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The Physiatric Approach to Upper Limb Pain Syndromes
PHYSIATRIC PEARLS
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THE PHYSIATRIC APPROACH TO UPPER LIMB PAIN SYNDROMES George H. Kraft, MD, MS
UNIQUE ASPECTS OF PHYSIATRIC TRAINING
Two characteristics of training give the physiatrist unique capabilities for the assessment and management of upper limb pain syndromes.* Physiatric training is broader than just rehabilitation-the concept that embraces restoration of function. By nature of the disorders seen and managed, emphasis is placed on musculoskeletal and neuromuscular diseases. This merging of knowledge and experience in these two separate but closely related organ systems is unique to physiatric training. It is the experience of assessing symptomatology stemming from dysfunction of these two systems (especially ways in which one interacts with the other, producing symptoms mimicking the other) that gives the physiatrist unique diagnostic capabilities.' In the following sections, the author presents several cases that show how this integration of training in the two organ systems helps clarify diagnoses of upper limb pain syndromes. The second characteristic of physiatric training teaches a unique approach to medical care. The physiatrist is taught to manage the whole patient, a concept that has evolved from the rehabilitative portion of *Upper limb, not upper extremity or arm. The limb refers to a projecting appendage of an animal body, whereas an extremity is the most remote segment (i.e., the hand in the upper limb), and the arm is that portion between the shoulder and the wrist.
From the Department of Rehabilitation Medicine, University of Washington School of Medicine, Seattle, Washington
PHYSICAL MEDICINE AND REHABILITATION CLINICS OF NORTH AMERICA VOLUME 7 . NUMBER 3 AUGUST 1996
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training. In diagnostic dilemmas, this skill can be exceedingly valuable, allowing the physiatrist to see how diseases in one organ system can affect the functions of another. True secondary symptoms at times are produced in the other system. As a result, a disorder of one organ system may produce a constellation of symptoms, the origin of which might not be clear to a physician who has not received this broad training. Because more than one organ system may be affected, effective treatment must be directed to all the affected systems, not just the primarily affected one. This principle is applicable to the treatment phase of upper limb pain syndromes. An accurate identification of what is wrong can be followed by effective implementation of treatments directed to all the affected systems. The physiatric approach consequently results in a better treatment outcome than if treatment had been directed to only one organ system. EXAMPLES OF PHYSlATRlC DIAGNOSES
The following cases represent examples of these principles and are gathered from patients the author has seen during the past 12 months. They show the value of the physiatric approach to the diagnosis and management of patients with upper limb pain syndromes. Case 1
This patient was a 61-year-old woman, the wife of a physician who recently had retired. She and her husband were active physically and appeared younger than their stated years. After her husband's retirement, they had spent a month in the Galdpagos Islands where they had engaged in many physical activities such as hiking and swimming. During this trip, she began snorkeling, and she did this for many days. As a result of this activity, she spent a considerable period swimming and looking about with her neck extended. The patient shortly thereafter developed numbness and tingling in her hands associated with pain in her shoulders. On return to Seattle, the patient saw a skilled rheumatologist who obtained cervical films, saw some degenerative changes, and prescribed a soft cervical collar. She then was referred for electrodiagnostic evaluation, with presumptive diagnoses of cervical radiculopathy, brachial plexopathy, or carpal tunnel syndrome. At the initial visit, the author had the patient remove the collar to examine her. The author noted that her head movement was normal and full; she did not appear to be splinting her neck. Physical examination confirmed that neck range of motion was good, and no Spurling's sign was identified. Deep tendon reflexes of the biceps and triceps were symmetrical and within the expected range. No proximal upper limb motor or sensory abnormalities were noted; however, distally, she had
THE PHYSIATRIC APPROACH TO UPPER LIMB PAIN SYNDROMES
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bilateral hypoesthesia in the median distribution and positive Phalen's signs. An electromyogram (EMG) confirmed bilateral carpal tunnel syndrome with conduction block and denervation on the left and only conduction block on the right. The patient was referred to a surgeon for carpal tunnel release because she and her husband were planning to take an extended trip to Europe and wanted definitive treatment and because there was the presence of fibrillation potentials on the left. Recent correspondence from the physician-husband indicated that she was doing well. This case shows the close interaction in the upper limb between neuromuscular and musculoskeletal diseases. The extended period of neck extension had been a red herring. The degenerative changes in the cervical spine were not clinically significant, and the concept of brachial plexopathy (a term frequently used in medical parlance) was not a real diagnosis to consider in this case. A focus exclusively on the musculoskeletal system emphasized the importance of the cervical radiographic changes and perhaps fully did not comprehend brachial neuropathy. Case 2 This patient was a 46-year-old woman with a diagnosis of amyotrophic lateral sclerosis referred for rehabilitative services by an excellent and experienced neurologist. She was right-handed, and her weakness was primarily in the right upper limb. The reason for the referral was to increase her ability to function using this limb. Her vocational history showed that she was an apartment manager who used her right hand to run an adding machine. During a period of months, she had developed increased clumsiness and inability to function at work. She had almost no movement in her wrist and fingers. Before seeing the author, she had seen three different physicians on several occasions and had received two EMGs. The diagnosis of amyotrophic lateral sclerosis appeared to be well established because she had progressive weakness in her right upper limb, no sensory deficit, and upper motor neuron signs. On evaluation, the author noted that her right upper limb was swollen and joint range of motion was limited and painful. She had lost the fine skinfolds over her distal joints, and her hand showed vasomotor changes. Her right shoulder was painful, with limited range of motion. Her chief complaint to the author was that she could not use the right upper limb because of pain. The author believed that in addition to her motor neuron disease she also had reflex sympathetic dystrophy. Treatment consisting of antiedematogenic wrapping, elevation, contrast baths, and active assistive range of motion was initiated. On seeing the patient subsequently, the author found that she was markedly improved, had much less pain, and was grateful for the intervention. Even though her weakness had progressed, her pain was reduced and her function actually had improved.
