Table 5.

Treatment of Manifestations in Individuals with Cold-Induced Sweating Syndrome / Crisponi Syndrome (CISS/CS)

w/respiratory distress
Provide for supplemental oxygen w/o delay.An apnea monitor is recommended.
Bouts of hyperthermia
(≤42º C [108° F])
Cooling blanketsTemperature-reducing medications are not effective.
Seizures Anti-seizure medication for acute episodesLong-term seizure medication past the infantile period will seldom be necessary as seizures are symptomatic of hyperthermia, not due to a primary seizure disorder.
Feeding difficulty in
NG or gastrostomy tube feeding until disorganized swallowing & esophageal dysmotility improve over 1st year of lifeManage gastroesophageal reflux pharmacologically.
Camptodactyly Bracing & PT/OTPlastic surgery may be considered at a later stage.
Prolonged bracing or surgical correction depending on rapidity of progression
Corneal injury Topical lubrication w/artificial tears at daytime, lubricating gels at nightFor severe corneal injury, tarsorrhaphy can be considered.
Dental decay Regular dental visitsW/focus on prevention of early dental decay
Paradoxic cold
induced sweating
in older children
& adults
See Pharmacologic Management of Cold-Induced Sweating.Normally noted by parents (w/earliest start at 2 yrs) but not a concern in childhood; treatment can be delayed until early teens.
Heat intolerance
(lack of sweating)
Cold drinks, splashing self w/or immersing in cold waterAvoid prolonged exposure to or physical activities in hot environmental temperatures.
Lack of temperature
& pain sensation in
fingers & toes
Patients should be made aware of the risks of burns or frostbite.Wear clothing (e.g., mittens & socks) to protect against burns & frostbite.

NG = nasogastric; OT = occupational therapy; PT = physical therapy

From: Cold-Induced Sweating Syndrome Including Crisponi Syndrome

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