1Department of Neurology, Dongsan Hospital, Keimyung University School of Medicine, Daegu, Korea
2Department of Neurology, Kyungpook National University Chilgok Hospital, School of Medicine, Kyungpook National University, Daegu, Korea
Copyright © 2022 The Korean Society of Clinical Neurophysiology
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Age (years)/sex | AChR-Ab | Symptoms |
EMG findings |
RNS | CK (titer, U/L) | Muscle biopsy | Initial misdiagnosis | |
---|---|---|---|---|---|---|---|---|
ASA: fibrillation potentials and positive sharp waves | Myopathic MUPs | |||||||
36/M (this case) | + | Dysarthria, dysphagia, bilateral ptosis, facial weakness, bilateral proximal limb weakness, and respiratory failure | FCR, PL, tongue, and cervical PSP | Tongue | Abnormal | Normal (124.1) | ND | None |
66/F 1 | + | Fatigable head drop, asymmetrical ptosis, dysarthria, and bilateral proximal weakness | Proximal upper and lower limb muscles | Proximal muscles | Abnormal | Normal | Not suggestive of inflammatory myopathy | Inflammatory myopathy |
74/M 1 | + | Dysphonia and respiratory failure | Bulbar muscles | NR | Abnormal | NR | NR | Anterior horn cell disease |
16/F 2 | + | Jaw drop, bulbar and limb-girdle fatigable weakness, and dyspnea | Deltoid, triceps, and iliopsoas | Deltoid, triceps, and iliopsoas | Normal | Abnormal (411) | Lymphorrhage | None |
55/F 2 | + | Head drop, bulbar and limb-girdle fatigable weakness, and shoulder atrophy | Deltoid, ISP, C5 PSP, C7 PSP, and T10 PSP | Deltoid, triceps, ISP, C5 PSP, and iliopsoas | Abnormal | Normal (114) | ND | None |
60/F 2 | + | Bulbar and limb-girdle fatigable weakness, head drop, shoulder atrophy, and dyspnea | Deltoid, trapezius, tongue, and temporalis | Deltoid | Abnormal | Normal (193) | ND | None |
58/F 3 | + | Dysarthria, dysphagia, limb-girdle weakness, and dyspnea | Deltoid and biceps | Biceps, deltoid, and VM | Abnormal | Normal (81) | Neurogenic muscle atrophy | Polymyositis |
74/M 3 | + | Dysarthria, dysphagia, masseter muscle weakness, and limb-girdle muscle weakness | Thoracic PSP | OO, trapezius, deltoid, FDI, VL, and TA | Abnormal | Abnormal (286) | Chronic neurogenic muscle atrophy, myopathic features, no signs of inflammation typical of myositis | Polymyositis |
60/F 4 | − | Diplopia, dysarthria, dysphagia, nasal regurgitation, mild proximal limb weakness, and respiratory failure | Diaphragm | NR | Abnormal | Normal | NR | None |
60/F 4 | − | Ptosis, bulbar muscle weakness, mild limb weakness, respiratory failure | Diaphragm and AEO | NR | Normal | Normal | NR | None |
38/F 4 | − | Dysphagia, diplopia, dysphonia, dyspnea, and weakness of ocular, facial, and bulbar muscles | Diaphragm and some limb muscles | Some limb muscles | Normal | Normal | NR | None |
42/M 5 | + | Diplopia, moderate-to-severe right ptosis, predominantly distal limb weakness | Distal hand and leg muscles | Distal hand and leg muscles | Abnormal | NR | NR | None |
37/F 5 | + | Ptosis, diplopia, and marked wrist extensor and intrinsic hand muscle weakness | Distal hand and forearm muscles | None | Abnormal | NR | NR | None |
28/M 6 | + | Diplopia, bilateral ptosis, mild dysphagia, and bilateral upper extremity weakness | Triceps, FCU, FDI, FDP III and IV, TA, and TP | None | Abnormal | Normal (185) | Neurogenic change | None |
77/M 7 | + | Mild left ptosis, intermittent diplopia, and severe bilateral proximal upper limb weakness | Deltoid | Deltoid | Abnormal | Normal | NR | None |
47/M 8 | + | Diplopia, right ptosis, and slowly progressive hand weakness | Distal upper limb muscles | Distal upper limb muscles | Abnormal | NR | NR | Distal upper limb myopathy |
28/F 9 | + | Non-fluctuating, progressive fatigue while talking and difficulty in swallowing, tongue atrophy and fasciculations, ptosis, and facial weakness | Tongue | Face and neck muscles | Abnormal | NR | NR | Myopathy |
AChR-Ab, acetylcholine receptor antibody; EMG, electromyography; ASA, abnormal spontaneous activity; MUPs, motor unit potentials; RNS, repetitive nerve stimulation; CK, serum creatine kinase; M, male; FCR, flexor carpi radialis; PL, peroneus longus; PSP, paraspinal muscles; ND, not done; F, female; NR, not reported; ISP, infraspinatus; VM, vastus medialis; OO, orbicularis oris; FDI, first dorsal interosseous; VL, vastus lateralis; TA, tibialis anterior; AEO, abdominal external oblique; FCU, flexor carpi ulnaris; FDP, flexor digitorum profundus; TP, tibialis posterior.
