5.

3
NEUROSURGICAL
TREATMENT OF DYSTONIA
Leland Albright

Dystonia, the involuntary sustained muscle contractions that cause twisting and abnormal
postures, particularly in people with cerebral palsy (CP), is the second most common
movement disorder (after spasticity) in CP, and is greatly underrecognized. Diagnosis and
treatment of dystonia is important to orthopaedists treating individuals with CP. Otherwise,
as one pediatric orthopaedist quipped, ‘Dystonia is when the orthopaedic surgeon does an
operation and it doesn’t work.’

Review of relevant pathophysiology

Dystonia appears to be caused by excessive excitation of the premotor and supplementary
motor cortices because of an abnormality in the two neural circuits connecting them with
the basal ganglia. The resultant abnormal output from the primary motor cortex travels
down the corticospinal tract and out via the ventral roots to cause the dystonic muscle
contractions. Treatment could thus be targeted at the excessively stimulated cortex,
the dysfunctional neural loops between the basal ganglia and the cortex, or the nerves
innervating dystonic muscles.

Historical perspective
Dystonia was treated – ineffectively – in the first half of the 20th century by ablation of the
motor cortex and premotor cortex. In the 1950s, Cooper made lesions in the globus pallidus
and thalamus and reported improvement in 70% of patients but similar results have not
been replicated by others (Cooper 1969). Thalamotomies, operations that made lesions in
the ventrolateral thalamus, improved dystonia in approximately 25% of patients, but results
were unpredictable and delayed for several months (Andrew et al. 1983). Bilateral thalamic
and globus pallidus lesions are often complicated by trouble speaking and swallowing, and
are no longer done. Focal dystonia causing torticollis has been treated since the mid-1970s
by selective denervation of C1–5, the Bertrand procedure (Bertrand 1993).

Key points
• Dystonia must be distinguished from spasticity and athetosis, with which it may be
associated.
• Unless dystonia is treated, the dystonic muscle contractions it causes will increase
the likelihood that the orthopaedic release of a dystonic muscle contracture will be
unsuccessful.

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Indications from patient assessment
It is essential to determine whether the patient’s dystonia is primary (in which case it will
probably respond well to deep brain stimulation), dopa-responsive (in which case dopa –
e.g. Sinemet – is virtually curative), heredo-degenerative (in which case the treatment should
be one whose effects can be adjusted as the disease progresses), or secondary – the
consequence of cerebral palsy, trauma, or other structural lesions. The vast majority of the
cases of dystonia that orthopaedists will encounter are secondary dystonia, in persons with
cerebral palsy, where it may be focal, regional, hemidystonic or generalized. The most
common form by far (~ 90%) is generalized.
Neurosurgical treatment of dystonia can be considered if the patient has not responded
satisfactorily to oral medications and/or botulinum toxin injections. The dystonic move-
ments impede function and caregiving, and cause discomfort. Sustained dystonic muscle
contractions occasionally lead to the development of muscle contractures, particularly of
the plantarflexors and posterior tibial muscles. Because dystonic muscle contractions are
usually somewhat migratory and not sustained for long periods of time, musculoskeletal
contractures are much less common than in people with spasticity.

Goals of treatment
The primary goals of treatment are most often to increase patient comfort and to facilitate
care. Severe dystonia causes painful muscle spasms: individuals arch their neck forcibly
backward and break the headrest on their wheelchair; they extend their trunk backward –
making seating in a wheelchair difficult without a lap bar; and they extend the legs and feet
tonically downward, breaking the wheelchair footrest.
Improving function is not often a primary goal of neurosurgical intervention,
particularly in dystonic cerebral palsy. Although function may improve after treatment of
primary dystonia, it improves less often in people with secondary dystonia – on average, in
one-third. Prevention of musculoskeletal contractures is almost never a treatment goal in
dystonia, in contradistinction to treating spasticity.

