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General assessment of the upper limb
Hand Clin 19 (2003) 557–564
General assessment of the upper limb Caroline Leclercq, MD Institut de la Main, 6 Square Jouvenet, Paris 75016, France Institut de la Main, Neurological Rehabilitation Centre, Coubert 75016, France
Clinical examination Clinical examination is the key to successful treatment. Unlike many other conditions, there is no standard clinical picture for spastic upper limbs, whatever their cause, as the condition primarily depends on the extent of the brain damage, which can vary greatly. A careful assessment of all parameters is therefore mandatory to get an accurate clinical picture of the deficits and potentials in each patient and to treat them accordingly. This examination is performed with a fourfold goal: Evaluate spasticity Evaluate the motor and sensory deficit in the upper limb Evaluate the existing function and functional needs of the upper limb Perform a complete general examination to seek associated neurologic disorders and seek contraindications to surgery This examination is lengthy and requires detailed knowledge of neurology, pediatrics, and physiatry. It is best performed with all the specialists involved in the child’s care (physiatrist, physical therapist, occupational therapist, pediatrician, and surgeon). It should be done in a warm, quiet, and friendly environment (eg, toys, adapted furniture). Trust must be established by the examiner at the beginning of the evaluation; this is essential because the child’s cooperation is mandatory for the sensorimotor evaluation and because spasticity may increase considerably if the child is frightened or recalcitrant. If painful
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procedures (eg, injections) are necessary, they must be left for last. Symptoms may vary with the child’s emotional state and fatigue level; even the weather can modify the clinical picture. Moreover, many children, especially younger ones, cannot cooperate throughout the entire examination and require that this examination be fragmented. It is therefore not wise to decide on surgery after a single examination, and at least a second session is recommended to have a more accurate view of the clinical picture. Video recording of this examination is most helpful in the decision making and in evaluation of surgical outcome. Resting posture of the upper limb Inspecting the limb at rest before examination provides information on the amount of spasticity. It is done while the child is sitting or lying down motionless, with his or her attention distracted elsewhere (eg, TV, movie). If predominant, spasticity usually leads to a resting posture in shoulder adduction and internal rotation, elbow flexion, forearm pronation, and wrist flexion. If it is moderate, however, the resting posture may be normal. The fingers may assume varied positions when spasticity is predominant. Most often they are curled in a clenched fist, but they also can display a swan neck deformity or a claw type deformity. A boutonniere type of deformity is less common. The thumb can assume either an adducted posture or an adducted and flexed posture. The adducted thumb is often in a slight retroposition with the metacarpophalangeal (MP) and interphalangeal (IP) joints extended. The flexusadductus thumb, often referred to as ‘‘thumbin-palm,’’ is embedded in the palm with full
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opposition and full flexion of MP and IP joints. Often the clenched fist is curled around the thumb. Any factor that aggravates spasticity increases these deformities. Evaluation of spasticity Spasticity is a muscle hypertonicity defined by five classic characteristics. 1. It is selective, predominant in flexor and adductor muscles, and is responsible for the characteristic flexion-pronation deformity of the upper limb. It is usually less evident in the shoulder and elbow and more severe in the distal part of the upper limb. In the shoulder it involves the adductor and internal rotator muscles and in the elbow the flexor muscles, predominantly the biceps brachii and the brachialis, but possibly also the brachioradialis [1]. The medial epicondylar muscles, often spastic, are responsible for the pronation deformity and also contribute to the elbow flexion deformity. The wrist flexors, especially the flexor carpi ulnaris (FCU), are frequently the muscles most involved by spasticity in the upper limb, leading to a characteristic hyperflexion and ulnar deviation of the wrist. If the finger flexors are also spastic, they contribute to this deformity. Spasticity of the finger extrinsic muscles is tested by tapping briefly on the pulps (flexors) or the nails (extensors), which produces an exaggerated response of the tested muscles. In such cases, spasticity of the intrinsic muscles of the hand is extremely difficult to evaluate because of associated deformities. An intrinsic-plus deformity (MP joint flexion and IP joint extension) results from spasticity of the interossei. A swan neck deformity (proximal interphalangeal [PIP] joint hyperextension and distal interphalangeal [DIP] joint flexion) may be the result of either excessive traction on the extensor tendons caused by excessive wrist flexion (extrinsic swan neck) or to spasticity of the interossei muscles (intrinsic swan neck), in which case it is often associated with MP joint flexion. Passively flexing the wrist usually reduces the intrinsic swan neck, whereas it increases the extrinsic swan neck. A claw-type deformity with the MP joints hyperextended and the PIP joints flexed usually is caused by
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a combination of excessive traction on the extensor tendons and paralysis of the intrinsic muscles. Thumb spasticity involves mainly the adductor pollicis, resulting in an adducted position. When the flexor pollicis longus (FPL) also is involved, a thumb-in-palm deformity develops. It is elastic. Attempts at stretching the involved muscles meet with resistance, which increases with the amount of strength applied and which correlates with the degree of spasticity. Unlike plastic contractures, the joint returns to its initial position as soon as the opposing force is stopped. If the opposing force is maintained long enough, however, the muscles usually yield, sometimes abruptly (this is referred to as the ‘‘penknife blade’’ phenomenon). The degree of resistance to stretching can be quantified by Ashworth’s scale, modified by Bohannon [2]. It is present at rest and exaggerated with voluntary movement, emotion, fatigue, and pain. Osteotendinous reflexes are exaggerated, brisk, diffuse, and polykinetic. Clonus is infrequent in the upper limb. Synkineses may be associated. Synkinesis is the phenomenon wherein paralyzed muscles, incapable of a certain voluntary movement, attempt to execute this movement in association with contraction of accompanying intact muscles. For example, in Souques synkinesis, the hand displays ‘‘active’’ extension and abduction of the fingers with voluntary shoulder abduction.