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This case presents an example of the intense focus on the neurologic system by those trained only in diseases of the neuromuscular system. This case also illustrates how a disease of the neuromuscular system can affect the musculoskeletal system and shows the importance of treating secondarily affected systems, as well. The patient's real complaint was the reflex sympathetic dystrophy; her upper limb was painful and markedly inhibited her remaining neurologic function. This case also shows the importance of taking into account the whole person, as emphasized in rehabilitative training.
Case 3 This patient was a 60-year-old white woman referred by a wellrespected neurologist who clinically had diagnosed Charcot-Marie-Tooth (CMT) disease. The patient's chief complaint was weakness of both hands and feet, and the patient was referred for electrodiagnostic testing and rehabilitation strategies to improve the function of her hands. The patient was employed and found that it was difficult for her to perform her job well because of fatigue and weakness of her hands. The patient's family history was significant in that she had been told that she had CMT more than a decade before because of complaints of ankle weakness and a history of her mother dying at age 32 with some type of weakness (variously described as polio or CMT). Significant occupational history was that the patient also said that she had had exposure to solvents in the past. The physical examination had been reported previously as showing weakness of grip and distal foot musculature. Deep tendon reflexes had been normal, but sensation was described as decreased in the lateral calves. On seeing the patient, the author noted that she had painful, swollen joints in her hands and that muscle strength could not be tested fully because of pain caused by her arthritis. The swollen joints made the intrinsic musculature of the hand appear atrophic. Nerve conduction studies were normal, as was the needle EMG examination, eliminating any type of hereditary motor sensory neuropathy. Her primary problem was joint disease not nerve disease, and appropriate treatment was implemented. This case nicely shows the interplay between the musculoskeletal and neuromuscular systems. Swollen finger joints make the intrinsic muscles of the hands appear atrophic. Painful joints prevent maximal contraction of muscles against resistance. This patient had a pseudoneuropathy, and her case shows how those trained in focusing on diseases of the neuromuscular system may not note changes in the musculoskeletal system. For patient satisfaction to occur, an accurate diagnosis must be made and appropriate treatment directed to the affected system or systems.
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PEARLS: UPPER LIMB PAIN SYNDROMES The following is a list of 22 pearls that will help the physician in assessment and treatment of patients with upper limb pain syndromes. This is not an exhaustive list. Many commonly used clinical tests have not been included (e.g., Spurling's sign) because they are well known and do not need to be repeated here. These clinical observations, however, have served the author well, and they are recommended to the reader. 1. In evaluating a patient, "think outside the box." As the three cases showed, it can be easy to direct one's attention to the neuromuscular system or the musculoskeletal system and exclude the other, thereby missing the significant contribution of the other system to the patient's complaints (see cases 2 and 3). The physiatrist is well trained in that he or she has experiences in disorders of the neuromuscular and musculoskeletal systems and can detect disorders of the complementary system that may account for pseudodisease in the other system (see case 3). 2. Treat all afected systems (not just the primary disease). A primary disease in one organ system may have secondary effects on other organ systems. For maximal patient improvement to occur, treat all the appropriate systems (e.g., cervical spondylosis triad; see pearl 6). 3. There are only three explanations for treatment failure. If there is no improvement in a patient's symptoms, only three possibilities exist. (1)The diagnosis is wrong; therefore, the wrong thing is being treated. (2) The treatment is wrong. In this option, the diagnosis is correct, but the wrong treatment has been chosen. (3) No adequate treatment exists. No other explanations exist for the failure of treatment. 4. Do not forget to observe the patient. Case 1 shows the importance of this simple and time-saving strategy. Look at the way the patient moves (or often does not move) the neck, shoulders, hands, and other possibly affected portions of the body. Do not forget the powers of observation. 5. Do not forget the history. Much history can be obtained by simply talking with patients while they are coming into the examining room, preparing for the examination, and being examined. For example, one of the tip-offs to the cervical spondylosis triad is the history of waking with neck pain. As another example, the author once saw a cervical cord-injured patient who had increased numbness in both little fingers. The patient had had extensive workup for a syrnix, and none was found. On seeing the patient, the author inquired about the onset of these symptoms and the history surrounding that event. The patient said that it started shortly after he bought a new television that he placed on the floor and that he watched television for many hours a day lying prone with his head propped on his hands while resting on his elbows. He had bilateral ulnar neuropathy at the elbows, a finding confirmed by electrodiagnosis. A careful history before the expensive interventions the patient under-