Treatment options
Treatment modalities for dystonia include oral medications, intramuscular medications,
intrathecal medications, nerve transection, and deep brain stimulation.Oral medications are
used mainly for generalized dystonia in younger children, e.g. 2–8 years of age, and are
mildly helpful. The common oral medications are baclofen, trihexyphenidyl (Artane), and
levodopa-carbidopa (Sinemet). The use of a GABA agonist (baclofen), an anticholinergic
(trihexyphenidyl), and a dopa-agonist (levodopa-carbidopa) indicates that no single
neurotransmitter abnormality is at work in dystonia. Baclofen doses are usually 10–30 mg
t.i.d., trihexyphenidyl doses 3–7 mg t.i.d., and levodopa-carbidopa doses 25/100mg 1–3
times a day. Oral tizanidine and dantrolene have been used less often. On average, 26% of
individuals have a beneficial response to oral medications (Chuang et al. 2002). Combination
therapy appears to be more beneficial than monotherapy.
The intrathecal medication is almost always baclofen; intrathecal use of clonidine for
dystonia is rare. Intrathecal baclofen (ITB) may affect dystonia at a cortical level by

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inhibiting the excessively stimulated premotor and supplementary motor cortex. The site
of action is uncertain, however. Electrophysiological data including motor-evoked potentials
and H reflex have indicated a spinal site of action (Dachy and Dan 2004). It is quite possible
that both cortical and spinal sites are affected. ITB involves the implantation of a
programmable pump to continuously infuse baclofen via an intrathecal catheter (Fig. 5.3.1).
ITB may be infused in a continuous rate or a variable rate, e.g. 200mg every 4 hours. There
are no criteria to determine if a continuous or variable rate is better; if children do not
respond to continuous infusion of 800–1000 mg/day, intermittent boluses can be tried.
Nerve transections such as peripheral neurectomies, ramisectomies and rhizotomies
are operations that divide motor components of nerves innervating dystonic muscles. These
procedures have been used far more often in Europe, India and developing countries than
in the USA, and used more often to treat focal spasticity than focal dystonia, but because
they divide motor roots or fascicles, they treat both spasticity and dystonia. They will be
discussed in greater detail below.

Fig. 5.3.1 Infusion pump

and intrathecal catheter.

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 Deep brain stimulation(DBS) involves the stereotactic implantation of electrodes into
the basal ganglia (usually the internal globus pallidus) and an implantable, programmable,
pulse generator (Fig. 5.3.2). The electrodes may be implanted according to coordinates
calculated from magnetic resonance (MR) scans or by supplementing those coordinates
with data from microelectrode recording. In the latter, microelectrodes are inserted to a
depth approximately 2.5 cm above the target, then advanced in sub-millimeter increments
while recording the electrical discharges of individual neurons. Neurons in the putamen,
external globus pallidus and internal globus pallidus have characteristic patterns of
discharging; those patterns can be observed on an oscilloscope and heard on an amplifier.
When the best target is identified by the microelectrode recording, the microelectrode is
removed and a permanent electrode is inserted and connected via extension wires to the pulse
generator, which is implanted subcutaneously in the infraclavicular region. Postoperatively,
programming often requires several months to identify the optimal stimulation parameters.
Programming is complex, adjusting voltage, pulse width, stimulation frequency, and
contacts in either monopolar or bipolar modes. Programming DBS is far more complex
than programming ITB.

FOCAL DYSTONIA
Oral medications are used infrequently for focal dystonia. Intramuscular injections
of botulinum toxins (serotype A – Botox, and serotype B – Myobloc) are the primary
nonsurgical treatment and may be effective for several years. The American Academy of
Neurology recently reviewed the published literature about botulinum toxin and found
seven class I studies of its usefulness for cervical dystonia, one class I study and three class
II studies for focal upper-extremity dystonia, and one class II study for focal lower-extremity
dystonia (Simpson et al. 2008). Doses of botulinum toxin needed to treat dystonia are often
higher than those required to treat spasticity.
Neurosurgical procedures are indicated when botulinum toxin is ineffective or a more
permanent therapy is desired. Although peripheral neurectomies are often appropriate for
focal extremity spasticity, they are infrequently used for focal dystonia in the upper
extremities because they partially denervate the limb and never improve function. However,
sometimes partial denervation and the consequent limp limb is a significant improvement
over a severely dystonic limb. Focal cervical dystonia (causing some variant of torticollis)
can be treated with division of the posterior rami of C1–5, a modification of the original
Bertrand procedure (Bouvier and Molina-Negro 2004). Focal extremity dystonia in non-
functional children, or in sites where ITB and DBS are not available, can be treated with
ventral rhizotomies, dividing approximately 85% of the ventral roots innervating the
dystonia muscles. That degree of denervation does not paralyze the muscle but markedly
decreases the severity of dystonic muscle contractions. If the dystonia coexists with
spasticity in the same extremity, a dorsal rhizotomy can be done at the same time to treat
the spasticity (Albright and Tyler-Kabara 2007).
We have used epidural motor cortex stimulation (EMCS) to treat three young adults with
focal secondary dystonia of the upper extremity. EMCS involves the implantation of an
epidural strip electrode over the motor cortex innervating the dystonic upper extremity

