Long-term follow-up in pediatric patients with paroxysmal hypothermia (Shapiro's syndrome)

Tambasco, Nicola; Paoletti, Federico Paolini; Prato, Giulia; Mancardi, Maria Margherita; Prontera, Paolo; Giordano, Lucio; Grosso, Salvatore; Romeo, Antonino; Pinto, Fausto J.; Savasta, Salvatore; Peruzzi, Cinzia; Romoli, Michele; Striano, Pasquale; Verrotti, Alberto; Belcastro, Vincenzo

doi:10.1016/j.ejpn.2018.08.004

Cited by 8 publications

(17 citation statements)

References 24 publications

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“…However, no epileptic signals have been identified and antiepileptic drugs seem to be ineffective in Shapiro’s syndrome. The remarkable effectiveness of clonidine in our patient has also been reported in some cases of Shapiro’s syndrome [ 2 , 8 ]. The prevalence of headaches would be 40.8 % in the PD population compared to 69.4 % in the general population and only 5.8 % in Shapiro’s syndrome patients [ 2 , 13 ].…”

Section: Discussionsupporting

confidence: 78%

“…Little is known about the pathophysiology of Shapiro’s syndrome. It could be secondary to hypothalamic abnormalities not visible on common 1.5 or 3 Tesla brain MRI [ 2 , 8 ]. Some authors also mention deregulation of neurotransmitters such as dopamine, serotonin, norepinephrine, or melatonin [ 10 , 11 ].…”

Section: Discussionmentioning

confidence: 99%

“…multiple sclerosis, brain tumors, autoimmune encephalitis, brain infection, subarachnoid hemorrhages…) [ 4 – 7 ]. Generally, headaches are uncommon and rarely in the foreground in Shapiro’s syndrome [ 2 , 8 ]. In this paper, we report an unusual case of Shapiro’s syndrome variant revealed by episodes of headache with spontaneous hypothermia in a parkinsonian patient.…”

Section: Introductionmentioning

confidence: 99%

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Episodic headache with spontaneous hypothermia reveal Shapiro’s syndrome variant with effectiveness of clonidine therapy

et al. 2021

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Background Episodic headache with spontaneous hypothermia constitute an uncommon association and is not well recognized in the International Classification of Headache Disorders (ICHD-3). Spontaneous periodic hypothermia, also called Shapiro’s syndrome, is a rare disease characterized by hypothermia attacks associated or not with hyperhidrosis without any triggering factor. Case presentation We report a rare case of Shapiro’s syndrome variantrevealed by episodes of headache with spontaneous hypothermia witheffectiveness of clonidine therapy in a 76-year-old Parkinson’s disease woman. Conclusions In the literature, apart from Shapiro’s syndrome, headache withhypothermia seem to occur very rarely. In our case,these symptoms may be considered as a very rare non-motor fluctuation ofParkinson’s disease.

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Section: Discussionsupporting

confidence: 78%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Episodic headache with spontaneous hypothermia reveal Shapiro’s syndrome variant with effectiveness of clonidine therapy

et al. 2021

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“…The thermoregulation centre is located in the brain, in the hypothalamus [22][23][24] . It has been shown that the rarely diagnosed Shapiro syndrome (spontaneous periodic hypothermia) is associated with hypothalamic dysfunction [36][37][38][39][40] . It has already been shown that in patients with brain injury (both traumatic and non-traumatic), thermoregulation disorders occur [41][42][43] .…”

Section: Discussion and Concluding Remarksmentioning

confidence: 99%

Theoretical basis for the use of non-invasive thermal measurements to assess the brain injury in newborns undergoing therapeutic hypothermia

Walas

Bandoła

Ostrowski

et al. 2020

Sci Rep

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The aim of this paper is to propose a new non-invasive methodology to estimate thermogenesis in newborns with perinatal asphyxia (PA) undergoing therapeutic hypothermia (TH). Metabolic heat production (with respect to either a neonate’s body mass or its body surface) is calculated from the newborn’s heat balance, estimating all remaining terms of this heat balance utilising results of only non-invasive thermal measurements. The measurement devices work with standard equipment used for therapeutic hypothermia and are equipped with the Global System for Mobile Communications (GSM), which allows one to record and monitor the course of the therapy remotely (using an internet browser) without disturbing the medical personnel. This methodology allows one to estimate thermogenesis in newborns with perinatal asphyxia undergoing therapeutic hypothermia. It also offers information about instantaneous values of the rate of cooling together with values of remaining rates of heat transfer. It also shows the trend of any changes, which are recorded during treatment. Having information about all components of the heat balance one is able to facilitate comparison of results obtained for different patients, in whom these components may differ. The proposed method can be a new tool for measuring heat balance with the possibility of offering better predictions regarding short-term neurologic outcomes and tailored management in newborns treated by TH.

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“…Additionally, thalamic injury would likely result in lifelong temperature dysregulation, where-as our patient had transient cycles, lasting only a few years. Tambasco et al report thirteen pediatric cases of CSS and VSS [7]. In each case, the cyclic hypothermia resolved after an average of three years regardless of treatment.…”

Section: Discussionmentioning

confidence: 99%

Carnitine Deficiency in a Patient with Shapiro Syndrome

Yager¹,

Alkhasova²,

Rudy³

2019

Intern Med

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Spontaneous hyperhidrosis with hypothermia and agenesis of the corpus callosum is known as Classical Shapiro Syndrome (CSS) and was first reported by Shapiro and Plum in 1967. We report a well-documented case of a 39-yearold woman with SS diagnosed at the age of 5-years-old who was diagnosed with Carnitine deficiency at 30-years-old. This is the first reported case of SS with Carnitine deficiency, which was treated with levocarnitine. Our patient also carries with her several comorbid diagnoses which have been reported in other cases of SS. These include primary amenorrhea (untreated), primary hypothyroidism (treated with levothyroxine and liothyronine), seizure disorder (treated with lamotrigine, levetiracetam, and eslicarbazepine acetates), and hypothermia-induced cytopenia (resolved with the addition of liothyronine). Additionally, this case offers unique insights gained from longitudinal care.

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Long-term follow-up in pediatric patients with paroxysmal hypothermia (Shapiro's syndrome)

Cited by 8 publications

References 24 publications

Episodic headache with spontaneous hypothermia reveal Shapiro’s syndrome variant with effectiveness of clonidine therapy

Episodic headache with spontaneous hypothermia reveal Shapiro’s syndrome variant with effectiveness of clonidine therapy

Theoretical basis for the use of non-invasive thermal measurements to assess the brain injury in newborns undergoing therapeutic hypothermia

Carnitine Deficiency in a Patient with Shapiro Syndrome

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