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went could have saved the health care system and the patient quite a few dollars. 6. Be aware of the cervical spondylosis triad. This is a disorder that in the author's opinion is common and accounts for many of the complaints of neck and upper limb pain that many patients express. It is the prototype of the interaction of different systems requiring multisystem treatment and without which pain relief is only partial. The three parts of the triad are degenerative spondylosis of the cervical spine, cervical radiculopathy, and cervical paraspinal muscle splinting. Based on the most pronounced symptomatology of the patient or the focus of the examining physician, one component of this triad often gets identified and treated. The result is that treatment is incomplete and patient improvement only partial. This syndrome needs to be viewed in context of its development and especially the interaction between the musculoskeletal and neuromuscular systems. During the years, with the development of degenerative loss of cervical disc height and secondary osteophytic proliferation, certain physical activities (commonly, sleeping position) will cause mild irritation of the cervical nerve roots, and the body will attempt to stabilize the cervical spine (muscle splinting). As a result, patients may have arthritic, radicular, and myalgic symptoms. Adequate treatment must be directed to identification and elimination of the precipitating causes (e.g., attention to nocturnal neck positioning), attention to the cervical radiculopathy with positioning and traction, and attention to what may evolve as a fibromyalgic paraspinal and upper shoulder muscle pain syndrome, as well as the underlying joint pathology, using physiatric treatment modalities as well as anti-inflammatory medication^.^ Ergonomic assessment and treatments also must be implqmented for many patients. 7. Myalgia frequently is seen in acute cervical radiculopathy. Patients whose radiculopathy is acute frequently have painful muscles on palpation. Chronic radiculopathies are associated much less frequently with muscle pain. 8. Pads over vulnerable sites of peripheral nerves can be helpful. On seeing patients who have symptoms of ulnar neuropathy at the elbow or peroneal neuropathy at the fibular head, conservative management frequently may not be given a reasonable trial, and patients may be referred for surgical intervention. There are few sites of true intrinsic nerve entrapment in the human. The classic is the median nerve under the volar carpal ligament. Most other peripheral nerve damage is associated with an external force causing pressure to a vulnerable peripheral nerve running over an unyielding bony structure. Ulnar neuropathies at the elbow most commonly are of this type. Another is the peroneal neuropathy occurring under the fibular head. Joint motion commonly can aggravate an already edematous and damaged peripheral nerve at the elbow, but this is rarely the initiating cause. In such cases, the patient should be queried by history (see pearl 5) as to whether they have any positional or occupational situations in which
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THE PHYSIATRIC APPROACH TO UPPER LIMB PAIN SYNDROMES
the nerve may be compromised externally. Even if patients do not relate such a history, an appropriate trial of some type of external protection is warranted (such as a foam pad over the vulnerable site of the ulnar nerve at the e l b ~ w ) . ~
9. Medial epicondylitis and ulnar entrapment at the elbow may coexist and potentiate each other. This is another example of the interrelationship behveen musculoskeletal and neuromuscula~pathology. ~nflammation of the medial epicondyle of the humerus may potentiate ulnar nerve injury at that site. Protection against external compression and trauma (see pearl 8) may help both condition^.^
10. It takes ~pproximatelytwice as long to heal a condition as to develop it. Under the best of circumstances and optimal treatment, this is a good
rule. This time frame also assumes that treatment is started vromvtlv and not after months of false diamoses and inadeauate treatments. As an example, elbow pads (see peazs 8 and 9) shoul2 be worn for about twice as long as the time between onset of symptoms and application of the pads. This means thev should be continued for some time, even after symptoms significantly abate. 11. Peripheral nerve diseases can be mimicked as a result of joint diseases (see case 3). Patients who have painful, swollen joints of the hands and feet appear to have atrophy of intrinsic muscles and will have decreased strength on manual examination of those muscles. In severe cases, true atrophy may be present, representing disuse atrophy secondary to joint disease. 12. Pain does not cause EMG abnormalities. This appears to be a difficult concept for referring physicians and patients to grasp. Patients frequently are referred for electrodiagnostic studies because of complaints of pain. Many times, they are not assessed carefully by a physical examination focusing on sensory deficit, reduction of deep tendon reflexes, or true muscle weakness and atrophy. EMG abnormalities relate to presence of muscle weakness or atrophy, and nerve conduction changes are associated with sensory deficits, whereas pain alone may have many causes. 13. A needle EMG examination can be done when a patient is not f i l l y relaxed. The author has seen physicians do ponderous EMG examinations because patients could not fully relax. Actually, a slight level of muscle tension can be useful in assessing insertional activity, and facilitation of positive sharp waves actually may be enhanced. In the less than fully relaxed state, the firing rate, phase, and size of motor unit potentials also can be determined. A ereat deal of information about the normalitv or abnormality of the motoy unit can be obtained in a short period whe; a muscle is not fully relaxed. 14. When doing an EMG, do not just stop with positive findings. It is the delineation of the boundaries between abnormal and normal areas that often helps identify a condition. The author has seen a patient with upper limb pain labeled as having a cervical radiculopathy when only a few symptomatic muscles were tested. A more complete EMG showed diffuse positive sharp wave activity throughout (even in asymptomatic limbs) in a patient who had musculoskeletal neck pain4 I
