Insights into thermoregulation: A clinico-radiological description of Shapiro syndrome.
AffiliationDepartment of Endocrinology, Mater Misericordiae University Hospital, Dublin 7, Ireland. Electronic address: firstname.lastname@example.org.
MetadataShow full item record
CitationInsights into thermoregulation: A clinico-radiological description of Shapiro syndrome. 2013, 329 (1-2):66-8 J. Neurol. Sci.
JournalJournal of the neurological sciences
AbstractShapiro syndrome is a rare entity, comprising a triad of recurrent hypothermia, hyperhidrosis and congenital agenesis of the corpus callosum. Fewer than 50 cases have been described, almost invariably in patients presenting in childhood or early adulthood. We present a case of an 80year old woman presenting with recurrent bouts of shivering, sweating and profound malaise, who sought medical attention because the frequency and severity of attacks worsened in her later years. MRI Brain demonstrated agenesis of the corpus callosum; a rigorous work-up excluded other causes for her symptomatology. The intricate interplay of neuronal networks involved in thermoregulation remains to be fully elucidated and as such, little is known about the pathophysiological mechanisms underlying the clinical manifestations of Shapiro syndrome. We present novel data from FDG-PET imaging of our patient, demonstrating hypermetabolism in a number of brainstem and cerebellar regions during the symptomatic phase. These findings imply that aberrant thermoregulation in Shapiro syndrome involves a number of structures remote from the callosal region. We also present neuropsychometric findings in our patient, of which there have been no reports to date. We postulate that the ageing brain may be more susceptible to the paroxysmal neurochemical fluxes implicated in the syndrome.
- Reverse Shapiro's syndrome: Hyperthermia in the scenario of agenesis of the corpus callosum.
- Authors: Kapoor S
- Issue date: 2013 Nov 15
- [A rare case of thermoregulation disorder: Shapiro syndrome].
- Authors: Szabó J, Zádori D, Varga ET, Vécsei L
- Issue date: 2016 Feb 14
- Early-Onset Shapiro Syndrome Variant Treated with Pizotifen: A Case Report.
- Authors: Denegri L, Prato G, Mancardi MM, Schiaffino C, Striano P, Baglietto MG
- Issue date: 2017
- Paroxysmal hypothermia and hyperhidrosis with exacerbation after COVID-19 Infection.
- Authors: Lamotte G, Benarroch EE, Coon EA
- Issue date: 2021 Apr
- Shapiro syndrome.
- Authors: Mathur S, Mathur A, Dubey T, Jain S, Mathur S, Agarwal H, Kulshrestha M, Jangid R, Ram C
- Issue date: 2013 Jun