Authors:
Kevin Cairns, MD, MPH
Joel Stein, MD

Spasticity

Affiliations:
From the Department of Physical
Medicine and Rehabilitation, Harvard
Medical School, Spaulding
Rehabilitation Hospital Network,
Boston, Massachusetts.

Case Report

Correspondence:
All correspondence and requests for
reprints should be addressed to Joel
Stein, MD, Department of Physical
Medicine and Rehabilitation, Harvard
Medical School, Spaulding
Rehabilitation Hospital Network, 125
Nashua Street, Boston, MA
02114-1198.
0894-9115/02/8104-0307/0
American Journal of Physical
Medicine & Rehabilitation
Copyright © 2002 by Lippincott
Williams & Wilkins

Motor Function Improvement
Following Intrathecal Baclofen
Pump Placement in a Patient with
Locked-in Syndrome
ABSTRACT
Cairns K, Stein J: Motor function improvement following intrathecal
baclofen pump placement in a patient with locked-in syndrome. Am J
Phys Med Rehabil 2002;81:307–309.
We describe a patient with locked-in syndrome who had minimal volitional motor function and severe spasticity in all four extremities. The
patient showed a significant improvement in volitional motor function
following intrathecal baclofen pump therapy to control spasticity. This
case study suggests that intrathecal baclofen pump therapy might improve motor function in select patients with locked-in syndrome.
Key Words: Locked-in Syndrome, Intrathecal Baclofen Pump, Spasticity, Motor Function

L

ocked-in syndrome (LIS) is a debilitating syndrome generally caused by
injury of the ventral pons. Its main clinical manifestations are quadriplegia,
lower cranial nerve paralysis, and mutism. Patients with classic LIS are unable
to talk or move. Consciousness remains intact, and patients are only able to
communicate by using eye blinking or eye movements.
There are three variants of LIS characterized in the literature: classical,
incomplete, and total.1 The classical type, originally noted by Plum and Posner,2 includes patients with the signs and symptoms described above. Patients
classified as having incomplete LIS are similar to the classical variety except
that they retain limited motor function beyond vertical eye movements. The
total category consists of patients who are totally immobile and cannot
communicate.
Cases of immediate and complete recovery from transient LIS have occurred.3
However, lack of recovery after 6 mo is strongly predictive of chronic and persisApril 2002

Motor Function Improvement

307

tent LIS.4,5 Patients who survive beyond the first 2 mo have a significantly
lower mortality rate.6 Rehabilitation efforts typically focus on establishing
communication strategies, often with
the use of computer-aided technologies, and establishing environmental
controls. Relatively small motor function gains can give a patient with LIS
the ability to interact with their environment by using computer-aided
technologies, motorized vehicles, and
greater spontaneous expression. We report a case of LIS in which intrathecal
baclofen (ITB) pump therapy utilized
for spasticity was found to have unanticipated benefits with regard to improved motor control.
Spasticity as described by Lance7 is
“a motor disorder characterized by a
velocity-dependent increase in tonic
stretch reflexes (muscle tone) with exaggerated tendon jerks.” Quantifying
spasticity can be challenging because
of observer variability, fluctuating temporal measurements, and the plethora
of different clinical scales in use. The
modified Ashworth scale is one clinical
scale widely used to quantify spasticity
because it has relatively high interrater
reliability.8

CASE REPORT
We describe the case of a otherwise healthy 37-yr-old man with a history of congenital coarctation of the
aorta who underwent an elective repair
of the coarctation via an endovascular
stent placement. Immediately after the
procedure, the patient was able to communicate normally. During the
evening of the procedure, he became
increasingly lethargic, had difficulty
with vocalization, and developed right
hemiplegia. The initial evaluation included a computed tomographic scan
of the head and lumbar puncture, both
of which were negative. A subsequent
magnetic resonance image revealed an
acute obstruction of the basilar artery
in the posterior circulation and a ventral pontine infarction (Fig. 1). The patient’s symptoms continued to deterio-

308

Cairns and Stein

Figure 1: T2-weighted flair magnetic
resonance image with an arrow
pointing to the area of infarction in the
ventral pons.
rate to the point that he was “locked
in” and unable to move his extremities
or verbalize. His volitional movements
were limited to vertical eye movements
and closing his eyelids. His acute hospital course was marked by mechanical
ventilation for airway protection and
gastrostomy tube placement for nutrition. Significant spasticity developed
in all four extremities. The patient was
transferred to a rehabilitation hospital
for a multidisciplinary rehabilitation 6
wk after the brainstem infarction.
During his rehabilitation hospitalization, the patient exhibited persistent four-limb spasticity without
observable volitional control. The patient’s reflexes were brisk in all four
limbs and his lower extremities exhibited clonus bilaterally. No cogwheeling was evident on exam and
his sensory exam was unreliable. The
patient was able to respond with reasonable consistency to yes or no
questions by using eye movements.
Progressive spasticity of his hip
flexors and knee flexors developed (3/4
on the modified Ashworth scale) and
resulted in the patient’s heels being in
contact with his buttocks. Painful spasticity affecting all four limbs interfered
with positioning, passive range of motion, and attempts to establish improved communication or environmental control. Trials of oral