Motor assessment Motor examination of the upper limb is not easy in children, especially in children younger than 5 years of age. The child should be provided with toys of different forms and colors and should be observed at play [3]. Each muscle or group of muscles is evaluated for (1) voluntary motor control, (2) fibrous contracture, and (3) joint range of motion. Voluntary motor control The palsy usually predominates in the extensor and supinator muscles and in the distal part of the upper limb. Rather than a true paralysis, it is a deficit in voluntary control linked with the pyramidal tract involvement (‘‘pseudo palsy’’).
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Voluntary motion is often slow and may be better in a limited range of motion. This examination may become difficult when the antagonist muscles are severely spastic. Weakness generally predominates in the distal muscles spared from spasticity (wrist and finger extensors, abductor, and extensor pollicis longus, and supinator muscles). The proximal muscles (shoulder external rotators and abductors and elbow extensors) are involved to a lesser degree. The lack of control varies with limb position. For instance, voluntary movement of the thenar muscles often is facilitated by elbow extension. In some cases, these pseudo-paralyzed muscles may be present but made ineffective by the spastic antagonist or by elongation caused by a severe deformity. Such is often the case for the wrist extensors in severe flexion deformities of the wrist. Careful palpation of the muscle belly during attempted active motion may confirm that the muscle is not paralyzed but cannot give information of its actual strength. In such cases, an accurate evaluation of individual muscles can be performed only after spasticity of the antagonists has been suppressed (see ‘‘Motor blocks’’ below). The spastic muscles (flexor, adductor, and pronator muscles) are also usually active, but their voluntary movements often are impaired by co-contractions of other muscle groups. Cocontractions are frequent in the hand, especially of the wrist flexors during attempted finger flexion. They may render clinical examination difficult. In difficult cases, motor blocks and electromyographic studies are helpful. Fibrous contracture Fibrous contracture involves only spastic muscles. Unlike spasticity, fibrous contracture is permanent and cannot be overcome. It can be alleviated by shortening the involved articular segment. For example, posturing the wrist in maximal flexion relieves fibrous contracture of the finger flexors. In cases of severe spasticity, however, clinical distinction between contracture and spasticity may be extremely difficult to establish. In such cases, motor blocks may be helpful [4]. Motor blocks. These blocks require a percutaneous injection. It is recommended that they be performed at the very end of the clinical examination so the child remains confident and cooperative throughout the whole session.
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Motor blocks provide several types of information: They differentiate spasticity from fibrous contracture in flexor, adductor, and pronator muscle groups They allow assessment of individual muscles after removing spasticity of the antagonists or co-contractions of adjacent muscles Within a spastic muscle group they help determine which muscles are spastic and which are not Several pharmacologic agents have been used percutaneously: anesthetics (lidocaine), diluted alcohol [5], and phenol [6]. As the last is toxic to surrounding soft tissues and sensory nerve fascicles, it is better to approach the nerve surgically for phenol blocks [7]. Whichever substance is used, it may be injected either around the nerve trunk or in the motor endplates of the involved muscles. Nerve trunks are fairly easy to locate. Injection at this site diffuses to other motor nerves, however, with an overlapping response. The motor endplates of individual muscles are much more difficult to locate and clinicians previously relied on chart drawings [5]. This has been facilitated greatly with the use of nerve stimulators. For intrinsic muscles in the hand, it is practically impossible to find the motor endplates of each individual muscle. One usually chooses to inject the motor branch of the ulnar nerve and, if required, the thenar motor branch. The effect of a motor block with lidocaine starts 15 minutes after injection and usually lasts for half an hour. The duration of efficacy is longer for alcohol and much longer for phenol. Spasticity yields completely, whereas contracture persists [7]. One then can assess muscle contracture in each muscle or muscle group. The most frequent example in the upper limb is severe spasticity of the wrist flexors. Following paralysis, muscles can be tested for contracture and the finger flexors can be assessed. Injection of a spastic pronator teres informs of a possible contracture of the interosseous membrane. One also can test the nonspastic muscles then, now relieved of their spastic antagonists, for voluntary motor control. In specific cases (eg, extensor carpi radiales muscles) the muscles are present and do contract, but are ineffective because of progressive lengthening over time, secondary to a severe wrist flexion deformity. Within a spastic group, one can perform a selective block of the predominant spastic
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muscle to evaluate voluntary control and spasticity of the other muscles. For example, after injecting the FCU, one can test flexor carpi radialis (FCR). In the same way, after injecting the biceps brachii, one can test the brachialis and the brachioradialis. Recently, botulinum toxin has partially supplanted motor blocks. It produces the same effects but with a much easier technique of injection, a lack of side effects, and a long lasting effect [8]. Injection of botulinum A within the body of a muscle induces a paralysis that starts 10–15 days after the injection and lasts 3–6 months [9,10]. Another article in this volume is dedicated to botulinum toxin and describes its indications in cerebral palsy (See article by Chin and Graham). Whether used as a diagnostic tool for preoperative assessment or as part of the therapeutic plan, botulinum effectively replaces motor blocks. Joint range of motion Passive range of motion rarely is affected in isolated cerebral palsy in children. It is impaired more frequently in adults [11] and, when severe, associated neurologic lesions coexist. The precise range of motion of many joints may be difficult to assess, not so much because of spasticity, but because of muscle fibrous contractures. Each individual joint is tested with the involved muscles fully relaxed. This is performed as gently as possible so as not to trigger spasticity. If fibrous contracture is present, motor blocks do not assist in assessment of the passive range of joint motion. Sometimes it is so difficult that preoperative examination under anesthesia is necessary to define the range of motion precisely. Some joints of the fingers and thumb may have excessive passive motion, resulting in joint instability. This occurs mainly at the thumb MP joint (hyperextension and lateral instability) and at the finger MP and PIP joints (hyperextension). Finally, a general motor assessment of the upper limb is performed to evaluate global motor control. Some standard tests are helpful, such as the head-to-knee test, in which the patient is asked to place his hand on his head and then move it to the contralateral knee. The speed and precision of the movement are recorded. These nonspecific tests involve many of the elements susceptible to perturbation (hypertonia and muscle contracture, ataxia, apraxia, and extrapyramidal lesions) and give information of the potential use of the limb should a surgical treatment be decided on.