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15. Finding decreased nerve conduction velocity of the ulnar nerve at the elbow does not require surgery. Assuming that the measurement was accurate (an assumption that may be too great because many electromyographers still have a difficult time measuring the ulnar nerve at the elbow) and that the nerve conduction across the elbow trulv is reduced and associated with a temporally dispersed compound &scle action potential, the only interpretation that should be made is that of the chronicity of the neuropathy. It tells what has happened in the past, not what is occurring today. The slowing and temporal dispersion seen in peripheral neuropathies is associated with areas of nerve that have been demyelinated and remyelinated repeatedly, resulting in increased loss of current to a differential degree in various fibers in that segment of peripheral nerve. This is a condition that may take months or years to develop and relates to past history and not current problems (see pearl 8). Protection against further damage is warranted, and surgical transposition may be unwarranted as well as potentially risky.5 16. The best indicator of acute axon loss is the presence of large amplitude fibrillation potentials. Immediately after a loss of axonal integrity, the fibrillating muscle fibers are normal in size and, as generators of spontaneous action potentials, generate fibrillation potentials of approximately 800 to 1000 kV. As time passes, atrophy occurs, the generators become progressively smaller, and the amplitude decay^.^ 17. Acute carpal tunnel syndrome may not produce prolonged median latency. A true but acute carpal tunnel syndrome may produce a considerable amount of symptoms but little conduction slowing. It takes time for latencies to become prolonged (see pearl 15). 18. In carpal tunnel syndrome, a diminution of symptoms does not mean the problem is abating. As nerve damage progresses, pain diminishes. Patients may believe they are improving because their pain is improving, whereas their carpal tunnel syndrome actually may be progressing. All patients with carpal tunnel syndrome should be monitored periodically by a physician, preferably with electrodiagnostic studies, if they are not corrected surgically. 19. Traumatic median neuro~athuat the wrist is not caroal tunnel sundrome. The author has seen this neuropathy in active paraplegic patients using manual wheelchairs. They can traumatize their median nerves at the wrist on the wheelchair rim, giving symptoms identical to those of carpal tunnel syndrome. Electrodiagnostic studies, however, generally show much more axon loss than conduction slowing. The author has seen them receive surgical decompression without relief. The only longterm treatment is to reduce the trauma to the median nerve when using: the wheelchair by means of padding (see pearl 8) or other ergonom; strategies. 20. When treating carpal tunnel syndrome z ~ ~ i at hsplint, the splint should be in a neutral position. The standard off-the-shelf splint (Colles cuff or splint) obtainable in a medical supply facility traditionally is in a "position of function" that is cocked approximately 20". This is not necessarily I
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Table 1. VALUE OF NEEDLE ELECTROMYOGRAPHY 1. Normal 2. Abnormal Distribution Generalized-proximal, distal Localized-peripheral nerve, nerve root, plexus Severity Time of onset Neuropathy or myopathy From Kraft GH: An approach to electrodiagnostic medicine: The power of needle electromyography. Physical Medicine and Rehabilitation Clinics of North America 5:496, 1994.
the position of function and also is not the position that gives the greatest space for the median nerve at the wrist.5, 21. Neutral splinting can improve nervefunction as well as symptoms in carpal tunnel syndrome. Empirically, splinting can be helpful (in those cases in which it is not, it often is because the splint is in a cocked up position, see pearl 20). Kruger et a18have shown improvement in median nerve conduction studies by use of splints only. 22. Do not forget the power of the needle EMG. Nerve conduction studies may be complementary to needle EMG evaluations, but the most information obtained from an electrodiagnostic examination is from the needle studies. From a needle examination, a completely normal motor unit can be differentiated from an abnormal one. (Even in peripheral neuropathies, action potential firing rates and configuration of motor unit action potentials are affected.) In most cases, needle EMG studies can differentiate nerve from muscle diseases. Areas of abnormality can be delineated to root, plexus, or peripheral nerve. The severity of the abnormality can be determined (Table 1).The needle EMG is a powerful instrument to evaluate motor unit pathology.' SUMMARY
Pearls by definition are small bits of information that can enhance the physician's diagnostic acumen and increase the likelihood of beneficial therapeutic intervention. They typically may not be based on scientific study, but rather stem from practical clinical experience. The approach to upper limb pain syndromes presented in the first part of this article and the 22 pearls listed in the last part have been useful to the author. It is hoped that they may be useful to the reader. References 1. Brandstater ME: Physical medicine a n d rehabilitation. JAMA 273:171O-l712, 1995 2. K r a f t GH: An approach t o electrodiagnostic medicine: The p o w e r o f needle electromy-
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3. 4.
5. 6. 7. 8.
