(BQ) Part 2 book “Neurological rehabilitation - Spasticity and contractures in clinical practice and research” has contents: Clinical management of spasticity and contractures in multiple sclerosis, hereditary spastic paraparesis and other hereditary myelopathies, clinical assessment and management of spasticity and contractures in traumatic brain injury,… and other contents.
6 Clinical Management of Spasticity and Contractures in Spinal Cord Injury Martin Schubert and Volker Dietz CONTENTS 6.1 Introduction 136 6.1.1 Epidemiology and Specific Aspects of Spasticity in SCI 137 6.1.2 Spinal Shock, Recovery of Spinal Excitability, and Development of Spastic Movement Disorder 139 6.1.3 Pattern of Spastic Movement Disorder Depends on Patho-Anatomy 141 6.2 Pathophysiology-Based Treatment of Spasticity 143 6.2.1 Clinical Signs of Spasticity 144 6.2.2 Spastic Movement Disorder 144 6.2.3 Therapeutic Consequences 145 6.3 Patient Selection and Therapeutic Approach 147 6.3.1 Indication for Treatment of Spasticity in SCI 147 6.3.2 Clinical Assessment of Spasticity in SCI 148 6.3.3 Clinical Presentation and Anatomical Distribution of Spasticity 149 6.3.4 Physiological Effects of Training 150 6.3.5 The Mainstay of Spasticity Treatment in SCI Is Physical Therapy 150 6.3.6 Oral Systemic Anti-Spastic Pharmacotherapy 152 6.3.7 Intrathecal Anti-Spastic Pharmacotherapy 155 6.3.8 Focal Anti-Spastic Pharmacotherapy: Chemodenervation 157 6.3.9 Surgical Correction of Contractures 160 6.3.10 Focal Anti-Spastic Surgical Treatment: Selective Dorsal Rhizotomy 161 6.4 The Complex Spastic SCI Patient: Selection of Therapeutic Approach 162 6.4.1 Case 1: Combination Therapies: Oral Systemic and Focal 163 6.4.2 Case 2: Combination Therapies: Intrathecal Systemic and Focal 164 References 164 135 136 Neurological Rehabilitation 6.1 Introduction As in other pathologies involving lesions of the central motor system, spasticity in SCI can be defined as disordered sensorimotor control, resulting from an upper motor neuron lesion and presenting as intermittent or sustained involuntary activation of muscles by sensory input Activation is independent of the type and location of triggering sensory input It can be touch, pain, temperature, or proprioceptive stimuli, or it can be mediated by vegetative stimuli As in other types of central nervous system (CNS) lesion, spasticity per se is a pathological condition that is part of a motor syndrome related to loss of voluntary motor control and related changes in sensory-motor integration and adaptation within the motor system These changes and adaptations may include adverse as much as beneficial effects for patients’ level of function and subjective well-being For instance, it can contribute to muscle strength and thus function where voluntary strength is lost, thereby supporting stance or gait in incomplete SCI Hence, spasticity in SCI as much as in other CNS pathologies may be seen as a compensatory state of a deficit of sensory-motor control that is usually associated with a lower level of functional CNS organisation This potentially leads to more disability if negative effects prevail and balance between voluntary and involuntary activation is lost Only in this case is treatment needed In any case, treatment should be focused only on these negative effects and should be done with a specific aim Such aims can be function, pain control, reducing of care burden, or prevention of complication such as impending contractures It must always involve an interdisciplinary consideration of the patient’s special situation of impairment Thus, treatment will usually require that medical staff, patient, and his/her relatives discuss the treatment aim and agree upon a treatment concept This chapter will first deal with the manifestation of spasticity in SCI and how it can be beneficial or detrimental to function It will then describe particular features of SCI spasticity based on spinal syndromes and their pathophysiology While there is good understanding of changing excitability of spinal motoneurons below the level of lesion as derived from animal models [1–3], these are not deemed representative of the spastic motor disorder in human SCI and thus have little meaning in the context of clinical practice Although there is some experimental work in the human that supports the notion of changing excitability of infra-lesional spinal motoneurons as a basis for the generation of muscle spasms [4], models derived from this work rely on several assumptions of analogy with animal models and have no significance for practical treatment of spasticity in human SCI This is mainly due to the fact that the anatomy of the spinal lesion is more relevant for clinical presentation than modeled excitability changes at the cellular level The anatomy of a human spinal lesion results in phenotypes with implications for functional deficits that have more effect on spasticity treatment than underlying pathophysiology of presumed neural interaction Clinical Management of Spasticity and Contractures in Spinal Cord Injury 137 at the spinal segmental level Therefore, the effects of spasticity in SCI will be discussed in terms of phenotypes and their implications for function and need for treatment 6.1.1 Epidemiology and Specific Aspects of Spasticity in SCI Spasticity is seen as a major health problem by many patients with SCI [5,6] Although spasticity can be seen as a compensatory adaptation to the loss of voluntary motor control, it may also severely limit patients’ mobility when overshooting and thus can negatively affect independence in activities of daily living (ADL) and work Prevalence of spasticity in SCI is reportedly as frequent as 40–74%, depending on the type of survey and whether external or self-reported outcomes were drawn upon [1,5–9] In most surveys, spasticity is rated as the most disabling complication, followed by pain, sexual, bowel, and bladder dysfunction and pressure ulcers There is an interrelation of spasticity, pain, reduced mobility, contractures, and pressure sores [5,6,10] Many patients report pain as a consequence of spasticity In fact, spastic and neuropathic pain can be inseparable in the clinical condition Independent of geographic region, the prevalence of secondary health conditions such as spasticity is known to vary across demographic and SCI characteristics Spasticity was more often reported in SCI with incomplete lesions or tetraplegia [5,7,8,10] SCI as a unique form of CNS damage comes with certain features that are characteristic to its patho-anatomy As the lesion is a focused one, severing the infra-lesional part of the cord from the supralesional CNS, characteristics of SCI will influence the manifestation and the distribution of spasticity Neural mechanisms are discussed to be the primary contributors to spasticity following SCI by some authors [9], whereas others emphasise the relevance of mechanisms underlying muscle hypertonia that are unrelated to increased stretch reflex activity Intrinsic changes in the muscle tissue itself, e.g loss of sarcomeres, histochemical changes, and composition of muscle fibres, ultrastructure and proportion of extracellular matrix, have been suggested to have a significant impact on spastic hypertonia [11–16] From a clinical viewpoint, the original definition by Lance [17] is not sufficient to understand resulting functional impairment It is also not helpful in delineating indication for treatment as it does not explain