title: Thermoregulatory Instability in Childhood: Linking the Normal Brain to Hypothalamic Storm.
abstract: Central core temperature is tightly controlled by hypothalamic centers, a feature that makes sudden changes in body temperature very unusual. A dysfunction of these hypothalamic pathways leads to Shapiro's syndrome, comprising spontaneous hypothermia, hyperhidrosis, and corpus callosum dysgenesis. Although it may affect any age, usually it presents in childhood. Variants to this syndrome with completely normal brain anatomy have been consistently reported, expanding the clinical spectrum of the syndrome. Herein, we report the case of a 4-year-old girl with Shapiro's syndrome and unaffected corpus callosum.
authors.affiliation: Service of Neurology, Faculty of Medicine, PUCRS, Porto Alegre, RS, Brazil.
authors.forename: William
authors.identifier: 0000-0002-8752-5760
authors.initials: W
authors.lastname: Alves Martins
authors.affiliation: Service of Neurology, Faculty of Medicine, PUCRS, Porto Alegre, RS, Brazil.
authors.forename: Rafael
authors.identifier: 0000-0002-7150-4455
authors.initials: R
authors.lastname: do Amaral Cristovam
authors.affiliation: Service of Neurology, Faculty of Medicine, PUCRS, Porto Alegre, RS, Brazil.
authors.forename: Helena
authors.initials: H
authors.lastname: Fussiger
authors.affiliation: Service of Neurology, Faculty of Medicine, PUCRS, Porto Alegre, RS, Brazil.
authors.forename: Viviane
authors.initials: V
authors.lastname: Maria Vedana
authors.affiliation: Service of Neurology, Faculty of Medicine, PUCRS, Porto Alegre, RS, Brazil; Internal Medicine, Division of Neurology, Faculty of Medicine, Hospital São Lucas, Pontifícia Universidade Católica do Rio Grande do Sul (PUCRS), Porto Alegre, RS, Brazil.
authors.forename: Marta
authors.initials: M
authors.lastname: Hemb