KRAF'I
ography. Physical Medicine and Rehabilitation Clinics of North America 5:495-508, 1994 Kraft GH: Fibrillation potential amplitude and muscle atrophy following peripheral nerve injury. Muscle Nerve 13:814-821, 1990 Kraft GH: Fibrillation potentials and sharp waves: Are they the same? Electroencephalogr Clin Neurophysiol 81:163-166, 1991 Kraft GH: Nonsurgical treatment of entrapment neuropathies. AAEM Plenary Session 1:39-55, 1992 Kraft GH, Detels P: Position of function of the wrist. Arch Phys Med Rehabil 53:272275, 1972 Kraft GH, Peck CL: Electromyographic biofeedback in neck and back pain [abstract]. Arch Phys Med Rehabil58:520, 1977 Kruger VL, Kraft GH, Deitz JC, et al: Carpal tunnel syndrome: Objective measures and splint use. Arch Phys Med Rehabil 72:517-520, 1991
Address reprint requests to George H . Kraft, MD, MS Department of Rehabilitation Medicine (Box 356490) University of Washington 1959 NE Pacific Street Seattle, WA 98195
1047-9651196 $0.00
+ .2O
THE PHYSIATRIC APPROACH TO UPPER LIMB PAIN SYNDROMES George H. Kraft, MD, MS
UNIQUE ASPECTS OF PHYSIATRIC TRAINING
Two characteristics of training give the physiatrist unique capabilities for the assessment and management of upper limb pain syndromes.* Physiatric training is broader than just rehabilitation-the concept that embraces restoration of function. By nature of the disorders seen and managed, emphasis is placed on musculoskeletal and neuromuscular diseases. This merging of knowledge and experience in these two separate but closely related organ systems is unique to physiatric training. It is the experience of assessing symptomatology stemming from dysfunction of these two systems (especially ways in which one interacts with the other, producing symptoms mimicking the other) that gives the physiatrist unique diagnostic capabilities.' In the following sections, the author presents several cases that show how this integration of training in the two organ systems helps clarify diagnoses of upper limb pain syndromes. The second characteristic of physiatric training teaches a unique approach to medical care. The physiatrist is taught to manage the whole patient, a concept that has evolved from the rehabilitative portion of *Upper limb, not upper extremity or arm. The limb refers to a projecting appendage of an animal body, whereas an extremity is the most remote segment (i.e., the hand in the upper limb), and the arm is that portion between the shoulder and the wrist.
From the Department of Rehabilitation Medicine, University of Washington School of Medicine, Seattle, Washington
PHYSICAL MEDICINE AND REHABILITATION CLINICS OF NORTH AMERICA VOLUME 7 . NUMBER 3 AUGUST 1996
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KRAFT
training. In diagnostic dilemmas, this skill can be exceedingly valuable, allowing the physiatrist to see how diseases in one organ system can affect the functions of another. True secondary symptoms at times are produced in the other system. As a result, a disorder of one organ system may produce a constellation of symptoms, the origin of which might not be clear to a physician who has not received this broad training. Because more than one organ system may be affected, effective treatment must be directed to all the affected systems, not just the primarily affected one. This principle is applicable to the treatment phase of upper limb pain syndromes. An accurate identification of what is wrong can be followed by effective implementation of treatments directed to all the affected systems. The physiatric approach consequently results in a better treatment outcome than if treatment had been directed to only one organ system. EXAMPLES OF PHYSlATRlC DIAGNOSES
The following cases represent examples of these principles and are gathered from patients the author has seen during the past 12 months. They show the value of the physiatric approach to the diagnosis and management of patients with upper limb pain syndromes. Case 1
This patient was a 61-year-old woman, the wife of a physician who recently had retired. She and her husband were active physically and appeared younger than their stated years. After her husband's retirement, they had spent a month in the Galdpagos Islands where they had engaged in many physical activities such as hiking and swimming. During this trip, she began snorkeling, and she did this for many days. As a result of this activity, she spent a considerable period swimming and looking about with her neck extended. The patient shortly thereafter developed numbness and tingling in her hands associated with pain in her shoulders. On return to Seattle, the patient saw a skilled rheumatologist who obtained cervical films, saw some degenerative changes, and prescribed a soft cervical collar. She then was referred for electrodiagnostic evaluation, with presumptive diagnoses of cervical radiculopathy, brachial plexopathy, or carpal tunnel syndrome. At the initial visit, the author had the patient remove the collar to examine her. The author noted that her head movement was normal and full; she did not appear to be splinting her neck. Physical examination confirmed that neck range of motion was good, and no Spurling's sign was identified. Deep tendon reflexes of the biceps and triceps were symmetrical and within the expected range. No proximal upper limb motor or sensory abnormalities were noted; however, distally, she had
THE PHYSIATRIC APPROACH TO UPPER LIMB PAIN SYNDROMES
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bilateral hypoesthesia in the median distribution and positive Phalen's signs. An electromyogram (EMG) confirmed bilateral carpal tunnel syndrome with conduction block and denervation on the left and only conduction block on the right. The patient was referred to a surgeon for carpal tunnel release because she and her husband were planning to take an extended trip to Europe and wanted definitive treatment and because there was the presence of fibrillation potentials on the left. Recent correspondence from the physician-husband indicated that she was doing well. This case shows the close interaction in the upper limb between neuromuscular and musculoskeletal diseases. The extended period of neck extension had been a red herring. The degenerative changes in the cervical spine were not clinically significant, and the concept of brachial plexopathy (a term frequently used in medical parlance) was not a real diagnosis to consider in this case. A focus exclusively on the musculoskeletal system emphasized the importance of the cervical radiographic changes and perhaps fully did not comprehend brachial neuropathy. Case 2 This patient was a 46-year-old woman with a diagnosis of amyotrophic lateral sclerosis referred for rehabilitative services by an excellent and experienced neurologist. She was right-handed, and her weakness was primarily in the right upper limb. The reason for the referral was to increase her ability to function using this limb. Her vocational history showed that she was an apartment manager who used her right hand to run an adding machine. During a period of months, she had developed increased clumsiness and inability to function at work. She had almost no movement in her wrist and fingers. Before seeing the author, she had seen three different physicians on several occasions and had received two EMGs. The diagnosis of amyotrophic lateral sclerosis appeared to be well established because she had progressive weakness in her right upper limb, no sensory deficit, and upper motor neuron signs. On evaluation, the author noted that her right upper limb was swollen and joint range of motion was limited and painful. She had lost the fine skinfolds over her distal joints, and her hand showed vasomotor changes. Her right shoulder was painful, with limited range of motion. Her chief complaint to the author was that she could not use the right upper limb because of pain. The author believed that in addition to her motor neuron disease she also had reflex sympathetic dystrophy. Treatment consisting of antiedematogenic wrapping, elevation, contrast baths, and active assistive range of motion was initiated. On seeing the patient subsequently, the author found that she was markedly improved, had much less pain, and was grateful for the intervention. Even though her weakness had progressed, her pain was reduced and her function actually had improved.
