Parkinsonism and Related Disorders 11 (2005) 195–198
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Case resport

Dystonia after a bone fracture of the arm in a patient with a history of
striato-pallidal ischemic stroke: a case report
Pantelis Stathis*, Caterina Hampipi
Department of Neurology, 1st Hospital of Social Security Services, Terma-Zaimi, 151-27, Melissia, Athens, Greece
Received 23 March 2004; revised 16 August 2004; accepted 1 September 2004

Abstract
We report on a female with segmental dystonia of the upper limb after an anatomically related ischemic stroke. Dystonia developed almost
3 years after the onset of the stroke and immediately following the removal of the cast because of a bone fracture in the same limb. We
discuss the case considering issues such as: delay-onset, lesion topography and pathophysiology, peripheral input and their possible
contribution to the development of secondary dystonia.
q 2004 Elsevier Ltd. All rights reserved.
Keywords: Dystonia; Basal ganglia; Stroke; Peripheral injury

1. Introduction
Dystonia is a syndrome characterized by sustained and
forceful muscle contractions, frequently causing twisting
and repetitive movements, or abnormal postures. According
to the etiological classification, dystonia can be divided into
two major categories: idiopathic or primary and symptomatic or secondary [1]. Delayed-onset focal or segmental
dystonia secondary to focal cerebral vascular injury, such as
infarction of the basal ganglia is a rare but well-described
entity [2–6]. Moreover selectivity of the lesions within the
basal ganglia that result in dystonia is a matter of great
interest because it may give clues to the functional
organization of the lesions. Finally, there is a growing
body of evidence that defective sensory inputs could play a
crucial role in the development of dystonic dyskinesia [7].
Our case describes a patient with a history of right striatopallidal ischemic stroke, who developed segmental dystonia
of the left upper limb 2 months after a peripheral injury
(bone fracture) of the left elbow and immediately following
the removal of the plaster cast. Our purpose is to consider
among others issues the following crucial question; was the
* Corresponding author. Tel.: C30 210 808 9363/613 8460x1107;
fax: C30 210 613 8463/598 2194.
E-mail address: statneur@hol.gr (P. Stathis).
1353-8020/$ - see front matter q 2004 Elsevier Ltd. All rights reserved.
doi:10.1016/j.parkreldis.2004.09.007

onset of the movement disorder related to the peripheral
trauma or could it have occurred by chance?
1.1. Case history
A 58-year-old right handed female was referred to a
movement disorder outpatient clinic complaining of ‘pain
and discomfort’ as well as marked involuntary movements
restricted to the left hand. Three years before she had a
stroke of the right basal ganglia territory (Brain CT scan:
hypodense area at the anatomical position of the right
putamen). resulting in a left hemiparesis involving mainly
the upper limb, together with mild paralysis of the left
lower face (asymmetry of the nasolabial fold). At the time
the patient had no sensory complaints or defects. An NIH
stroke scale performed immediately after the insult
showed only minor motor impairment [score 1 for ‘facial
facial palsy’ (item 4) and score 1 for ‘motor arm and leg’
(item 5 and 6)] and the neuropsychological evaluation
revealed some degree of apathy and loss of drive. Apart
from mild hypertension for the previous 5 years, medical
history was unremarkable and she had no family or
personal history of psychiatric or movement disorders.
Following the stroke she was seen every 6 months in the
outpatient clinic, and by the end of the first year
the patient had no residual weakness of the left hand.

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About two and a half years after the stroke she fractured
left elbow and she was in a plaster cast for the next 2
months in order to immobilize the joint. Immediately after
the removal of the cast at the end of 2 months, the patient
experienced marked discomfort of the hand, which was
not helped by physiotherapy. The discomfort ‘forced’ the
patient to make continual ‘irregular, mainly twisting,

movements’ of the hand mostly characterized by finger
extension and abduction, or flexion of fingers III and IV in
the metacarpophalangeal joints with extension of the other
finger joints, pronation of the forearm, and to a lesser
degree by internal rotation, as well as adduction of the
arm. She also stated that when walking she had less
discomfort when the limb ‘goes’ at behind her. Muscle
strength in the hand was almost normal and the clinical
examination did not reveal additional signs. The continuous writhing character of the movements added an
athetotic component to them. There were no signs of
autonomic dysfunction of the limb as seen in cases of
complex regional pain syndrome (CRPS). There was no
history of toxic exposure or treatment with neuroleptic
drugs. The patient underwent a brain MRI (Fig. 1), which
showed the old lesion of the right putamen and the globus
pallidus with additional involvement to a minor degree of
the right internal capsule and gliosis at the border zone
around the lesion. The pattern of the dystonic movements
did not change over a 2-year follow-up period.

2. Discussion

Fig. 1. Brain MRI of the patient. The lesion is visible as hypodense and
hyperdense area on T1 and T2 weighted images, respectively, involving the
posterior part of the right putamen and the globus pallidus.