the syndrome of spastic motor disorder Clinical signs of spasticity are not related to spastic movement disorder The functional impairment that follows a central motor lesion will be influenced and modified by spasticity However, it is not a direct consequence of the clinical syndrome that was clinically defined by Lance as ‘a velocity-dependent increase in tonic stretch reflex with exaggerated tendon jerks, clonus, and spasms, resulting from hyper-excitability of the stretch reflex’ [2,18] This is due to several aspects On the one hand, the definition by Lance does not capture the signs and symptoms of what is usually referred to as spastic motor disorder It does not include the impending secondary 138 Neurological Rehabilitation changes within muscle and connective tissue leading to contractures as an unwanted final point of missed treatment On the other hand, it overemphasises the significance of the hyperexcitability of the stretch reflex while negating the functional significance of loss of polysynaptic reflex activity [19] Spasticity in SCI evolves with time after lesion It varies with location of lesion level and other SCI characteristics such as central cord damage and completeness of the lesion Clinical aspects of spasticity are diverse, including muscle hypertonia, flexor or adductor spasms, clonus, and dyssynergic patterns of contraction Muscle hypertonia, an abnormal increase in muscle stiffness, can be regarded as a defining feature of spasticity Other than exaggerated reflexes, it has both diagnostic and therapeutic significance [16] This heterogeneity in clinical presentation cannot be explained by exaggeration of the stretch reflex alone There is abundance of clinical and experimental neurophysiological work extending on the suspected mechanisms of spasticity in SCI and the reader is referred to the respective chapter However, it should be mentioned that there is controversy about the putative role of hyper-excitability of spinal motoneurons as a major cause in the emergence of spinal spasticity This was put forward based on the observation of low-frequency invariant spontaneous self-sustained firing in motor units from out of 15 SCI patients [4] It was explained as a consequence of altered intrinsic voltage-dependent persistent inward currents (PICs; e.g., persistent inward calcium currents) [1] The hypothesis was primarily derived from animal work and then indirectly tested in human SCI [4] Under normal circumstances, PICs are assumed to have physiological roles at the MN level in amplifying synaptic inputs to provide a sustained excitatory drive that allows motoneurons to fire repetitively following a brief synaptic excitation In SCI patients in whom involuntary muscle spasms could be elicited by various types of afferent stimulation, a self-sustained firing of motoneurons was observed which would last for seconds at unusually low and regular discharge frequency Based on several assumptions derived from animal experiments it was suggested, that this slow spontaneous firing likely occurs without appreciable synaptic noise and is driven to a substantial degree by PICs intrinsic to the motoneuron [4] This would not necessarily be in contradiction with observations of reduced motor unit action potentials [20] and reduced overall activity of the motor units during functional movement [12,21–23] as well as a reduction of functional long-latency reflexes on the one, and enhanced short latency reflex excitability and spontaneous muscle spasms on the other side [19,24] However, self-sustained firing of motoneurons was only observed and described following induced muscle spasms and not during functional movement It is unclear whether it could commonly be observed in chronic spinal injury or if it is only present during induced spasms Long-term intramuscular single-motor unit recordings in the human, which could substantiate the finding, are lacking It remains to be determined if there is a relation with functional impairment or if there is a significant role of the phenomenon in the development of contractures Clinical Management of Spasticity and Contractures in Spinal Cord Injury 139 There is more human experimental data supporting the idea that spasticity involves synaptic mechanisms such as recurrent inhibition [25], reduction in Ia-reciprocal inhibition [26,27], and reciprocal inhibition of flexor reflex afferents [28] In summary, changes of motoneuron and interneuron plasticity are assumed to play a significant role in spinal spasticity, which early after an SCI are thought related to postsynaptic mechanisms such as receptor upregulation, and later during the recovery phase would be associated primarily with pre-synaptic mechanisms [1,9,29] However, these changes are not observed immediately after spinal trauma They evolve with time, suggesting gradual changes of neural adaptation following SCI 6.1.2 Spinal Shock, Recovery of Spinal Excitability, and Development of Spastic Movement Disorder When describing the natural course of disease following SCI it must be distinguished between pathologies with acute onset and those that result in slow alteration of the cord, e.g., due to tumor or other etiology with increasing compression Following an acute onset there will be a phenomenon of a sudden loss of reflexes and muscle tone, commonly referred to as ‘spinal shock’ The term was introduced by Hall in 1841, who, in describing the sudden loss and recovery of reflexes, for the first time linked it with the term ‘reflex arc’ [30] Our present idea is that a flaccid motor paresis is observed immediately after acute onset of a complete SCI when there are no motor responses to external stimuli below the level of lesion During the subsequent days and weeks, motor reactions to external stimuli and reflex activity gradually reappear in a more or less systematic manner [24] The phenomenon of spinal shock remains an issue of debate and controversy Due to involvement of the autonomous system in acute SCI, there is some overlap with cardiovascular symptoms, i.e., arterial hypotension and cardiac compensatory response The question of duration of spinal shock can be seen as a matter of definition of the delimiting type of motor reaction or reflex [31] Depending on what is chosen as the distinguishing motor criterion, cessation of spinal shock may be assumed with the appearance of a ‘delayed plantar response’ (DPR), which occurs within hours after SCI and persists for hours to a few days [32,33] If deep tendon reflexes (DTR) are chosen as the criterion, then duration of spinal shock is longer and will comprise several weeks DTR return in the majority of patients but the Babinski sign may or may not be present, which seems to be related to the presence of spasticity [34] Appearance of interlimb reflexes indicates late changes reflecting increased polysegmental spinal reflex excitability 6–12 months after SCI [35] Competitive synapse growth originating from preserved long descending motor input [36] and segmental reflex inputs [29] are postulated as underlying the individual outcome and clinical presentation of recovery of voluntary motor control and spastic motor disorder [35] 140 Neurological Rehabilitation Complete and incomplete SCI were claimed to be distinguishable by the extent and duration of spinal shock in several studies lasting only minutes to hours in ‘slight’ injuries [32,37] Furthermore, response amplitude to tendon tap and reflex spread to adjacent segments