Reversible posterior leukoencephalopathy syndrome in a case of adult onset Still's disease with concurrent thrombotic thrombocytopenic purpura: response to high dose immunoglobulin infusions.

Thrombotic thrombocytopenic purpura (TTP) is a multisystem disorder characterized by a pentad consisting of thrombocytopenic, microangiopathic hemolytic anemia, renal dysfunction, neurological signs and fever. Coexistence of thrombotic thrombocytopenic purpura and Adult Onset Still's Disease (AOSD) is extremely rare. We report a case of 18 year old girl with AOSD who developed TTP. Neuroimaging of brain demonstrated white matter edema consistent with reversible posterior leukoencephalopathy syndrome (RPLS). Complete recovery occurred with prompt anti-hypertensive treatment and high dose immunoglobulin infusions (IVIg). Plasma exchange is the standard of care and the first line treatment for patient with TTP. We used IVIg alone in our case and this showed a gratifying response. Use of IVIG before considering plasmapharesis is justifiable or not requires randomized control clinical trials. This should determine the optimal therapeutic strategies for TTP.

Gaucher's disease.

Gaucher's disease is the most common group of lysosomal storage disorders caused by defective activity of an enzyme beta-glucosidase leading to accumulation of glucocerebroside in cells of macrophage lineage. Accumulation of glucosylceramide in tissues leads to multisystem organ involvement viz. liver, spleen, bone marrow, lungs and central nervous system. Serum beta=glucosidase levels <15% of mean normal activity confirms the diagnosis, enzyme replacement being the only definitive treatment. We report a clinical case of a 21 year male with Gaucher's disease. To the best of our knowledge only six cases of Gaucher's disease have been reported from India so far.

Electroconvulsive therapy in drug resistant neuroleptic malignant syndrome.

We report a case of a 20 years female referred to us with a history of a brief psychotic episode for which she was given inj. Haloperidol. The patient presented in an unconscious state with high grade fever. The diagnosis was kept as neuroleptic malignant syndrome after ruling out other possibilities. The patient did not respond to Bromocriptine and Dantrolene. With the recent evidence of electroconvulsive therapy being useful in these patients, we went ahead with the same. We present this case to share our experience of the excellent response of neuroleptic malignant syndrome to electroconvulsive therapy.