CLINICAL/SCIENTIFIC NOTES
exclude a non-dystonic, “peripheral nerve-induced” spasm of
the cervical muscles. Moreover, the postural abnormalities observed in our patient resembled those described in several
published series of patients13,14 with disturbance of head and
neck posture resulting from muscular or radiologically identifiable disorders (such as posterior fossa and cervical spinal cord
tumors, craniocervical junction or cervical spine abnormalities,
focal myositis, or fibrosis). The authors13,14 consider this motor
disorder as a non-dystonic spasm or pseudodystonia, suggesting that it might have a different (“peripheral”) pathophysiology than true dystonia.
Probably, our report of a single case does not allow us to
definitively resolve the controversy in the literature4,5,13–16 as
to whether or not a post-traumatic abnormal shoulder elevation
and hypertrophy of the ipsilateral trapezius muscle can be
considered as dystonia. Further patient studies with careful
analysis of both central and peripheral motor control pathways
might be helpful. Nevertheless, we are convinced that not all
patients with this motor disorder should have the diagnosis of
isolated focal shoulder elevation dystonia and that this is less
likely when EMG demonstrates the presence of spontaneous
CRDs or other signs of abnormal peripheral nerve-induced
activity in the hypertrophied muscles.

Legends to the Video
Segment 1. Before treatment, the patient showed increased
muscle bulk of his left trapezius muscle with impairment of
shoulder elevation and arm abduction and inability to completely
abduct his arm. EMG findings of the left trapezius muscle were
consistent with continuous complex repetitive discharges of simple or polyphasic potentials at all sites in the completely relaxed
muscle.
Segment 2. After treatment, an almost complete resolution of
muscle hypertrophy, abnormal posture, and patient discomfort has
been observed but EMG findings seemed unchanged. The patient
was now able to raise and abduct the shoulder. The abnormal
displacement of the scapula observed in the extreme arm abduction was due to a slight weakness of the trapezius muscle that
might be related in part to botulinum toxin treatment.
Acknowledgments: We thank Mr. F. Matta for providing technical
assistance in preparing the videotape.

References
1. Schott GD. Induction of involuntary movements by peripheral
trauma. An analogy with causalgia. Lancet 1986;2:712–716.
2. Brin MF, Fahn S, Bressman SB, Burke RE. Dystonia precipitated
by peripheral trauma. Neurology 1986;36(Suppl.):119.
3. Jankovic J, Van der Linden C. Dystonia and tremor induced by
peripheral trauma: predisposing factor. J Neurol Neurosurg Psychiatry 1988:51:1512–1519.
4. Jankovic J. Post traumatic movement disorders: central and peripheral mechanisms. Neurology 1994;44:2006 –2014.
5. Jankovic J. Can peripheral trauma induce dystonia and other movement disorders? Yes! Mov Disord 2001;16:7–12.
6. Emeryk B, Hausmanova-Petrusewicz J, Nowak T. Spontaneous
volley of bizarre high frequency potentials in neuromuscular diseases. Electromyogr Clin Neurophysiol 1974;14:303–312.
7. Nix WA, Butler IJ, Roontga S, Gutmann L, Hopf HC. Persistent
unilateral tibialis anterior muscle hypertrophy with complex
repetitive discharges and myalgia: report of two unique
cases and response to botulinum toxin. Neurology 1992;42:
602– 606.

