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Maladaptive Responses

Rasmussen’s syndrome is an autoimmune disease involving one hemisphere, with cortical inflammation and atrophy, and thus this is a secondary myoclonus. Pathophysiology is unknown. Despite the identification of antiglutamate receptor antibodies in some children, immune-modulating therapies do not have long-term benefit and only hemispherectomy is curative.66 A case has also been described after gliomatosis cerebri in two children.67 Some authors have suggested that EPC be used to designate cases with cortical origin, and myoclonia continua be used for those originating elsewhere in the nervous system.68

Does the Patient Have a Disorder that Increases Electroneutral Reabsorption of Na+ Ions in the CDN?

The pathophysiology in these patients may be an increase in parallel transport activity of pendrin and NDBCE. This may be the pathophysiology for what used to be thought of as a “chloride shunt disorder.” These patients will also have an expanded EABV and suppressed PRenin and PAldosterone (hyporeninemic hypoaldosteronism). Patients with this disorder are expected to have an increase in K+ ion excretion with increasing the delivery of HCO3−ions to the CDN with the administration of the carbonic anhydrase inhibitoracetazolamide. Examples of this pathophysiology may include some patients with diabetic nephropathy and hyporeninemic hypoaldosteronism.