radiculopathies have revealed sensitivity values ranging from 29 to 92% and specificity values ranging from 37 to 100%.2 A normal EMG does not necessarily exclude pathology and should not bar patients from receiving clinically indicated treatment. As our study demonstrated, it is important to state the EMG findings as clearly as possible.1 When the electrophysiologic findings do not correlate with the patient’s symptoms, perhaps electromyographers should describe the limitations of the study within the EMG report as well. Elizabeth A. Mauricio, MD Elliot L. Dimberg, MD Kathleen D. Kennelly, MD, PhD Devon I. Rubin, MD Department of Neurology, Mayo Clinic, Jacksonville, Florida, USA 1. Mauricio EA, Dimberg EL, Kennelly KD, Rubin DI. Improving referring physicians’ understanding of electromyography reports when qualifying radiculopathies: a need for standardized terminology. Muscle Nerve 2014;49:129–130. 2. Cho SC, Ferrante MA, Levin KH, Harmon RL, So YT. Utility of electrodiagnostic testing in evaluating patients with lumbosacral radiculopathy: an evidence-based review. Muscle Nerve 2010;42:276–282.
Published online 11 February 2014 in Wiley Online Library (wileyonlinelibrary.com). DOI 10.1002/mus.24206
--------------------------------------------------------FIGURE 1. MRC grading scores for the 3 main shoulder and elbow muscles over the training: (A) left and (B) right upper limb.
ROBOTIC-ASSISTED REHABILITATION FOR PARSONAGE-TURNER SYNDROME We describe upper limb robot-mediated training, an innovative therapy primarily recommended after stroke,1,2 for a patient with severe subacute brachial plexopathy. A 41-year-old right-handed man was admitted to our neurorehabilitation unit 13 days after onset of typical bilateral Parsonage-Turner syndrome (PTS), a rare acute brachial plexopathy.3,4 The patient’s symptoms included neuropathic pain with severe bilateral weakness (MRC 3/5)5,6 in the deltoid, biceps, and triceps muscles (Fig. 1). EMG recordings were consistent with severe bilateral axonopathy in the C5 to C7 territories. Although PTS has a good prognosis,6,7 optimal recovery usually requires 2 to 3 years, particularly because no treatments currently exist to accelerate recovery.8 However, recent studies in animals9,10 have suggested that physical therapies could be beneficial. Thus, our patient integrated a conventional rehabilitation program (1 h of occupational therapy, 5 days/week) complemented by robot-aided training (InMotion 2.0, Interactive Motion Technologies, Inc., Watertown, MA; 45 min, 3 days/ week) for 15 weeks. The patient signed a consent form that was approved by our local ethics committee. The robotic training consisted of a high number of shoulder and elbow movements (960 movements/ session, 46 sessions). During the period of most severe weakness (baseline MRC, deltoid R, 2/5; L, 2/5; biceps R, 3/5; L, 2/5; triceps R, 3/5; L, 2/5; extensor carpi radialis R, 4/5; L, 4/5), the assist-as-needed program was Letters to the Editor
used. During the last 10 sessions, the patient had improved sufficiently to carry out exercises against resistance. Every 2–3 weeks, each impaired muscle was evaluated using the MRC scale, and it showed unusually rapid4,5 improvement, with an average score of 4.5 6 0.5 at the end of the program, less than 5 months after onset (Fig. 1A,B). The program was well tolerated and did not increase pain. This report shows that intensive exercises can be performed after a severe neuropathic injury without any damaging effects (i.e., no undue fatigue or increase in pain), despite the fact that some studies have shown that intensive training can have detrimental effects on muscle function.10 The difference might be due to the use of a robotic algorithm that adapted the level of assistance to the muscle weakness and took into account motor changes which occurred during the training. Another positive effect is likely to have come from the motivational and distraction effects on pain of a virtual realitylike therapy that enabled the patient to perform such a high number of movements,11 even if the necessity of such intensity is not known. Whether the training impacted motor outcomes cannot be determined from this study, however comparison with results in the literature suggests that recovery was earlier than expected.6 Tsairis et al. (1972)7 reported that 75% of patients affected by PTS achieved full recovery after 2 years. However, although there is still no MUSCLE & NERVE
