A tic is a brief involuntary movement that
can be suppressed by the individual but at the
expense of mounting inner tension, often with
subsequent rebound [11. Gilles de la
Tourette syndrome (GTS) is a neurodevelopmental
disorder characterized by multiple motor tics
and one or more vocal tics that usually begin
before the age of 18. Estimates of the
prevalence of GTS vary. The generally accepted
figure was 0.5/1000, but recent research
suggests that between 1% and 3% of school
children are affected. The nature and
phenomenology of tics vary widely, even in
documented familial cases. They may take the
form of a movement, a vocalization (phonic tic),
or a complex phenomenon such as an
echophenomenon or coprolalia (the
inappropriate.utterng of obscenities) which is a
rare manifestation of GTS. Tics can be increased
in conditions of stre$s, boredom, or fatigue.
Patients often report that tics are preceded by
distinct premonitory feelings or sensations,
they are temporarily suppressible, and patients
experience relief after the tic has occurred.
Tics can also fluctuate in severity and change
character within the same person.
It is increasingly recognized that tics can
be unusual in their manifestations and even
self-injurious with reports of teeth grinding,
vomiting, and retching, and other selfinjurious
behaviors.
Aerophagy is a pattern of repetitive air
swallowing and troublesome belching that is
objectively observed and has been present for
the last 3 months with symptom onset at least 6
months before diagnosis. It may be undetected
until the patient develops a distended abdomen.
It is often associated with abdominal discomfort
and distension and patients may present to
gastroenterologists with pseudoobstruction or
malabsorption, although life-threatening
complications are rare. Aerophagy has been
described as a cause of irritable bowel syndrome
and is frequently reported in association with
neurological disorders and learning
disabilities, with a high incidence in
institutionalized patients. In particular, it
has been linked with Huntington's disease and
Rett syndrome in which it is particularly common
and is thought to be due to abnormal feeding
patterns during feeding or to specific
oropharyngeal problems. Adults with aerophagy
have recently been shown to have an increased
prevalence of anxiety, and anxiety per se has
been linked to increased swallowing rates in
normal subjects [18], which may suggest
a possible precipitating factor in predisposed
individuals. Zella et al. reported incessant
"eructation" as the presenting symptom of
obsessive-compulsive disorder, the latter being
a comorbid psychopathology in 30-60% of patients
with GTS. Aerophagy has also been described as a
phenomenon in children with no neurological
abnormalities.
Although air swallowing can be secondary to
a surgical or interventional procedure, as in
nasal intermittent positive pressure
ventilation, and latyngectomy [231, usually
no organic cause is found. The exact mechanism
of air swallowing in aerophagy is not fully
understood, but a recent study has shown that
distraction and attention have a significant
effect on belching in patients with aerophagia.
This may provide some insight into a possible
mechanism for air swallowing in GIS and also has
important implications for behavioral
therapy.
To the best of our knowledge, there have
been very few reports of air swallowing as a tic
in the literature, apart from one recent report
of air swallowing in GIS leading to ileus. The
lack of documentation of air swallowing as a tic
may suggest that it is a rare phenomenon, but it
is also likely that it is an unrecognized and
underreported feature of GIS. We are therefore
documenting this patient with air swallowing as
a tic in GIS to highlight the importance of
unusual features of GIS which may be amenable to
treatment.
Case report
A 22-year-old man with mild learning
disability was referred to the National Hospital
for Neurology and Neurosurgery where he was
diagnosed with GIS. The patient was assessed
with the National Hospital Interview Schedule,
the Yale Global Tic Severity Scale, the MOVES
self-rating scale for GIS, and the burette
Syndrome Diagnostic Confidence Index. The first
tic that the patient exhibited took the form of
barely audible muttering, a complex tic, at the
age of 14. This was followed by a wide variety
of tics. He had at least 47 motor tics including
frowning, blinking, facial grimacing, and
smelling things, as well as some complex
movements such as hopping and skipping. He had
at least 17 vocalization tics which included
yawning, barking, snorting, and saying
"yes" very loudly. He also had mental
coprolalia, echolalia, palilalia, forced
touching, aggression, and some obsessive
compulsive behaviors. On the Yale Global Tic
Severity Scale h scpred 48/55, and on the
Diagnostic Confidence Index he scored 69%,
suggesting that there was no doubt at all about
the diagnosis and that he had GIS of moderate
seventy. Family history of note included a
maternal aunt with facial tics in childhood,
lasting into adulthood. He had been prescribed
clomipramine 85 mg daily since the age of 16 for
depressive symptoms associated with his GIS.