are sensitive indicators of preserved supraspinal control over lower limb musculature in subjects with acute SCI and may thus be helpful for prediction of recovery [32] Conversely, this would be well in line with the clinical observation of long-lasting flaccidity as an indicator of complete SCI Within this spectrum of motor responses and gradually increasing motor activity following spinal shock it is difficult to distinguish spasticity as a single and clearly defined motor phenomenon Spreading reflex activity and clonus is regarded a clinical sign of evolving spasticity Muscle hypertonia and polysegmental reflexes may appear as involuntary contractions and spasms, thus adding to the picture of spastic motor syndrome of SCI [35] In the clinical view, the transition from spinal shock to spasticity is a continuum of an initially gradual increase in motor excitability [24] with characteristic changes in muscle stiffness, spasms, and subsequent reduction of short- and increase in long-latency reflex excitability In contrast to tetraplegic patients, paraplegia resulted in M-wave and flexor reflex amplitudes that were found to decrease, indicating that spastic motor disorder eventually is not associated with increased excitability of motoneurons and premotoneuronal network [12,24] Neurophysiological methods have deepened our understanding of underlying excitability changes in spinal circuits and peripheral nerves during this transition [20,24,29,38,39] During spinal shock, the loss of tendon tap reflexes and flaccid muscle tone is associated with low excitability of spinal motor neurons, as tested by neurographic methods (F-waves) and with a loss of flexor reflexes, whereas only H-reflexes can be elicited because the unexcitable intrafusal gamma fibre system is bypassed by direct electrical stimulation of 1a afferents Reduced excitability of peripheral mixed nerves was shown to be based on high threshold stimulus–response relationships that were apparent from the early phase of spinal shock This coincided with depolarisation-like features reaching a peak after 12 and 17 days for the median and common peroneal nerves, respectively [20,38,40] Between Days 68 and 215 after SCI at the end of rehabilitation Boland and coworkers (2011) found that excitability for upper and lower limbs had returned towards normative values, but not for all parameters These reductions of excitability of the peripheral motor axon were described to be paralleled by the development of spasticity despite reduced excitability of the motor axon This supports the notion that spasticity occurs without overactivity of the motoneurons and their axons During the transition to spasticity, the reappearance of tendon tap reflexes and muscle tone can parallel the occurrence of spasms and is associated with the recovery of excitability of spinal motoneurons as indicated by increasing F-wave persistence and flexor reflex excitability [24] but there is no excess activity of the motor system causing spasticity Little change in spinal excitability can be shown after Clinical Management of Spasticity and Contractures in Spinal Cord Injury 141 this transition phase as the decrease in compound muscle action potentials (CMAP/M-wave) and reduced flexor reflex amplitude suggest a secondary degeneration of spinal circuits and motoneurons subsequent to severe spinal trauma [20,24,41] Furthermore, flexor reflex excitability depends on the level of lesion, indicating that spinal interneurons and pre-motoneuronal circuits may depend on the extent of infra-lesional intact spinal network [24,32] As an overall conclusion of these neurophysiological observations during transition from spinal shock to spasticity, it must be emphasised that spasticity in SCI develops without a net increase in spinal excitability 6.1.3 Pattern of Spastic Movement Disorder Depends on Patho-Anatomy Traumatic SCI usually results in a diffuse damage zone of the spinal cord extending for 2–3 segments, clinically reflected by a ‘zone of partial preservation’ In incomplete SCI, the distribution and extent of segmental damage is of great relevance for recovery Contusion injuries inherently represent the combined damage of both segmental central and peripheral neural structures [42] Preserved function of neuronal circuits below the level of the lesion is the target of rehabilitation training Spasticity develops only in this zone Next to severity and completeness of the injury, clinical spinal syndromes are relevant as they can show distinct patterns of recovery and spastic motor disturbance due to specific epidemiology and anatomical distribution of lesion in the spinal cord [43] The anterior cord syndrome (ACS), due to a flexion injury of the spine, results in predominant damage of the ventral cord, the segmental ventral horn cells, and spinothalamic and long motor tracts This is also possible when a minor mechanical impact triggers a disturbance of the blood supply from the anterior spinal artery [44] In patients with diffuse non-penetrating spinal injuries, the clinical syndrome is characterised by segmental flaccid paresis and spastic paresis with disturbance of pain and temperature sensation caudal to the lesion level but sparing of light touch and proprioception, which are mediated in the dorsal tracts of the cord Incidence is low, accounting for only 2.7% of all traumatic spinal injuries [45] and less than 1% of all spinal syndromes [43] Traumatic ACS as defined by Schneider [46] affects the anterior two-thirds of the cord and hence involves damage of the lateral corticospinal tracts This is associated with a poor prognosis and minor recovery rates of muscle force and poor coordination Traumatic central cord syndrome (CCS) is the most common acute incomplete cervical spinal cord injury, accounting for 44% of all spinal syndromes and for 9% of all SCI in a recent study of 839 spinal cord injuries [43,47] About 20% of patients with cervical spinal cord injuries present a clinical CCS [47] The syndrome is characterised by predominant upper extremity weakness and clumsy hands, and less severe lower extremity dysfunction and sensory and bladder dysfunction Spasticity will be generalised with a focus on the hands as paresis and loss of motor function is most pronounced 142 Neurological Rehabilitation here unless lesion level is within the range of the motoneurons supplying the hand muscles, as this will result in peripheral-type lesion with atrophy and flaccid paresis However, most cervical lesions occur at cervical levels C4 to C6, maximum at C5, while very few affect C7 or C8 segmental levels [43,48], thus mostly sparing motoneurons of the hand muscles, which are localised below CSS represents the oldest age group, with the lowest admission functional level of all SCI clinical syndromes, which is a cofactor in determining relatively poor recovery of hand function in this group, despite its favorable outcome compared to traumatic incomplete cervical SCI in general [43], which is in the range of the group of Brown-Sequard [49] Hand spasticity in these patients can add to their functional impairment in activities of daily life due to loss of manual dexterity However, walking ability can also be severely impaired by spasticity of the trunk and legs CCS was originally thought to result from post-traumatic centro-medullary hemorrhage and edema [50], or from a Wallerian degeneration, as a consequence of spinal cord compression in a narrowed canal [47] The