Delayed-onset dystonia is a rare sequel of stroke.
Delayed-onset of a movement disorder after ischemichypoxic injury may reflect the time required for remyelination, inflammatory changes, ephaptic transmission,
oxidation reactions, maturation or aberrant synaptic reorganization, trans-synaptic neuronal degeneration, or denervation supersensitivity [11].
The anatomical basis and pathogenesis of secondary
dystonia are both uncertain. The involvement of the
internal capsule together with the adjacent pallido-striatal
complex in stroke patients with dystonia confined to the
upper limb has been also demonstrated by others [5,6,13].
Moreover, the literature concerning the question of which
structure among putamen and globus pallidus, internal
(GPi) or external (GPe), is mainly responsible for the
development of secondary dystonia, is conflicting and
confusing. It has been suggested that putaminal lesions
might affect both direct and indirect pathways [14]. This
disruption may predominantly affect the indirect pathway
resulting in dystonia by increased thalamocortical drive
due to disinhibition of the thalamo-cortical projections
[6,15]. On the other hand, there is indirect evidence in
primates and humans showing that, at least in primary
dystonia, both the direct and indirect pathways are
overactive, and certain models of primary dystonia share
this notion [15]. If this is a valid point, then secondary
dystonias associated with putaminal lesions are difficult to
explain based on striatal overactivity. The same difficulty
exists in the case of involvement of the external globus
pallidus (GPe) since lesions of GPe tend to produce
parkinsonism by disinhibition of the subthalamic nucleus
(STN) [17], and certain movement disorders like chorea

P. Stathis, C. Hampipi / Parkinsonism and Related Disorders 11 (2005) 195–198

result from increased neuronal activity of GPe neurons
[19]. Finally in the case of GPi involvement there is also
the paradox that although GPi pallidotomy alleviates
symptoms in patients with dystonia, by altering the
neuronal activity [16], involvement of GPi in secondary
dystonia has also been reported by many investigators [4–
6,10,13,18]. All these findings suggest the possibility that
either a different central pathophysiological mechanism
may be responsible for the development of primary vs
secondary dystonia, or that in the case of primary dystonia,
the palliative result of pallidotomy could be due to the
precise lesion location within the GPi (posteroventral) [19].
Another issue in our case has to do with whether the
onset of the movement disorder (dystonia) was related to the
peripheral trauma or whether it could have occurred by
chance? Both peripheral injury and immobilization are
responsible for the peripheral input. Although the opposite
idea has also been stressed [9], a recent review provides
evidence that different types of peripheral injury, including
direct trauma and chronic immobilization with plaster cast
or splint, may lead to certain movement disorders like focal
or segmental dystonia [8].
In contrast to Jankovic assertion [8], namely that
peripherally induced post-traumatic dystonia is often
characterized by the presence of CRPS, this was not the
case in our patient, as has also been reported by others [20].
In addition, our patient’s dystonia was not characterized by
inconsistency, spontaneous remissions, disappearing with
distraction, incongruity of movements, etc. that could
suggest a psychogenic origin [24–26].
It has been suggested that one of the functions of the
basal ganglia is to regulate sensorimotor interactions in a
way that determines which sensory stimuli are used to
initiate motor action and which are disregarded [12,14].
Moreover, the participation of the somatosensory system in
the mechanism of dystonia has been suggested [7]. One
important indication in favour of this notion is the alteration
of dystonic muscular patterns after successful botulinum
toxin treatment [21]. There is a growing body of evidence
that a preexisting dysfunction of the central nervous system
plays an important role in the pathogenesis of this kind of
movement disorder, and predisposes some patients to
develop it. Animal studies also provide evidence that
peripheral trauma can ‘unmask’ subclinical disturbance of
basal ganglia function [8], and that localized lesions in the
GPe produced certain hyperkinetic syndromes triggered by
sensory stimulation [22]. If this is the case, then we can
argue that our patient could possibly be an example in which
an underlying central nervous system disorder was silent
until a peripheral cause triggered the development of the
dystonic movements. This may also explain why the
dystonia was restricted to the upper limb since we can
assume that the bone fracture or immobilization could
induce a peripheral sensory modification that ‘marked’ the
left upper limb as the body part that will develop dystonia.
Instead, if we reject the contribution of peripheral sensory

197

input, we can possibly accept that the dystonia was confined
to the left hand and arm because of the topography of the
lesion within the striato-pallidal complex [23].
In summary, although we realize that it is impossible to
draw conclusions in such complicated issues in a single
patient report, we suggest that our patient ‘meets both
criteria’ for a mixed delay-onset secondary to stroke and
also a peripherally induced dystonia [8,11], and that this
case could be an example supporting the notion that in the
case of secondary dystonia, the sensory abnormalities are
probably not responsible for the primary pathology and that
people who develop peripheral trauma-induced dystonia
may be somehow predisposed to the development of
dystonia [9]. It is possible that changes in neuronal activity
in basal ganglia or thalamus or even cortex together with
changes in the receptive field properties of neurons of these
areas could contribute to the development of dystonia. This
perhaps points towards the necessity for studies that can
compare baseline characteristics of neuronal activity in
putamen and also in GPe and GPi in different clinical and
pathophysiological types of dystonia, taking also into
consideration the somatosensory input as an important
component in the development of this particular movement
disorder.

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