The neck-tongue syndrome

Abstract

The neck-tongue syndrome, consisting of pain in the neck and altered sensation in the ipsilateral half of the tongue aggravated by neck movement, has been attributed to damage to lingual afferent fibres travelling in the hypoglossal nerve to the C2 spinal roots. The lingual afferents in the hypoglossal nerve are thought to be proprioceptive. Two further cases of the neck-tongue syndrome are described, the spectrum of its clinical manifestations is explored, and the phenomenon of lingual pseudoathetosis is illustrated as a result of the presumed lingual deafferentation.

Full text

38ournal
of
Neurology,
Neurosurgery,
and
Psychiatry
1994;57:348-352
The
neck-tongue
syndrome
Richard
W
Orrell,
C
David
Marsden
Abstract
The
neck-tongue
syndrome,
consisting
of
pain
in
the
neck
and
altered
sensation
in
the
ipsilateral
half
of
the
tongue
aggra-
vated
by
neck
movement,
has
been
attributed
to
damage
to
lingual
afferent
fibres
travelling
in
the
hypoglossal
nerve
to
the
C2
spinal
roots.
The
lingual
affer-
ents
in
the
hypoglossal
nerve
are
thought
to
be
proprioceptive.
Two
further
cases
of
the
neck-tongue
syndrome
are
described,
the
spectrum
of
its
clinical
manifestations
is
explored,
and
the
phe-
nomenon
of
lingual
pseudoathetosis
is
illustrated
as
a
result
of
the
presumed
lingual
deafferentation.
(7
Neurol
Neurosurg
Psychiatry
1994;57:348-352)
University
Department
of
Clinical
Neurology,
The
National
Hospital,
Queen
Square,
London
WClN
3BG,
UK
R
W
Orrell
C
D
Marsden
Correspondence
to:
Dr
R
W
Orrell.
Received
14
April
1993
and
in
final
revised
form
14
June
1993.
Accepted
21
June
1993
Lance
and
Anthony'
described
four
patients,
aged
8
to
14
years
at
onset,
with
a
brief
(sec-
onds
to
a
minute)
sharp
pain
on
one
side
of
the
upper
neck
or
occiput
on
sudden
rotation
of
the
neck,
followed
immediately
by
tran-
sient
(seconds
to
minutes)
ipsilateral
numb-
ness
of
the
tongue.
Two
of
these
cases
had
congenital
anomalies
of
the
upper
cervical
spine
on
radiography.
Cyriax2
had
previously
noted
a
similar
syndrome
in
two
patients,
one
of
whom
was
a
45-year-old
woman
with
attacks
of
severe
pain
at
the
occiput
and
left
side
of
the
neck
and
numbness
of
the
tongue
and
back
of
the
palate,
precipitated
by
turn-
ing
her
head
sharply
to
the
right.
The
other
patient
had
a
cervical
disc
lesion
and
a
sensa-
tion
of
pins
and
needles
felt
in
the
tongue
only.
Lance
and
Anthony'
concluded
that
the
pain
was
due
to
irritation
of
the
second
(and
third)
cervical
root,
which
is
vulnerable
to
compression
in
its
course
between
the
atlas
and
axis
during
neck
rotation.
They
explained
the
unilateral
numbness
of
the
tongue
on
the
basis
of
afferent
impulses
(notably,
propriocep-
tive)
travelling
from
the
lingual
nerve
via
the
hypoglossal
nerve
to
the
second
cervical
root.
Bogduk3
clarified
the
anatomical
basis
for
the
syndrome
by
cadaveric
dissection,
demonstrating
that
the
C2
ganglion
and
spinal
nerve
lie
dorsal
to
the
lateral
atlantoax-
ial
joint;
the
joint
is
innervated
by
the
C2
ventral
ramus.
He
proposed
that
the
pain
was
due
to
temporary
abnormal
subluxation
of
the
lateral
atlantoaxial
joint
on
rotation
of
the
head.