central focus of spinal damage in combination with the special somatotopic organisation of the corticospinal tract, where motor tracts for the upper are localised more centrally than those for the lower extremities, were assumed to be responsible for the predominance of motor deficits in the hands in CSS However, more recent anatomical analysis and primate animal studies suggest that the syndrome is due to the specific effects of a cervical spinal lesion on direct corticomotor (pyramidal) tracts given their significant role in manual motor control [51] This would be in line with the seminal findings of these direct cortico-motoneuronal projections by Bernhard and Bohm [52] and with these authors’ appreciation and consideration of this anatomical feature, which is unique in primates and humans A loss of the capacity for ‘fractionation’ of movements and control of small groups of muscles in a highly selective manner [53] is as much characteristic of CCS as an impairment of the acquisition of new motor skills [54] Therefore, when considering the significance of direct cortico-motoneuronal control in human manual dexterity [51], CSS may be considered a prototypical condition where spinal cervical lesion inflicts damage predominantly on pyramidal tract axons affecting fine motor control and coordination of the hand Loss of fine motor control in general and, hence, particularly in the condition of CSS is associated with spastic motor disorder, which can lead to contracture and pain, predominantly in the upper extremity This mostly concerns the flexor muscles of the hands A hemisection of the cord leads to Brown-Séquard Syndrome (BSS), which was first described in 1851 by the neurologist Charles Edouard Brown-Séquard [55] as ipsilateral ataxia and spastic paresis due to proprioceptive and motor loss in association with contralateral loss of pain and temperature sensation below the level of lesion A surgical unilateral lesion dividing most of the ipsilateral tracts of the spinal cord resulted in complete flaccid paresis of the ipsilateral limbs only for a few hours, after Clinical Management of Spasticity and Contractures in Spinal Cord Injury 143 which voluntary movements began to reappear [56] Within days after such a sharp lesion, patients were able to exert slow digital movements, and walking ability was attained within weeks Slow and feeble manual function recovered within less than weeks of the operation This indicates that recovery and redundancy in corticospinal control is strong in human SCI However, this syndrome is rare in traumatic SCI and its recovery is generally less favorable than in the cases with a sharp penetrating spinal lesion, as described by Nathan, indicating that there must be more extensive and diffuse lesion of spinal tracts in lateralised traumatic SCI [57] Although BSS-like syndromes with more or less lateralisation of lesion are relatively rare in Europe and account for less than 4% of all traumatic SCI [43], they are nevertheless relevant as prognosis is known to be most favorable among incomplete traumatic SCI [43,57,58], particularly with regard to ambulation Physiologically, recovery occurs in a rather characteristic order, with proximal extensors prior to distal flexors on the more affected side and vice versa on the less affected side [58]) This is attributed to the unilateral (distal flexors) and bilateral (proximal extensors) distribution of preserved fibres and their recovery due to sprouting and formation of collaterals The recovery is most likely owed to lumbar midline crossing fibres [59,60] Spasticity usually is present, but does not pose a problem in these patients Conus medullaris syndromes amount to 1.7% and posterior cord syndrome to less than 1% in the analysis of McKinley and coworkers [43] Data on these groups are sparse In general, spinal syndromes tend to need shorter rehabilitation length of stay, indicating that sufficient functional outcome is reached after shorter duration of rehabilitation, which is likely secondary to an in-complete pattern of lesion and high proportion of preserved spinal nerve fibres [43] Spasticity usually only occurs in the plantar-flexors and digital muscles where there is an epi-conus lesion leaving intact ventral horn motoneuron cells that are disconnected from supraspinal input 6.2 Pathophysiology-Based Treatment of Spasticity Spasticity even today is frequently thought to be reflected in an ‘extraactivity’ in limb muscles mediated by exaggerated reflexes leading to muscle overactivity Also, most articles in this volume are focused on these phenomena The consequence of this thinking is that spasticity should be treated by attenuating reflex and muscle activity by antispastic drugs or botulinum toxin injections However, for over 40 years convincing evidence has been available indicating that these assumptions hold only partially for ‘clinical spasticity’ but not for spastic movement disorder, which hampers the patient (for review [61]) 144 Neurological Rehabilitation In contrast to clinical signs of spasticity, it is characterised by a reduced limb muscle activation According to the studies on spastic movement disorder, secondary to a CNS lesion, alterations of mechanical muscle fibre properties occur in association with low tonic muscle activity, which allows the development of spastic muscle activity to compensate the reduced dynamic muscle activation during functional movements after, e.g., a stroke This enables the patient, for example, to support the body during stepping The consequence of this compensatory mechanism in mobile patients is that anti-spastic drugs can accentuate paresis In the following paragraphs, we will discuss the multiple aspects of evidence in more detail 6.2.1 Clinical Signs of Spasticity The diagnosis of a spastic paresis is based on the examination of tendon tap reflexes and muscle stiffness in the passive subject Early after an acute damage of the CNS, tendon tap reflexes are exaggerated, but muscle stiffness develops only after some weeks When stretching a limb muscle of a spastic patient (Ashworth Test) during the clinical examination a tonic muscle, activation occurs in this muscle, leading to an increased resistance [62] This observation has led to the assumption that exaggerated reflexes result in an increased muscle activity and, consequently, are responsible for the movement disorder However, electrophysiological investigations on the neuronal adaptations after a complete spinal cord injury indicate a divergent course of increasing clinical signs of spasticity but decreasing or stable values of their potential neuronal correlates (M-wave, F-wave, H-reflex, and flexor reflex) [24] Consequently, non-neuronal mechanisms were assumed to contribute to spastic muscle stiffness In addition, according to all investigations of natural, complex movements in patients with spasticity, the assumption of a relevant ‘extra-activity’ contributing to spastic muscle stiffness could not be confirmed [19] 6.2.2 Spastic Movement Disorder For a patient with spasticity, the impaired performance of hand or leg/ stepping movements and their treatment are of importance, not the clinical signs found during examination During active movements such as gait a low amplitude, tonic activation of upper and lower limb muscles can be observed, i.e., a normal modulation of EMG activity is lacking while a normal timing of muscle activity is largely preserved [12,63] The reduction of limb