Table 5.

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

Manifestation/ConcernTreatmentConsiderations/Other
Laryngospasm
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
infancy
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.
Thoracolumbar
scoliosis
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

Cover of GeneReviews®
GeneReviews® [Internet].
Adam MP, Feldman J, Mirzaa GM, et al., editors.
Seattle (WA): University of Washington, Seattle; 1993-2024.
Copyright © 1993-2024, University of Washington, Seattle. GeneReviews is a registered trademark of the University of Washington, Seattle. All rights reserved.

GeneReviews® chapters are owned by the University of Washington. Permission is hereby granted to reproduce, distribute, and translate copies of content materials for noncommercial research purposes only, provided that (i) credit for source (http://www.genereviews.org/) and copyright (© 1993-2024 University of Washington) are included with each copy; (ii) a link to the original material is provided whenever the material is published elsewhere on the Web; and (iii) reproducers, distributors, and/or translators comply with the GeneReviews® Copyright Notice and Usage Disclaimer. No further modifications are allowed. For clarity, excerpts of GeneReviews chapters for use in lab reports and clinic notes are a permitted use.

For more information, see the GeneReviews® Copyright Notice and Usage Disclaimer.

For questions regarding permissions or whether a specified use is allowed, contact: ude.wu@tssamda.

NCBI Bookshelf. A service of the National Library of Medicine, National Institutes of Health.