This
strains
the
joint
capsule,
and
thereby
causes
pain
in
the
suboccipital
region.
He
attributed
the
unilateral
numbness
of
the
tongue
(and
of
the
skin
behind
the
ear)
to
compression
of
the
C2
ventral
ramus
by
movement
or
subluxation
of
the
lateral
atlantoaxial
joint.
He
suggested
that
the
abnormal
sensation
on
compression
of
pro-
prioceptive
afferents
from
the
tongue
is
simi-
lar
to
the
altered
sensation
or
numbness
recognised
in
Bell's
palsy,
despite
intact
trigeminal
sensation,
due
to
compression
of
proprioceptive
fibres
in
the
facial
nerve.
Since
these
original
descriptions
of
the
neck-tongue
syndrome,
a
few
other
cases
have
been
described.
Lance4
briefly
mentions
a
further
four
patients
in
whom
the
syndrome
started
in
late
childhood
or
adolescence.
Elisevich
et
all
reported
a
53-year-old
woman
with
assimilation
of
the
atlas
to
the
occiput
presenting
with
neck
pain
and
ipsilateral
paraesthesiae
of
the
tongue
on
head
turning,
in
whom
the
symptoms
became
persistent
and
incapacitating.
At
operation,
the
C2
spinal.
nerves
were
found
to
be
compressed
by
a
protuberant
atlantoaxial
joint.
Resection
of
the
superficial
parts
of
the
C2
spinal
nerves
led
to
partial
relief.
Webb
et
a16
described
three
cases
with
ipsi-
lateral
neck
pain
and
tongue
numbness
pre-
cipitated
by
neck
movement,
first
developing
symptoms
at
ages
of
10
to
61
years.
Degen-
erative
changes
of
the
anterior
atlantoaxial
joint
were
present
in
two
of
the
cases.
Restriction
of
neck
movement,
including
use
of
a
surgical
collar
in
two
of
the
cases,
con-
trolled
the
symptoms.
Fortin
and
Biller7
described
a
57-year-old
woman
with
repeated
episodes
of
numbness
of
the
posterior
right
half
of
the
tongue
and
right
posterolateral
oropharynx,
an
ipsilateral
"ear
pressure",
and
a
sharp
aching
pain
radi-
ating
along
the
border
of
the
trapezius
to
the
acromioclavicular
joint.
At
the
same
time
she
noticed
a
pressure
sensation
in
the
occiput
and
paraesthesiae
in
the
fourth
and
fifth
fin-
gers
of
the
right
hand.
The
episodes
lasted
one
minute,
and
she
herself
noted
no
clear
precipitant,
but
they
were
precipitated
by
extreme
lateral
rotation
of
the
neck.
The
region
of
the
atlantoaxial
joint
was
normal
radiologically.
They
also
described
a
29-year-
old
man,
with
previous
head
injury
and
lower
thoracic
vertebral
compression
fracture
after
a
motorcycle
accident,
who
complained
of
mainly
right,
but
sometimes
left
sided
(never
both
sides
simultaneously),
hemiglossal
and
facial
paraethesiae,
sharp
occipital
pain
shoot-
ing
to
the
periorbital
area,
dysarthria,
nasal
348
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The
neck-tongue
syndrome
congestion,
sialorrhoea,
and
paraesthesiae
in
the
ulnar
distribution
of
the
upper
limb.
The
symptoms
in
both
patients
were
relieved
by
use
of
a
surgical
collar.
Bertoft
and
Westerberg8
reported
a
further
six
patients
with
a
complete
syndrome,
and
three
with
an
incomplete
syndrome,
which
they
define
as
having
characteristic
attacks,
but
lacking
the
paroxysmal
numbness
of
the
tongue.
We
have
not
included
these
cases
of
an
incomplete
syndrome
in
the
present
dis-
cussion,
but
two
of
the
patients
with
a
com-
plete
syndrome
had
radiological
evidence
of
arthritis
of
the
lateral
atlantoaxial
joints,
and
in
one
the
symptoms
were
abolished
after
surgical
atlantoaxial
fusion.