muscle activity is suggested to be due to a diminished excitatory drive from supraspinal centers and an attenuated activity of certain polysynaptic (or long-latency) reflexes [64,65] Polysynaptic reflexes are known to modulate limb muscle activity [64] and thereby adapt the movement pattern to the environmental requirements Hereditary Spastic Paraparesis and Other Hereditary Myelopathies 287 200 Keller, J L et al Strength: a relevant link to functional performance in the neurodegenerative disease of adrenomyeloneuropathy Neurorehabil Neural Repair 26, 1080–1088 (2012) 201 van Geel, B M., Koelman Jh Fau - Barth, P G., Barth Pg Fau - Ongerboer de Visser, B W & Ongerboer de Visser, B W Peripheral nerve abnormalities in adrenomyeloneuropathy: a clinical and Neurology 46, 112–118 (1996) 202 Zhan, Z X et al Exome sequencing released a case of X-linked adrenoleukodystrophy mimicking recessive hereditary spastic paraplegia Eur J Med Genet 56, 375–378 (2013) 203 Holmberg, B H., E., H., Duchek, M., & Hagenfeldt, L Screening of patients with hereditary spastic paraparesis and Addison’s disease Acta Neurol Scand 85, 147–149 (1992) 204 Engelen, M et al X-linked adrenoleukodystrophy in women: a cross-sectional cohort study Brain 137, 693–706 doi: 610.1093/brain/awt1361 (2014) 205 Semmler, A., Kohler, W., Jung, H., Weller, M., & Linnebank, M Therapy of X-linked adrenoleukodystrophy Expert Rev Neurother 8, 1367–1379 (2008) 206 van Karnebeek, C et al Deep Brain Stimulation and Dantrolene for Secondary Dystonia in X-Linked in x-linked adrenoleukodystrophy JIMD Rep 113–116 (2015) http://taylorandfrancis.com Index Page numbers followed by f, t, and n represent figures, tables, and notes, respectively A AbobotulinumtoxinA, 220 Accelerometers, 18 Achilles tendon, 127 Action research arm test (ARAT) score, 15f, 107, 121 Activation of muscles, 69 Activities of daily life (ADL), 137, 148, 154, 180, 225 Activity and participation, 121 Addison-only presentation, 275 Adolescents, 83, 254 Adrenal hormone replacement therapy, 275 Adrenoleukodystrophy (ADL); see also Hereditary spastic paraparesis (HSP) clinical presentation, 274–275 interventions, 275 prevalence and genetics, 274 Adrenomyeloneuropathy (AMN) about, 237, 274–275 management of, 275 Adults acquired brain injury, 222 adult-onset stroke, 244 affected by cerebral palsy, 82 cerebral palsy in, 244 dorsal rhizotomy in, 195 femoral anteversion in, 84 with hereditary spastic paraparesis, 254, 255 hyperexcitable stretch reflexes in, 63 inhibitory spinal cord circuit alterations in, 244 motor branch to soleus in, 102 routine walking, 108 spasticity in, 220 with stroke, 65 tizanidine dosage scheduling in, 183 traumatic brain injury in, 225 Alcohol neurolysis, 223 Altered tone, 9–12; see also Spasticity Ambulatory stroke patients, 126 Amyotrophic lateral sclerosis (ALS), 268–269 Ankle dorsiflexion, 127 Ankle dorsiflexors, 68, 113 Ankle-foot orthosis, 83f, 94 Ankle kinetics, 96f Antagonist muscles, regulation of, 63 Anterior cord syndrome (ACS), 141 Anti-gravity distribution, 104 Antigravity strength, 93 Anti-spastic drug therapy, 146, 152 Areflexic flaccid paresis, 105 Arm and hand function, role of spasticity in about, 115–116 spasticity in patients with mildly affected upper limb (UAT 4-7), 118 with moderately affected upper limb (UAT 2-3), 117–118 with severely affected upper limb (UAT 0-1), 117 Ashworth scale, 94, 120, 148, 211t Ashworth score, 17 ASIA impairment scale C, 163, 164 Athetosis, 80n Autogenetic Ib inhibition, 43; see also Postsynaptic inhibition of motoneurons Autosomal dominant cerebellar ataxias (ADCA), 258–259, 260t–261t; see also Spinocerebellar degenerations Autosomal recessive ataxias, 266; see also Spinocerebellar degenerations 289 290 Axonal degeneration, 179 Axonal sensory neuropathy, 266 B Baclofen, 37, 123, 152, 163, 182 pump implantation, 190 Balance dysfunction, 262 Basal ganglia lesion, 46 Beck depression inventory, 248 Benzodiazepines, 85–86, 124, 152, 186 Biomechanical evaluation of spasticity, 27; see also Spasticity Biomechanical measurement methods, 120 Bladder dysfunction, 247 Body function/structure, 119–121, 119t, 120t Bone growth, 84 Bony changes, 247 Botulinum neurotoxin (BoNT), 158–160 treatment of toe-walking in children, 64 Botulinum toxin, 124–125 focal therapies for TBI, 220–222 management of spasticity in MS, 188 type A, 86–87 Brain-derived neurotrophic factor (BDNF), 273 Brain injury complications on spasticity, 209; see also Spasticity Brown-Séquard Syndrome (BSS), 142 Brunnstrom stages, 106f Bulbar symptoms, 270 C Calf spasticity, 83f, 84f; see also Spasticity Canadian Occupational Performance Measure, 121 Cannabinoids, 152, 184–186 Casting, 217 for contracture vs spasticity management, 217–218 Central cord syndrome (CCS), 141, 142 Central pattern generators (CPG), 108 Cerebral inflammatory presentation, 274 Cerebral palsy, characteristics of, 80–82 Index Cerebral palsy and spastic diplegia (CP-SD), 247, 250 Cerebral palsy/clinical management of spasticity cerebral palsy, characteristics of, 80–82 injection therapies botulinum toxin type A, 86–87 phenol, 87–88 oral medication benzodiazepines, 85–86 gabapentin and pregabalin, 86 oral baclofen, 86 overview, 70–80 surgical treatment intrathecal baclofen, 89–91 neurotomy, 89 selective dorsal rhizotomy, 91–96, 95t therapy strengthening spastic muscles, 88 stretching, 88 treatment objectives, 82–85 Chemodenervation, 148, 157–160 Children botulinum toxin injections, 87 with cerebral palsy, 64 contractures in, 94 dorsal rhizotomy for, 195 with dystonia, 224 with HSP, 247, 251 hyperexcitable stretch reflexes in, 63 neronal migration disorder in, 81 oral baclofen for, 86 selective dorsal rhizotomy (SDR) for, 161 SPARCLE study of in, 82 with spastic cerebral palsy, 88, 254 surgical management of spasticity, 226 upper extremity in, with cerebral palsy, 159 Clasp-knife response, 11f, 16 Clonidine, 152 Clonus, 8–9, 13, 44–45 Closed-nerve blockade, 187 Clostridium botulinum, 188 Co-contraction; see also Spasticity abnormal movement patterns and, 12–13 for joint stiffness, 69–70 Index Cognitive dysfunctions, 107 Collateral sprouting, 35 Coma, 90, 186, 205, 224, 225 Coma Recovery Scale (Revised) (CRS-R), 225 Commissural interneurons, 68 Complex spastic SCI patient combination therapies intrathecal systemic/focal (case study), 164 oral systemic/focal (case study), 163 Composite Spasticity Index, 17 Compound muscle action potentials (CMAP), 141 Contractures biomechanical methods to measure, 18 in cerebral palsy, see Cerebral palsy/ clinical management of spasticity defined, 14 measurement of, 17–19 in patients with upper motoneuron syndrome, 14–16 by spasticity/immobilisation, 204, 205f surgical correction of, 160–161 surgical treatment of, 81 Contralateral ankle spasticity, 218; see also Spasticity Conus medullaris syndromes, 143 Coordination, 141, 142, 161, 205f, 265t interjoint, 67–68 interlimb, 68–69 Cortical activation with movement, 241 Cortical hyperexcitability, 269 Cortical stimulation, 273 Corticospinal tract, 266 Cramps, 243 Cystic disorders, 274; see also Leukodystrophies D Dantrolene, 152, 183 Dantrolene sodium, 124 Deep tendon reflexes (DTR), 139 291 Delayed plantar response (DPR), 139 Demyelinating/dysmyelinating disorders, 273; see also Leukodystrophies De novo mutations, 274 Depression, 40, 43, 62, 63, 65, 156, 266 CNS, 223 post-activation, 36, 37–39, 244 respiratory, 157, 186 Diazepam, 37, 85, 186 Dietary therapy, 275 Diffusor tensor imaging (DTI), 241 Disability-adjusted life years (DALY), 102 Disability Rating Scale, 225 Disease-modifying therapy, 271 Disordered motor control, 70 over-activity and, 70 Disordered sensori-motor control, Distal axonopathies; see also Hereditary spastic paraparesis (HSP) balance in HSP, 248–249 cellular changes, 238–240, 239t clinical presentation, 237–238 cortical activation with movement, 241 descending/ascending tract function, changes in, 240–241 outcome measurement, 252 physical interventions, 257–258 prevalence and genetics, 237 service delivery, 258 spasticity, pharmacological/surgical treatment of, 253–255 spasticity/associated symptoms on functional ability, 248 walking difficulties, 249–252 Disynaptic reciprocal Ia inhibition, 40–41; see also Postsynaptic inhibition of motoneurons Dorsal rhizotomy, 195 Dorsiflexors, 40, 41 Dynamometry, 251 Dysdiadochokinesis, 118 Dysfunctional equinovarus posture, 113 Dystonia, 80n about, 46 clinical management, 81 292 E Education of patient, 123 Electrical stimulation, 206, 218–219 Electromyography (EMG), 10, 64, 148 Endocytosis, 240 Endosomes, 240 Endurance training, 268 EPSP, 34, 64–65 Epstein Barr Virus (EBV) infection, 176 Exaggerated reflexes, Exaggerated stretch reflex activity; see also Spasticity, pathophysiology of about, 31–32 motoneuronal changes, pathophysiological role of, 32–35 postsynaptic inhibition of motoneurons, pathophysiological role of changes in, 40–44 presynaptic sites, regulation at, 35–39 sprouting, 35 transmission in group II pathways, 39–40 Exercise in ALS, 272 in MS, 191 Exocystosis, 240 Extensor hallucis longus, 114 Extrapyramidal signs, 270 F Familial ALS (FALS), 269; see also Motor neuron disorders/ familial ALS Fatigue, 248 Femoral anteversion, 84 Flexor reflex afferent (FRA) circuitries, 45 FMRI, 241, 245 Focal anti-spastic pharmacotherapy, 157–160 Focal anti-spastic surgical treatment, 161–162 Index Focal chemodenervation, 159, 164 Focal spasticity, 125; see also Spasticity treatments for, 187 Focal therapies botulinum toxin injection, 220–222 case study, 222–223 phenol/alcohol neurolysis, 223 Force vital capacity (FVC), 271 Frataxin, 266 Frenchay Scale, 214 Frequency, intensity type, and time (FITT), 191 Friedreich’s ataxia (FRDA) about, 266–267; see also Spinocerebellar degenerations management of Co-enzyme Q10 and idebenone, 267 symptomatic management, 267–268 Frontal lobe white matter, 241 Functional ambulation category (FAC), 254 Functional electrical stimulation (FES), 257 Fusimotor drive, gamma-spasticity, 43–45; see also Postsynaptic inhibition of motoneurons F-wave measurements, 19 G GABAergic drugs, 37 Gabapentin, 86, 152, 183–184, 254 GABA receptor, 36, 86, 89, 184 Gait control, role of spasticity in, see Postural control/gait control, role of spasticity in Gamma-aminobutyric acid (GABA), 123, 152, 245 Gamma-spasticity, 43–45; see also Spasticity Glasgow Outcome Scale, 217 Global Spasticity Score, 192 Goal Attainment Scale, 121 Goal Attainment Scaling (GAS), 212 Golgi tendon organs, 39, 43 293 Index Graded Redefined Assessment of Strength, Sensibility, and Prehension (GRASSP), 149 Gross motor function measure (GMFM), 254 H Handheld dynamometers, 27 Hand spasticity, 142, 160 Hematopoietic stem cell transplantation, 275 Hemiplegic gait, 109 Hereditary myelopathies, 236 Hereditary spastic paraparesis (HSP) adrenoleukodystrophy (ADL) clinical presentation, 274–275 interventions, 275 prevalence and genetics, 274 case study, 242, 253b, 255b–256b distal axonopathies balance in HSP, 248–249 cellular changes, 238–240, 239t clinical presentation, 237–238 cortical activation with movement, 241 descending/ascending tract function, changes in, 240–241 outcome measurement, 252 physical interventions, 257–258 prevalence and genetics, 237 service delivery, 258 spasticity, pharmacological/ surgical treatment of, 253–255 spasticity/associated symptoms on functional ability, 248 walking difficulties, 249–252 leukodystrophies cystic disorders, 274 demyelinating/dysmyelinating disorders, 273 hypomyelinating disorders, 273 spongiform disorders, 273 motor neuron disorders/familial amyotrophic lateral sclerosis amyotrophic lateral sclerosis (ALS), 268–269 clinical presentation, 269–271 disease-modifying therapy, 271 pathology, 269 symptomatic management, 271–273, 272t overview, 236–237 spinocerebellar degenerations autosomal dominant cerebellar ataxias (ADCA), 258–259, 260t–261t autosomal recessive ataxias, 266 Friedreich’s ataxia (FRDA)/ late-onset Friedreich’s ataxia (LOFA), 266–268 SCA3 or Machado-Joseph disease, 259–262, 261t, 266 symptoms associated with bladder dysfunction, 247 bony changes, 247 fatigue, 248 limb stiffness, 243–246 mood/quality of life, 248 paresis, 246–247 sensory loss, 247 Heterotopic ossification (HO), 209 High tone, Hippotherapy, 194 Hoffmann reflex, 121 H-reflex about, 43, 140 measurements, 19 technique, 41 5-HT receptors, 34, 35, 45 Hydrotherapy, 257 Hyper-excitable reflexes, Hyperexcitable stretch reflexes, 62–63 Hypertonia, Hypertonicity in paralysis, Hypomyelinating disorders, 273; see also Leukodystrophies Hypotonia, 4–6 I Idebenone, 267 Impairments, classification of, 17 IncobotulinumtoxinA, 220 294 Increased reflexes, Injection therapies; see also Cerebral palsy/clinical management of spasticity botulinum toxin type A, 86–87 phenol, 87–88 Interjoint coordination, 67–68 Interlimb coordination, 68–69 Interlimb reflexes, 139 Intermittent theta burst stimulation (iTBS), 190 Intermittent theta burst TMS (iTBS), 193 Intramuscular botulinum toxin injection, 220 Intrathecal anti-spastic pharmacotherapy, 155–157 Intrathecal application of baclofen (ITB), 155, 255 Intrathecal baclofen (ITB), 89–91, 125–126, 146, 189–190, 254 Intrathecal therapies, for TBI, 223–226, 226t Intrinsic stiffness, 28 Invasive/permanent methods, for stroke patients, 126–128 Invasive/reversible methods, for stroke patients, 124–126 Isometric muscle strength, 246 J Joint posture, measurement of, 119 Joint stiffness, 214 K Kernicterus, 80 King’s Hypertonicity Scale, 214 L Late-onset Friedreich’s ataxia (LOFA), 266–268; see also Spinocerebellar degenerations Leukodystrophies; see also Hereditary spastic paraparesis (HSP) cystic disorders, 274 demyelinating/dysmyelinating disorders, 273 Index hypomyelinating disorders, 273 spongiform disorders, 273 Limb stiffness, 243–246 Lipids, 240 Locomotor training (LT), 151, 152 Long-latency, defined, 29 Long-latency stretch reflexes/ coordination of movement, 66–67 Lower extremity spasticity, 206 Lower limb weakness, 246 Lower motor neuron (LMN), 268 M Machado-Joseph disease, 259–262, 261t, 266; see also Spinocerebellar degenerations Maculopathy, 259 Mechanical resistance, 29 Medication possession ration (MPR), 219 Microneurography, 43 Mildly affected upper limb (UAT 4-7), spasticity in patients with, 118 Moderately affected upper limb (UAT 2-3), spasticity in patients with, 117–118 Modified Ashworth Scale (MAS), 26, 102, 115, 148, 191, 193 Monoaminergic neurotransmitters, 39 Monosynaptic Ia afferent pathway, 65 Mood/quality of life, 248 Motoneuronal changes, pathophysiological role of, 32–35 Motoneurons, 32 postsynaptic inhibition of, see Postsynaptic inhibition of motoneurons Motor-evoked potentials (MEP), 240 Motor nerve, 89 Motor neuron disorders/familial ALS amyotrophic lateral sclerosis (ALS), 268–269 clinical presentation, 269–271 disease-modifying therapy, 271 pathology, 269 symptomatic management, 271–273, 272t 295 Index Motor recovery/motor control after