Age (years)/sex | AChR-Ab | Symptoms | EMG findings |
RNS | CK (titer, U/L) | Muscle biopsy | Initial misdiagnosis | |
---|---|---|---|---|---|---|---|---|
ASA: fibrillation potentials and positive sharp waves | Myopathic MUPs | |||||||
36/M (this case) | + | Dysarthria, dysphagia, bilateral ptosis, facial weakness, bilateral proximal limb weakness, and respiratory failure | FCR, PL, tongue, and cervical PSP | Tongue | Abnormal | Normal (124.1) | ND | None |
66/F 1 | + | Fatigable head drop, asymmetrical ptosis, dysarthria, and bilateral proximal weakness | Proximal upper and lower limb muscles | Proximal muscles | Abnormal | Normal | Not suggestive of inflammatory myopathy | Inflammatory myopathy |
74/M 1 | + | Dysphonia and respiratory failure | Bulbar muscles | NR | Abnormal | NR | NR | Anterior horn cell disease |
16/F 2 | + | Jaw drop, bulbar and limb-girdle fatigable weakness, and dyspnea | Deltoid, triceps, and iliopsoas | Deltoid, triceps, and iliopsoas | Normal | Abnormal (411) | Lymphorrhage | None |
55/F 2 | + | Head drop, bulbar and limb-girdle fatigable weakness, and shoulder atrophy | Deltoid, ISP, C5 PSP, C7 PSP, and T10 PSP | Deltoid, triceps, ISP, C5 PSP, and iliopsoas | Abnormal | Normal (114) | ND | None |
60/F 2 | + | Bulbar and limb-girdle fatigable weakness, head drop, shoulder atrophy, and dyspnea | Deltoid, trapezius, tongue, and temporalis | Deltoid | Abnormal | Normal (193) | ND | None |
58/F 3 | + | Dysarthria, dysphagia, limb-girdle weakness, and dyspnea | Deltoid and biceps | Biceps, deltoid, and VM | Abnormal | Normal (81) | Neurogenic muscle atrophy | Polymyositis |
74/M 3 | + | Dysarthria, dysphagia, masseter muscle weakness, and limb-girdle muscle weakness | Thoracic PSP | OO, trapezius, deltoid, FDI, VL, and TA | Abnormal | Abnormal (286) | Chronic neurogenic muscle atrophy, myopathic features, no signs of inflammation typical of myositis | Polymyositis |
60/F 4 | − | Diplopia, dysarthria, dysphagia, nasal regurgitation, mild proximal limb weakness, and respiratory failure | Diaphragm | NR | Abnormal | Normal | NR | None |
60/F 4 | − | Ptosis, bulbar muscle weakness, mild limb weakness, respiratory failure | Diaphragm and AEO | NR | Normal | Normal | NR | None |
38/F 4 | − | Dysphagia, diplopia, dysphonia, dyspnea, and weakness of ocular, facial, and bulbar muscles | Diaphragm and some limb muscles | Some limb muscles | Normal | Normal | NR | None |
42/M 5 | + | Diplopia, moderate-to-severe right ptosis, predominantly distal limb weakness | Distal hand and leg muscles | Distal hand and leg muscles | Abnormal | NR | NR | None |
37/F 5 | + | Ptosis, diplopia, and marked wrist extensor and intrinsic hand muscle weakness | Distal hand and forearm muscles | None | Abnormal | NR | NR | None |
28/M 6 | + | Diplopia, bilateral ptosis, mild dysphagia, and bilateral upper extremity weakness | Triceps, FCU, FDI, FDP III and IV, TA, and TP | None | Abnormal | Normal (185) | Neurogenic change | None |
77/M 7 | + | Mild left ptosis, intermittent diplopia, and severe bilateral proximal upper limb weakness | Deltoid | Deltoid | Abnormal | Normal | NR | None |
47/M 8 | + | Diplopia, right ptosis, and slowly progressive hand weakness | Distal upper limb muscles | Distal upper limb muscles | Abnormal | NR | NR | Distal upper limb myopathy |
28/F 9 | + | Non-fluctuating, progressive fatigue while talking and difficulty in swallowing, tongue atrophy and fasciculations, ptosis, and facial weakness | Tongue | Face and neck muscles | Abnormal | NR | NR | Myopathy |
AChR-Ab, acetylcholine receptor antibody; EMG, electromyography; ASA, abnormal spontaneous activity; MUPs, motor unit potentials; RNS, repetitive nerve stimulation; CK, serum creatine kinase; M, male; FCR, flexor carpi radialis; PL, peroneus longus; PSP, paraspinal muscles; ND, not done; F, female; NR, not reported; ISP, infraspinatus; VM, vastus medialis; OO, orbicularis oris; FDI, first dorsal interosseous; VL, vastus lateralis; TA, tibialis anterior; AEO, abdominal external oblique; FCU, flexor carpi ulnaris; FDP, flexor digitorum profundus; TP, tibialis posterior.