Cassidy
et
a19
described
three
patients
with
the
neck-tongue
syndrome,
who
were
suc-
cessfully
treated
with
rotational
manipulation
of
the
cervical
spine.
Terrett
et
al'°
described
eight
cases
of
the
neck-tongue
syndrome,
which
were
treated
with
spinal
manipulative
therapy.
They
also
referred
to
an
additional
54-year-old
woman
with
headaches,
who,
after
cervical
manipulation,
developed
left
sided
tongue
numbness,
persisting
for
two
years,
together
with
an
intermittent
dysarthria.
Hankey"
described
a
more
classical
neck-tongue
syndrome
in,
a
14-year-
old
girl
with
recurrent
episodes
of
unilateral
upper
nuchal
pain
radiating
to
the
ipsilateral
side
of
the
tongue
on
ipsilateral
rotation
of
the
neck.
Noda
and
Umezaki"2
described
a
35-year-
old
woman
with
paroxysmal
episodes,
lasting
up
to
one
minute,
of
numbness
of
the
left
neck
and
occiput,
with
simultaneous
numb-
ness
of
the
left
half
of
the
tongue,
preceded
by
tonic
spasm
of
the
left
arm.
They
suggest
this
is
a
component
of
a
spinal
tonic
seizure
related
to
an
upper
cervical
inflammatory
myelopathy
involving
the
dorsal
funiculus,
proximal
to
the
C2
and
C3
dorsal
root
gan-
glia,
and
hence
a
spinal
neck-tongue
syn-
drome.
In
total,
36
cases
of
this
syndrome
have
been
previously
described
in
the
literature,
some
very
briefly,48
although
Bogduk13
com-
ments
that
on
discussion
with
other
neurolo-
gists
and
neurosurgeons
it
seems
that
the
syndrome
is
not
rare.
Accordingly
we
describe
two
further
cases
of
the
syndrome,
due
in
one
case
to
atlantoaxial
osteoarthritis,
and
in
the
other
to
a
traumatic
fracture
and
displacement
of
the
occipital
condyle.
A
strik-
ing
feature
was
dysarthria
and,
in
the
second
case,
pseudoathetosis
of
the
tongue.
Patients
CASE
1
A
51-year-old
woman
complained
of
a
pro-
gressive
stiffness
and
pain
in
the
neck
for
six
years.
Three
years
previously
she
had
noticed
that
the
pain
was
exacerbated
by
movement
of
the
neck
to a
critical
position,
with
the
chin
down
and
to
the
right.
She
was
aware
of
a
grating
sensation
in
the
neck,
and
the
pain
appeared
to
be
deep
to
the
angle
of
the
jaw
on
the
left,
radiating
to
the
occiput.
These
exacerbations
were
associated
with
a
tingling
sensation
of
the
left
side
of
the
tongue,
and
a
sensation
of
the
tongue
being
pulled
into
the
back
of
the
throat
on
the
left,
causing
a
feel-
ing
of
choking,
with
difficulty
speaking.
Neurological
examination
was
normal,
and
in
particular
touch
and
pin
prick
sensation
on
both
sides
of
the
tongue
was
preserved.
The
tongue
and
palate
appeared
normal,
with
nor-
mal
movement.
Cervical
spine
movement
was
severely
limited
due
to
pain.
A
cervical
spine
radiograph
showed
degen-
erative
changes
in
the
mid
and
lower
cervical
spine,
with
rotation
at
the
atlantoaxial
joint,
but
no
horizontal
subluxation.
A
cervical
spine
CT
scan
with
the
head
turned
to
the
right
and
left
showed
no
structural
lesion
or
abnormal
movement.
Rotatory
movement
was
equal
to
the
right
and
left,
with
no
dislo-
cation
of
the
lateral
atlantoaxial
joint.
The
rotation
was
about
40
degrees
on
each
side,
a
little
more
than
usually
seen,
but
probably
not
pathological.