1111

8. Gutmann L. AAEM minimonograph #46: neurogenic muscle hypertrophy. Muscle Nerve 1986;19:811– 818.
9. Mattle HP, Hess CW, Ludin HP, Mumenthaler M. Isolated muscle
hypertrophy as a sign of radicular or peripheral nerve injury.
J Neurol Neurosurg Psychiatry 1991;54:325–329.
10. Pihko H, Leitinen I, Tikkanen H, Harkonen M, Rapola J, Lamminen A, Sahlman A, Somer H. Progressive unilateral hypertrophic
myopathy. A case study. Muscle Nerve 1993;16:63– 68.
11. Wright RA, Ahlskog JE. Focal shoulder-elevation dystonia. Mov
Disord 2000;15:709 –713.
12. Berardelli A, Rothwell JC, Hallet M, Thompson PD, Manfredi M,
Marsden CD. The pathophysiology of primary dystonia. Brain
1998;121:1195–1212.
13. Suchowersky O, Calne DB. Non-dystonic cause of torticollis. Adv
Neurol 1988;50:501–508.
14. Tarsy D. Comparison of acute- and delayed-onset posttraumatic
cervical dystonia. Mov Disord 1998;13:481– 485.
15. Thyaragarajan D, Kompoliti K, Ford B. Post-traumatic shoulder
“dystonia”: persistent abnormal postures of the shoulder after
minor trauma. Neurology 1998;51:1205–1207.
16. Weiner J. Can peripheral trauma induce dystonia and other movement disorders? No! Mov Disord 2001;16:13–22.

Response of Postapoplectic Hemichorea/Ballism
to GPi Pallidotomy: Progressive Improvement
Resulting in Complete Relief
Kazumichi Yamada, MD, PhD,1 Mikio Harada, MD, PhD,2
and Satoshi Goto, MD, PhD1*
1

Department of Neurosurgery, Graduate School of Medical
Sciences, Kumamoto University, Kumamoto, Japan
2
Department of Neurosurgery, Kumamoto Neurosurgical
Hospital, Kumamoto, Japan

Abstract: We report on a 66-year-old woman in whom GPi
pallidotomy produced progressive and eventually complete relief of hemichorea/ballism (HCB) after a subthalamic hemorrhage. Although choreoballistic movements were unchanged
during and immediately after the surgery, the symptoms were
gradually improved and consequently abolished by 5 days
postoperatively. HCB has never recurred up to the present (9
months follow-up period). This note is the first report describing detailed postsurgical process in HCB relief after pallidotomy. © 2004 Movement Disorder Society
Key words: ballism; chorea; pallidotomy

This article contains supplementary video clips, available online at
http://www.interscience.wiley.com/jpages/0885-3185/suppmat.
*Correspondence to: Dr. Satoshi Goto, Department of Neurosurgery,
Graduate School of Medical Sciences, Kumamoto University, 1-1-1
Honjo, Kumamoto 860-8556, Japan.
E-mail: sgoto@kaiju.medic.kumamoto-u.ac.jp
Received 20 August 2003; Revised 2 February 2003; Accepted 24
February 2004
Published online 22 April 2004 in Wiley InterScience (www.
interscience.wiley.com). DOI: 10.1002/mds.20143

Movement Disorders, Vol. 19, No. 9, 2004

1112

CLINICAL/SCIENTIFIC NOTES

Chorea and ballism are characterized by rapid, brief, irregular,
involuntary movements that occur spontaneously and interrupt
ongoing voluntary movements.1,2 They often coexist, and usually involve hemibody.1,3 Stereotactic surgery has long been
used to treat hemichorea/ballism (HCB)4 that appears in a
variety of diseases.1 The globus pallidus internus (GPi) is now
reintroduced as the major target in ablative surgery or deep
brain stimulation (DBS) to treat HCB.5–9 Lesioning or blocking
the activity of the GPi is thought to release the motor circuit
from the abnormally patterned neuronal activities in the GPi
that are suspected to underlie the manifestation of HCB and
other hyperkinetic disorders.7,8 However, it is currently not
clear how HCB responds to pallidal surgery or whether pallidotomy or pallidal stimulation offers the greater beneficial
effect. We report a patient in whom GPi pallidotomy produced
progressive and eventually complete relief of HCB caused by
subthalamic hemorrhage.