Primitive reflexes also are sought. They are caused by an abnormal sensory motor development and impair greatly the functional capacity of the limb. One classic example of these primitive reflexes is the asymmetric neck reflex: when the head is turned actively or passively to one side, it produces abduction of the shoulder and extension of the elbow, wrist, and fingers of the ipsilateral upper limb, whereas the contralateral limb flexes in all joints. Once the motor examination has been completed, an attempt at classification can be made. Zancolli’s classification [12] is probably the most popular one: Type I includes the spastic intrinsic-plus hands, in which spasticity of the interossei and lumbrical muscles causes flexion of the MP joints and extension of the IP joints, sometimes associated with a swan neck deformity. In this type a wrist flexion deformity is rare. Type II includes the spastic flexion-pronation hands with (hyper)flexion of the wrist and pronation of the forearm. Three groups are individualized depending on the degree of active finger extension. In the first group, with the wrist in neutral or near neutral, there is full active extension of the fingers. In the second group there is nearly complete active extension of the fingers, but only with some degree of wrist flexion. This group is subdivided further based on the presence (subgroup A) or absence (subgroup B) of active wrist extension. In the third group there is no active finger extension, even with maximum wrist flexion. Goldner [4] has produced another classification: In group I the wrist and MP joint can be extended at least to neutral. There is active grasp and release. The main deficiencies are delayed speed, slow coordination, and minimal dexterity. In group II there is weakness of wrist and finger extension with mild contracture of the wrist, finger, and thumb flexors. The thumb remains in the palm during hand extension. The hand is used only as an assist and a stabilizer. In group III the wrist and finger flexors are contracted severely. The primary goal of surgery is cosmetic improvement.
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In group IV the hands are spastic and athetoid. House [13] has classified thumb deformities in four types: Type I is an isolated adduction of the first metacarpal. Type II is adduction of the first metacarpal with flexion of the MP joint. Type III is adduction of the first metacarpal with MP joint hyperextension. Type IV is adduction of the first metacarpal with flexion of MP and IP joints. Aside from the thumb classification of House, which is purely descriptive, the author does not find any of the available classifications particularly helpful, as the clinical picture always varies from one child to another, depending on the amount and extent of spasticity and paralysis. There are no two identical cases and often they do not fit accurately in any of the described categories. Sensory examination Sensory examination requires, besides the child’s cooperation, a certain level of intellectual capacity and language ability. It is practically impossible in the small child; it becomes feasible at age 4–5 years, and more complicated tests such as two-point discrimination become reliable only at age 6–7 years. The basic sensory functions (light touch, pain, temperature) are essentially intact in cerebral palsy, whereas complex sensations (epicritic sensibility, proprioception, gnosis) are affected more severely. Light touch is explored with a smooth point or a finger, pain with a needle, and temperature with tubes of hot (40 C) and cold (melting ice) water. Epicritic sensibility is explored with discriminatory tests such as two-point discrimination. Proprioception is tested by vibration (tuning fork) and by the sense of position of the limb: the patient is blindfolded, has the unaffected limb placed in one position and is asked to reproduce the position with the affected limb. Proprioception is usually more disturbed in the distal part of the limb. Gnosis is most affected. Placing an object in the child’s hand and asking him or her to identify it tests stereognosis. Graphesthesia is tested by drawing figures or forms in the patient’s palm. Sensation is considered satisfactory when the child identifies at least three out of five objects,
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can recognize large figures drawn in the palm, and has a two-point discrimination test of no greater than 5-10 mm (according to the child’s age) [14]. Pain may be present but is difficult to evaluate, especially in children who may not report it and may not know how to describe it. It may be linked to severe contractures or to a deformed joint. Other causes of pain should be considered. For example, the author has treated a young adult with CP with wrist pain caused by Kienbo¨ck disease, perhaps related to the severe flexion deformity of his wrist [15]. Functional examination This part of the examination tests the actual use of the upper limb. It is done with standard reproducible tests using a pre-established scenario and simple objects. It should be video recorded. This usually proves to be a great assessment tool, as it can be visualized as many times as necessary without necessitating the child’s presence and cooperation. The same tests and video recording are repeated after surgery and serve as a comparison for evaluation of the results of surgery. Grasping tests Handing objects to the child tests prehension. Thumb-finger pinch often is limited to a lateral (key) pinch because of the lack of fine voluntary control and because of the adducted posture of the thumb. Grasp generally is preserved, although not always functional because of finger flexor contractures, extensor weakness, and wrist flexor co-contractions. Release often proves difficult because of weakness of active finger extension. The pick up and release test is done with several objects of different size and shape displayed on a table in front of the child, asking him or her to pick up each of them and displace it to a different spot. This test evaluates not only the prehensile capacity of the hand but also the contribution of the whole limb to that function. Quantitative measurements can be made if one introduces the time factor. Many different tests have been described for this assessment. In the ‘‘Box and Block Test,’’ the patient moves as many wooden blocks as possible from one compartment to another in 1 minute [16]. Enjalbert’s test [17], designed for stroke patients, involves grabbing a pen presented 40 cm away from the involved upper limb and handing it back to the examiner.