A
radioisotope
bone
scan,
however,
revealed
increased
uptake
in
the
left
atlantoaxial
joint,
attributed
to
osteoarthritis.
Injection
of
a
local
anaesthetic
and
steroid
into
this
joint
led
to
brief
relief
of
the
pain.
CASE
2
A
32-year-old
woman
accidentally
fell
down
a
ffight
of
12
stairs
at
night,
hitting
the
right
frontoparietal
region
of
her
head
against
a
table.
This
caused
a
scalp
laceration
but
no
loss
of
consciousness
or
immediately
appar-
ent
neurological
deficit.
The
next
day
she
awoke
with
a
continuous
right
sided
occipital
headache
and
neck
pain
exacerbated
by
attempts
at
neck
movement.
She
had
slurring
of
speech,
continuous
altered
sensation
of
the
tongue
and
pharynx
on
the
right
with
a
feel-
ing
of
the
tongue
moving
to
the
left,
and
diffi-
culty
manipulating
food
in
the
mouth.
On
examination
she
had
tenderness
over
the
upper
cervical
spine,
and
neck
move-
ments
were
severely
restricted
by
pain.
The
tongue
was
rotated
anticlockwise
in
the
mouth,
with
consequent
appearance
of
decreased
bulk
of
the
left
side
of
the
tongue
(fig
A).
On
forward
protrusion
of
the
tongue
there
was
equal
bulk
of
the
two
sides,
but
great
difficulty
in
maintaining
a
sustained
steady
forward
position,
the
tongue
moving
involuntarily
to
right
and
left
(fig
B).
There
were
no
visible
fasciculations.
There
was
full
movement
of
the
tongue
to
the
left
(fig
C)
despite
partial
denervation
of
the
right
half
of
the
tongue
demonstrated
by
EMG.
Tongue
protrusion
to
the
right
was
incomplete
and
poorly
sustained
although
no
weakness
of
the
left
half
of
the
tongue
could
be
detected
(fig
D).
On
fibreoptic
endoscopy
the
base
of
the
tongue
was
more
posterior
than
usual,
and
partially
obstructing
the
larynx.
She
had
a
mild
lingual
dysarthria.
Sensation
of
the
tongue
and
pharynx
to
touch,
taste,
and
pin
prick
was
normal.
Neurological
examination
was
otherwise
normal.
Cervical
spine
radiography
was
normal.
An
upper
cervical
CT
scan
was
normal,
but
a
CT
scan
of
the
skull
base
showed
a
fracture
of
the
349
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Movement
of
the
tongue
in
case
2;
postures
A,B,C,
and
D
as
described
in
the
text.
right
occipital
condyle,
with
slight
medial
dis-
placement
of
the
fragment
just
distal
to
the
right
hypoglossal
canal.
Discussion
The
clinical
spectrum
of
the
neck-tongue
syn-
drome,
including
age
at
presentation,
chronicity,
and
disablement,
has
broadened
since
the
original
description.
The
two
patients
we
describe
had
a
feeling
of
abnor-
mal
posturing
or
movement
of
the
tongue
as
a
significant
feature
of
their
symptomatology.
In
case
2
the
positioning
of
the
tongue
was
clearly
abnormal
and
she
was
unable
to
main-
tain
a
sustained
normal
posture
of
the
tongue
in
the
absence
of
any
specific
weakness.
In
particular
there
was
no
weakness
of
tongue
movement
to
the
left,
which
would
be
affected
by
motor
denervation
of
the
right
side
of
the
tongue.
We
propose
that
this
is
a
result
of
proprioceptive
deafferentation
of
the
tongue,
with
the
clinical
sign
of
lingual
pseudoathetosis.
This
is
similar
to
pseudo-
athetosis
as
previously
described
in
the
limbs.14
Pseudoathetoid
movements
of
the
tongue
seem
particularly
complex
in
the
absence
of
any
restriction
of
direction
of
movement
imposed
by
a
joint.