466
KRAFT
This case presents an example of the intense focus on the neurologic system by those trained only in diseases of the neuromuscular system. This case also illustrates how a disease of the neuromuscular system can affect the musculoskeletal system and shows the importance of treating secondarily affected systems, as well. The patient's real complaint was the reflex sympathetic dystrophy; her upper limb was painful and markedly inhibited her remaining neurologic function. This case also shows the importance of taking into account the whole person, as emphasized in rehabilitative training.
Case 3 This patient was a 60-year-old white woman referred by a wellrespected neurologist who clinically had diagnosed Charcot-Marie-Tooth (CMT) disease. The patient's chief complaint was weakness of both hands and feet, and the patient was referred for electrodiagnostic testing and rehabilitation strategies to improve the function of her hands. The patient was employed and found that it was difficult for her to perform her job well because of fatigue and weakness of her hands. The patient's family history was significant in that she had been told that she had CMT more than a decade before because of complaints of ankle weakness and a history of her mother dying at age 32 with some type of weakness (variously described as polio or CMT). Significant occupational history was that the patient also said that she had had exposure to solvents in the past. The physical examination had been reported previously as showing weakness of grip and distal foot musculature. Deep tendon reflexes had been normal, but sensation was described as decreased in the lateral calves. On seeing the patient, the author noted that she had painful, swollen joints in her hands and that muscle strength could not be tested fully because of pain caused by her arthritis. The swollen joints made the intrinsic musculature of the hand appear atrophic. Nerve conduction studies were normal, as was the needle EMG examination, eliminating any type of hereditary motor sensory neuropathy. Her primary problem was joint disease not nerve disease, and appropriate treatment was implemented. This case nicely shows the interplay between the musculoskeletal and neuromuscular systems. Swollen finger joints make the intrinsic muscles of the hands appear atrophic. Painful joints prevent maximal contraction of muscles against resistance. This patient had a pseudoneuropathy, and her case shows how those trained in focusing on diseases of the neuromuscular system may not note changes in the musculoskeletal system. For patient satisfaction to occur, an accurate diagnosis must be made and appropriate treatment directed to the affected system or systems.
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PEARLS: UPPER LIMB PAIN SYNDROMES The following is a list of 22 pearls that will help the physician in assessment and treatment of patients with upper limb pain syndromes. This is not an exhaustive list. Many commonly used clinical tests have not been included (e.g., Spurling's sign) because they are well known and do not need to be repeated here. These clinical observations, however, have served the author well, and they are recommended to the reader. 1. In evaluating a patient, "think outside the box." As the three cases showed, it can be easy to direct one's attention to the neuromuscular system or the musculoskeletal system and exclude the other, thereby missing the significant contribution of the other system to the patient's complaints (see cases 2 and 3). The physiatrist is well trained in that he or she has experiences in disorders of the neuromuscular and musculoskeletal systems and can detect disorders of the complementary system that may account for pseudodisease in the other system (see case 3). 2. Treat all afected systems (not just the primary disease). A primary disease in one organ system may have secondary effects on other organ systems. For maximal patient improvement to occur, treat all the appropriate systems (e.g., cervical spondylosis triad; see pearl 6). 3. There are only three explanations for treatment failure. If there is no improvement in a patient's symptoms, only three possibilities exist. (1)The diagnosis is wrong; therefore, the wrong thing is being treated. (2) The treatment is wrong. In this option, the diagnosis is correct, but the wrong treatment has been chosen. (3) No adequate treatment exists. No other explanations exist for the failure of treatment. 4. Do not forget to observe the patient. Case 1 shows the importance of this simple and time-saving strategy. Look at the way the patient moves (or often does not move) the neck, shoulders, hands, and other possibly affected portions of the body. Do not forget the powers of observation. 5. Do not forget the history. Much history can be obtained by simply talking with patients while they are coming into the examining room, preparing for the examination, and being examined. For example, one of the tip-offs to the cervical spondylosis triad is the history of waking with neck pain. As another example, the author once saw a cervical cord-injured patient who had increased numbness in both little fingers. The patient had had extensive workup for a syrnix, and none was found. On seeing the patient, the author inquired about the onset of these symptoms and the history surrounding that event. The patient said that it started shortly after he bought a new television that he placed on the floor and that he watched television for many hours a day lying prone with his head propped on his hands while resting on his elbows. He had bilateral ulnar neuropathy at the elbows, a finding confirmed by electrodiagnosis. A careful history before the expensive interventions the patient under-