Treatment with clonidine had previously been
attempted for his tics, but as it had no effect
on them it was withdrawn.
At the age of 18, the patient presented with
longstanding constipation and 5 weeks of
bloating, abdominal distension, and repeated
regurgitation associated with profuse
belching.
This was relieved by the passage of flatus.
He also had reduced appetite and had lost half a
stone (3.2 kg) in weight. His parents had
noticed the sounds of air swallowing. We
enquired and it appeared that he was probably
not able to suppress the air swallowing, but it
was unclear if air swallowing increased in times
of anxiety: his being learning disabled made the
subjective history less clear.
On examination, the abdomen was distended
and resonant to percussion. Bowel sounds were
normal but continuous. Plain abdominal X-ray
revealed a grossly distended stomach, air in the
intestine but no mechanical obstruction. A small
bowel meal, which followed the initial plain
abdominal film by 1 week, was within normal
limits, excluding a volvulus. Blood tests were
normal and the patient was diagnosed with a
"windy abdomen" due to air swallowing. The air
swallowing and associated symptoms resolved
spontaneously, within 7 weeks of onset.
The symptoms of abdominal distension and
discomfort recurred more mildly 5 months later
and resolved within 6 weeks, soon after stopping
clomipramine. Although the gastrointestinal
symptoms and air swallowing improved, other
motor and vocal tics worsened and he was treated
with sulpiride and paroxetine. He continued on
these medications until he suffered a further
relapse of air swallowing 3 years later. This
was also milder than the initial episodes and
associated with abdominal distension, but far
less than on the earlier occasions. Supine
abdominal X-ray showed generalized distension of
the gastrointestinal tract. The distension
resolved with conservative management. The
patient has remained well on the same treatment
for the past 7 years. He has had no further
periods of air swallowing or abdominal
distension. His motor and vocal tics are also
well controlled and he has been able to work in
a supermarket for the past 3 years.
Discussion
This patient with GTS was noted to be
swallowing air. The symptoms lasted longer than
12 weeks in total, but were not consecutive and
so do not quite fulfil the latest diagnostic
criteria for aerophagy [11, although he
fulfils the previous diagnostic criteria. In
this case, there were several possible causes
for the air swallowing. Our patient was learning
disabled, which has been linked to air
swallowing. However, our patient was able to
articulate the sensation of air swallowing and
his parents gave a very clear description. It
was felt overall that this was a tic rather than
any other clinical phenomenon.
The patient was also taking clomipramine,
which has been linked to aerophagy. However,
that report only links tricyclic antidepressants
with air swallowing caused by increased water
consumption due to a dry mouth. This was not a
feature of the presenting case, who was noted by
his parents to be swallowing air at times both
when he was and was not drinking fluids.
Moreover, the patient had been stable on
clomipramine for 2 years, without air
swallowing, and the air swallowing resolved
whilst he was still taking clomipramine. Other
causes of air swallowing such as excessive
talking whilst eating, drinking carbonated
drinks, and rapid eating were ruled out as they
were not present in this case. We thus suggest
that this air swallowing may have been a
tic.
There are three descriptions of possible air
swallowing as a tic in the literature. The first
is in the form of a debate in which an apparent
sequence of hiccups was identified by the author
as possible aerophagy as a tic. Alternative
diagnoses were pharyngeal dystonia and branchial
myoclonus. The second is in the Shapiro et al.
series of patients with GTS where several
demonstrated hiçcupping and one
demonstrated belching as vocal tics, suggesting
that air swallowing may have been previously
associated with GTS.
More recently, Frye and Hait described a
16-year-old boy diagnosed with GTS at the age of
7, who developed clinically significant air
swallowing over a 2-month perio& The air
swallowing was paralleled by an acute increase
in vocal tics and psychological stressors, and
resulted in abdominal cramping, eructation, and
flatus, eventually leading to "aeroenteria" and
recurrent ileus. Of note, according to the
authors the air swallowing resulted from the
aggravation of already present tics, rather than
representing a newly developed and isolated tic,
as in our patient.
In most cases, air swallowing resolves
spontaneously within weeks or months. Where
symptoms persist, treatment may be difficult.
Some groups have successfully used carbon
tablets and others have attempted behavioral
therapy with some success. However, in cases
where air swallowing is suspected to be a tic,
it could be treated pharmacologically, as for
GTS, with neuroleptic drugs such as haloperidol,
pimozide, and sulpiride [341, or the newer
atypical antipsychotics risperidone and
aripiprazole. Of these, risperidone could prove
particularly useful in this context, since it is
associated with fewer gastrointestinal side
effects compared to aripiprazole.