May 2014
773
evidence on the effects of exercise-based therapy on motor outcomes, with only moderate effectiveness for strengthening exercises on muscle strength in peripheral neuropathy,12 some studies in animals have shown that exercise can promote reinnervation after nerve injury.10 This case study demonstrates that upper-limb robotaided rehabilitation is a feasible and safe adjunct to a standard therapy program that enhanced motor recovery for a patient with severe PTS. Christophe Duret, MD1 Laurent Lehenaff, PT,1 Emilie Hutin, PhD2 Manvel Aghasaryan, MD3 Jean-Michel Gracies, MD, PhD2 1 decine Physique et de CRF Les Trois Soleils, Me adaptation, Unite de Re e ducation Neurologique, Re Boissise-Le-Roi (77), France
7. Tsairis P, Dyck PJ, Mulder DW. Natural history of brachial plexus neuropathy. Report of 99 patients. Arch Neurol 1972;27:109–117. 8. Van Alfen N, Van Engelen BG, Hughes RA. Treatment for idiopathic and hereditary neuralgic amyotrophy (brachial neuritis). Cochrane Database Syst Rev 2009;3:CD006976. 9. Udina E, Puigdemasa A, Navarro X. Passive and active exercise improve regeneration and muscle reinnervation after peripheral nerve injury in the rat. Muscle Nerve 2011;43:500–509. 10. Udina E, Cobianchi S, Allodi I, Navarro X. Effects of activitydependent strategies on regeneration and plasticity after peripheral nerve injuries. Ann Anat 2011;193:347–353. 11. Malloy KM, Milling LS. The effectiveness of virtual reality distraction for pain reduction: a systematic review. Clin Psychol Rev 2010;30:1011–1018. 12. White CM, Pritchard J, Turner-Stokes L. Exercise for people with peripheral neuropathy. Cochrane Database Syst Rev 2004;18:CD003904.
Published online 30 November 2013 in Wiley Online Library (wileyonlinelibrary.com). DOI 10.1002/mus.24139
---------------------------------------------------------
2
Analyse et Restauration du Mouvement, Groupe Hospitalier e ducation Neurolocomotrice, AP-HP, Henri Mondor, Re teil (94), France Cre
3
Centre Hospitalier Sud Francilien, Neurologie, Corbeil-Essonnes (91), France 1. Aisen ML, Krebs HI, Hogan N, McDowell F, Volpe BT. The effect of robot-assisted therapy and rehabilitative training on motor recovery following stroke. Arch Neurol 1997;54:443–446. 2. Lo AC, Guarino PD, Richards LG, Haselkorn JK, Wittenberg GF, Federman DG, et al. Robot-assisted therapy for long-term upper-limb impairment after stroke. N Engl J Med 2010;362:1772–1783. 3. MacDonald BK, Cockerell OC, Sander JW, Shorvon SD. The incidence and lifetime prevalence of neurological disorders in a prospective community-based study in the UK. Brain 2000;123:665–676. 4. Parsonage M, Turner J. Neuralgic amyotrophy: the shoulder-girdle syndrome. Lancet 1948;1:973–978. 5. Medical Research Council. Aids to the examination of the peripheral nervous system. London: HMSO; 1976. 6. Van Alfen N, Van Engelen BGM. The clinical spectrum of neuralgic amyotrophy in 246 cases. Brain 2006;126:438–450.
MUTATION IN FAM134B CAUSING HEREDITARY SENSORY NEUROPATHY WITH SPASTICITY IN A TURKISH FAMILY Hereditary sensory and autonomic neuropathy (HSAN) type 2 is a rare autosomal recessive disease that was first described by Ota et al. in 1973.1 We report the clinical and pathological findings of 2 siblings with HSAN type 2 due to a homozygous mutation in FAM134B. Patient 1. A 41-year-old man, born from first degree cousins, did not show any obvious motor delay until the age of 2, when he started to have difficulty in walking. He experienced recurrent painless foot infections and ulcers from age 8. He was admitted to the Neurology Depart_ ment of the Istanbul Medical Faculty at age 24 and was followed up afterward. On examination, he had both
FIGURE 1. Clinical presentation and pedigree of FAM 134B. (A) Palmar hyperkeratosis of the index man. (B) Amputated greater toe of his sister. (C) Pedigree: The parents of the patients are first-degree cousins. Only DNA samples from the father, mother, sister, and son were available. The filled arrow shows our index patient, the striped arrow his affected sister. A square indicates a man; a circle a woman; a filled symbol represents “affected”; a slanted line through a symbol indicates that the individual is deceased.
