Rehabilitation in Transverse Myelitis - Kennedy Krieger Institute

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These limitations present an opportunity for rehabilitation management to preventsecondarycomplicationsandfurther improve function in the long term. In our clinical practice we have identified a subgroup of children who presented with CSF and multilevel MRI changes consistent with ATM. These children subsequently recovered most of the function that was acutely lost, except for one extremity, either upper or lower, that was left with persistent flaccid monoplegia (see Case 6-1). On physical examination, the affected limb has generally demonstrated intact sensation but severely decreased motor function, rapid and severe muscle atrophy, areflexia, and no muscle contraction with trials of electrical stimulation. Because of this unusual outcome, electrodiagnostic studies have been completed on all of these children. Results of these studies have been consistent with severe motor neuronopathy. We postulate that these children have had an immunologic or inflammatory reaction to either the anterior horn cells or their proximal nerve roots, resulting in a lower motor neuron pattern of paralysis. We anticipated that these children would demonstrate long-term sequelae similar to those of children affected with poliomyelitis or brachial plexus injury, including asymmetric limb growth, weakness, joint dislocation or contracture, scoliosis, and scapular winging or gait abnormalities. 28 Upper extremity fractures (multiple in several children), including buckle fractures in the wrists, have been observed in several children with plegic upper extremities due to rapid onset and progression of disuse or neurogenic osteoporosis. Indeed, children with affected upper extremities demonstrated shoulder subluxation, scapular winging, and mild scoliosis, and children with lower extremity involvement demonstrated lax- ity of the hip, hyperextension at the knee, poor or absent dorsiflexion, and plantar flexion at the ankle. Bracing and splinting have been prescribed to protect affected joints. During observation of some of these children for over 2 years, recovery of motor function has been poor despite aggressive outpatient and home rehabilitation programs including strengthening, weight-bearing activities, gait training or fine motor skill remediation, joint stabilization, and bracing. 29 All children were given trials of FES cycle ergometry (either upper or lower extremity). In general, muscle contraction was not elicited, and, as sensation was intact, the children’s tolerance for FES was poor. Because of poor spontaneous recovery of neurologic function, these children have been evaluated for nerve, muscle, or tendon transfers in the hope of improving function or at least slowing the severe muscle and bone wasting. Three of our patients in this subgroup have undergone nerve transfers. Two have received nerve transfers with or without grafts to the biceps muscle with the goal of restoring elbow flexion. One has had a nerve transfer to the quadriceps with the goal of improving knee extension. Currently, the results of these procedures are unknown. Referral for nerve transfers must be made early in the course of recovery, as experience with the neonatal and traumatic nerve injury populations shows that better outcomes are achieved if these procedures are performed within 3 to 9 months, as fewer muscle fibers and less motor endplate atrophy will be present. 30 The goal of muscle and tendon transfer procedures is to stabilize joints (such as the shoulder or ankle) and to potentially improve function (such as elbow flexion or ankle plantar flexion or dorsiflexion). Because these procedures are not as time-sensitive, Continuum Lifelong Learning Neurol 2011;17(4):816–830 www.aan.com/continuum Copyright @ 201 1 Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited. 823
Rehabilitation Case 6-1 A 6-year, 8-month-old boy (Patient 1) presented with sudden-onset right upper and lower limb weakness with associated pain and muscle spasm. A 3-year, 9-month-old girl (Patient 2) presented with left upper limb weakness progressing to left lower limb weakness, pain, and spasms. Acutely, both patients demonstrated gait disturbance, and neither lost bowel or bladder function. Both children reported symptoms of a mild viral illness several days prior to the onset of paresis. Neither child had a personal or family history of neurologic or autoimmune disorders. In both cases, CSF analysis was consistent with inflammation (Patient 1 had a white blood cell count of 138, a protein of 35 mg/dL, and a glucose of 60 mg/dL; Patient 2 had a white blood cell count of 21, a protein of 26 mg/dL, and a glucose of 51 mg/dL). Workup for other causes of myelopathy, including viral and bacterial cultures, oligoclonal bands, and serum neuromyelitis optica titers was negative. In both cases, MRI revealed multilevel cervical T2 changes consistent with spinal cord edema (Figure 6-1). Ultimately, a diagnosis of transverse myelitis was made in both cases. Patient 1 was treated with antibiotics, antiviral medication, and IV immunoglobulin. Patient 2 was treated with IV methylprednisolone sodium succinate for 5 days with taper over 6 weeks. Both recovered full lower extremity motor function within 10 days; however, unilateral upper extremity paresis persisted. Subsequent electrodiagnostic studies revealed evidence of severe motor neuronopathy. Evaluation of the affected upper extremity 14 months (Patient 1) and 3 months (Patient 2) after onset demonstrated areflexia at the biceps, triceps, and brachioradialis muscles with flaccid tone and severe wasting of proximal and distal shoulder muscles (Figure 6-2 and Table 6-1). Sensation was intact and no residual pain was reported. Significant shoulder subluxation in the affected limb and upper limb residual paresis were present in the proximal and distal muscle groups with sparing of the finger flexors. Both children underwent 2 weeks of intensive occupational therapy, which included functional electrical stimulation (FES)-assisted upper extremity (UE) ergometry, scapular stabilization FIGURE 6-1 T2-weighted axial and sagittal MRI images of the cervical spine depicting Patient 1 (A) and Patient 2 (B). A hyperintense T2 signal is present within the cervical spinal cord ranging from level C2 through T1 in Patient 1 and C3 through C7 in Patient 2 (white arrows), corresponding with cervical gray matter areas. FIGURE 6-2 Photographs of Patient 1 (A) and Patient 2 (B) depicting muscular wasting and disuse atrophy when compared to the unaffected extremity. Glenohumeral subluxation is notable, as is shoulder girdle and trunk asymmetry. Scoliosis is evident in both cases, indicating that the posterior rami are affected. exercises, supported UE weight bearing, and fine motor skill remediation. Mechanical approximation of the glenohumeral joint was also included, either via FES or supportive bracing. FES-assisted UE ergometry was performed on the RT300-SA arm cycle, and the deltoids, rotator cuff, biceps, triceps, finger flexors, and extensors were stimulated sequentially. The children’s tolerance for FES was poor, as sensation was intact. Alternate exercises were performed with conventional facilitation techniques. A home therapy program was developed consisting of closed-chain proximal strengthening, fine motor Continued on page 825 824 www.aan.com/continuum August 2011 Copyright @ 201 1 Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited.
Page 1 and 2: Address correspondence to Dr Cristi
Page 3 and 4: KEY POINTS h Some muscle tightness
Page 5 and 6: KEY POINTS h Activity-based restora
Page 7: KEY POINT h Functional electrical s
Page 11 and 12: Rehabilitation they may be delayed
Page 13 and 14: KEY POINT h Applying conceptually s
Page 15: Rehabilitation unique complication

Rehabilitation in Transverse Myelitis - Kennedy Krieger Institute

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