Cyclosporine in the treatment of a case of fulminant and refractory acute disseminated encephalomyelitis

Acute disseminated encephalomyelitis [ADEM] is a rare, monophasic, demyelinating disease of the CNS which sometimes could be refractory to traditional treatment. We present a case of fulminant ADEM which is treated with combination of corticosteroid, intravenous immunoglobulin and cyclosporine. Immunosuppressive agents such as cyclosporine may be effective especially in fulminant form of the disease

Status epilepticus as a presenting sign of reversible posterior leukoencephalopathy syndrome in a liver transplant recipient

Reversible posterior leukoencephalopathy syndrome [RPLE] is an increasingly recognized disorder, most commonly associated with immunosuppressive and cytotoxic agent, malignant hypertension and toxemia of pregnancy. Neurological symptoms are headache, mental and visual disturbance, and seizures. Brain MRI findings are symmetrical bilateral hyper intense areas of occipital and parietal lobes in T2-weighted magnetic resonance [MR] images. This finding is reversible with prompt successful treatment which may take days to weeks for full reversal. We observed a case of status epilepticus which occurred 32 days after liver transplantation in a patient on regimen consisting of Tacrolimus [FK 506]. He had no history of neurologic disease or metabolic abnormalities. Typical findings of RPLE were present on brain MRI. The recurrence of seizures terminated after a few hours with adding Na-valproate to Phenytoin, Phenobarbital and Midazolam. The radiological finding of brain MRI gradually diminished after several weeks. A physician should be alert of seizures during early post transplant period of liver transplantation. He should bear in mind the diagnosis of RPLE and consider discontinuation of immunosuppressive drugs

Continuous ambulatory peritoneal dialysis for a patient with Bardet-Biedl syndrome

Bardet-Biedl syndrome [BBS] is a multisystem syndrome with a range of primary and secondary features. Kidney abnormalities are a major cause of morbidity and mortality in BBS and it is a significant genetic cause of chronic kidney disease in children. Conventional approach to end-stage renal disease in these patients is hemodialysis and kidney transplantation afterwards. Continuous ambulatory peritoneal dialysis, however, is not a commonly advocated modality in literature. In this report, we present a boy with BBS who underwent continuous ambulatory peritoneal dialysis, which resulted in control of kidney function impairment and better compliance for his family than hemodialysis before kidney transplantation. Of note, this is a rare case of BBS complicated with end-stage renal disease in the first decade of life