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What Is Curly Coat, Dry Eye, and Episodic Falling Down Syndrome
(CC, DE, EFS)?
 

DRY EYE CURLY COAT SYNDROME

The first thing to understand is, though the condition of dry eye curly coat syndrome (CKCSIDA) affects only the Cavalier King Charles Spaniel breed, a recognized research study estimated that of the dogs tested, only 10.8% of Cavaliers were carriers, and 0.4% were affected.

The scientific name is quite a mouthful: ichthyosis keratoconjunctivitis sicca and also as congenital keratoconjunctivitis sicca and ichthyosiform dermatosis (or CKCSIDA). It is a severe developmental disease of the eyes, skin, coat, and nails in some Cavalier King Charles Spaniels. Affected dogs are prone to corneal ulcers, itchy skin, and thick, cracked paw pads. When a puppy is born affected, they are born with an unusually rough, curly hair coat. They do not produce tears, which leads to the early symptoms of corneal ulcers, infection, and ocular discharge.

NOTE: This severe type of dry eye should not be confused with the more common dry eye (as with humans) that can be easily treated with eye drops. The dry eye affiliated with curly coat is a separate, genetic disorder.

 

The syndrome is also known as rough coat syndrome. Affected puppies are usually diagnosed between the age of 2 - 10 weeks. The coat will have frizzy sparse hair, skin will be dry and flakey with unusually thickened foot pads, and malformed nails. Combined with the accompanying dry eye syndrome often green discharge around the eyes is seen due to abnormal tear production, meaning lack of the ability to produce adequate tears for the eye to develop healthily. All this can lead to severe corneal ulcers.  Additionally, such dogs are predisposed to dental disease, even more so than what is already considered normal for all toy breed dogs and specifically the Cavalier King Charles Spaniel.

Figure 1

cavalier puppy affeted with curly coat syndrome
image of canine eye affected with severe dry eye as part of dry eye curly coat syndrome

Figure 2

Medications & Treatments:

Gel Artificial Tear Drops: Given to relieve tissue to the cornea  and conjunctiva. 

Acetylcysteine: A mucolytics agent, to aid in controlling excess mucus on the ocular surface.

Antibiotics: Used topically to aid in the control of bacterial infection. 

Corticosteroids: Usually given short-term to help improve symptoms, but with caution due to the high risk of ulcer. 

Pilocarpine: It is a parasympathomimetic agent that mimics or modifies the effects of acetylcholine, which plays a role in brain functions, such as memory, and body functions, such as muscle contractions to move your muscles.

Immunosuppressive drugs: A therapy that stimulates tear production in dogs.

Surgery Options:

If medical management proves unsuccessful, surgical intervention is considered, although options for surgical management are, unfortunately, very limited.

EPISODIC FALLING DOWN SYNDROME

The first thing to understand is, though the condition of  episodic falling syndrome (EFS) also only affects the Cavalier King Charles Spaniel breed, again the research study shows that a low number of Cavaliers were affected, that number being 3.7%

Episodic falling syndrome is marked by seizure-like episodes (though not actually a medically defined seizure). Symptoms generally appear between the ages of 3 months - 4 years. Signified by rigid limbs, abnormal postures, loss of coordination, and collapse, though alarming to witness, these are not accompanied by loss of consciousness, and dogs generally recover rather quickly afterward. In very rare cases permanent neurological injury can occur, but typically the disorder improves with therapy. It is also worthy to note that with this disorder, lifespan is not affected.

cavalier king charles spaniel with episodic falling syndrome having a spasm

Figure 3

Medications & Treatments

Diazapam: (Valium®) Used as a sedative. 

Acetazolamide: (Diamox®, Dazamide®) Used to balance internal enzymes. 

Clonazepam: (Klonopin®) Used to enhance GABA neurotransmissions, the main inhibitory neurotransmitter in the brain, improves chemical messages that pass between nerve cells.

Natural TreatmentsSuch as acupuncture, chiropractic, and vitamin therapy have been shown to aid in relief and control of symptoms. 

Though as a breeder I am not able to promise I will never have a puppy born with any of the above disorders, to date I never have. I also only know a few breeders of my breed who have seen it or had a puppy born with it. The odds of this occurring is reflected in the above-mentioned conducted research studies. Unlike MVD and COMS/SM, as breeders we can breed away from these disorders because the genes have been identified, and through testing, we are able to identify "clear", "carrier" and "affected" dogs and breed or eliminate dogs accordingly. 

Definitions:

Clear: This dog does not carry the gene - Can be bred to "clear" or "carrier" and will not produce affected puppies.

Carrier: This dog carries 1 (one) copy of the gene - Can be bred to a "clear" and will not produce affected puppies.

Affected: This dog carries 2 (two) copies of the gene (1 from each parent) - Should not be bred.

All active Cavaliers of Fairhaven dams and studs have been tested for CC, DE, and EFS. Though there is not a public database, I have the results posted as part of each of my dogs' biographies on our BOYS and GIRLS pages. Also, I have all results in paper form in my home in each of my dogs' personal health files.

Author: Angela Schuller

Disclaimer: As a breeder who cares deeply for my dogs and puppies I act as responsibly as I can.  The information provided is intended to inform and help to lessen the alarm when inherited disorders such as CC, DE, and EFS become a topic with your veterinarian. Ultimately, once a puppy leaves my home relinquished to an owner's care, owners must then take on the responsibility for their decisions. As always, if your pet has an urgent medical concern, please consult your veterinarian. Finally, as your trusted breeder, I am always available to offer support and answer questions. 

SOURCES:

Gensol Genetic Testing Website, https://www.gensoldx.com/tests/dry-eye-curly-coat-syndrome-ckcsid/

"Figure 1" jpg, Cavalier Health Website, courtesy Dr. K. C. Barnett,  also health information obtained, "Figure 2" jpg and "Figure 3" jpg (no photo credits given on site), https://cavalierhealth.org/curly_coat.htm , Accessed 23 July 2023

National Library of Medicine, https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6067592/

Dog Heirs Website, https://www.dogheirs.com/hypertonicity-and-episodic-falling-in-dogs/

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