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 Fig. 5.3.2 Anteroposterior radiograph
(top) and coronal MRI (bottom)
showing bilateral DBS electrodes in
the internal globus pallidus.

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(Priori and Lefaucheur 2007) (Fig. 5.3.3). The electrode is connected by extension wires to
the same type pulse-generator that is used in DBS. We obtained sustained mild improvement
in dystonia in one of the three patients but only transient improvement in the other two.
EMCS has the potential to treat focal upper-extremity dystonia with considerably less
complexity than is involved with DBS but its use is in its infancy.

HEMIDYSTONIA
The typical appearance of a person with hemidystonic CP is demonstrated in Figure 5.3.4.
The appearance is different from that of an individual with spastic hemiparesis – lacking
the characteristic flexion at multiple joints – and muscle bulk in dystonia is typically
accentuated because of the sustained ‘isometric’ muscle contractions. As shown in the
figure, muscle contractures develop and require orthopaedic releases. However, recurrence
of the contractures is likely if the dystonia is not addressed, analogous to the recurrence of
contractures if significant spasticity is not addressed.
Hemidystonia is usually secondary to a structural lesion in the basal ganglia or
thalamus and may be treated by either ITB or deep brain stimulation if it is refractory to oral
medications. No comparisons of the outcomes of ITB and DBS have been done. ITB is
a simpler treatment for hemidystonia than DBS. As an initial neurosurgical step, it is

Fig. 5.3.3 Strip electrode used

for epidural motor cortex
stimulation.

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reasonable to evaluate the response of hemidystonia to ITB by a continuous infusion
screening trial.
In such a trial, an intrathecal catheter is inserted and connected to an external micro-
infusion pump. ITB is infused continuously, beginning at 200 mg/day and increasing in
increments of 50 mg every 8 hours until improvement of the dystonia is evident, adverse
side effects occur, or a total daily dose of 900 mg/day is given without reduction in the
dystonia. Too few patients with hemidystonic CP have been treated to know accurately
how effective it is; extrapolation from data about ITB for generalized dystonia would
indicate improvement in 85%–90%. Ideally, dystonia is graded before the trial and at
intervals during it by a physical therapist. Grading can be with either of two validated scales,
the Barry–Albright Dystonia (BAD) scale or the Burke–Fahn–Marsden (BFM) scale (Barry
et al. 1999). The BAD scale was developed to grade secondary dystonia in children, the BFM
scale to grade primary dystonia in adults.
DBS has been used to treat hemidystonia in a small number of patients. In Zhang et al.’s
(2006) series of nine patients with secondary dystonia, two patients with hemidystonia were
treated by DBS of the subthalamic nucleus. One had slight improvement and the other had
less stiffness of his shoulder and mildly improved posture. Loher et al. (2000) used DBS of
the internal globus pallidus to treat a 24-year-old with hemidystonia secondary to a head

Fig. 5.3.4 Characteristic hemidystonic

posture.

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injury at age 15 with sustained improvement in dystonia, pain and posture after 4 years of
follow-up.