stroke, 105–108 Movement patterns, abnormal, 12–13; see also Spasticity MS, see Multiple sclerosis (MS) Multiple sclerosis (MS) disease-modifying treatments for, 179 incidence/epidemiology/disease course, 176–178 pathophysiology of/spasticity, 178–179 spasticity in, 179–181 spasticity management in MS hippotherapy, 194 neuromuscular electrical stimulation cycling (NMES), 194 non-pharmacological treatments, 190–194 pharmacological treatments, 181–190, 186t strategy for, 195–197 surgery, 195 whole-body vibration (WBV), 194 Muscle afferents, 35 Muscle fibre atrophy, 105 Muscle hypertonia, 138, 140 Muscle overactivity, 104 and increased co-contraction, 118 during stance phase, 111–113 during swing phase, 113–115 Muscle reinnervation, 223 Muscle resistance, 26 Muscle stiffness, 38 Muscle weakness, 249 Myotonometer, 216 N National Institute for Care and Clinical Excellence (NICE), 179, 181 Neuroleptic malignant syndrome, 89 Neurolysis, 124 Neuromuscular electrical stimulation cycling (NMES), 194 Neurons within ascending/descending tracts, 238 Neurotomy, 89 NIHSS score, 15 Nocturnal oximetry, 271 Noninvasive treatment, for stroke patients with spasticity, 123–124 Non-invasive ventilation (NIV), 271, 272t Non-pharmacological treatments, in MS about, 190–191 physical activity/exercise for spasticity management in MS, 191 transcranial magnetic stimulation (TMS) for, 193–194 transcutaneous electrical nerve stimulation (TENS) for, 191–193 Non-reflex stiffness, 214, 215 Nonsurgical management of spasticity, 126 Normal tone, Nutritional management, 271 O Obturator nerve, 124 Ocrelizumab, 179 Onabotulinum toxin A, 217, 220 Oral anti-spasticity medications, 254 evidence-based guidelines for, 186–187, 186t Oral baclofen, 86 Oral medication; see also Cerebral palsy/clinical management of spasticity about, 181, 219 benzodiazepines, 85–86 gabapentin and pregabalin, 86 oral baclofen, 86 Oral spasmolytic drugs, 123 Oral systemic anti-spastic pharmacotherapy, 152–155, 153t Orthopaedic surgical procedures, 195 Orthoses, 257 Orthotic devices, 88, 123 Orthotic management, 82 Over activity phenomena, 35, 70 P Paralysis, hypertonic, Paresis, 246–247 Participation, activity and, 121 296 Passive stretching, 216 Penn Spasm Frequency Scale, 17, 214 Perceived resistance to passive movement (PRPM) test, 120t Percutaneous endoscopic gastrostomy (PEG), 271 Persistent inward currents (PIC), 32, 34, 45, 138 Persistent vegetative state (PVS), 225 Phenol and alcohol neurolysis, 223 chemodenervation, 187–188 in injection therapies, 87–88, 158, 223 Physical activity for spasticity management in MS, 191 Physical modalities, 216–217 Physical therapy, 123, 150, 257 Physiotherapy, 151, 191 Pigmentary retinopathy, 259 Plantarflexor, 40, 41, 64 spasticity, 257 Plastic surgery, 126 Polyglutamate (polyQ) disease, 259 Polysegmental reflexes, 140 Polysynaptic reflexes, 144 Position-dependent spasticity, 10f–11f, 16 Post-activation depression, 36, 38 Postsynaptic inhibition of motoneurons autogenetic Ib inhibition, 43 disynaptic reciprocal Ia inhibition, 40–41 fusimotor drive, gamma-spasticity, 43–44 recurrent inhibition, 41 Postural control/gait control, role of spasticity in about, 108–111 muscle overactivity during stance phase, 111–113 muscle overactivity during swing phase, 113–115 Pregabalin, 86 Premature birth, 81 Presynaptic inhibition, 36–37 Presynaptic sites, regulation at; see also Spasticity, pathophysiology of about, 35–36 post-activation depression, 37–39 presynaptic inhibition, 36–37 Index Primary adrenal insufficiency, 275 Primary afferent depolarisation (PAD), 36 Problematic spasticity, 110 Progressive-relapsing disease, 177 Proprioceptive feedback, 107 Proteins, 240 Pyramidal tract, lesion of, 29–30 Q Quality of life, 248 R Ramp and hold method, 10f Randomised controlled trials (RCT), 154, 182, 185 Range of Motion (ROM), measurement of, 119 Recurrent inhibition, 41; see also Postsynaptic inhibition of motoneurons Reduced range of motion (ROM), 160 Reflexes in antagonist, control of, 63 excitability, 61 increased, integrated part of voluntary movement, 59–60 modulation in simple contraction of agonist muscle, 60–62 in swing phase, suppression of, 63–64 Reflex hyperexcitability, 31 Reflex-mediated stiffness, 28–29 Reflex response, Rehabilitation about, 266 after stroke, 106 Rehabilitation Medicine spasticity, 102; see also Spasticity Relapsing-remitting MS (RRMS), 177 Relaxed muscle, response of, 9–12 Renshaw cells, 41 Renshaw-mediated recurrent inhibition, 270 Repetitive cortical stimulation, 273 Repetitive TMS (rTMS), 193 Index Respiratory muscle weakness, 270 Rigidity, Rimabotulinumtoxin B, 220 Routine walking, 108 S Sativex, 185 SCI, see Spinal cord injury (SCI) Scottish Intercollegiate Guidelines Network (SIGN) guidance, 181 Sedation, 123 Selective dorsal rhizotomy (SDR), 91–96, 95t, 161–162 Self-report instruments, 121 Sensitivity, 8n Sensory feedback contribution to movement, 64–66 Sensory loss, 247 Severely affected upper limb (UAT 0-1), spasticity in patients with, 117 Short-interval intracortcal inhibition (SICI), 269 Smooth rectified EMG (SRE), 111f Sniff nasal inspiratory pressures, 271 Soleus H-reflex, 37, 62, 244 SPARCLE study, 82 Spasm about, 8–9, 13 causes of, 45 defined, SPASM Consortium defining spasticity, 7, 7t Spasm Frequency Score, 17 Spastic dystonia, 16, 45–46, 70 Spasticity and associated symptoms on functional ability, 248 biomechanical evaluation of, 27–28 biomechanical methods to measure, 18 clinical evaluation of, 26–27 clinical presentation/anatomical distribution of, 149–150 clinical signs of, 144 definition abnormal movement patterns/ co-contractions, 12–13 about, 2–3, 3t 297 contractures in patients with upper motoneuron syndrome, 14–16 framework development for, 6–12, 7t altered tone or response of relaxed muscle, 9–12 increased (hyper-excitable/ exaggerated) reflexes, spasms and clonus, 8–9 hypertonia (or high tone), hypotonia, 4–6 measurement of spasticity/ contracture, 17–19 by SPASM Consortium, 7, 7t in upper motoneuron syndrome, 13–14 in multiple sclerosis (MS), 179–181 neurophysiological methods to measure, 19 overview, 1–2 pathophysiology-based treatment of about, 143–144 clinical signs of spasticity, 144 spastic movement disorder, 144–145 therapeutic consequences, 145–147 pharmacological/surgical treatment of, 253–255 in SCI, clinical assessment of, 148–149 treatment in SCI, 150–152 Spasticity, pathophysiology of by adaptive changes in spinal networks, 30–31 clonus, relation to spasticity, 44–45 exaggerated stretch reflex activity about, 31–32 motoneuronal changes, 32–35 postsynaptic inhibition of motoneurons, 40–44 presynaptic sites, regulation at, 35–39 sprouting, 35 transmission in group II pathways, 39–40 by lesion of pyramidal tract, 29–30 muscle response to stretch, nature of, 28–29 spasms, causes of, 45 298 spastic dystonia, 45–46 spasticity measurement (from clinical evaluation to biomechanical techniques), 26–28 Spasticity management in MS; see also Multiple sclerosis (MS) hippotherapy, 194 neuromuscular electrical stimulation cycling (NMES), 194 non-pharmacological treatments about, 190–191 physical activity/exercise for, 191 transcranial magnetic stimulation (TMS) for, 193–194 transcutaneous electrical nerve stimulation (TENS) for, 191–193 pharmacological treatments baclofen, 182 benzodiazepines, 186 botulinum toxin, 188 cannabinoids, 184–186 dantrolene, 183 focal spasticity, treatments for, 187 gabapentin, 183–184 intrathecal (IT) baclofen, 189–190 