 3 with IgA deficiency], 3 [7.5%] with IgG subclass deficiency and 7 [17.5%] patients had Specific antibody deficiency [SAD] against polysaccharide antigen despite normal levels of serum immunoglobulins and IgG subclasses. Our study along with several other studies confirmed that all patients with bronchiectasis should undergo thorough immunological evaluation in order to identify the presence of the underlying immunologic defect. This evaluation should include serum immunoglobulins, IgG subclasses concentrations and also determination of serum antibodies against pneumococcal antigens. Early diagnosis and appropriate treatment will prevent the subsequent complications and improve quality of life of affected individuals