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went could have saved the health care system and the patient quite a few dollars. 6. Be aware of the cervical spondylosis triad. This is a disorder that in the author's opinion is common and accounts for many of the complaints of neck and upper limb pain that many patients express. It is the prototype of the interaction of different systems requiring multisystem treatment and without which pain relief is only partial. The three parts of the triad are degenerative spondylosis of the cervical spine, cervical radiculopathy, and cervical paraspinal muscle splinting. Based on the most pronounced symptomatology of the patient or the focus of the examining physician, one component of this triad often gets identified and treated. The result is that treatment is incomplete and patient improvement only partial. This syndrome needs to be viewed in context of its development and especially the interaction between the musculoskeletal and neuromuscular systems. During the years, with the development of degenerative loss of cervical disc height and secondary osteophytic proliferation, certain physical activities (commonly, sleeping position) will cause mild irritation of the cervical nerve roots, and the body will attempt to stabilize the cervical spine (muscle splinting). As a result, patients may have arthritic, radicular, and myalgic symptoms. Adequate treatment must be directed to identification and elimination of the precipitating causes (e.g., attention to nocturnal neck positioning), attention to the cervical radiculopathy with positioning and traction, and attention to what may evolve as a fibromyalgic paraspinal and upper shoulder muscle pain syndrome, as well as the underlying joint pathology, using physiatric treatment modalities as well as anti-inflammatory medication^.^ Ergonomic assessment and treatments also must be implqmented for many patients. 7. Myalgia frequently is seen in acute cervical radiculopathy. Patients whose radiculopathy is acute frequently have painful muscles on palpation. Chronic radiculopathies are associated much less frequently with muscle pain. 8. Pads over vulnerable sites of peripheral nerves can be helpful. On seeing patients who have symptoms of ulnar neuropathy at the elbow or peroneal neuropathy at the fibular head, conservative management frequently may not be given a reasonable trial, and patients may be referred for surgical intervention. There are few sites of true intrinsic nerve entrapment in the human. The classic is the median nerve under the volar carpal ligament. Most other peripheral nerve damage is associated with an external force causing pressure to a vulnerable peripheral nerve running over an unyielding bony structure. Ulnar neuropathies at the elbow most commonly are of this type. Another is the peroneal neuropathy occurring under the fibular head. Joint motion commonly can aggravate an already edematous and damaged peripheral nerve at the elbow, but this is rarely the initiating cause. In such cases, the patient should be queried by history (see pearl 5) as to whether they have any positional or occupational situations in which
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the nerve may be compromised externally. Even if patients do not relate such a history, an appropriate trial of some type of external protection is warranted (such as a foam pad over the vulnerable site of the ulnar nerve at the e l b ~ w ) . ~
9. Medial epicondylitis and ulnar entrapment at the elbow may coexist and potentiate each other. This is another example of the interrelationship behveen musculoskeletal and neuromuscula~pathology. ~nflammation of the medial epicondyle of the humerus may potentiate ulnar nerve injury at that site. Protection against external compression and trauma (see pearl 8) may help both condition^.^
10. It takes ~pproximatelytwice as long to heal a condition as to develop it. Under the best of circumstances and optimal treatment, this is a good
rule. This time frame also assumes that treatment is started vromvtlv and not after months of false diamoses and inadeauate treatments. As an example, elbow pads (see peazs 8 and 9) shoul2 be worn for about twice as long as the time between onset of symptoms and application of the pads. This means thev should be continued for some time, even after symptoms significantly abate. 11. Peripheral nerve diseases can be mimicked as a result of joint diseases (see case 3). Patients who have painful, swollen joints of the hands and feet appear to have atrophy of intrinsic muscles and will have decreased strength on manual examination of those muscles. In severe cases, true atrophy may be present, representing disuse atrophy secondary to joint disease. 12. Pain does not cause EMG abnormalities. This appears to be a difficult concept for referring physicians and patients to grasp. Patients frequently are referred for electrodiagnostic studies because of complaints of pain. Many times, they are not assessed carefully by a physical examination focusing on sensory deficit, reduction of deep tendon reflexes, or true muscle weakness and atrophy. EMG abnormalities relate to presence of muscle weakness or atrophy, and nerve conduction changes are associated with sensory deficits, whereas pain alone may have many causes. 13. A needle EMG examination can be done when a patient is not f i l l y relaxed. The author has seen physicians do ponderous EMG examinations because patients could not fully relax. Actually, a slight level of muscle tension can be useful in assessing insertional activity, and facilitation of positive sharp waves actually may be enhanced. In the less than fully relaxed state, the firing rate, phase, and size of motor unit potentials also can be determined. A ereat deal of information about the normalitv or abnormality of the motoy unit can be obtained in a short period whe; a muscle is not fully relaxed. 14. When doing an EMG, do not just stop with positive findings. It is the delineation of the boundaries between abnormal and normal areas that often helps identify a condition. The author has seen a patient with upper limb pain labeled as having a cervical radiculopathy when only a few symptomatic muscles were tested. A more complete EMG showed diffuse positive sharp wave activity throughout (even in asymptomatic limbs) in a patient who had musculoskeletal neck pain4 I