oral antispasticity medications, evidence-based guidelines for, 186–187, 186t oral medications, 181 phenol chemodenervation, 187–188 tizanidine, 182–183 strategy for, 195–197 surgery, 195 whole-body vibration (WBV), 194 Spastic movement disorder development of, 139–141 on patho-anatomy, 141–143 pathophysiology-based treatment of, 144–145 Spastic paraplegia rating Scale (SPRS), 252 Spastic patients, functional problems in co-contraction for joint stiffness, 69–70 interjoint coordination, 67–68 interlimb coordination, 68–69 long-latency stretch reflexes/ coordination of movement, 66–67 Index over-activity as general adaptation to central lesion, 70 reflexes, integrated part of voluntary movement, 59–60 sensory feedback contribution to movement, 64–66 stretch reflex modulation in spastic subjects control of reflexes in antagonist, 63 hyperexcitable stretch reflexes in stance phase of gait, 62–63 reflex modulation during simple contraction of agonist muscle, 60–62 suppression of reflexes in swing phase, 63–64 training for learning, 70–71 Specificity, 8n Spinal cord atrophy, 179 Spinal Cord Independence Measure (SCIM), 149 Spinal cord injury (SCI) complex spastic SCI patient combination therapies, intrathecal systemic/focal (case study), 164 combination therapies, oral systemic/focal (case study), 163 epidemiology/specific aspects of spasticity in, 137–139 overview, 136–137 patient selection/therapeutic approach clinical assessment of spasticity in, 148–149 clinical presentation/anatomical distribution of spasticity, 149–150 contractures, surgical correction of, 160–161 focal anti-spastic pharmacotherapy, chemodenervation, 157–160 focal anti-spastic surgical treatment, selective dorsal rhizotomy (SDR), 161–162 indication for treatment, spasticity in SCI, 147–148 intrathecal anti-spastic pharmacotherapy, 155–157 mainstay of spasticity treatment in SCI, physical therapy, 150–152 Index oral systemic anti-spastic pharmacotherapy, 152–155, 153t physiological effects of training, 150 spasticity, pathophysiology-based treatment of about, 143–144 clinical signs of spasticity, 144 spastic movement disorder, 144–145 therapeutic consequences, 145–147 spastic movement disorder and patho-anatomy, 141–143 spinal shock/recovery of spinal excitability/development of spastic movement disorder, 139–141 Spinal excitability, recovery of, 139–141 Spinal networks, adaptive changes in, 30–31 Spinal shock, 31, 139–141 Spinocerebellar ataxias (SCA), 259–262, 261t, 266 Spinocerebellar degenerations autosomal dominant cerebellar ataxias (ADCA), 258–259, 260t–261t autosomal recessive ataxias, 266 Friedreich’s ataxia (FRDA)/late-onset Friedreich’s ataxia (LOFA), 266–268 spinocerebellar ataxia3 (SCA3)/ Machado-Joseph disease, 259–262, 261t, 266 Splinting, 218 Splints and orthoses, 266 Spongiform disorders, 273; see also Leukodystrophies Sprouting, 35 Standing posture, 109 Stiff knee gait, 113, 251 Stiffness at elbow, 5f measured at knee joint, 7f Strengthening spastic muscles, 88 Stretching, 88 for contracture vs spasticity management, 217–218 Stretch reflex activation, 251 Stretch reflexes, 38, 43, 62 299 Stretch reflex-mediated resistance, 28 Stretch reflex modulation in spastic subjects control of reflexes in antagonist, 63 hyperexcitable stretch reflexes in stance phase of gait, 62–63 reflex modulation during simple contraction of agonist muscle, 60–62 suppression of reflexes in swing phase, 63–64 Striatal toe, 113, 114 Stroke about, 101–102 motor recovery/motor control after, 105–108 pathophysiology of spasticity after, 102–105 spasticity management after about, 122–123, 122t invasive, permanent methods, 126–128 invasive, reversible methods, 124–126 management strategy for, 128 noninvasive treatment, 123–124 Stroke, contractures in/clinical management of spasticity about stroke, 101–102 in arm and hand function about, 115–116 with mildly affected upper limb (UAT 4–7), 118 with moderately affected upper limb (UAT 2–3), 117–118 with severely affected upper limb (UAT 0–1), 117 motor recovery/motor control after stroke, 105–108 in postural control/gait control about, 108–111 muscle overactivity during stance phase, 111–113 muscle overactivity during swing phase, 113–115 stroke, pathophysiology of spasticity after, 102–105 stroke, spasticity management after about, 122–123, 122t 300 invasive, permanent methods, 126–128 invasive, reversible methods, 124–126 management strategy for stroke patients with spasticity, 128 noninvasive treatment, 123–124 stroke patients, spasticity assessment in about, 118–119 activity and participation, 121 body function and structure, 119–121, 119t, 120t Stroke patients, spasticity assessment in about, 118–119 activity and participation, 121 body function/structure, 119–121, 119t, 120t Stroke Upper Limb Capacity Scale, 121 Strumpell-Lorrain syndrome, 237 Supraspinal control of spinal networks, 69 Surface electromyography (sEMG), 121 Surface neuromuscular electrical stimulation, 123 Surgical treatment intrathecal baclofen, 89–91 in MS, 195 neurotomy, 89 selective dorsal rhizotomy, 91–96, 95t for TBI, 226–227 Symptomatic management, SCA, 262 T Tardieu scale, 27, 243 Tardieu Score, 17, 212t TBI, see Traumatic brain injury (TBI) Tendon-lengthening procedures, 195 Tendon transfers, 128 Tenotomy, 195 Tetrahydrocannabinol (THC), 155 Tetraplegia, 160 Tibial nerve, 124, 126 Timed Up and Go Test, 121 Tizanidine, 40, 124, 152, 163, 182–183 Tone about, 3, altered, 9–12 Tone Assessment Scale, 102, 214 Tongue movements, 270 Index Tonic supraspinal inhibition, 31 Tonus, 3, Training for learning, 70–71 physiological effects of, 150 Transcranial magnetic stimulation (TMS), 193–194, 240 Transcutaneous electrical nerve stimulation (TENS), 191–193 Transmission in group II pathways, 39–40 Traumatic brain injury (TBI) biomechanical assessment, 214–216 brain injury complications on spasticity, 209 clinical assessment, 212–214, 213t clinical presentations, 207–209, 208t contractures/spasticity on recovery, 206 management options electrical stimulation, 218–219 focal therapies, 220–223 intrathecal therapies, 223–226, 226t oral medications, 219 physical modalities, 216–217 stretching/casting for contracture vs spasticity management, 217–218 surgical interventions, 226–227 treatment modalities, 227 overview, 204–206 treatment goals, 209–212, 210t–212t Treadmill training, 69 Trunk control, 108 U Upper limb symptoms, 270 Upper motoneuron syndrome spasticity in, 13–14 Upper motor neuron (UMN), 204, 236, 268 Utrecht Arm/Hand Test (UAT), 116, 116f V Velocity-dependent response, 10f–11f Very late-onset Friedreich’s ataxia (VLOFA), 267 301 Index Visual Analogue Scaling, 121 Visual problems, 259 W Walking difficulties, 249–252 Walking index in SCI (WISCI), 149 Walking Test, 121 Wallerian degeneration, 88, 142 Water therapy, 151 Whole-body vibration (WBV), 194 Wolff’s law, 85n Z Zone of partial preservation, 141 ... observed in chronic spinal injury or if it is only present during induced spasms Long-term intramuscular single-motor unit recordings in the human, which could substantiate the finding, are lacking... training They allow longer training times and can provide useful feedback information to the patient about the course of Clinical Management of Spasticity and Contractures in Spinal Cord Injury... 91(5): pp 22 47–58 Clinical Management of Spasticity and Contractures in Spinal Cord Injury 165 Gorassini, M.A et al., Role of motoneurons in the generation of muscle spasms after spinal cord injury