Case Report
A 66-year-old woman suffered from mild right hemiparesis
due to a left thalamic hemorrhage that had occurred 8 years
earlier. Then while watching television, she experienced transient motor weakness in her left extremities followed by the
sudden development of rapid, brief, irregular involuntary
movements. On admission the following day, she showed ballistic and choreatic movements involving the proximal and
distal part of both the upper and lower limbs on the left side.
Computed tomography disclosed a cerebral hematoma in the
right subthalamic nucleus (STN; Fig. 1); an old hematoma in
the left thalamus and multiple lacunar lesions in the bilateral
cerebral hemispheres were also noted. Over the next few
weeks, her symptoms gradually worsened and spread to her
neck. This condition was sustained throughout the day and was
exacerbated by emotional stress. She could neither sit nor stand
by herself and had to be fed. Her condition kept her confined to
the bed, and the continuous volitional movements resulted in
complete exhaustion. Treatment with haloperidol and tiapride
brought no relief. As she wanted to be cured of HCB by any
means, we decided to perform stereotaxy after obtaining informed consent.
With the patient under transient general anesthesia with
propofol, right GPi pallidotomy was carried out with the aid of
magnetic resonance imaging (MRI), third ventriculography,
and microelectrode guidance.10,11 The initial target coordinates
for the posteroventral part of the GPi were calculated with MRI
and the Leksell stereotactic frame (G frame; Elekta, Stockholm,
Sweden). Semi-microelectrode stimulation (100-␮sec pulse
width, 160 Hz frequency, and up to 3.0 V) was then applied to
determine the effect on vision, speech, and sensory motor
function of the limbs on the contralateral. The optimal target
was determined to be 2 mm anterior and 20 mm lateral to the
midpoint of the anterior-to-posterior commissure line, and 1
mm dorsal to the floor of the third ventricle. After creating a
test lesion (42°C, 60 seconds), a permanent anatomic lesion
was made by heating the electrode tip to 72°C for up to 70
seconds. The electrode was moved in 2-mm increments in the
medial, lateral, and dorsal direction, and the lesioning process
was repeated to increase the overall size of lesion.
Upon completion of the lesioning process, while the patient
was still in the operating room, we did not observe an obvious
change in the severity of her HCB and thought it might necessary to perform additional stereotaxy (e.g., thalamic sur-

Movement Disorders, Vol. 19, No. 9, 2004

FIG. 1. Computed tomography on admission, demonstrating a cerebral hematoma confined to the right subthalamic nucleus.

gery)11 at a later time. However, 2 hours later while moving her
to the recovery room, the involuntary movements in her neck
and left shoulder ceased completely and the choreatic and
ballistic movements of her left hand and leg were considerably
diminished. By the first postoperative day, all ballistic movements had almost disappeared and only mild chorea persisted in
the distal portions of her left hand and leg. Three days after
surgery, slight choreic movements were noted in the distal
portion of her left hand but not in her leg. Her HCB had
completely resolved by 5 days after surgery, and no adverse
effects attributable to surgery occurred during her postoperative
course. Magnetic resonance images obtained at 18 days after
surgery showed that the lesion in the posterior portion of the
right GPi was approximately 8 mm in diameter (Fig. 2). The
patient has resumed her normal activities and, at present (9
months follow-up period), she is doing well and exhibits no
deficits, except for mild right hemiparesis due to the left thalamic hemorrhage that had occurred 8 years earlier.

Discussion
HCB has been thought to be the result of a reduced excitatory output from the STN to the GPi.3,13 Several studies6 – 8
have shown decreased discharge rate of GPi neurons in patients
with hemiballismus or hemichorea. Since GPi pallidotomy
should give rise to a further decrease in the neuronal activity of
the GPi, its beneficial effect on HCB presents a paradox. Vitek
and coworkers8 have proposed an alternative model for movement disorders. They suggest that an alteration in the pattern,
but not the rate, of neuronal discharges in the basal ganglia

CLINICAL/SCIENTIFIC NOTES

1113

toms involved the neck and the distal and proximal portions of
her left upper and lower extremities.
Of special interest in our patient is the progressive, although
not immediate, improvement of her HCB after pallidotomy. To
our knowledge, ours is the first report of such alleviation of
HCB after stereotaxy. Another type of hyperkinetic disorder,
dystonia, is known to respond to pallidal surgery and pallidotomy and pallidal stimulation often produces progressive
improvement of dystonic movements and postures.8,11,17,18 Although the mechanism(s) underlying these phenomena remains
unclear, our observations may provide helpful information in
postsurgical managements of HCB after pallidotomy.