The
associated
dysarthria
has
features
of
both
impaired
lin-
gual
control
and
pharyngeal
obstruction
as
manifestations
of
lingual
pseudoathetosis
pre-
dominantly
of
the
tip
and
base
respectively.
Proprioception
in
the
tongue
has
been
studied
in
animals
and
humans,"5
with
con-
troversy
initially
concerning
whether
the
tongue
musculature
has
proprioception.
As
a
generalisation,
it
seems
that
muscle
spindles
are
not
found
in
the
tongue
of
mammals,
except
in
moles,
primates,
and
humans.16
Muscle
spindles
have
been
demonstrated
his-
tologically
in
the
human
tongue.'7
18
The
afferent
pathways
seem
to
be
in
the
hypoglos-
sal
nerve,'6
19
which
is
otherwise
thought
of
as
being
a
purely
motor
nerve.
Lingual
pseudoathetosis
is
suggested
in
previous
descriptions
of
neck-tongue
syn-
drome.
A
15-year-old
boy
had
a
right
occipi-
tal
pain
and
numbness
of
the
right
side
of
the
tongue
on
sudden
neck
rotation,
and
a
feeling
that
the
tongue
was
twisted
sideways
in
his
mouth.'
The
symptoms
lasted
a
few
seconds
only.
A
29-year-old
man
had
ipsilateral
occip-
ital
pain
and
tingling
in
his
tongue,
with
dysarthria
and
nasal
congestion,
precipitated
by
neck
movement.7
A
60-year-old
man,
with
symptoms
since
the
age
of
10
years,
had
experienced
a
sensation
of
his
tongue
being
twisted
sideways
in
the
mouth
during
an
attack,
"as
though
the
whole
tongue
is
cleav-
ing
to
the
roof
of
the
mouth".4
A
19-year-old
woman
with
pain
in
the
left
side
of
the
neck
on
sudden
head
turning
also
noticed
devia-
tion
of
her
tongue
to
the
right
for
10
seconds,
with
anarthria.4
A
33-year-old
woman
described
paralysis
of
her
tongue
for
about
five
seconds
on
sharp
rotation
of
the
neck.'0
A
19-year-old
woman
described
her
tongue
as
seeming
to
take
up
all
the
room
in
her
mouth,
having
a
mind
of
its
own,
being
uncontrol-
lable,
and
resulting
in
dysarthria.'0
A
46-year-
old
man
experienced
the
symptoms
of
the
neck-tongue
syndrome,
his
tongue
being
numb
and
rigid,
with
dysarthria.10
A
26-year-
old
woman
experienced
the
neck-tongue
syn-
drome,
with
an
inability
to
move
her
tongue.'0
In
these
cases
the
dysarthria,
as
in
case
2,
may
be
related
to
proprioceptive
deaf-
ferentation
rather
than
a
primary
motor
deficit.
A
53-year-old
woman
had
pain
in
the
right
side
of
her
neck
and
tingling
of
the
ipsilateral
side
of
her
tongue,
initially
intermittent
but
then
constant.
This
was
aggravated
by
head
turning
and
talking,
and
when
severe
the
pain
radiated
to
the
back
of
her
head
or
right
arm
and
caused
mild
dysphagia
related
to
a
feeling
of
muscle
spasm
in
the
throat.5
A
34-year-old
man
complained
of
intermittent
left
sided
neck
pain
radiating
to
the
occiput,
with
ipsi-
lateral
aching
and
numbers
of
the
tongue.
The
symptoms
were
precipitated
by
rapid
350
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The
neck-tongue
syndrome
neck
flexion
and
rotation
to
either
side
when
breathing
during
competitive
free-style
swim-
ming.
Occasionally
the
symptoms
were
so
intense
and
associated
with
a
choking
sensa-
tion
that
he
had
to
stop
swimming
during
a
race.
(He
had
experienced
the
same
symp-
toms
while
swimming
between
the
ages
of
10
and
22
years).6
Most
reported
cases
have
had
transient
symptoms
and
signs,
but
in
case
2
these
were
constant,
enabling
the
tongue
posture
and
movement
to
be
observed.