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Instrumented objects have been used more recently in an attempt to quantify hand grasp. Computerized systems of movement analysis now allow analysis of the different sequences of motor events and captors integrated in gloves can measure the forces generated by grasp [18,19]. Bimanual activities These give accurate information of the spastic upper limb’s actual functional ability. Several tests can be used, such as carrying a container with two handles, holding one object into which another one should be placed (eg, envelope and letter, nest of dolls) or holding a ruler while drawing a line with the other hand. Questionnaire The child and family are asked to describe precisely how the hand is used in activities of daily living, such as dressing, self-care, and eating. This can be done in the form of a questionnaire that must be adapted to the child’s age. The questionnaire can be completed during the session or given to the child and family to be completed at home (self-questionnaire). In many cases the upper extremity is neglected by the child despite functional capacity. In these cases it is possible, if not probable, that the child persists in ignoring the limb even if functional ability can be improved by surgery. There are several validated nonspecific questionnaires evaluating hand function (Michigan Hand Outcome Questionnaire [20]. To the best of the author’s knowledge, however, there is no validated questionnaire adapted to the cerebral palsied child. Tests Many tests have been designed to assess the functional value of the upper limb. Among the nonspecific tests, only those testing the simplest functions can be used in patients with CP (Frenchay Arm Test, with only five items [21], Jebsen test, with seven items [22]). There are few tests specifically designed for the spastic upper limb [4]. The ‘‘400 points’’ test [23], which analyzes mobility, grip strength, pick up and release, and bimanual function, can be used in children starting from 7 years of age. In children younger than 7 years, Rumeau et al [24] use a bimanual test adapted to each age group. The author’s group has chosen to use that described by Hoffer [14], which tests dressing, personal hygiene, feeding, bimanual activities, grasp and release, and lateral pinch.
General preoperative examination The aim of this general examination is to evaluate the real benefit the child will get from surgery, taking into account other neurologic impairments, the patient’s age, intelligence, motivation, and environment. Other neurologic impairments As these children are hemiplegic, the lower limb deficit also must be assessed. It is especially important to know about the child’s walking ability and the possible need for walking aids (eg, crutch, wheelchair). If operations are necessary for improvement of the lower limb, they usually are planned before upper extremity surgery. Associated extrapyramidal signs also should be detected. These include the following: Athetosis, which consists of unexpected, nonvoluntary movements causing a slow oscillation of the limbs. It is reduced at rest, abolished at sleep, and increased by noise, fatigue, and emotions. Chorea consists of brisk, rapid, and anarchic nonvoluntary movements of variable amplitude that can involve all territories. In the upper limb these contortions of the forearm, hand, and fingers make activities of daily living impossible. Parkinson syndrome is characterized by the classic triad: resting tremor, plastic hypertonia (predominant in the proximal muscles) with the cogwheel sign, and akinesia. If these extrapyramidal signs are predominant, they preclude surgical treatment, because the child is unable to use the hand because of these nonvoluntary movements. When they are mild, surgery may be performed when indicated. The capacity of the child to communicate must be evaluated, seeking visual, hearing, and language deficits. Behavioral problems, such as irritability, inability to concentrate, and emotional instability, also may constitute contraindications to surgical treatment if they predominate. Intelligence is evaluated through the intelligence quotient (IQ). It usually is stated that rehabilitation surgery is not indicated when the IQ is lower than 70, but this is not absolute. Surgical procedures, aimed at improving comfort, cosmesis, and personal hygiene, still are indicated [25]. Age Because the neurologic deficit in cerebral palsy is not progressive, early surgery can be planned.
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Sometimes it is necessary to operate very early because of an increasing deformity. In most cases, however, one can wait until the child is old enough that motor and sensory capacities can be evaluated accurately and he or she can cooperate with surgery and the postoperative rehabilitation. Sequelae of cerebral palsy in adults should be evaluated cautiously before surgical planning, because usually the patients have adapted already, functionally and socially, to the handicap, and surgery may be more disturbing than beneficial [26]. Motivation and environment Evaluation of motivation should take into account the patient’s ability to understand the modalities and benefits of the proposed treatment and to participate actively in the postoperative regimen. Understanding and motivation on the part of the parents are also mandatory. Environmental factors during the surgical period are also important, such as a rehabilitation center with an integrated school system and physiotherapists experienced in the management of children with CP. All are necessary components of the preoperative evaluation. According to Tonkin [27], ‘‘the ideal candidate is a cooperative 6-year-old child, with stable family support, who has a predominantly spastic upper limb deformity, with satisfactory hand sensibility, hemiplegic or monoplegic and without significant neurological deficits.’’
Electromyography EMG studies are often helpful. Static and dynamic studies are necessary, but they require cooperation on the child’s part [28]. This may be difficult to achieve in children younger than 5 years of age. In spastic muscles, EMG studies give information on the voluntary control, the possibility of relaxation, and possible co-contractions with another active movement. This is of the utmost importance when planning a tendon transfer, as a spastic muscle may be considered for potential transfer only if it displays satisfactory relaxation at rest and no co-contraction with other voluntary movements [28]. In other muscles, EMG studies are able to identify voluntary control that is not clinically detectable, for example, when spastic or retracted antagonists, joint deformity, or stiffness prevent functional contraction. EMG studies can help in
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determining the most appropriate muscles for transfer.
Radiography Radiographs are part of the preoperative evaluation. They are aimed at assessing any growth disturbance and joint deformity linked to the spasticity. Satisfactory assessment, however, may not be easy when there is a severe deformity such as wrist hyperflexion. Contralateral views in the same position may be helpful then. In such cases, standard radiographs may reveal growth disturbances of the distal radius and ulna and the carpus. They also may reveal avascularity of the lunate (Kienbo¨ck disease) [15]. In the elbow there may be a rare dislocation of the radial head [29,30].
Summary Clinical examination of the upper limb of a spastic patient is a lengthy and somewhat complicated procedure that is best performed together with all the practitioners involved in the child’s care. It should be repeated before any decision is made regarding treatment. Standard procedures in surgery of the upper limb in cerebral palsy are not appropriate, as the clinical picture is different for each individual patient. Surgery should be aimed at correcting spasticity, muscle/joint contracture, and motor deficit as required. All these elements therefore must have been assessed carefully before treatment. Finally, clinical examination detects all local and general contraindications to surgery and selects the appropriate candidates.