Legends to the Video

FIG. 2. Coronal view of T2-weighted magnetic resonance image obtained at 18 days after the surgery. A thermocoagulative lesion (arrow)
is made in the posterior portion of the right globus pallidus internus.

provokes anomalous activities throughout the pallido-thalamocortical circuit and results in the observed involuntary movements. This concept, if confirmed, justifies the use of pallidal
surgery to treat patients with HCB or other hyperkinetic disorders such as dystonia. On the other hand, Hutchison and colleagues14 more recently reported lack of global suppression of
the GPi neuron activities in dystonic patients, suggesting that
the current model of a “hypoactive basal ganglia output” is not
a consistent feature of hyperkinetic disorders. Thus, the neural
mechanism underlying the occurrence of dystonia is still debated.
Both GPi pallidotomy and pallidal stimulation are reported
to be highly effective for levodopa-induced dyskinesias that
may present with ballistic, choreic, athetoid, and dystonic features in patients with Parkinson’s disease.15,16 However, there
is evidence that GPi pallidotomy did, while pallidal stimulation
did not, produce relief of chorea subsequent to a vascular
insult6 – 8 or neuroacanthocytosis.5,9 On the basis of the negative
results of pallidal stimulation in a patient with chorea–acanthocytosis, Wihl and coworkers9 suggested that DBS could not
sufficiently suppress the GPi activities that provoke choreatic
movements. We suggest that ablative surgery has the advantage
of producing a pallidal lesion of the ideal volume, size, and
shape to abolish hyperkinesias. This consideration led us to
choose pallidotomy to treat our patient, and we believe the
eventual complete resolution of her HCB justifies our decision,
but more experience is required before GPi pallidotomy rather
than stimulation can be recommended for treatment of HCB.
Outflow pathway from GPi is known to influence not only
the thalamocortical pathway that controls distal appendicular
musculature by means of descending corticospinal and corticobulbar tracts but also the brainstem motor centers such as
pedunculopontine nucleus relating to the mesencephalic tegmental field that controls axial and proximal appendicular
musculature by means of the descending reticulospinal tract.17
Therefore, unlike thalamic surgery, pallidal surgery may have
an effect on the control of both distal and axial muscles. On the
basis of these considerations, we chose pallidal rather than
thalamic surgery to treat our patient whose hyperkinetic symp-

Segment 1. Before surgery, the patient manifested continuous volitional choreoballistic movements in her left upper and
lower extremities.
Segment 2. At 2 weeks after surgery, choreoballistic movements were completely resolved. Mild right hemiparesis due to
the left thalamic hematoma that had occurred 8 years earlier
was additionally noted.

