ABSTRACT
Central pontine myelinosys [CPM] is a pathological and clinical syndrome due to destruction of medullated sheaths with relative sparing of the axis cylinders and nerve cells in the pons. Clinicaly it is characterised by rapidly evolving quadriplegia and pseudobulbar palsy. In children, the most common etiology is the rapid correction of hyponatremia, and rarely other disorders like adrenal insufficiency, hyperglycaemic coma, liver disease and leukemia have been associated with CPM. The condition is often fatal and survivors have been reported to suffer from permanent neurological deficit in the form of 'locked in' stated. We report a girl who developed steroid resistant nephrotic syndrome, and during the course of her illness became hyponatremic also. Over enthusiastic correction of the latter, she developed CPM but recovered totally in a few days
Subject(s)
Humans , Female , Myelinolysis, Central Pontine/diagnosis , HyponatremiaABSTRACT
This case clinical study describes a 10-years old boy with idiopathic adipsic hypernatremia associated with reset [shift to right] osmostat for arginine vasopressin [AVP] secretion, combined with a relative deficiency of AVP. His clinical course was characterised by intermittent exacerbation of chronic hypernatremia due to lack of thirst. The pathophysiology of adipsic hypernatremia and the practical difficulties in the management are discussed