The
tongue
seemed
to
be
drawn
back
in
the
mouth,
com-
patible
with
the
symptom
described
in
case
1,
and
possibly
explaining
the
muscle
spasm
in
the
throat
of
the
53-year-old
woman5
and
34-
year-old
swimmer.6
Of
the
cases
of
the
neck-tongue
syndrome
previously
described,
four
involved
abnormal-
ities
at
the
atlantoaxial
joints,
two
fusion
of
the
atlas
to
the
occiput,
and
another
a
minor
abnormality
of
the
occipital
condylar
processes.
A
cervical
disc
lesion
was
noted
in
another.
In
the
other
cases
no
clear
pathology
of
the
upper
cervical
joints
was
noted
(although
in
one
an
intrinsic
myelopathy
was
suggested).
In
case
1
the
structural
abnormal-
ity
was
osteoarthritis
at
the
left
atlantoaxial
joint
demonstrated
by
radioisotope
bone
scan.
Irregular
loss
of
the
left
lateral
atlantoaxial
joint
space
with
reactive
sclerosis
and
osteophytic
lipping
was
noted
in
a
36-
year-old
man,6
and
moderate
degenerative
changes
at
the
anterior
atlantoaxial
joint
in
a
65-year-old
woman.6
Another
possible
example
of
the
condition
was
described
by
Lees
et
al20
as
"paroxysmal
hemiglossal
twisting".
These
authors
reported
two
patients,
and
tentatively
suggested
dysto-
nia
or
tonic
seizure
as
the
cause.
The
first
case
was
a
61-year-old
woman
who,
on
review
of
the
original
records,
described
a
tin-
gling
sensation
at
the
back
of
the
tongue
on
the
left,
the
tongue
then
deviated
to
the
right,
and
she
was
unable
to
move
her
tongue
to
the
left,
associated
with
dysarthria.
On
other
occasions
the
tongue
twisted
from
side
to
side,
and
she
had
bitten
the
left
side
of
her
tongue
several
times.
Touch
sensation
of
the
tongue
was
normal
during
an
episode.
She
had
repeated
episodes,
lasting
for
about
one
minute,
which
were
infrequent,
with
no
clear
precipitating
neck
movement,
but
appeared
to
be
related
to
exercise.
The
attacks
could
be
associated
with
discomfort
down
the
left
side
of
the
neck
and
behind
the
left
ear.
A
cervical
spine
radiograph
showed
no
abnormality
of
the
upper
cervical
region,
and
CT
scan
of
the
atlantoaxial
joint
was
normal.
Except
for
the
absence
of
precipitation
by
neck
movement,
this
case
has
features
in
common
with
the
neck-tongue
syndrome,
and
the
abnormal
twisting
of
the
tongue
may
be
a
lingual
pseudoathetosis
due
to
lingual
proprioceptive
deafferentation,
with
associated
C2
pain.
(The
second
patient
had
brief
spontaneous
attacks
of
tongue
twisting
and
dysarthria
without
pain
or
paraesthesiae).
In
case
1,
the
site
of
deafferentation
is
assumed
to
be
in
the
neck,
related
to
the
atlantoaxial
joint.
In
case
2,
the
site
of
deaf-
ferentation
is
uncertain,
and
could
be
at
the
atlantoaxial
joint
related
to
the
fall
and
twist-
ing
of
the
neck,
related
to
the
displaced
occipital
condyle
and
abnormal
atlanto-
occipital
joint,
or
in
the
hypoglossal
nerve
after
exit
from
the
hypoglossal
canal
related
to
the
displaced
bone
fragment.
Previous
abnormalities
of
the
atlanto-occipital
joint
were
described
in
a
26-year-old
man
with
anteromedial
bosses
of
bone
on
the
condylar
processes
of
the
occiput
representing
a
minor
anomaly
of
the
occipital
assimilation.'