References
1. Fahn S. Sydenham and other forms of chorea. In: Rowland LP,
editor. Merritt’s neurology. 10th ed. Philadelphia: Lippincott Williams and Wilkins; 2000. p 662– 666.
2. Lang AE, Weiner WJ. Movement disorders: a comprehensive
survey. Mount Kisco, NY: Futura Publishing; 1989.
3. Shannon KM. Hemiballismus. Clin Neuropharmacol 1990;13:
413– 425.
4. Krauss JK, Mundinger F. Functional stereotactic surgery for hemiballism. J Neurosurg 1996;85:278 –286.
5. Fujimoto Y, Isozaki E, Yokochi F, Yamakawa K, Takahashi H,
Hirain S. A case of chorea-acanthocytosis successfully treated with
posteroventral pallidotomy. Rinsho Shinkeigaku 1997;37:891–
894.
6. Hashimoto T, Morita H, Tada T, Maruyama T, Yamada Y, Ikeda
S. Neuronal activity in the globus pallidus in chorea caused by
striatal lacunar infarction. Ann Neurol 2001;50:528 –531.
7. Suarez JI, Verhagen Metman L, Reich SG, Dougherty PM, Hallett
M, Lenz FA. Pallidotomy for hemiballismus: efficacy and characteristics of neuronal activity. Ann Neurol 1997;42:807– 811.
8. Vitek JL, Chockkan V, Zhang J-Y, et al. Neuronal activity in the
basal ganglia in patients with generalized dystonia and hemiballismus. Ann Neruol 1999;46:22–35.
9. Wihl G, Volkmann J, Allert N, Lehrke R, Sturm V, Freund H-J.
Deep brain stimulation of the internal pallidum did not improve
chorea in a patient with neuro-acanthocytosis. Mov Disord 2001;
16:572–575.
10. Goto S, Hamasaki T, Nishikawa S, et al. Temporal sequence of
response to unilateral GPi pallidotomy of motor symptoms in
Parkinson’s disease. Stereotact Funct Neurosurg 2000;75:160 –
166.
11. Muta D, Goto S, Nishikawa S, et al. Bilateral pallidal stimulation
for idiopathic segmental axial dystonia advanced from Meige
syndrome refractory to bilateral thalamotomy. Mov Disord 2001;
16:774 –777.
12. Goto S, Kunitoku N, Hamasaki T, Nishikawa S, Ushio Y. Abolition of postapopletic hemichorea by Vo-complex thalamotomy:
long-term follow-up study. Mov Disord 2001;16:771–774.
13. DeLong MR. Primate models of movement disorders of basal
ganglia origin. Trends Neurosci 1990;13:281–285.
14. Hutchison WE, Lang AE, Dostrovsky JO, Lozano AM. Pallidal
neuronal activity: implication for models of dystonia. Ann Neurol
2003;53:480 – 488.

Movement Disorders, Vol. 19, No. 9, 2004

1114

CLINICAL/SCIENTIFIC NOTES

15. Lang AE. Surgery for levodopa-induced dyskinesias. Ann Neurol
2000;47(Suppl. 1):S193–S202.
16. Volkmann J, Sturm V, Weiss P, et al. Bilateral high-frequency
stimulation of the internal globus pallidus in advanced Parkinson’s
disease. Ann Neurol 1998;44:953–961.
17. Iacono RP, Kuniyoshi SM, Schoonenberg TR. Experience with
stereotactics for dystonia: case examples. Adv Neruol 1998;78:
221–226.
18. Kumar R. Methods for programming and patient management with
deep brain stimulation of the globus pallidus for the treatment of
advanced Parkinson’s disease and dystonia. Mov Disord 2002;
17(Suppl. 3):S198 –S207.

Pramipexole-Treated Parkinson’s Disease
During Pregnancy
Marco Mucchiut, MD,1* Enrico Belgrado, MD,1
Daniela Cutuli, MD,1 Angelo Antonini, MD,2
and Paolo Bergonzi, MD1
1

Clinica Neurologica, Policlinico Universitario, Udine, Italy
2
Centro Parkinson, Istituti Clinici di Perfezionamento,
Milano, Italy

Abstract: There are few reports about drug-related effects on
PD pregnancy. We describe the case of a woman affected by
PD treated with pramipexole monotherapy during pregnancy.
The child, born by caesarean delivery, is healthy, whereas
motor disability of the mother progressively increased to the
point that levodopa therapy was necessary. © 2004 Movement
Disorder Society
Key words: Parkinson’s disease; pregnancy; pramipexole
There are few clinical reports concerning the pregnancy of
parkinsonian women because of the rarity of this condition;
consequently, we have little information about the safety of
antiparkinsonian drugs during pregnancy. Most of the reported
cases of PD in pregnancy were treated with levodopa,1–5 some
others with bromocriptine2,3 and pergolide.6 In these descriptions antiparkinsonian treatment with L-dopa or ergot dopamine-agonists has not been related to any teratogenic effect. To
our knowledge, there is no report available on treatment of
parkinsonian women with the non-ergot dopamine agonist
pramipexole during their pregnancy. We describe the case of a
woman with PD treated with high dose of pramipexole during
pregnancy.

