Hyperhidrosis, another hallmark of SS, was present in only 42.3% of the cases and mainly in adult onset. [ncbi.nlm.nih.gov]

The presentation is one of periodic hypothermia and hyperhidrosis, without warning and often lasting hours before spontaneous resolution back to baseline 1-3. [radiopaedia.org]

As with our patient, the majority of reported cases of Shapiro's syndrome have presented spontaneously in adulthood [1, 3–7]. The reason for late presentation, as well as the reason symptoms tend to spontaneously occur and remit, is unknown. [academic.oup.com]

CASE A 8-year-old boy in otherwise good health, who, since 5 years of age, presented with sporadic episodes of nocturnal hypothermia, associated with pallor and profuse sweating. [scielo.br]

[…] hypothermia syndrome. 61 52 24594427 2014 2 Spontaneous periodic hypothermia and hyperhidrosis. 61 52 Dundar NO...Haspolat S 19027594 2008 3 Mode of death in Shapiro syndrome: a case report. 61 Braams NJ...van Halm VP 31449626 2019 4 Shapiro syndrome presenting [malacards.org]

• Hypothermia

  SG 2910261 1989 34 Spontaneous periodic hypothermia. 61 Mooradian AD...Morley JE 6537857 1984 35 Spontaneous periodic hypothermia with lipoma of the corpus callosum. 61 Summers GD...Jones RA 7334403 1981 36 Spontaneous periodic hypothermia: diencephalic [malacards.org]

  Episodic spontaneous hypothermia with hyperhidrosis syndrome (ESHH) is a rare syndrome characterized by periodic hypothermia and hyperhidrosis. [radiopaedia.org]

  KEYWORDS: Corpus callosum; Hyperhidrosis; Hypothermia; Paroxysmal; Shapiro; Shapiro Syndrome [ncbi.nlm.nih.gov]

  A case of spontaneous hypothermia, Scott Med J, 1992, vol. 37 (pg. 85 - 6 ) 7, ,, ,,. [academic.oup.com]

  Nonenvironmental hypothermia in pediatric patients is uncommon. Life-threatening causes of nonenvironmental hypothermia include sepsis, drug ingestions, brain tumors, and endocrinologic abnormalities. [scielo.br]

• Chills

  Over the next few days, frequent episodes of sweats and chills were observed. A typical sweating episode lasted 1–2 h and was followed by shaking chills lasting 30 min to 1 h. [academic.oup.com]

  […] hipotireoidismo hipotalâmico Ronaldo ArkaderI; Carlos Augusto TakeuchiII IMD, Pediatrician IIMD, Child Neurologist Shapiro syndrome, first described in 1969, is an extremely rare condition consisting of the clinical triad of recurrent hypothermia, sweating, and chills [scielo.br]

• Poikilothermia

  A problem in this area could lead to hypothermia or poikilothermia. The anterior center controls heat dissipation by stimulating a caudal hypothalamic region, thus inducing vasodilation and sweating. A problem here may lead to hyperthermia [3]. [academic.oup.com]

• Cold Intolerance

  The symptoms of central hypothyroidism include fatigue, apathy, weight gain, dry skin, constipation and cold intolerance deficiency of TRH secretion gives rise to hypothalamic hypothyroidism, also called tertiary hypothyroidism, which can occur in hypothalamic [scielo.br]

• Epilepsy

  2910261 1989 34 Spontaneous periodic hypothermia. 61 Mooradian AD...Morley JE 6537857 1984 35 Spontaneous periodic hypothermia with lipoma of the corpus callosum. 61 Summers GD...Jones RA 7334403 1981 36 Spontaneous periodic hypothermia: diencephalic epilepsy [malacards.org]

• Constipation

  The symptoms of central hypothyroidism include fatigue, apathy, weight gain, dry skin, constipation and cold intolerance deficiency of TRH secretion gives rise to hypothalamic hypothyroidism, also called tertiary hypothyroidism, which can occur in hypothalamic [scielo.br]

  Constipation and LUTS - how do they affect each other? International braz j urol 2011;37(1):16-28. 13. Henderson AM, Vallis CJ, Sumner E. Anaesthesia in the prunebelly syndrome. A review of 36 cases. Anaesthesia 198;42(1):54-60. 14. [revistapediatria.org]

• Hyperhidrosis

  Episodic spontaneous hypothermia with hyperhidrosis syndrome (ESHH) is a rare syndrome characterized by periodic hypothermia and hyperhidrosis. [radiopaedia.org]

  1 Shapiro's syndrome: Defining the clinical spectrum of the spontaneous paroxysmal hypothermia syndrome. 61 52 24594427 2014 2 Spontaneous periodic hypothermia and hyperhidrosis. 61 52 Dundar NO...Haspolat S 19027594 2008 3 Mode of death in Shapiro syndrome [malacards.org]