The
spinous
processes
of
the
atlas
were
fused
to
the
adjoining
occipital
bones
in
a
15-year-old
girl,'
and
assimilation
of
the
atlas
to
the
occiput
was
noted
in
a
53-year-old
woman.5
Thus
of
the
38
cases
now
described,
pathology
of
the
occipitoatlantoaxial
joints
has
been
noted
in
10.
The
onset
of
symptoms
in
case
2
was
clearly
related
to
trauma,
as
were
10
previous
cases.
A
29-year-old
man
had
a
motorcycle
accident,
with
head
and
thoracic
spine
injury,
two
years
before
developing
the
neck-tongue
syndrome.7
A
26-year-old
man
was
hit
in
the
midfrontal
region
by
a
child's
swing,
with
lac-
eration
of
the
forehead.
Shortly
afterwards
he
developed
numbness
and
weakness
of
the
right
hand
lasting
for
several
days.
Ever
since
he
has
had
transient
symptoms
of
numbness
of
the
right
side
of
the
tongue
and
right
fourth
and
fifth
fingers,
and
right
sided
neck
pain,
lasting
a
few
seconds
only,
on
sudden
rotation
of
the
neck.'
A
36-year-old
man
acci-
dentally
jarred
his
neck,
and
subsequently,
whenever
jolted
unexpectedly,
experienced
high
cervical
pain
radiating
to
the
left
side
of
his
tongue
"like
the
aftermath
of
a
dental
anaesthetic".6
A
28-year-old
woman
devel-
oped
the
neck-tongue
syndrome
the
day
after
a
whiplash
injury,
which
persisted
for
one
year,
but
resolved
with
spinal
manipulation.9
Other
cases
associated
with
trauma
include
four
after
a
motor
vehicle
collision,
and
one
developing
eight
years
after
a
water
skiing
accident.10
The
transient
relief
of
the
symptoms
in
case
1
by
injection
of
local
anaesthetic
and
steroid
into
the
lateral
atlantoaxial
joint
is
supportive
of
the
symptoms
being
related
to
this
joint,
and
raises
the
possibility
of
treat-
ment
by
permanent
cervical
fusion.
Previously
reported
surgery
with
bilateral
resection
of
C2
spinal
nerves
with
their
dorsal
and
ventral
rami,
initially
relieved
the
symp-
toms,
but
after
one
week
there
was
a
recur-
rence
of
mild
tingling
sensations
in
the
tongue
when
the
patient
was
tired.
The
neck
pain
also
recurred,
but
less
severely
than
before
the
operation.
The
authors
suggested
that
the
recurrence
of
tongue
symptoms
could
be
explained
by
facilitation
of
residual
proprioceptive
fibres
in
the
adjacent
unin-
jured
spinal
roots.
In
another
patient,
surgical
atlantoaxial
fusion
abolished
the
symptoms.8
Other
patients
have
had
their
symptoms
relieved
by
spinal
manipulation.9
10
Symptoms
have
been
improved
by
preventing
the
precip-
itative
neck
movement
with
a
surgical
collar,
351
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Onrell,
Marsden
but
the
benefit
is
not
necessarily
permanent,
and
with
no
long
term
follow
up
of
this
syn-
drome
the
natural
history
is
unclear.
Since
the
initial
description
by
Lance
and
Anthony'
of
patients
aged
15
to
26
years,
with
an
onset
age
of
eight
to
15
years,
it
has
become
apparent
that
the
same
syndrome
may
present
up
to
at
least
the
age
of
61
years,
and
may
present
as
transient
symptoms
which
may
persist,
may
resolve
and
relapse,
or
may
become
permanent
and
present
as
a
perma-
nent
deficit.
In
a
significant
proportion
there
is
pathology
at
the
atlantoaxial
and
atlanto-
occipital
joints,
and
in
some
cases
the
syn-
drome
may
result
from
trauma.
We
thank
Professor
A
E
Harding
for
permission
to
include
details
of
the
patient
under
her
care.
1
Lance
JW,
Anthony
M.
Neck-tongue
syndrome
on
sud-
den
turning
of
the
head.
