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1555 B. Katirji Et Al. (Eds.), Neuromuscular Disorders In Index A Acute motor and sensory axonal neuropathy (AMSAN), Abetalipoproteinemia. See Bassen-Kornzweig disease 54, 579–580 Abnormal muscle movements, 8–9 Acute motor axonal neuropathy (AMAN), 54, 579 Abnormal SNAPs, 582 Acute myocardial infarction, CK elevation in, Abnormal temporal dispersion, 608–609 41–42 Abscess. See also Spinal epidural abscess (SEA) Acute necrotizing alcoholic myopathy, 1419 bacterial, 1067 Acute necrotizing myelopathy, PND, 1504 compressive disorders, and LS plexopathy, 1067 Acute neuromuscular weakness perirectal, 1067 clinical presentation, 1523 psoas, 1067 differential diagnosis, 1523–1525 Absolute muscle mass, 42 epidemiology Accessory deep peroneal nerve, 119 critical illness myopathy, 1516–1517 Acetaminophen, 1021 critical illness polyneuropathy, 1516 Acetazolamide, 726 prolonged NMJ blockade, 1517 Acetylcholine receptors (AChR), 28 evaluation and diagnosis kinetic abnormalities, 1119 critical illness myopathy, 1526–1527 mutations, 1116, 1117 critical illness polyneuropathy, 1525–1526 neuromuscular junction disorders, 69–71 laboratory abnormalities, 1527 AChR. See Acetylcholine receptors (AChR) neuromuscular junction blockade, 1527 Acidic-hexosaminidase deficiency, 411 pathology and pathogenesis Acid maltase deficiency, 374 critical illness myopathy, 1518 Acromegaly, endocrine neuropathies, 700 critical illness polyneuropathy, 1517 Acrylamide spectrum, 1515–1516 clinical presentation, 709 treatment, management, and prognosis electrodiagnostic testing, 709 critical illness myopathy, 1528 treatment and management, 709–710 critical illness polyneuropathy, 1528 Action myotonias, 8 general principles, 1527–1528 Action potentials prolonged neuromuscular junction blockade, activation and inactivation gates, 26 1528–1529 depolarizations, 24 Acute nonspecific neck pain, 993 electrical circuit, 24–25 Acute radiculopathy, 951. See also Cervical radiculopathy ions, 23 with neurological deficits, 994 membrane potential, change in, 26 with sensory features, 993–994 origin, 23 Acute ventilatory management, 366–368 Acute ataxic neuropathy, 595 Acyclovir, 1023 Acute autonomic neuropathy, 596 Adrenoleukodystrophy (ALD) Acute axonal degeneration, 239–240 phenotypic heterogeneity, 560, 561 Acute hypokalemic alcoholic myopathy, 1419–1420 zebra bodies, 561 Acute inflammatory demyelinating polyradiculoneuropathy Adrenomyeloneuropathy (AMN) (AIDP), 573–574 phenotypic heterogeneity, 560, 561 Acute intermittent porphyria (AIP), 586 zebra bodies, 561 Acute ischemic mononeuropathy, nonvasculitic ischemic Adult intestinal toxemia botulism, 1067. See also Botulism neuropathy Adult-onset hexosaminidase A deficiency clinical presentation, 832 clinical presentation, 452–453 diagnosis, 833 course, 453 differential diagnosis, 832 diagnosis, 453 etiology, 832 differential diagnosis, 453 evaluation, 832–833 etiology, 452 management, 833 evaluation, 453 pathogenesis, 832 management, 453 prognosis, 833 pathogenesis, 452 treatment, 833 prognosis, 453 B. Katirji et al. (eds.), Neuromuscular Disorders in Clinical Practice, 1555 DOI 10.1007/978-1-4614-6567-6, © Springer Science+Business Media New York 2014 1556 Index Adult-onset myotonic dystrophy type 1, 1261 AMSAN. See Acute motor and sensory axonal neuropathy (AMSAN) Adult polyglucosan body disease (APBD) Amyotrophic lateral sclerosis (ALS), 996 clinical manifestations, 454 atypical motor neuron disorders, 441, 442, 444, 445, 447–449, course, 455 452–460 diagnosis, 455 characteristics, 395 differential diagnosis, 455 clinical presentation etiology, 454 atypical features, 398–400 evaluation, 455 typical features, 397–398 pathogenesis, 454 clinicoanatomic correlation, 396–397 prognosis, 455 diagnosis treatment, 455 diagnostic algorithm, 405 AECAs. See Anti-endothelial cell antibodies (AECAs) EDX examination, 405–407 AFOs. See Ankle-foot orthoses (AFOs) laboratory testing, 