Paroxysmal gluten-sensitive dyskinesia in Border terriers
Firstly, yes the name is unfortunately a mouthful, and doesn’t really roll-off-the-tongue very
nicely! Probably, the (somewhat) easier term to remember is canine epileptoid cramping
syndrome, or CECS. Another name sometimes/previously used to describe this disorder is
that of Spike’s Disease (Spike was one of the first Border terriers to be recognised with the disorder).
CECS is characterised by episodes or attacks of involuntary movements which may include any combination of the following: difficulty in walking or falling over, tremors/muscle fasciculations, and dystonia (sustained or repetitive muscle contractions). These episodes most frequently affect all four limbs, along with the head and neck. Despite some obviously alarming clinical signs, this disorder does not impair consciousness. Interestingly, one study by Black et al. 2014, reported concurrent borborygmi in nearly half of the animals assessed during these episodes. In this same study, approximately 50% of the dogs showed signs of vomiting and/or diarrhoea, either before or
after the episode of CECS.
Currently, no specific genetic test exists to discriminate between affected and disorder-free
dogs, most likely indicating a complex pattern of inheritance. Serological testing (using a
blood sample) can however be performed to determine the presence of anti-canine
transglutaminase-2 and anti-canine gliadin antibodies. Both of these types of antibodies are
increased in Border Terriers with CECS, when compared with healthy animals and even
animals with epilepsy.
It seems that most Border Terriers will experience their first episode before the age of 3 years
(range: 6 weeks – 7 years). These episodes, however, can be extremely variable based on
several studies detailing owners accounts (questionnaire-based) of onset, duration, frequency,
and severity. Some owners describe episodes lasting anywhere from 2 up to 30 minutes, and
frequency ranging from one every few months to multiple attacks per day. In addition, most
episodes occur at rest, or even during waking from sleep, however, there are also several
reports of an association with either stress or excitement.
What is unique about this syndrome, is its apparent responsiveness to dietary adjustments,
particularly changing to a gluten-free diet. Lowrie et al. 2015 demonstrated this diet-
associated improvement in six dogs, and also showed that with re-introduction of gluten this
improvement is reversible, with relapse of clinical signs in two dogs in which the diet was not
adhered to. Several studies have reported on the response to owners changing their dog’s diet,
with a reduction in the frequency of episodes being noted in anywhere from approximately
50% (14/26 dogs) up to 100% (5/5 dogs) of cases, in which owners strictly maintained their
dog on a gluten-free diet.
In humans, the term non-coeliac gluten sensitivity (NCGS) has been used as an umbrella term
for a spectrum of conditions that are all characterised by an immune response to the ingestion
of gluten. Disorders included in this group include an enteropathy (intestinal disease, distinct
in nature from celiac disease), dermatopathy (dermatitis herpetiformis – a chronic
autoimmune blistering skin condition), and neurological disorders such as gluten ataxia
(impaired balance or coordination). Often, the disease manifests in a systemic fashion in
humans, involving one or more of the above processes.
In fact, Lowrie et al. 2016 described the first case of a Border Terrier that had a combination of neurological signs, atopy (allergic skin disease), positive serological results consistent with CECS, along with signs suggestive of gastrointestinal disease. This report suggested that Border Terriers may manifest CECS as a multisystem disease, similar to that described in humans, and was later further supported by another study by Lowrie et al. 2018.
While CECS is not an epileptic disorder, Stassen et al. 2017, did describe a beneficial effect
of anti-epileptic therapy in approximately 70% (29/43) of dogs treated with such drugs. That
being said, and particularly given the potential side effects experienced with most of the
commonly used anti-epileptics, this would not normally be the first treatment recommendation.
If you’re concerned that your Border Terrier may have CECS, try taking a video of an episode to show to your veterinarian, and potentially discuss serological testing with him/her to try and confirm the diagnosis.
If you’re in need of advice on a gluten-free diet, don’t hesitate to contact us: firstname.lastname@example.org
Please check out the link below to watch a YouTube video on CECS: