Clinical Neurophysiology 129 (2018) 89–94

Contents lists available at ScienceDirect

Clinical Neurophysiology
journal homepage: www.elsevier.com/locate/clinph

Review

On Denny-Brown’s ‘spastic dystonia’ – What is it and what causes it?

Jakob Lorentzen a,b,⇑, Maud Pradines c, Jean-Michel Gracies c, Jens Bo Nielsen a,b
a
Section for Integrative Neuroscience, Center for Neuroscience, University of Copenhagen, Denmark
b
Elsass Institute, Holmegårdsvej 28, 2920 Charlottenlund, Denmark
c
EA 7377 BIOTN, Université Paris-Est, Hospital Albert Chenevier-Henri Mondor, Service de Rééducation Neurolocomotrice, APHP, Créteil, France

a r t i c l e i n f o h i g h l i g h t s

Article history:
 Stretch and effort-unrelated sustained involuntary muscle activity following central motor lesions
Accepted 2 October 2017
Available online 4 November 2017
may be caused by:
 Plastic changes at a spinal level involving upregulation and sprouting of surviving descending fibres

Keywords:
and/or changes in intrinsic properties of motoneurones.
Spasticity  Re-organization in the motor cortex.
Spastic dystonia  Lesions in basal ganglia.
Hypertonia

a b s t r a c t

In this review, we will work around two simple definitions of two different entities, which most often co-
exist in patients with lesions to central motor pathways: Spasticity is ‘‘Enhanced excitability of velocity-
dependent responses to phasic stretch at rest”, which will not be the subject of this review, while Spastic
dystonia is tonic, chronic, involuntary muscle contraction in the absence of any stretch or any voluntary
command (Gracies, 2005). Spastic dystonia is a much less well understood entity that will be the subject
this review.
Denny-Brown (1966) observed involuntary sustained muscle activity in monkeys with lesions
restricted to the motor cortices . He further observed that such involuntary muscle activity persisted fol-
lowing abolition of sensory input to the spinal cord and concluded that a central mechanism rather than
exaggerated stretch reflex activity had to be involved. He coined the term spastic dystonia to describe this
involuntary tonic activity in the context of otherwise exaggerated stretch reflexes. Sustained involuntary
muscle activity in the absence of any stretch or any voluntary command contributes to burdensome and
disabling body deformities in patients with spastic paresis. Yet, little has been done since Denny-Brown’s
studies to determine the pathophysiology of this non- stretch or effort related sustained involuntary
muscle activity following motor lesions and there is a clear need for research studies in order to improve
current therapy.
The purpose of the present review is to discuss some of the possible mechanisms that may be involved
in the hope that this may guide future research. We discuss the existence of persistent inward currents in
spinal motoneurones and present the evidence that the channels involved may be upregulated following
central motor lesions. We also discuss a possible contribution from alterations in synaptic inputs from
surviving or abnormally branched sensory and descending fibres leading to over-activity and lack of
motor coordination. We finally discuss evidence of alterations in motor cortical representational maps
and basal ganglia lesions.
Ó 2017 International Federation of Clinical Neurophysiology. Published by Elsevier Ireland Ltd. All rights
reserved.

⇑ Corresponding author at: Neural Control of Movement Research Group, Department of Neuroscience and Pharmacology, University of Copenhagen, Denmark.
E-mail address: Jlo@elsassfonden.dk (J. Lorentzen).

https://doi.org/10.1016/j.clinph.2017.10.023
1388-2457/Ó 2017 International Federation of Clinical Neurophysiology. Published by Elsevier Ireland Ltd. All rights reserved.
90 J. Lorentzen et al. / Clinical Neurophysiology 129 (2018) 89–94

Contents

1. Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 90
2. When does spastic dystonia occur and how frequent is it? . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 91
3. How should spastic dystonia be evaluated?. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 91
4. What causes spastic dystonia? . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 92
4.1. Plastic changes at a spinal level involving upregulation and sprouting of surviving descending fibres . . . . . . . . . . . . . . . . . . . . . . . . . . . . 92
4.2. Changes in intrinsic properties of spinal motoneurons and interneurons . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 92
4.3. Re-organization in the motor cortex . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 92
4.4. Role of basal ganglia lesions . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 93
5. Where to go from here . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 93
Conflict of interest. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 93
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 93

1. Introduction
There has been considerable debate in the scientific literature in
recent years regarding the proper definition of spasticity (Biering-
Sorensen et al., 2006; Burridge et al., 2005; Gracies, 2005a, 2005b;
Lorentzen et al., 2010; Malhotra et al., 2008; Pandyan et al., 2005a,
2005b; Sheean, 2002). Much of this debate stems from docu-
mented differences in the understanding of which clinical signs
define spasticity as well as a presumed variability in the clinical
use of the term (Pandyan et al., 2005a, 2005b). Without a clear con-
sensus of what we understand by ‘spasticity’ there is a risk that
research in the field remains confused and potentially leads to
misunderstandings and wrong treatment decisions in the clinic.
Spasticity has commonly been linked to an increase in the
velocity-dependent stretch responses of a muscle, manifested as
a ‘catch’ that can be felt when stretching the muscle quickly
enough (Gracies, 2005a; Lance, 1980; Sheean, 2002; Sheean and
McGuire, 2009). The implicit assumption is that the ‘catch’ is
caused by hyperactive stretch reflexes and several definitions,
including that of Lance (1980), consequently include hyperactive
stretch reflex activity as a central component (Lance, 1980) or
the only component of the definition (Gracies, 2005b). Yet, research
in the past 20–30 years has documented that hyperactive stretch
reflexes generally do not cause functional problems and do not
show any clear relation to the main clinical problems experienced
by the patients and clinicians (Dietz and Sinkjaer, 2007, 2012;
Lorentzen et al., 2010; Salazar-Torres Jde et al., 2004; Willerslev-
Olsen et al., 2013). In the clinic, the term spasticity has in fact often
been used much more broadly, also including spasms, involuntary
movements, unwanted muscle activity and alterations of elastic
muscle properties leading to reduced movement range and eventu-
ally contractures (Pandyan et al., 2005b). It has especially been a
confounding factor that the alterations of elastic muscle properties
are difficult to distinguish clinically from the muscle resistance
caused by hyperactive stretch reflexes and many of the functional
problems observed in patients with central motor lesions have
therefore been falsely attributed to hyperactive stretch reflex activ-
ity (Dietz and Sinkjaer, 2007, 2012). Research through three dec-
ades have instead documented that it is the reduced movement
range and lack of extensibility of the muscle and connective tissue,
which are dominant causes of reduced functional capacity in
‘‘spastic” patients, particularly those with cerebral palsy (Berger
et al., 1982; Geertsen et al., 2015; Willerslev-Olsen et al., 2014),
but also with stroke (Pradines et al., 2015). Spasms are a frequent
cause of painful muscle contractions, which may interfere with
sleep (Biering-Sorensen and Biering-Sorensen, 2001). They are
caused by activation of hyperactive spinal networks, which are dis-
tinct from the stretch reflex circuitry (Sheean and McGuire, 2009).
Recent research has implicated altered persistent inward currents
in both motoneurones and interneurons as important pathophysi-
ological factors in the development of spasms (Bellardita et al.,
2017; Gorassini et al., 2004; Kong et al., 2011; Murray et al.,
2010, 2011).
Little is known of the involuntary movements and unwanted
muscle activity, which are frequently observed in patients with
central motor lesions and which constitute the core of what is also
often called spastic movement disorder. There may be some kind of
‘overactivity’ causing unwanted muscle activity when the patient
attempts to remain at rest. This clinical symptom is what has been
named spastic dystonia (Denny-Brown, 1966; Gracies, 2005b). In
addition to this involuntary resting activity, one can often observe
in the same patients involuntary activation of antagonist muscles
to the muscles targeted by the command, and of muscles other
than those that the patient attempts to activate. These phenomena
have been termed spastic co-contraction and extrasegmental co-
contraction respectively (Gracies, 2005b). As Spastic Dystonia is
caused by an actual brain lesion (vascular, traumatic, etc.), being
usually accompanied by a number of other symptoms (spasticity,
spastic cocontraction, etc.), Spastic Dystonia is one type of what
has been labelled secondary dystonias (Gracies and Simpson,
2004).
The term ‘spastic dystonia’ may be seen as a misnomer by
some, since it mixes terms that have traditionally been linked
to either lesions of descending motor pathways or the basal gan-
glia. Despite these problems we have decided to maintain the
term in this review, since it is already used widely in the litera-
ture and since an alternative term such as ‘dystonic spasticity’
would be equally misleading and would imply a false pathophys-
iological relationship with spasticity. We do not believe that it
would add to clarity in the field if we were to introduce yet
another term for which there is no general consensus and which
in our opinion would not help in achieving what we aim for with
this review, which is to point out that the sustained involuntary
muscle activity observed in patients with central motor lesions
should not be mistaken for spasticity. For that purpose spastic
dystonia is a better term than dystonic spasticity. A descriptive
term such as ‘stretch- and effort-unrelated sustained involuntary
muscle activity following central motor lesions’ may avoid some
of the confusion, but it is lengthy and awkward. The reader
should therefore see our use of the term ‘spastic dystonia’ in this
review as a convenient abbreviation for ‘stretch- and effort-
unrelated sustained involuntary muscle activity following central
motor lesions’.
In humans, spastic dystonia is seen most conspicuously in the
upper limb where it contributes to the so-called hemiparetic pos-
ture, particularly in subjects with stroke or cerebral palsy (Gracies,
2005b; Sheean, 2002, Sheean and McGuire, 2009). An aggravated
expression of spastic dystonia may also be seen during standing
and gait where the subject may adopt a posture with plantarflex-
ion and/or inversion at the ankle, toe flexion, pronounced exten-
sion at the knee and associated flexion at the elbow (Gracies,
2005b; Sheean, 2002; Sheean and McGuire, 2009).
 J. Lorentzen et al. / Clinical Neurophysiology 129 (2018) 89–94
We have little knowledge of the exact cause of spastic dystonia
except for the observation by Denny-Brown (1966) that monkeys
would still show cortical lesion-induced involuntary static muscle
activity following dorsal root section, suggesting that stretch reflex
activity plays little or no role in the pathophysiology of this phe-
nomenon. This finding appears to have been generally overlooked
in the clinic, since sustained involuntary muscle activity following
motor lesion is still treated in many centres by classical ‘‘antispas-
tic” therapy designed to – and approved based on their capacity to
– diminish hyperactive stretch reflex activity. Selective dorsal rhi-
zotomy, which is now used in children with cerebral palsy in a
number of centers is another example where it is essential to
determine whether the functional disability of the child is caused
by hyperactive stretch reflexes or rather a centrally mediated
involuntary muscle activity. The lack of research on the possible
pathophysiological mechanisms involved in spastic dystonia sug-
gests that much of the research community has also failed to real-
ize that a distinct pathophysiology, unrelated to stretch reflex
activity, is involved. Given that spastic dystonia may frequently
be responsible for what is wrongly assumed to be spasticity and
may also be involved in the functional challenges often ascribed
to spasticity, we find it of paramount importance to initiate
research aimed at disclosing the pathophysiological mechanisms
involved. It is the purpose of this review to highlight and discuss
some of the possible pathophysiological mechanisms, which may
be involved, in order to facilitate future research on this topic.
2. When does spastic dystonia occur and how frequent is it?
Spastic dystonia has so far not been explicitly mentioned or
described in studies on the incidence or prevalence of spasticity
in populations with lesions of central motor pathways such as
stroke (Malhotra et al., 2009; Sommerfeld et al., 2012), spinal cord
injury (Sheean, 2002), multiple sclerosis (Sinkjaer et al., 1993) or
cerebral palsy (Gracies, 2005a). As pointed out by (Malhotra
et al., 2009) spasticity is often poorly defined and poorly described
and it is therefore not possible to provide any valid estimate of the
frequency of occurrence of spastic dystonia based on the literature.
It is the personal experience of the present authors that spastic
central motor lesion
Fig. 1. Proposed contributing components to hypertonia.
91
dystonia is often seen in the upper limb of people with stroke,
whereas it appears perhaps less prominent in either upper or lower
limbs of people with traumatic spinal cord injury. However, we
have no scientific evidence to support these claims and our obser-
vations may have been biased by location and time since lesion.
Therefore, controlled studies with appropriate definition and eval-
uation of spastic dystonia are necessary in order to clarify this
issue.
3. How should spastic dystonia be evaluated?
Muscle tone was defined by Lance and McLeod as ‘‘the sensation
of resistance felt as one manipulates a joint through a range of motion,
with the subject attempting to relax” (Lance and McLeod, 1981). This
encompasses several contributing components to the tone. The
three main components to increased muscle tone (hypertonia) in
neurological patients are altered mechanical properties of the mus-
cle tendon complex, reflex mediated stiffness, and stretch and
effort-unrelated sustained involuntary muscle activity following
motor lesion (Fig. 1).
Altered mechanical properties (‘passive’ stiffness) may be dis-
tinguished from other (neural) causes of increased muscle resis-
tance by the absence of any muscle activity during testing
(Lorentzen et al., 2010; Malhotra et al., 2009). This absence of mus-
cle activity can only be detected by appropriate recording of the
electrical activity in the muscle, which explains the failure of clin-
ical assessment tools in distinguishing the different causes of mus-
cle resistance (Biering-Sorensen et al., 2006; Malhotra et al., 2009).
Thus characterization of EMG also appears to be a useful tool for
clinicians to decide treatment, and a recent study with the aim of
characterizing activation patterns of spastic and dystonic muscles
evaluated by EMG at rest, during volitional movement and pas-
sively induced movements found that use of EMG is useful in sep-
arating these parameters (Beattie et al., 2016).
Treatment of clinical manifestations should be directed by the
underlying mechanisms and differentiation between spasticity
and the sustained, stretch- and effort- unrelated involuntary mus-
cle activity, present at rest in the absence of phasic stretch follow-
ing central motor lesions and spasticity is therefore important in
92 J. Lorentzen et al. / Clinical Neurophysiology 129 (2018) 89–94

Fig. 2. Proposed mechanisms involved in the pathophysiology of sustained involuntary muscle activation following central motor lesions, in the absence of phasic stretch or
voluntary effort.

the clinic. For clinical manifestations such as postural deformities Wienecke et al., 2014). This period corresponds quite well to the
caused by sustained involuntary muscle activations, therapies that period of so called spinal shock following the lesion and appears
focus on reducing the hyperexcitability of the spinal reflex mecha- to correlate also well to reduced monoaminergic innervation and
nisms that constitute spasticity may not be effective. reduced expression of 5-HT1 receptors in the membranes of the
motoneurones (Hultborn et al., 2013). When reflex hyperexcitabil-
ity develops following the spinal shock it appears to be linked to
4. What causes spastic dystonia?
upregulation of 5-HT receptors in both motoneurones and
interneurons as well as production of monoaminergic substances
4.1. Plastic changes at a spinal level involving upregulation and
from a number of cell populations distal to the lesion (Bennett
sprouting of surviving descending fibres
et al., 2004; D’Amico et al., 2013, 2014; Heckman et al., 2005;
Hultborn et al., 2013; Kong et al., 2010; Murray et al., 2010; Rank
Degeneration of descending fibres leaving vacant synaptic sites
et al., 2007; Ren et al., 2013; Wienecke et al., 2014). These changes
that may subsequently be overtaken by other descending fibres
in monoaminergic innervation and their receptors in both
and/or sensory afferents is well documented following spinal cord
motoneurones and interneurons may play a central role in the
lesions both in animal models and in human patients (Bareyre
pathophysiology of spasms. Especially upregulation of persistent
et al., 2004; Calancie et al., 1996, 2002). There is less documenta-
inward currents (PICs) which involve both Ca and Na channels
tion following subcortical lesions of the corticospinal tract or the
may lead to prolonged episodes of continuous motoneuronal dis-
motor cortex in relation to stroke or other brain lesions and it does
charges following a relatively brief synaptic input (D’Amico et al.,
not appear clarified whether any significant sprouting and hyper-
2013, 2014; Heckman et al., 2005; Murray et al., 2010). There is
sensitivity develops in such patients (Fig. 2).
also evidence from human subjects that PICs may be essential in
Increased involvement of descending pathways (rubro-,
the pathophysiology of spasms in spinal cord injured subjects
vestibulo-, tecto-, and ipsilateral reticulo-spinal pathways) under-
(D’Amico et al., 2013). It would not be unlikely if upregulation of
going abnormal branching onto motor neurons, which have lost a
PICs also played a role in the pathophysiology of stretch- and
considerable part of their normal descending innervation after
effort-unrelated sustained involuntary muscle activity following
lesions in the brain or spinal cord may be one mechanism con-
motor lesion. The observation of increased activation of PICs in
tributing to spastic dystonia (Ellis et al., 2012; Krainak et al.,
the biceps muscle of chronic stroke survivors may indeed be
2011; Miller et al., 2014; Raineteau et al., 2002; Sukal-Moulton
related to spastic dystonia rather than hyperexcitable stretch
et al., 2014a, 2014b). One mechanism contributing to stretch and
reflexes. However, it should be noted that other studies have failed
effort-unrelated sustained involuntary muscle activity following
to find evidence that would support upregulation of PICs in stroke
central motor lesions may be increased activity in the properties
(Mottram et al., 2009) or spinal cord injured subjects (Nickolls
of these pathways, since most of them are excitatory and show
et al., 2004).
more spontaneous neuronal activity than corticospinal pathways
(Du Beau et al., 2012; Drew et al., 1986, 1996). However, no conclu-
sive evidence exists. 4.3. Re-organization in the motor cortex