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General assessment of the upper limb Caroline Leclercq, MD Institut de la Main, 6 Square Jouvenet, Paris 75016, France Institut de la Main, Neurological Rehabilitation Centre, Coubert 75016, France
Clinical examination Clinical examination is the key to successful treatment. Unlike many other conditions, there is no standard clinical picture for spastic upper limbs, whatever their cause, as the condition primarily depends on the extent of the brain damage, which can vary greatly. A careful assessment of all parameters is therefore mandatory to get an accurate clinical picture of the deficits and potentials in each patient and to treat them accordingly. This examination is performed with a fourfold goal: Evaluate spasticity Evaluate the motor and sensory deficit in the upper limb Evaluate the existing function and functional needs of the upper limb Perform a complete general examination to seek associated neurologic disorders and seek contraindications to surgery This examination is lengthy and requires detailed knowledge of neurology, pediatrics, and physiatry. It is best performed with all the specialists involved in the child’s care (physiatrist, physical therapist, occupational therapist, pediatrician, and surgeon). It should be done in a warm, quiet, and friendly environment (eg, toys, adapted furniture). Trust must be established by the examiner at the beginning of the evaluation; this is essential because the child’s cooperation is mandatory for the sensorimotor evaluation and because spasticity may increase considerably if the child is frightened or recalcitrant. If painful
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procedures (eg, injections) are necessary, they must be left for last. Symptoms may vary with the child’s emotional state and fatigue level; even the weather can modify the clinical picture. Moreover, many children, especially younger ones, cannot cooperate throughout the entire examination and require that this examination be fragmented. It is therefore not wise to decide on surgery after a single examination, and at least a second session is recommended to have a more accurate view of the clinical picture. Video recording of this examination is most helpful in the decision making and in evaluation of surgical outcome. Resting posture of the upper limb Inspecting the limb at rest before examination provides information on the amount of spasticity. It is done while the child is sitting or lying down motionless, with his or her attention distracted elsewhere (eg, TV, movie). If predominant, spasticity usually leads to a resting posture in shoulder adduction and internal rotation, elbow flexion, forearm pronation, and wrist flexion. If it is moderate, however, the resting posture may be normal. The fingers may assume varied positions when spasticity is predominant. Most often they are curled in a clenched fist, but they also can display a swan neck deformity or a claw type deformity. A boutonniere type of deformity is less common. The thumb can assume either an adducted posture or an adducted and flexed posture. The adducted thumb is often in a slight retroposition with the metacarpophalangeal (MP) and interphalangeal (IP) joints extended. The flexusadductus thumb, often referred to as ‘‘thumbin-palm,’’ is embedded in the palm with full
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opposition and full flexion of MP and IP joints. Often the clenched fist is curled around the thumb. Any factor that aggravates spasticity increases these deformities. Evaluation of spasticity Spasticity is a muscle hypertonicity defined by five classic characteristics. 1. It is selective, predominant in flexor and adductor muscles, and is responsible for the characteristic flexion-pronation deformity of the upper limb. It is usually less evident in the shoulder and elbow and more severe in the distal part of the upper limb. In the shoulder it involves the adductor and internal rotator muscles and in the elbow the flexor muscles, predominantly the biceps brachii and the brachialis, but possibly also the brachioradialis [1]. The medial epicondylar muscles, often spastic, are responsible for the pronation deformity and also contribute to the elbow flexion deformity. The wrist flexors, especially the flexor carpi ulnaris (FCU), are frequently the muscles most involved by spasticity in the upper limb, leading to a characteristic hyperflexion and ulnar deviation of the wrist. If the finger flexors are also spastic, they contribute to this deformity. Spasticity of the finger extrinsic muscles is tested by tapping briefly on the pulps (flexors) or the nails (extensors), which produces an exaggerated response of the tested muscles. In such cases, spasticity of the intrinsic muscles of the hand is extremely difficult to evaluate because of associated deformities. An intrinsic-plus deformity (MP joint flexion and IP joint extension) results from spasticity of the interossei. A swan neck deformity (proximal interphalangeal [PIP] joint hyperextension and distal interphalangeal [DIP] joint flexion) may be the result of either excessive traction on the extensor tendons caused by excessive wrist flexion (extrinsic swan neck) or to spasticity of the interossei muscles (intrinsic swan neck), in which case it is often associated with MP joint flexion. Passively flexing the wrist usually reduces the intrinsic swan neck, whereas it increases the extrinsic swan neck. A claw-type deformity with the MP joints hyperextended and the PIP joints flexed usually is caused by
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a combination of excessive traction on the extensor tendons and paralysis of the intrinsic muscles. Thumb spasticity involves mainly the adductor pollicis, resulting in an adducted position. When the flexor pollicis longus (FPL) also is involved, a thumb-in-palm deformity develops. It is elastic. Attempts at stretching the involved muscles meet with resistance, which increases with the amount of strength applied and which correlates with the degree of spasticity. Unlike plastic contractures, the joint returns to its initial position as soon as the opposing force is stopped. If the opposing force is maintained long enough, however, the muscles usually yield, sometimes abruptly (this is referred to as the ‘‘penknife blade’’ phenomenon). The degree of resistance to stretching can be quantified by Ashworth’s scale, modified by Bohannon [2]. It is present at rest and exaggerated with voluntary movement, emotion, fatigue, and pain. Osteotendinous reflexes are exaggerated, brisk, diffuse, and polykinetic. Clonus is infrequent in the upper limb. Synkineses may be associated. Synkinesis is the phenomenon wherein paralyzed muscles, incapable of a certain voluntary movement, attempt to execute this movement in association with contraction of accompanying intact muscles. For example, in Souques synkinesis, the hand displays ‘‘active’’ extension and abduction of the fingers with voluntary shoulder abduction.