774
Letters to the Editor
MUSCLE & NERVE
May 2014
Published online 11 February 2014 in Wiley Online Library (wileyonlinelibrary.com). DOI 10.1002/mus.24206
--------------------------------------------------------FIGURE 1. MRC grading scores for the 3 main shoulder and elbow muscles over the training: (A) left and (B) right upper limb.
ROBOTIC-ASSISTED REHABILITATION FOR PARSONAGE-TURNER SYNDROME We describe upper limb robot-mediated training, an innovative therapy primarily recommended after stroke,1,2 for a patient with severe subacute brachial plexopathy. A 41-year-old right-handed man was admitted to our neurorehabilitation unit 13 days after onset of typical bilateral Parsonage-Turner syndrome (PTS), a rare acute brachial plexopathy.3,4 The patient’s symptoms included neuropathic pain with severe bilateral weakness (MRC 3/5)5,6 in the deltoid, biceps, and triceps muscles (Fig. 1). EMG recordings were consistent with severe bilateral axonopathy in the C5 to C7 territories. Although PTS has a good prognosis,6,7 optimal recovery usually requires 2 to 3 years, particularly because no treatments currently exist to accelerate recovery.8 However, recent studies in animals9,10 have suggested that physical therapies could be beneficial. Thus, our patient integrated a conventional rehabilitation program (1 h of occupational therapy, 5 days/week) complemented by robot-aided training (InMotion 2.0, Interactive Motion Technologies, Inc., Watertown, MA; 45 min, 3 days/ week) for 15 weeks. The patient signed a consent form that was approved by our local ethics committee. The robotic training consisted of a high number of shoulder and elbow movements (960 movements/ session, 46 sessions). During the period of most severe weakness (baseline MRC, deltoid R, 2/5; L, 2/5; biceps R, 3/5; L, 2/5; triceps R, 3/5; L, 2/5; extensor carpi radialis R, 4/5; L, 4/5), the assist-as-needed program was Letters to the Editor
used. During the last 10 sessions, the patient had improved sufficiently to carry out exercises against resistance. Every 2–3 weeks, each impaired muscle was evaluated using the MRC scale, and it showed unusually rapid4,5 improvement, with an average score of 4.5 6 0.5 at the end of the program, less than 5 months after onset (Fig. 1A,B). The program was well tolerated and did not increase pain. This report shows that intensive exercises can be performed after a severe neuropathic injury without any damaging effects (i.e., no undue fatigue or increase in pain), despite the fact that some studies have shown that intensive training can have detrimental effects on muscle function.10 The difference might be due to the use of a robotic algorithm that adapted the level of assistance to the muscle weakness and took into account motor changes which occurred during the training. Another positive effect is likely to have come from the motivational and distraction effects on pain of a virtual realitylike therapy that enabled the patient to perform such a high number of movements,11 even if the necessity of such intensity is not known. Whether the training impacted motor outcomes cannot be determined from this study, however comparison with results in the literature suggests that recovery was earlier than expected.6 Tsairis et al. (1972)7 reported that 75% of patients affected by PTS achieved full recovery after 2 years. However, although there is still no MUSCLE & NERVE
May 2014
773
evidence on the effects of exercise-based therapy on motor outcomes, with only moderate effectiveness for strengthening exercises on muscle strength in peripheral neuropathy,12 some studies in animals have shown that exercise can promote reinnervation after nerve injury.10 This case study demonstrates that upper-limb robotaided rehabilitation is a feasible and safe adjunct to a standard therapy program that enhanced motor recovery for a patient with severe PTS. Christophe Duret, MD1 Laurent Lehenaff, PT,1 Emilie Hutin, PhD2 Manvel Aghasaryan, MD3 Jean-Michel Gracies, MD, PhD2 1 decine Physique et de CRF Les Trois Soleils, Me adaptation, Unite de Re e ducation Neurologique, Re Boissise-Le-Roi (77), France
