ABSTRACT
The Brugada syndrome is an autosomal dominant disease with incomplete penetrance, which may cause syncope and sudden cardiac death in young individuals with a normal heart. It is characterized by an electrocardiographic pattern of complete or incomplete right bundle branch block and ST-segment elevation in leads V1-V3. Mutations in gene encoding for cardiac sodium channel SCN5A is linked to this syndrome in 20-25% patients. Several conditions producing Brugada-like ECG patterns should be borne in mind and excluded before making a diagnosis of the Brugada syndrome. The management of this syndrome is difficult as pharmacological agents are not universally effective. The mode of treatment recommended by the majority of cardiac electro-physiologists is implantation of a cardioverter defibrillator. We report the first case of a 40-year-old Indian lady who presented with a history of aborted sudden cardiac death with recurrent ventricular arrhythmias and an electrocardiogram (ECG) pattern of type 1 Brugada syndrome, and underwent successful implantable cardioverter defibrillator implantation.
ABSTRACT
Autoimmune diseases in the form of cutaneous leucocytoclastic Vasculitis (CLCV), polyarteritis nodosa (PAN), cerebral arteritis and cryoglobulinemia are uncommon associations of Hairy Cell Leukaemia (HCL). We report a case of a 55 year old male with HCL, carcinomatous meningitis, cryoglobulinemia and cutaneous leukocytoclastic vasculitis, which preceded the diagnosis of HCL, leucocytoclastic vasculitis was confirmed on skin biopsy, the cutaneous lesions regressed on steroid therapy.