Hypoglycemia as a Manifestation of Shapiro Syndrome

Abstract

Shapiro syndrome is an extremely rare disorder of dysautonomia characterized by paroxysmal episodic hypothermia to below 95°F. Many patients with Shapiro syndrome improve with medical management, though a minority of cases are refractory to treatment. Our patient with adult-onset Shapiro syndrome is an atypical case. Our patient has been refractory to medical treatment as well as chemical sympathectomy. Based on a review of the literature, this is also the first reported case of hypoglycemia with Shapiro syndrome episodes in the absence of other metabolic diseases. This case suggests that hypoglycemia could be a potential manifestation of Shapiro syndrome.