J7
Neurol
Neurosurg
Psychiatry
1980;43:97-101.
2
Cyriax
J.
Textbook
of
orthopaedic
medicine.
4th
ed.
Vol
1.
London:
Cassell,
1962:158.
3
Bogduk
N.
An
anatomical
basis
for
the
neck-tongue
syn-
drome.
J
Neurol
Neurosurg
Psychiatry
198
1;44:202-8.
4
Lance
JW.
Unusual
syndromes
in
neurological
practice.
Med
JAust
1984;140:409-16.
5
Elisevich
K,
Stratford
J,
Bray
G,
Finlayson
M.
Neck-
tongue
syndrome:
operative
management.
J
Neurol
Neurosurg
Psychiatry
1984;47:407-9.
6
Webb
J,
March
L,
Tyndall
A.
The
neck-tongue
syndrome:
occurrence
with
cervical
arthritis
as
well
as
normals.
J
Rheumatol
1984;11:530-3.
7
Fortin
CJ,
Biller
J.
Neck-tongue
syndrome.
Headache
1985;25:255-8.
8
Bertoft
ES,
Westerberg
CE.
Further
observations
on
the
neck-tongue
syndrome.
Cephalgia
1985;5(suppl
3):
312-3.
9
Cassidy
JD,
Diakow
PRP,
De
Korompay
VL,
Munkacsi
I,
Yong-Hing
K.
Treatment
of
neck-tongue
syndrome
by
spinal
manipulation:
a
report
of
three
cases.
The
Pain
Clinic
1986;1:41-6.
10
Terrett
AGJ.
Neck-tongue
syndrome
and
spinal
manipu-
lative
therapy.
In:
Vernon
H,
ed.
Upper
cervical
syn-
drome:
chiropractic
diagnosis
and
treatment.
Baltimore:
WiHiams
and
Wilkins,
1988:223-9.
11
Hankey
GJ.
"Neck-tongue"
syndrome
on
sudden
neck
rotation.
Aust
N
ZJ
Med
1988;18:181.
12
Noda
SN,
Umezaki
H.
Spinal
neck-tongue
syndrome.
J
Neurol
Neurosurg
Psychiatry
1984;47:75
1.
13
Bogduk
N.
Neck-tongue
syndrome.
Med,JAust
1980;2:4.
14
Liversedge
LA.
Involuntary
movements.
In:
Vinken
PJ,
Bruyn
GW,
eds.
Handbook
of
clinical
neurology.
Vol
1.
Amsterdam:
North-Holland,
1969:277-292.
15
Tier
GA,
Rees
RT,
Rood
JP.
The
sensory
nerve
supply
to
the
tongue:
a
clinical
reappraisal.
Br
Dent
Jf
1984;157:
354-7.
16
Kubota
K,
Narita
N,
Takada
K,
et
al.
Origin
of
lingual
proprioceptive
afferents
in
Japanese
monkey,
M
fuscata
fuscata.
Studied
by
HRP-labelling
technique.
Anat
Anz
1988;166:
141-8.
17
Cooper
S.
Muscle
spindles
in
the
intrinsic
muscles
of
the
human
tongue.
J
Physiol
(Lond)
1953;122:193-202.
18
Kubota
K,
Negishi
T,
Masegi
T.
Topological
distribution
of
muscle
spindles
in
the
human
tongue
and
its
signifi-
cance
in
proprioception.
Bull
Tokyo
Med
Dent
Univ
1975;22:235-42.
19
Adatia
AK,
Gehring
EN.
Proprioceptive
innervation
of
the
tongue.
JAnat
1971;110:215-20.
20
Lees
AJ,
Blau
JN,
Schon
F.
Paroxysmal
hemiglossal
twist-
ing.
Lancet
1986;ii:812-3.
352
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doi: 10.1136/jnnp.57.3.348
1994 57: 348-352J Neurol Neurosurg Psychiatry
R W Orrell and C D Marsden
The neck-tongue syndrome.
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