medications were undertaken, including oral baclofen, tizanidine, and dantrolene. The patient continued to experience an abnormal resting position
with his lower extremities flexed at the
knee without being able to be stretched
to an extended position. The patient
was unable to perform head movements such as flexion or extension, lateral rotation, and shrugging of his
shoulders on a consistent basis. Oral
medications were attempted, but they
were limited by dose-related sedation.
Because of his persistent wide-spread
spasticity refractory to oral medical
therapy, a test dose of 50 ␮g of baclofen
was injected intraspinally. The patient
showed a substantial reduction in spasticity of 1–2 points on the modified
Ashworth scale in affected limbs. The
patient subsequently had an ITB pump
placed 16 wk after transfer. An infusion
rate of baclofen beginning at 50 ␮g/day
was gradually increased. Four days after initiating ITB pump therapy, the
patient had a marked decrease in spasticity most evident in his lower extremities. Furthermore, the patient demonstrated left thumb abduction to
command with a 3/5 strength rating.
The baclofen dosage was titrated to 230
␮g/day, which appeared to provide optimal control of spasticity. The patient
continued to exhibit further motor
function gains, including being able to
perform flexion and extension movements and lateral rotation of his head
on a consistent basis and being able to
shrug his left shoulder.
His subsequent rehabilitation
hospital course was notable for gradual return of spasticity with attendant loss of motor control. This was
attributed to ITB tolerance, and improved with a progressive increase of
dosage to 330 ␮g/day. This restored
control of the patient’s spasticity, and
his motor control returned to his
new baseline. The patient now utilizes head nodding and shoulder
movements routinely for communication. Effort is being made to develop
more consistent control of switches
with his left thumb.

Am. J. Phys. Med. Rehabil. ● Vol. 81, No. 4

DISCUSSION
This case illustrates the potential
of ITB pump therapy to unmask volitional motor function that may be
hidden in extreme cases of spasticity.
It has been shown that continuous
ITB decreases spasticity in individuals with long-standing hemiplegia.9
This patient showed both a significant reduction in spasticity after continuous ITB and also improved motor
function. Although in many individuals the return of slight motor function may seem insignificant, in the
case of LIS, these movements can be
critical. In the case of the patient
described, limited movements allowed the patient a much broader
range of spontaneous expression
without the use of technological aids.
Furthermore, limited motor function
of his left thumb enhanced efforts to
operate computer-aided devices that
could aid in many different functions,
including operating a motorized
wheelchair, communication, and environmental control.
We distinguished spasticity in our

patient from other forms of increased
muscle tone on the basis of the clinical
exam. The patient had brisk reflexes in
all four extremities and lower extremity clonus bilaterally. Furthermore,
tone in his lower extremities was velocity dependent, consistent with Lance’s
definition of spasticity.
Although it is possible that the patient’s improved motor control was
simply coincidental, this seems unlikely. The patient was noted to regain
function 4 days after the ITB pump
therapy was initiated. Furthermore,
when the patient’s spasticity worsened
because of ITB tolerance, his control of
head movements declined, only to improve with an increase in ITB dosage.
This case illustrates the potential use of
ITB therapy in some patients with LIS
to unmask motor function that is being hindered by severe spasticity.

REFERENCES
1. Bauer G, Gerstenbrand F, Rump E:
Variables of the locked-in syndrome.
J Neurol 1979;221:77–91

2. Plum F, Posner JB: The Diagnosis of
Stupor and Coma. Philadelphia, Davis,
1966
3. Khurana RK, Genut AA, Yannakis GD.
Locked-in syndrome with recovery. Ann
Neurol 1980;8:439 – 41
4. Katz RT, Haig AJ, Clark BB: Longterm survival, prognosis, and life care
planning for 29 patients with chronic
locked-in syndrome. Arch Phys Med Rehabil 1992;73:403– 8
5. Patterson JR, Grabois M: Locked-in
syndrome: A review of 139 cases. Stroke
1986;17:758 – 64
6. Haig AJ, Katz RT: Locked in Syndrome:
A Review. Curr Concepts Rehabil Med
1986;3:12– 4
7. Lance JW: Symposium synopsis, in
Feldman RG, Young RR, Koella WP
(eds): Spasticity: Disordered Motor
Control. Chicago, Year Book Medical
Publishers, 1980
8. Bohannon RW, Smith MB: Interrater
reliability on a modified Ashworth scale of
muscle spasticity. Phys Ther 1987;67:
206 –7
9. Meythaler JM, Guin-Renfroe S, Hadley
MN: Continuously infused intrathecal baclofen for spasticity/dystonic hemiplegia:
A preliminary report. Am J Phys Med Rehabil 1999;78:247–54

Letters to the Editor
The American Journal of Physical Medicine & Rehabilitation welcomes letters to the Editor that objectively
critique information appearing in the Journal. Letters should be limited to a maximum of 500 words (approximately
two double-spaced pages). No unsigned letters will be accepted for publication. If a letter is accepted for publication,
a copy will be sent to the principal author of the material being discussed, who will have an opportunity to submit
a response for publication. All letters are subject to editing by the Editor.

April 2002

Motor Function Improvement

309