Motor assessment Motor examination of the upper limb is not easy in children, especially in children younger than 5 years of age. The child should be provided with toys of different forms and colors and should be observed at play [3]. Each muscle or group of muscles is evaluated for (1) voluntary motor control, (2) fibrous contracture, and (3) joint range of motion. Voluntary motor control The palsy usually predominates in the extensor and supinator muscles and in the distal part of the upper limb. Rather than a true paralysis, it is a deficit in voluntary control linked with the pyramidal tract involvement (‘‘pseudo palsy’’).
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Voluntary motion is often slow and may be better in a limited range of motion. This examination may become difficult when the antagonist muscles are severely spastic. Weakness generally predominates in the distal muscles spared from spasticity (wrist and finger extensors, abductor, and extensor pollicis longus, and supinator muscles). The proximal muscles (shoulder external rotators and abductors and elbow extensors) are involved to a lesser degree. The lack of control varies with limb position. For instance, voluntary movement of the thenar muscles often is facilitated by elbow extension. In some cases, these pseudo-paralyzed muscles may be present but made ineffective by the spastic antagonist or by elongation caused by a severe deformity. Such is often the case for the wrist extensors in severe flexion deformities of the wrist. Careful palpation of the muscle belly during attempted active motion may confirm that the muscle is not paralyzed but cannot give information of its actual strength. In such cases, an accurate evaluation of individual muscles can be performed only after spasticity of the antagonists has been suppressed (see ‘‘Motor blocks’’ below). The spastic muscles (flexor, adductor, and pronator muscles) are also usually active, but their voluntary movements often are impaired by co-contractions of other muscle groups. Cocontractions are frequent in the hand, especially of the wrist flexors during attempted finger flexion. They may render clinical examination difficult. In difficult cases, motor blocks and electromyographic studies are helpful. Fibrous contracture Fibrous contracture involves only spastic muscles. Unlike spasticity, fibrous contracture is permanent and cannot be overcome. It can be alleviated by shortening the involved articular segment. For example, posturing the wrist in maximal flexion relieves fibrous contracture of the finger flexors. In cases of severe spasticity, however, clinical distinction between contracture and spasticity may be extremely difficult to establish. In such cases, motor blocks may be helpful [4]. Motor blocks. These blocks require a percutaneous injection. It is recommended that they be performed at the very end of the clinical examination so the child remains confident and cooperative throughout the whole session.
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Motor blocks provide several types of information: They differentiate spasticity from fibrous contracture in flexor, adductor, and pronator muscle groups They allow assessment of individual muscles after removing spasticity of the antagonists or co-contractions of adjacent muscles Within a spastic muscle group they help determine which muscles are spastic and which are not Several pharmacologic agents have been used percutaneously: anesthetics (lidocaine), diluted alcohol [5], and phenol [6]. As the last is toxic to surrounding soft tissues and sensory nerve fascicles, it is better to approach the nerve surgically for phenol blocks [7]. Whichever substance is used, it may be injected either around the nerve trunk or in the motor endplates of the involved muscles. Nerve trunks are fairly easy to locate. Injection at this site diffuses to other motor nerves, however, with an overlapping response. The motor endplates of individual muscles are much more difficult to locate and clinicians previously relied on chart drawings [5]. This has been facilitated greatly with the use of nerve stimulators. For intrinsic muscles in the hand, it is practically impossible to find the motor endplates of each individual muscle. One usually chooses to inject the motor branch of the ulnar nerve and, if required, the thenar motor branch. The effect of a motor block with lidocaine starts 15 minutes after injection and usually lasts for half an hour. The duration of efficacy is longer for alcohol and much longer for phenol. Spasticity yields completely, whereas contracture persists [7]. One then can assess muscle contracture in each muscle or muscle group. The most frequent example in the upper limb is severe spasticity of the wrist flexors. Following paralysis, muscles can be tested for contracture and the finger flexors can be assessed. Injection of a spastic pronator teres informs of a possible contracture of the interosseous membrane. One also can test the nonspastic muscles then, now relieved of their spastic antagonists, for voluntary motor control. In specific cases (eg, extensor carpi radiales muscles) the muscles are present and do contract, but are ineffective because of progressive lengthening over time, secondary to a severe wrist flexion deformity. Within a spastic group, one can perform a selective block of the predominant spastic
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muscle to evaluate voluntary control and spasticity of the other muscles. For example, after injecting the FCU, one can test flexor carpi radialis (FCR). In the same way, after injecting the biceps brachii, one can test the brachialis and the brachioradialis. Recently, botulinum toxin has partially supplanted motor blocks. It produces the same effects but with a much easier technique of injection, a lack of side effects, and a long lasting effect [8]. Injection of botulinum A within the body of a muscle induces a paralysis that starts 10–15 days after the injection and lasts 3–6 months [9,10]. Another article in this volume is dedicated to botulinum toxin and describes its indications in cerebral palsy (See article by Chin and Graham). Whether used as a diagnostic tool for preoperative assessment or as part of the therapeutic plan, botulinum effectively replaces motor blocks. Joint range of motion Passive range of motion rarely is affected in isolated cerebral palsy in children. It is impaired more frequently in adults [11] and, when severe, associated neurologic lesions coexist. The precise range of motion of many joints may be difficult to assess, not so much because of spasticity, but because of muscle fibrous contractures. Each individual joint is tested with the involved muscles fully relaxed. This is performed as gently as possible so as not to trigger spasticity. If fibrous contracture is present, motor blocks do not assist in assessment of the passive range of joint motion. Sometimes it is so difficult that preoperative examination under anesthesia is necessary to define the range of motion precisely. Some joints of the fingers and thumb may have excessive passive motion, resulting in joint instability. This occurs mainly at the thumb MP joint (hyperextension and lateral instability) and at the finger MP and PIP joints (hyperextension). Finally, a general motor assessment of the upper limb is performed to evaluate global motor control. Some standard tests are helpful, such as the head-to-knee test, in which the patient is asked to place his hand on his head and then move it to the contralateral knee. The speed and precision of the movement are recorded. These nonspecific tests involve many of the elements susceptible to perturbation (hypertonia and muscle contracture, ataxia, apraxia, and extrapyramidal lesions) and give information of the potential use of the limb should a surgical treatment be decided on.
