Spontaneous Periodic Hypothermia and Hyperhidrosis

Dündar, Nihal Olgaç; Boz, Adil; Duman, Özgür; Aydın, Funda; Haspolat, Şenay

doi:10.1016/j.pediatrneurol.2008.08.010

Cited by 26 publications

(36 citation statements)

References 10 publications

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“…e16 In a single pediatric case, the patient had fewer episodes while being treated with carbamazepine. 14 Seizures originating from the hypothalamus may be difficult to detect with a routine surface EEG due to their deep location. If there is a high clinical suspicion for seizures, extended EEG Testing should include an MRI of the brain with contrast to evaluate for agenesis of the corpus callosum, a tumor in the hypothalamic region, or other structural abnormalities.…”

Section: E11mentioning

confidence: 99%

Diagnosis and management of periodic hypothermia

Blondin

2014

Neur Clin Pract

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SummaryHypothermia, defined as a core temperature below 35°C, can occur in a variety of clinical settings, including environmental exposure, shock, infection, metabolic disorders (such as hypothyroidism, adrenal insufficiency, and Wernicke encephalopathy), malnutrition, and alcohol or drug toxicity. Typically, hypothermia should resolve with treatment of the underlying disorder. However, in rare cases patients experience recurrent episodes of hypothermia in the context of a stereotyped syndrome due to a hypothalamic lesion, which can be either congenital or acquired. The episodes are characterized by progressive confusion and a decreased level of arousal, hypothermia, and eventual resolution with a return to baseline. Additional clinical findings during episodes may include diaphoresis, asterixis, bradycardia, and thrombocytopenia. These recurrent episodes represent periodic hypothermia. I n healthy individuals, several mechanisms exist to prevent hypothermia from occurring. When a reduced body temperature is detected by peripheral temperature receptors, several reflexes are activated: skin vasoconstriction and contraction of erector pili muscles reduce heat radiation, shivering produces heat, and sympathetic excitation and thyroxine production increase the basal metabolic rate. Control of these reflexes occurs in the preoptic nucleus of the anterior hypothalamus and adjacent regions of the septum, which is referred to as the pre-optic area.e1 In this region, thermoafferent signals are mediated by a variety of neurotransmitters, including catecholamines, serotonin, melatonin, peptides, and cytokines. Dysfunction, injury, or destruction of the preoptic area, whether congenital or acquired, can lead to the development of periodic hypothermia. The cognitive impairment and other neurologic symptoms seen during hypothermia may be due to the reduction in cerebral blood flow that occurs once the body temperature falls below 33°C.e2 EEGs performed during episodes

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Section: E11mentioning

confidence: 99%

Diagnosis and management of periodic hypothermia

Blondin

2014

Neur Clin Pract

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“…4 The hypothermic episodes can occur at varying intervals, ranging from hours to months, and episodes can last from minutes to years. 3 In our patient, the attacks occurred monthly and lasted approximately one hour. She did not experience any shivering during the attacks.…”

Section: Discussionmentioning

confidence: 57%

“…2,3 It has been reported in all age groups with the youngest case being a sixmonth-old girl. 4 The hypothermic episodes can occur at varying intervals, ranging from hours to months, and episodes can last from minutes to years.…”

Section: Discussionmentioning

confidence: 99%

“…4 In the differential diagnosis, Shapiro's syndrome, which is characterized by agenesis of the corpus callosum and ESHH should be ruled out. 3 The episodic nature of this disorder has led some authors to believe that the episodes represented "diencephalic epilepsy". Although ESHH can occasionally be accompanied with epilepsy, it does not appear to be a manifestation of a seizure disorder.…”

Section: Discussionmentioning

confidence: 99%

“…Anticonvulsants, clonidine, bromocriptine, and chlorpromazine are all used in the treatment of ESHH, with varying degrees of success. 3 Cyproheptadine, an antiserotonergic agent is the most commonly recommended with variable therapeutic success rates.…”

Section: Discussionmentioning

confidence: 99%

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