4.2. Changes in intrinsic properties of spinal motoneurons and
interneurons
Following spinal cord lesions, spinal motoneurones undergo a
period in which they are less responsive to synaptic inputs than
normal (D’Amico et al., 2013, 2014; Hultborn et al., 2013;
Although it is natural to focus on the spinal cord and its cir-
cuitry when considering possible pathophysiological mechanisms
involved in stretch- and effort-unrelated sustained involuntary
muscle activity following central motor lesions, given its history
of association with spasticity and hyperreflexia, it should not be
overlooked that lesions anywhere in the nervous system are bound
 J. Lorentzen et al. / Clinical Neurophysiology 129 (2018) 89–94
to cause adaptive changes in connected circuitries in other parts of
the nervous system. Adaptive changes in the representation of
muscles in the motor cortex are well documented following stroke,
spinal cord injury and cerebral palsy (Basu et al., 2010; Frost et al.,
2003; Liu and Rouiller, 1999). Could these changes play a role in
the pathophysiology of stretch- and effort- unrelated sustained
involuntary muscle activity following central motor lesions? We
believe that there is a reasonably good likelihood for this hypoth-
esis. Firstly, the motor cortex exerts its control of muscles not only
through the corticospinal tract, but also more indirectly through its
connections to brain stem nuclei and their descending projections
to the spinal cord (i.e. vestibulo-, reticulo- and rubrospinal path-
ways). Following lesions that involve the corticospinal tract it
would therefore not be unlikely if the motor cortex would undergo
re-organisation with the aim of optimizing the surviving output to
the spinal cord and that this would involve a heightened excitabil-
ity and activity of indirect connections to the spinal cord through
brain stem nuclei. Increased input to the spinal motoneurones
from these descending pathways may in other words not necessar-
ily be caused by sprouting and increased synaptic efficiency of
their terminals, but maybe more simply because they are them-
selves more active due to increased drive from the motor cortex.
There is indeed evidence that the functional re-organization of cor-
tical activity in relation to movement in patients with brain lesions
involves activation of far more cortical neurones and cortical areas
than what is seen normally (Basu et al., 2010). It thus appears as if
the patients needed to activate more cortical neurones in order to
compensate for the loss of specific descending drive through the
lesioned corticospinal fibres and this might lead to less precise
activation of the spinal cord circuitries and inadvertent activation
of brain stem nuclei already at ‘rest’. It may be argued that there
is no apparent cortical hyperexcitability in patients with stretch-
and effort-unrelated sustained involuntary muscle activity follow-
ing central motor lesions and that motor evoked potentials are in
contrast greatly diminished (Cortes et al., 2012; Dimyan and
Cohen, 2010). This is true but it should be kept in mind that motor
evoked potentials only provide information about the (lesioned)
corticospinal tract and therefore do not explore excitability of
more indirect pathways through brain stem nuclei.
4.4. Role of basal ganglia lesions
Stretch- and effort-unrelated sustained involuntary muscle
activity following central motor lesions may turn out to be not less
common in spinal cord than in brain injuries, which thus might be
a clue to its pathophysiology. Classic textbooks often present
stroke and cerebral palsy as lesions which produce distinct symp-
toms that may be associated to lesions of the corticospinal tract,
such as paresis and spasticity, others that may be associated to
the cerebellum such as ataxia and others again which may be asso-
ciated to the basal ganglia such as rigidity, resting tremor and ‘dys-
tonia’. However, the clinical reality is that the lesion seldom obeys
the rules laid out in the textbooks. The basal ganglia are located so
close to the internal capsule and to the corticospinal tract that it
would require great agility of the lesion to carefully affect one
without the other. Stretch- and effort-unrelated sustained involun-
tary muscle activity following central motor lesions may thus be
the end-result of reduced voluntary control of muscles due to (par-
tial) corticospinal lesion and increased involuntary activation due to
some forms of basal ganglia lesion. It should in this relation be kept
in mind that the basal ganglia do not only project to the motor cor-
tex through the thalamus, but also to the motor nuclei in the brain
stem as well as the spinal cord (Jordan et al., 1992).
So could it be that stretch- and effort-unrelated sustained invol-
untary muscle activity is simply caused by a lesion, which involves
both the cortical spinal tract and the basal ganglia? An answer to
93
this question could be provided by a careful comparison of the
prevalence of stretch- and effort- unrelated sustained involuntary
muscle activity in patients with pure cortical vs subcortical stroke.
However, this would require that stretch- and effort- unrelated
sustained involuntary muscle activity be diagnosed and measured
appropriately and that especially increased passive muscle stiff-
ness and spasticity be ruled out. So far such a study has not been
performed to our knowledge.
5. Where to go from here
We have pointed to a number of possible pathophysiological
mechanisms that may be involved in the development of spastic
dystonia (Fig. 2). There may be others that we have not thought
about, but we hope that the suggestions put forward here may
serve as an inspiration for researchers in the field. There is a great
need for a general consensus on whether we should continue to
use the term ‘ spastic dystonia’ or whether we can find a better
term to use when we talk about stretch- and effort- unrelated sus-
tained involuntary muscle activity following central motor lesions
in the absence of phasic stretch or voluntary effort. We also need to
decide how it should be evaluated and distinguished from other
causes of increased muscle resistance. This will provide us with a
tool to determine the incidence and prevalence of spastic dystonia
in different limbs following different types of lesions to the central
motor pathways. With that knowledge in hand, we should obtain
valuable clues to the pathophysiology of spastic dystonia, but
experimental studies designed to explore directly the mechanisms
involved will be necessary. The new knowledge and understanding
that such studies will generate, should lead to development of new
and hopefully also more appropriate and efficient treatment of not
only spastic dystonia, but all the different causes of increased mus-
cle resistance. The general recognition of sustained involuntary
muscle activity in the absence of phasic stretch or voluntary effort
as a separate and distinct symptom of central motor lesions is the
first step in this direction.
Conflict of interest
The authors report no conflicts of interest.
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