Primitive reflexes also are sought. They are caused by an abnormal sensory motor development and impair greatly the functional capacity of the limb. One classic example of these primitive reflexes is the asymmetric neck reflex: when the head is turned actively or passively to one side, it produces abduction of the shoulder and extension of the elbow, wrist, and fingers of the ipsilateral upper limb, whereas the contralateral limb flexes in all joints. Once the motor examination has been completed, an attempt at classification can be made. Zancolli’s classification [12] is probably the most popular one: Type I includes the spastic intrinsic-plus hands, in which spasticity of the interossei and lumbrical muscles causes flexion of the MP joints and extension of the IP joints, sometimes associated with a swan neck deformity. In this type a wrist flexion deformity is rare. Type II includes the spastic flexion-pronation hands with (hyper)flexion of the wrist and pronation of the forearm. Three groups are individualized depending on the degree of active finger extension. In the first group, with the wrist in neutral or near neutral, there is full active extension of the fingers. In the second group there is nearly complete active extension of the fingers, but only with some degree of wrist flexion. This group is subdivided further based on the presence (subgroup A) or absence (subgroup B) of active wrist extension. In the third group there is no active finger extension, even with maximum wrist flexion. Goldner [4] has produced another classification: In group I the wrist and MP joint can be extended at least to neutral. There is active grasp and release. The main deficiencies are delayed speed, slow coordination, and minimal dexterity. In group II there is weakness of wrist and finger extension with mild contracture of the wrist, finger, and thumb flexors. The thumb remains in the palm during hand extension. The hand is used only as an assist and a stabilizer. In group III the wrist and finger flexors are contracted severely. The primary goal of surgery is cosmetic improvement.
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In group IV the hands are spastic and athetoid. House [13] has classified thumb deformities in four types: Type I is an isolated adduction of the first metacarpal. Type II is adduction of the first metacarpal with flexion of the MP joint. Type III is adduction of the first metacarpal with MP joint hyperextension. Type IV is adduction of the first metacarpal with flexion of MP and IP joints. Aside from the thumb classification of House, which is purely descriptive, the author does not find any of the available classifications particularly helpful, as the clinical picture always varies from one child to another, depending on the amount and extent of spasticity and paralysis. There are no two identical cases and often they do not fit accurately in any of the described categories. Sensory examination Sensory examination requires, besides the child’s cooperation, a certain level of intellectual capacity and language ability. It is practically impossible in the small child; it becomes feasible at age 4–5 years, and more complicated tests such as two-point discrimination become reliable only at age 6–7 years. The basic sensory functions (light touch, pain, temperature) are essentially intact in cerebral palsy, whereas complex sensations (epicritic sensibility, proprioception, gnosis) are affected more severely. Light touch is explored with a smooth point or a finger, pain with a needle, and temperature with tubes of hot (40 C) and cold (melting ice) water. Epicritic sensibility is explored with discriminatory tests such as two-point discrimination. Proprioception is tested by vibration (tuning fork) and by the sense of position of the limb: the patient is blindfolded, has the unaffected limb placed in one position and is asked to reproduce the position with the affected limb. Proprioception is usually more disturbed in the distal part of the limb. Gnosis is most affected. Placing an object in the child’s hand and asking him or her to identify it tests stereognosis. Graphesthesia is tested by drawing figures or forms in the patient’s palm. Sensation is considered satisfactory when the child identifies at least three out of five objects,
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can recognize large figures drawn in the palm, and has a two-point discrimination test of no greater than 5-10 mm (according to the child’s age) [14]. Pain may be present but is difficult to evaluate, especially in children who may not report it and may not know how to describe it. It may be linked to severe contractures or to a deformed joint. Other causes of pain should be considered. For example, the author has treated a young adult with CP with wrist pain caused by Kienbo¨ck disease, perhaps related to the severe flexion deformity of his wrist [15]. Functional examination This part of the examination tests the actual use of the upper limb. It is done with standard reproducible tests using a pre-established scenario and simple objects. It should be video recorded. This usually proves to be a great assessment tool, as it can be visualized as many times as necessary without necessitating the child’s presence and cooperation. The same tests and video recording are repeated after surgery and serve as a comparison for evaluation of the results of surgery. Grasping tests Handing objects to the child tests prehension. Thumb-finger pinch often is limited to a lateral (key) pinch because of the lack of fine voluntary control and because of the adducted posture of the thumb. Grasp generally is preserved, although not always functional because of finger flexor contractures, extensor weakness, and wrist flexor co-contractions. Release often proves difficult because of weakness of active finger extension. The pick up and release test is done with several objects of different size and shape displayed on a table in front of the child, asking him or her to pick up each of them and displace it to a different spot. This test evaluates not only the prehensile capacity of the hand but also the contribution of the whole limb to that function. Quantitative measurements can be made if one introduces the time factor. Many different tests have been described for this assessment. In the ‘‘Box and Block Test,’’ the patient moves as many wooden blocks as possible from one compartment to another in 1 minute [16]. Enjalbert’s test [17], designed for stroke patients, involves grabbing a pen presented 40 cm away from the involved upper limb and handing it back to the examiner.
