Pathophysiological basis of a paroxysmal, episodic, central thermoregulatory failure

F. Magnifico, G. Pierangeli, G. Barletta, C. Candela, G. Bonavina, P. Cortelli

Research output: Contribution to journalArticlepeer-review


Abnormal sweating may occur as a compensatory reaction to the loss of thermoregulatory sweating on the denervated side (Harlequin syndrome) or it may be associated with poikilothermia and agenesis of the corpus callosum (Shapiro syndrome). We evaluated the pathophysiological basis of a possible new syndrome characterized by idiopathic unilateral crisis of sweating with hypothermia. A 51-year-old male, with a 2-month history of paroxysmal, prolonged attacks of unilateral sweating over the left side of the upper body and face, following an aspecific flu infection, was studied. These crises occurred when getting up in the morning or, less frequently, after a meal. Microbiologic, biochemical, and endocrinological studies were normal. The corpus callosum on brain MRI was normal. EMG, sympathetic skin response and cardiovascular autonomic function tests in the interjetal state were performed, along with thermoregulatory sweat test (TST). Circadian rhythm of body core temperature (CRT°), by continuous monitoring of rectal temperature (rT°), and skin temperature, (left, L-sT°; right side R-sT°) were evaluated for 4 days. During body heating, when the rectal temperature rose by 1 C° (from a low baseline 34.82 C° to 36.15 C°), sweating on the left side of the face, neck, and upper body, including the hand, occurred, while the right side and lower legs remained almost dry. The onset of sweating was accompanied by a fall in rectal temperature by 1 C° (to 35 C°). A spontaneous sweating episode recorded during standing up was characterized by a slight increase in BP and HR and by noradrenaline increase from a baseline level of 728 pg/ml to 1012 pg/ml, with adrenaline in a normal range. The continuous recording of rT°showed that the onset of the sweating "crisis" was immediately followed by a progressive fall in rectal temperature, taking about 85 min (from 37.27 C° to a nadir of 33.9 C°) as well as a fall in right (from 36.04 C° to 31.67 C°) and left (35.45 C° to 30.90 C°) skin temperature. At this stage, the unilateral upper sweating stopped while the hypothermia (33.9 C°) lasted about 2 h and corresponded clinically to the severe weakness, shivering, and confusion phase. Then, in about 2 h, the rectal and skin temperatures returned slowly to base-line values (rT° = 36.5 C°; R-sT°= 35.6 C°; L-sT°= 35.45 C°). In our patient, a sympathetic noradrenergic storm of unknown origin determined the fall in cutaneous and rectal temperature, leading to a severe episodic hypothermia. The normal response to the TST indicated a normal function of the heat loss mechanisms. However, the spontaneous sweating "crisis" leading to hypothermia indicates an abnormal downward shift of the thermoregulatory set-point and a delay in the effect of those mechanisms blocking the heat dissipation. Clinical and laboratory findings established the diagnosis of Shapiro syndrome and Harlequin syndrome.

Original languageEnglish
JournalNeurological Sciences
Issue number4 SUPPL.
Publication statusPublished - 2000

ASJC Scopus subject areas

  • Clinical Neurology
  • Neuroscience(all)


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