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15. Finding decreased nerve conduction velocity of the ulnar nerve at the elbow does not require surgery. Assuming that the measurement was accurate (an assumption that may be too great because many electromyographers still have a difficult time measuring the ulnar nerve at the elbow) and that the nerve conduction across the elbow trulv is reduced and associated with a temporally dispersed compound &scle action potential, the only interpretation that should be made is that of the chronicity of the neuropathy. It tells what has happened in the past, not what is occurring today. The slowing and temporal dispersion seen in peripheral neuropathies is associated with areas of nerve that have been demyelinated and remyelinated repeatedly, resulting in increased loss of current to a differential degree in various fibers in that segment of peripheral nerve. This is a condition that may take months or years to develop and relates to past history and not current problems (see pearl 8). Protection against further damage is warranted, and surgical transposition may be unwarranted as well as potentially risky.5 16. The best indicator of acute axon loss is the presence of large amplitude fibrillation potentials. Immediately after a loss of axonal integrity, the fibrillating muscle fibers are normal in size and, as generators of spontaneous action potentials, generate fibrillation potentials of approximately 800 to 1000 kV. As time passes, atrophy occurs, the generators become progressively smaller, and the amplitude decay^.^ 17. Acute carpal tunnel syndrome may not produce prolonged median latency. A true but acute carpal tunnel syndrome may produce a considerable amount of symptoms but little conduction slowing. It takes time for latencies to become prolonged (see pearl 15). 18. In carpal tunnel syndrome, a diminution of symptoms does not mean the problem is abating. As nerve damage progresses, pain diminishes. Patients may believe they are improving because their pain is improving, whereas their carpal tunnel syndrome actually may be progressing. All patients with carpal tunnel syndrome should be monitored periodically by a physician, preferably with electrodiagnostic studies, if they are not corrected surgically. 19. Traumatic median neuro~athuat the wrist is not caroal tunnel sundrome. The author has seen this neuropathy in active paraplegic patients using manual wheelchairs. They can traumatize their median nerves at the wrist on the wheelchair rim, giving symptoms identical to those of carpal tunnel syndrome. Electrodiagnostic studies, however, generally show much more axon loss than conduction slowing. The author has seen them receive surgical decompression without relief. The only longterm treatment is to reduce the trauma to the median nerve when using: the wheelchair by means of padding (see pearl 8) or other ergonom; strategies. 20. When treating carpal tunnel syndrome z ~ ~ i at hsplint, the splint should be in a neutral position. The standard off-the-shelf splint (Colles cuff or splint) obtainable in a medical supply facility traditionally is in a "position of function" that is cocked approximately 20". This is not necessarily I
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Table 1. VALUE OF NEEDLE ELECTROMYOGRAPHY 1. Normal 2. Abnormal Distribution Generalized-proximal, distal Localized-peripheral nerve, nerve root, plexus Severity Time of onset Neuropathy or myopathy From Kraft GH: An approach to electrodiagnostic medicine: The power of needle electromyography. Physical Medicine and Rehabilitation Clinics of North America 5:496, 1994.
the position of function and also is not the position that gives the greatest space for the median nerve at the wrist.5, 21. Neutral splinting can improve nervefunction as well as symptoms in carpal tunnel syndrome. Empirically, splinting can be helpful (in those cases in which it is not, it often is because the splint is in a cocked up position, see pearl 20). Kruger et a18have shown improvement in median nerve conduction studies by use of splints only. 22. Do not forget the power of the needle EMG. Nerve conduction studies may be complementary to needle EMG evaluations, but the most information obtained from an electrodiagnostic examination is from the needle studies. From a needle examination, a completely normal motor unit can be differentiated from an abnormal one. (Even in peripheral neuropathies, action potential firing rates and configuration of motor unit action potentials are affected.) In most cases, needle EMG studies can differentiate nerve from muscle diseases. Areas of abnormality can be delineated to root, plexus, or peripheral nerve. The severity of the abnormality can be determined (Table 1).The needle EMG is a powerful instrument to evaluate motor unit pathology.' SUMMARY
Pearls by definition are small bits of information that can enhance the physician's diagnostic acumen and increase the likelihood of beneficial therapeutic intervention. They typically may not be based on scientific study, but rather stem from practical clinical experience. The approach to upper limb pain syndromes presented in the first part of this article and the 22 pearls listed in the last part have been useful to the author. It is hoped that they may be useful to the reader. References 1. Brandstater ME: Physical medicine a n d rehabilitation. JAMA 273:171O-l712, 1995 2. K r a f t GH: An approach t o electrodiagnostic medicine: The p o w e r o f needle electromy-
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5. 6. 7. 8.
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ography. Physical Medicine and Rehabilitation Clinics of North America 5:495-508, 1994 Kraft GH: Fibrillation potential amplitude and muscle atrophy following peripheral nerve injury. Muscle Nerve 13:814-821, 1990 Kraft GH: Fibrillation potentials and sharp waves: Are they the same? Electroencephalogr Clin Neurophysiol 81:163-166, 1991 Kraft GH: Nonsurgical treatment of entrapment neuropathies. AAEM Plenary Session 1:39-55, 1992 Kraft GH, Detels P: Position of function of the wrist. Arch Phys Med Rehabil 53:272275, 1972 Kraft GH, Peck CL: Electromyographic biofeedback in neck and back pain [abstract]. Arch Phys Med Rehabil58:520, 1977 Kruger VL, Kraft GH, Deitz JC, et al: Carpal tunnel syndrome: Objective measures and splint use. Arch Phys Med Rehabil 72:517-520, 1991
Address reprint requests to George H . Kraft, MD, MS Department of Rehabilitation Medicine (Box 356490) University of Washington 1959 NE Pacific Street Seattle, WA 98195