7. Tsairis P, Dyck PJ, Mulder DW. Natural history of brachial plexus neuropathy. Report of 99 patients. Arch Neurol 1972;27:109–117. 8. Van Alfen N, Van Engelen BG, Hughes RA. Treatment for idiopathic and hereditary neuralgic amyotrophy (brachial neuritis). Cochrane Database Syst Rev 2009;3:CD006976. 9. Udina E, Puigdemasa A, Navarro X. Passive and active exercise improve regeneration and muscle reinnervation after peripheral nerve injury in the rat. Muscle Nerve 2011;43:500–509. 10. Udina E, Cobianchi S, Allodi I, Navarro X. Effects of activitydependent strategies on regeneration and plasticity after peripheral nerve injuries. Ann Anat 2011;193:347–353. 11. Malloy KM, Milling LS. The effectiveness of virtual reality distraction for pain reduction: a systematic review. Clin Psychol Rev 2010;30:1011–1018. 12. White CM, Pritchard J, Turner-Stokes L. Exercise for people with peripheral neuropathy. Cochrane Database Syst Rev 2004;18:CD003904.
Published online 30 November 2013 in Wiley Online Library (wileyonlinelibrary.com). DOI 10.1002/mus.24139
---------------------------------------------------------
2
Analyse et Restauration du Mouvement, Groupe Hospitalier e ducation Neurolocomotrice, AP-HP, Henri Mondor, Re teil (94), France Cre
3
Centre Hospitalier Sud Francilien, Neurologie, Corbeil-Essonnes (91), France 1. Aisen ML, Krebs HI, Hogan N, McDowell F, Volpe BT. The effect of robot-assisted therapy and rehabilitative training on motor recovery following stroke. Arch Neurol 1997;54:443–446. 2. Lo AC, Guarino PD, Richards LG, Haselkorn JK, Wittenberg GF, Federman DG, et al. Robot-assisted therapy for long-term upper-limb impairment after stroke. N Engl J Med 2010;362:1772–1783. 3. MacDonald BK, Cockerell OC, Sander JW, Shorvon SD. The incidence and lifetime prevalence of neurological disorders in a prospective community-based study in the UK. Brain 2000;123:665–676. 4. Parsonage M, Turner J. Neuralgic amyotrophy: the shoulder-girdle syndrome. Lancet 1948;1:973–978. 5. Medical Research Council. Aids to the examination of the peripheral nervous system. London: HMSO; 1976. 6. Van Alfen N, Van Engelen BGM. The clinical spectrum of neuralgic amyotrophy in 246 cases. Brain 2006;126:438–450.
MUTATION IN FAM134B CAUSING HEREDITARY SENSORY NEUROPATHY WITH SPASTICITY IN A TURKISH FAMILY Hereditary sensory and autonomic neuropathy (HSAN) type 2 is a rare autosomal recessive disease that was first described by Ota et al. in 1973.1 We report the clinical and pathological findings of 2 siblings with HSAN type 2 due to a homozygous mutation in FAM134B. Patient 1. A 41-year-old man, born from first degree cousins, did not show any obvious motor delay until the age of 2, when he started to have difficulty in walking. He experienced recurrent painless foot infections and ulcers from age 8. He was admitted to the Neurology Depart_ ment of the Istanbul Medical Faculty at age 24 and was followed up afterward. On examination, he had both
FIGURE 1. Clinical presentation and pedigree of FAM 134B. (A) Palmar hyperkeratosis of the index man. (B) Amputated greater toe of his sister. (C) Pedigree: The parents of the patients are first-degree cousins. Only DNA samples from the father, mother, sister, and son were available. The filled arrow shows our index patient, the striped arrow his affected sister. A square indicates a man; a circle a woman; a filled symbol represents “affected”; a slanted line through a symbol indicates that the individual is deceased.
774
Letters to the Editor
MUSCLE & NERVE
May 2014