  KEYWORDS: Corpus callosum; Hyperhidrosis; Hypothermia; Paroxysmal; Shapiro; Shapiro Syndrome [ncbi.nlm.nih.gov]

  PMID 18641886 "Subtotal corpus callosum agenesis with recurrent hyperhidrosis-hypothermia (Shapiro syndrome)" Tambasco N, Corea F, Bocola V. Neurology. 2005 Jul 12;65(1):124. [en.wikipedia.org]

  Basal forebrain malformation with hyperhidrosis and hypothermia: variant of Shapiro's syndrome, Neurology, 2001, vol. 56 (pg. 254 - 6 ) © 2004 by the Infectious Diseases Society of America [academic.oup.com]

• Sweating

  Over the next few days, frequent episodes of sweats and chills were observed. A typical sweating episode lasted 1–2 h and was followed by shaking chills lasting 30 min to 1 h. [academic.oup.com]

  CASE A 8-year-old boy in otherwise good health, who, since 5 years of age, presented with sporadic episodes of nocturnal hypothermia, associated with pallor and profuse sweating. [scielo.br]

  Shapiro syndrome Other names Spontaneous periodic hypothermia Specialty Neurology Shapiro syndrome is an extremely rare disorder consisting of paroxysmal hypothermia (due to hypothalamic dysfunction of thermoregulation), hyperhydrosis (sweating), and [en.wikipedia.org]

  […] thermoregulation disorder: Shapiro syndrome]. 61 Szabo J...Vecsei L 26853729 2016 10 Spontaneous periodic hypothermia in the postoperative phase of a brain tumor of the third ventricle. 61 Vinetti M...Hantson P 25577333 2015 11 Remission of Episodic Sweating [malacards.org]

• Dry Skin

  The symptoms of central hypothyroidism include fatigue, apathy, weight gain, dry skin, constipation and cold intolerance deficiency of TRH secretion gives rise to hypothalamic hypothyroidism, also called tertiary hypothyroidism, which can occur in hypothalamic [scielo.br]

• Suggestibility

  The presence of SS in siblings of different genders suggests an autosomal recessive inheritance model, however a gonadic mosaicism responsible for an autosomal de novo mutation cannot be ruled out. [ncbi.nlm.nih.gov]

• Catatonia

  […] syndrome. 61 52 24594427 2014 2 Spontaneous periodic hypothermia and hyperhidrosis. 61 52 Dundar NO...Haspolat S 19027594 2008 3 Mode of death in Shapiro syndrome: a case report. 61 Braams NJ...van Halm VP 31449626 2019 4 Shapiro syndrome presenting with catatonia [malacards.org]

• Bicytopenia

  Shapiro's syndrome. 61 Topcu Y...Kurul SH 24339619 2013 13 Shapiro syndrome. 61 Mathur S...Ram C 24640213 2013 14 Insights into thermoregulation: a clinico-radiological description of Shapiro syndrome. 61 Pazderska A...O'Dowd S 23578790 2013 15 Cyclic bicytopenia [malacards.org]

Treatment and prognosis Management is guided by case report-level evidence, with one notably effective agent being cyproheptadine 1-3. Shapiro syndrome Promoted articles (advertising) [radiopaedia.org]

No definitive treatment for this disorder has ever been found. After 2 weeks of clonidine therapy, our patient's core temperature and symptoms were unchanged. [academic.oup.com]

We emphasize the importance of recognition the syndrome and associated endocrine manisfestations and the current treatment. REFERENCES 1. Sheth RD, Barron TF, Hartlage PL. [scielo.br]

Treatment and prognosis Management is guided by case report-level evidence, with one notably effective agent being cyproheptadine 1-3. Shapiro syndrome Promoted articles (advertising) [radiopaedia.org]

Contemporary epidemiology and characterization of newborn males with prune belly syndrome. Urology 2010;76(1):44-8. PubMed PMID: 20381841. Epub 2010/04/13. eng. 7. Salihu HM, Tchuinguem G, Aliyu MH, Kouam L. [revistapediatria.org]

Genetic epidemiology of hereditary hemorrhagic telangiectasia in a local community in the northern part of Japan. Hum Mutat. 2002;19(2):140-8. https://doi.org/10.1002/humu.10026 Grosse SD, Boulet SL, Grant AM, Hulihan MM, Faughnan ME. [revistagastrocol.com]

Pathophysiologic and anesthetic correlations of the prune-belly syndrome. AANA journal 1989;57(2):137-41. 16. Woodard JR. Prune Belly syndrome. En: King LR, Panayotis KP, Belman AB, editores. Clinical Pediatric Urology. [revistapediatria.org]