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Instrumented objects have been used more recently in an attempt to quantify hand grasp. Computerized systems of movement analysis now allow analysis of the different sequences of motor events and captors integrated in gloves can measure the forces generated by grasp [18,19]. Bimanual activities These give accurate information of the spastic upper limb’s actual functional ability. Several tests can be used, such as carrying a container with two handles, holding one object into which another one should be placed (eg, envelope and letter, nest of dolls) or holding a ruler while drawing a line with the other hand. Questionnaire The child and family are asked to describe precisely how the hand is used in activities of daily living, such as dressing, self-care, and eating. This can be done in the form of a questionnaire that must be adapted to the child’s age. The questionnaire can be completed during the session or given to the child and family to be completed at home (self-questionnaire). In many cases the upper extremity is neglected by the child despite functional capacity. In these cases it is possible, if not probable, that the child persists in ignoring the limb even if functional ability can be improved by surgery. There are several validated nonspecific questionnaires evaluating hand function (Michigan Hand Outcome Questionnaire [20]. To the best of the author’s knowledge, however, there is no validated questionnaire adapted to the cerebral palsied child. Tests Many tests have been designed to assess the functional value of the upper limb. Among the nonspecific tests, only those testing the simplest functions can be used in patients with CP (Frenchay Arm Test, with only five items [21], Jebsen test, with seven items [22]). There are few tests specifically designed for the spastic upper limb [4]. The ‘‘400 points’’ test [23], which analyzes mobility, grip strength, pick up and release, and bimanual function, can be used in children starting from 7 years of age. In children younger than 7 years, Rumeau et al [24] use a bimanual test adapted to each age group. The author’s group has chosen to use that described by Hoffer [14], which tests dressing, personal hygiene, feeding, bimanual activities, grasp and release, and lateral pinch.
General preoperative examination The aim of this general examination is to evaluate the real benefit the child will get from surgery, taking into account other neurologic impairments, the patient’s age, intelligence, motivation, and environment. Other neurologic impairments As these children are hemiplegic, the lower limb deficit also must be assessed. It is especially important to know about the child’s walking ability and the possible need for walking aids (eg, crutch, wheelchair). If operations are necessary for improvement of the lower limb, they usually are planned before upper extremity surgery. Associated extrapyramidal signs also should be detected. These include the following: Athetosis, which consists of unexpected, nonvoluntary movements causing a slow oscillation of the limbs. It is reduced at rest, abolished at sleep, and increased by noise, fatigue, and emotions. Chorea consists of brisk, rapid, and anarchic nonvoluntary movements of variable amplitude that can involve all territories. In the upper limb these contortions of the forearm, hand, and fingers make activities of daily living impossible. Parkinson syndrome is characterized by the classic triad: resting tremor, plastic hypertonia (predominant in the proximal muscles) with the cogwheel sign, and akinesia. If these extrapyramidal signs are predominant, they preclude surgical treatment, because the child is unable to use the hand because of these nonvoluntary movements. When they are mild, surgery may be performed when indicated. The capacity of the child to communicate must be evaluated, seeking visual, hearing, and language deficits. Behavioral problems, such as irritability, inability to concentrate, and emotional instability, also may constitute contraindications to surgical treatment if they predominate. Intelligence is evaluated through the intelligence quotient (IQ). It usually is stated that rehabilitation surgery is not indicated when the IQ is lower than 70, but this is not absolute. Surgical procedures, aimed at improving comfort, cosmesis, and personal hygiene, still are indicated [25]. Age Because the neurologic deficit in cerebral palsy is not progressive, early surgery can be planned.
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Sometimes it is necessary to operate very early because of an increasing deformity. In most cases, however, one can wait until the child is old enough that motor and sensory capacities can be evaluated accurately and he or she can cooperate with surgery and the postoperative rehabilitation. Sequelae of cerebral palsy in adults should be evaluated cautiously before surgical planning, because usually the patients have adapted already, functionally and socially, to the handicap, and surgery may be more disturbing than beneficial [26]. Motivation and environment Evaluation of motivation should take into account the patient’s ability to understand the modalities and benefits of the proposed treatment and to participate actively in the postoperative regimen. Understanding and motivation on the part of the parents are also mandatory. Environmental factors during the surgical period are also important, such as a rehabilitation center with an integrated school system and physiotherapists experienced in the management of children with CP. All are necessary components of the preoperative evaluation. According to Tonkin [27], ‘‘the ideal candidate is a cooperative 6-year-old child, with stable family support, who has a predominantly spastic upper limb deformity, with satisfactory hand sensibility, hemiplegic or monoplegic and without significant neurological deficits.’’
Electromyography EMG studies are often helpful. Static and dynamic studies are necessary, but they require cooperation on the child’s part [28]. This may be difficult to achieve in children younger than 5 years of age. In spastic muscles, EMG studies give information on the voluntary control, the possibility of relaxation, and possible co-contractions with another active movement. This is of the utmost importance when planning a tendon transfer, as a spastic muscle may be considered for potential transfer only if it displays satisfactory relaxation at rest and no co-contraction with other voluntary movements [28]. In other muscles, EMG studies are able to identify voluntary control that is not clinically detectable, for example, when spastic or retracted antagonists, joint deformity, or stiffness prevent functional contraction. EMG studies can help in
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determining the most appropriate muscles for transfer.
Radiography Radiographs are part of the preoperative evaluation. They are aimed at assessing any growth disturbance and joint deformity linked to the spasticity. Satisfactory assessment, however, may not be easy when there is a severe deformity such as wrist hyperflexion. Contralateral views in the same position may be helpful then. In such cases, standard radiographs may reveal growth disturbances of the distal radius and ulna and the carpus. They also may reveal avascularity of the lunate (Kienbo¨ck disease) [15]. In the elbow there may be a rare dislocation of the radial head [29,30].
Summary Clinical examination of the upper limb of a spastic patient is a lengthy and somewhat complicated procedure that is best performed together with all the practitioners involved in the child’s care. It should be repeated before any decision is made regarding treatment. Standard procedures in surgery of the upper limb in cerebral palsy are not appropriate, as the clinical picture is different for each individual patient. Surgery should be aimed at correcting spasticity, muscle/joint contracture, and motor deficit as required. All these elements therefore must have been assessed carefully before treatment. Finally, clinical examination detects all local and general contraindications to surgery and selects the appropriate candidates.
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