GENERALIZED DYSTONIA
Perhaps 90% of dystonia in persons with CP is generalized; most of these are non-
ambulatory and in Gross Motor Function Classification System (GMFCS) levels IV and V.
Neurosurgical intervention is appropriate if they have failed oral medications, but is also
appropriate if their dystonia is severe, since the likelihood that it can be effectively treated
with oral medications, even multiple oral medications, is small.
ITB is the initial treatment for severe generalized dystonic CP. We used continuous ITB
infusion screening trials in the first hundred or more patients and found that 90% or more
responded to the infusion with significant reductions in dystonia, and now no longer do the
screening trials. The intrathecal catheters are positioned higher, e.g. C1–4, when treating
dystonia than when treating spastic quadriparesis. In general, ITB doses needed to treat
dystonia are twice or more the doses needed to treat spasticity (where the site of action is
clearly at the spinal cord level). Recently intraventricular baclofen (IVB) has been used to
treat generalized dystonic CP (Albright 2009). IVB is particularly useful in children with
fused spines who need infusion into spinal fluid. IVB doses needed to obtain satisfactory
improvement in dystonia appear to be lower than when the infusion is intrathecal.
In our 2001 report of ITB in the first 89 cases with dystonia treated for 1 year or longer,
we found that dystonia scores on the BAD scale improved signifi cantly, from a mean score
of 18 (out of a maximum possible of 32) pre-ITB, to 13 at 3 months, 10 at 6 months, and 7
at 12 months postoperatively, p < 0.001 (Albright et al. 2001). 92% of the patients retained
their improvement during 2 years of follow-up. Patients and caregivers reported that quality
of life improved in 85%, ease of care increased in 86%, speech improved in 33%, and
extremity function improved in 34/36% (upper/lower extremities). Since that publication,
other authors have reported benefit of ITB for individuals with secondary dystonia (Dachy
and Dan 2004, Woon et al. 2007, Motta et al. 2008). Motta et al. (2008) evaluated the effects
of ITB in children in level V of the Gross Motor Function Classification Sysyem (GMFCS)
by its effects on their BFM and BAD scores at 3, 6 and 12 months after ITB. Scores were
significantly decreased on both scales, to approximately the same extent.
The use of ITB in dystonia appears to be associated with a higher frequency of
complications than are seen when treating spasticity. In our 2001 publication, surgical
complications occurred in 36% of patients and included CSF leaks in 8%, infections in 14%
and catheter problems in 21% (Albright et al. 2001). Several patients had more than one
complication. However, the complication rate has decreased substantially since then. The
infection rate has decreased with better antimicrobial therapy, including prepping with
chlorhexidine and other modalities listed in a recent publication about best practices in ITB
therapy (Albright et al. 2006). The currently available intrathecal catheters are far better than
the ones used prior to the 2001 publication, and have been associated with complication rates
in the 5% range in the past 2 years.
For patients with generalized secondary dystonia who do not respond adequately to
ITB, DBS should be considered. DBS is the treatment of choice for primary dystonia but

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for secondary generalized dystonia it is generally used only if ITB is found to be ineffective.
To treat generalized dystonia, electrodes are usually inserted bilaterally into the internal
globus palllidus (GPi) (Starr et al. 2006). Electrodes have been inserted recently into the
subthalmic nucleus; data are not available to know which of the two sites is better.
It seems clear that the response of secondary dystonia to DBS is substantially less than
the response of primary dystonia. Cif et al. (2003) reported DBS of GPi in 21 patients. At
a mean follow-up of 23 months, their Burke–Fahn–Marsden (BFM) scores were decreased
by 31%. Alterman and Tagliati (2007) treated five children with secondary dystonia with
DBS of the internal globus pallidus and observed 33% reductions in their BFM scores one
year postoperatively. Zhang et al. (2006) treated nine patients who had secondary dystonia
with DBS, primarily of the subthalamic nucleus, and reported only mild improvement. Of
the six patients reported by Eltahawy et al. (2004) with DBS of the GPi, patients had either
mild or no improvement in their dystonia and no functional improvement.

Summary
The treatment of dystonia in individuals with CP has improved substantially in the past
decade. Focal dystonia is being treated with peripheral neurectomies or ventral rhizotomies.
Hemidystonic CP is being treated with ITB or DBS, although which procedure is better is
unknown. Generalized dystonic CP, the most common form, can be substantially improved
in most individuals with ITB, and for those who do not respond sufficiently to ITB, DBS
may be helpful. Although the improvement of dystonia by DBS is only 30%–35% or so,
that improvement may be of substantial clinical significance in persons with severe dystonia
who have been refractory to oral medications, botulinum toxin injections and ITB.

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