Case Report
A 42-year-old woman developed mild rest tremor and motor
slowness in the right limbs at the age of 37. At the first

*Correspondence to: Dr. Marco Mucchiut, Clinica Neurologica,
Policlinico Universitario, pzza Rodolone 2, 33013 Gemona del Friuli
(UD), Italy. E-mail: marco.mucchiut@med.uniud.it
Received 12 July 2003; Revised 11 February 2004; Accepted 24
February 2004
Published online 22 April 2004 in Wiley InterScience (www.
interscience.wiley.com). DOI: 10.1002/mds.20148

Movement Disorders, Vol. 19, No. 9, 2004

neurological examination, she presented bradykinesia and rigidity of the right limbs and mild (infrequently present) rest
tremor of the ipsilateral hand; the remaining neurological examination was normal. Hoehn and Yahr stage was I; the total
Unified Parkinson’s Disease Rating Scale (UPDRS) score was
14 (11 in section III); the Schwab and England scale was 80%.
Family history was not significant for extrapyramidal diseases.
Routine blood examination, ceruloplasmin, and CU⫹⫹ levels
were normal; cerebral magnetic resonance imaging scan was
normal; 18-fluorodopa positron emission tomography showed
mild decreased dopamine uptake in the left striatum. Treatment
with pramipexole (4.5 mg per day) resulted in optimal control
of motor symptoms.
At the age of 41, the patient became pregnant and refused to
discontinue pramipexole because of concern of motor disability. Approximately 3 months before starting pregnancy, the
total UPDRS score was 16 (12 in section III). During pregnancy, motor disability (mostly bradykinesia and rigidity) did
progressively worsen, and by the sixth month, total UPDRS
score was 36 (25 in section III). The patient underwent amniotic fluid examination with normal results (46 XX karyotype);
the morphological fetal echography at the 22nd week of pregnancy showed no abnormalities.
She gave birth to a normal-term girl with an Apgar score of
9, by caesarean section because of motor impairment. General
and neurological examination of the baby revealed no abnormalities and the routine neonatal blood tests were normal. At 6
months, the baby was healthy and showed normal development.
During the first month of the puerperal period, bradykinesia
and rigidity improved, but 2 months later, these symptoms
worsened again, reaching an UPDRS total score of 50 (34 in
section III). In consideration of impairment with nursing tasks,
we introduced small doses of L-dopa (150 mg/day) with complete motor control after 4 weeks. In agreement with the patient, we decided to avoid breastfeeding because medical information on this topic is lacking.

Discussion
The effect of pregnancy on PD course is controversial: some
authors report unchanged symptoms,2,3,5 but in the case described by Shulman and colleagues,1 a marked increase of
motor disability was noted, and Hagell and coworkers2 in their
review reported a significant worsening of motor disability
during and after pregnancy in a PD patient.
In our case, worsening occurred in the second part of pregnancy and in the postpartum period and disease progression
cannot be excluded. The mechanism underlying the increase of
PD disability during pregnancy is poorly understood: some
authors suggest both positive7 and negative8,9 dopaminergic
effects of estrogens, others that changes of PD disability during
pregnancy could be related to disease progression2 or to pregnancy-induced pharmacokinetic variations of drug levels.10 –12
As recently suggested by Shulman and colleagues1 and De
Mari and coworkers,6 it could be related to the low levels of
estrogens.
Medical experience on the management of pregnancy in PD
patients is limited. Whereas for L-dopa we find descriptions in
the medical literature in favor of its substantial safety during
human pregnancy in parkinsonian patients, 1–5,10 only anecdotal
data are available for dopamine agonists,2,3,6 and to date, no
published data exist on ropinirole or cabergoline. To our