407–408 AIDP. See Acute inflammatory demyelinating neuroimaging, 407 polyradiculoneuropathy (AIDP) differential diagnosis, 410 AIP. See Acute intermittent porphyria (AIP) anterior horn cell, 411–412 Alcoholic cardiomyopathy, 1420 brain, 409 Alcoholic polyneuropathy brainstem and spinal cord, 409–411 vs. chronic alcoholism, 677–678 myopathies, 413 clinical presentation, 679–680 neuromuscular junction disorders, 413 differential diagnosis, 680 neuropathies and plexopathies, 412 EDX features, 680 polyradiculoneuropathies, 412 etiology and pathogenesis polyradiculopathies, 412 animal studies, 679 radiculopathies, 412 multivariate analysis, 678 systemic disease, 413–414 nutritional history, 678 epidemiology, 395–396 thiamine-deficient polyneuropathy, 678–679 etiology, 403–404 toxic etiology, 678 familial, 401–402 treatment, 680 history, 414–415 Alcohol-related myopathy pathogenesis, 403–404 acute hypokalemic myopathy, 1419–1420 pathology, 404 acute necrotizing myopathy, 1419 peripheral nerve disorders, 503 cardiomyopathy, 1420 prognosis, 414–415 chronic myopathy, 1420 treatment ALD. See Adrenoleukodystrophy (ALD) simplified algorithm, 416 Aldolase, 45–46 symptom management, 417–418 Alemtuzumab, 349 trials in, 317–319 Alexander’s disease, 277 western Pacific, 403 Allgrove syndrome, 409–410 Amyotrophic lateral sclerosis functional rating scale (ALSFRS-R), All-or-none response, 23 295–297 Alpha-sarcoglycanopathy, 1241–1242 ANCAs. See Antineutrophil cytoplasmic autoantibodies (ANCAs) ALS. See Amyotrophic lateral sclerosis (ALS) Andersen-Tawil syndrome (ATS) AMAN. See Acute motor axonal neuropathy (AMAN) clinical presentation, 1175–1176 American association of neuromuscular and electrodiagnostic diagnosis, 1176 medicine (AANEM), 620 differential diagnosis, 1176 Aminolevulinic acid (ALA), 557 etiology, 1176 Aminotransferases, 47 evaluation, 1176 Amiodarone, 722 exercise testing, 1176 Amitriptyline, 724 laboratory testing, 1176 Ammoniated mercury, 726 management, 1176–1177 AMN. See Adrenomyeloneuropathy (AMN) pathogenesis, 1176 Amphetamines, 726 prognosis, 1177 Amphiphilic drug myopathy treatment, 1176–1177 amiodarone, 1414 Aneurysms and large vessel disease, 1067 chloroquine, hydroxychloroquine, 1414 Ankle-foot orthoses (AFOs), 329, 539, 1255 differential diagnosis, 1414 Anoctaminopathy, 1244–1245 etiology, 1413 ANS. See Autonomic nervous system (ANS) lysosomes producing vacuolization, 1413 Anterior horn cells, 441, 447–450, 454, 456, 457, 460 membranes causing myofiber necrosis, 1413 Anterior primary rami disorders, 997 NVP-LDE225, 1415 Anterior tarsal tunnel syndrome, 918–919 pathogenesis, 1413 Antibiotics, 1130 prognosis, 1415 Anti-endothelial cell antibodies (AECAs), 738–739 rimmed vacuole myopathies, 1413–1414 Anti-microtubular myopathy Amphiphysin antibodies (amphiphysin Ab), 73 clinical presentation, 1415 Amplification-refractory mutation system colchicine myopathy, predisposing factors for, 1415–1416 (ARMS), 277 differential diagnosis, 1416–1417 Index 1557 drug interactions, 1416 IgG-AAb2 etiology, 1415 GD1b ganglioside, 55 pathogenesis, 1415 glycolipid/carbohydrate antigens, 55 prognosis, 1417 GM1 ganglioside, 54 treatment, 1417 GQ1b/GT1a gangliosides, 54–55 Antineutrophil cytoplasmic antibodies (ANCAs), 55–56, 738 IgG-AAb3, 55–56 Antineutrophil cytoplasmic autoantibodies (ANCAs), 738 IgG-AAb4 Anti-voltage-gated calcium channel antibody, 1093 CRMP5, 56–57 Aortic aneurysms, 1017 Hu antigens, 56 APBD. See Adult polyglucosan body disease (APBD) IgG4-AAb, 57 Appel ALS scale, 297–298 IgM-AAb Array comparative genomic hybridization (aCGH), 282–284 GD1b ganglioside, 59 Arsenic GM1 ganglioside/NS6S, 58 clinical presentation, 705–706 MAG, 58–59 diagnosis, 706 monoclonal proteins, 60–62 evaluation, 706 properties, 57 management, 706 sulfatide, 59–60 pathogenesis, 705 target antigens, 60 treatment, 706 TS-HDS, 59 Arthrogryposis multiplex congenita (AMC) Autoantibody testing causes, 1541–1542 with hyperexcitability disorders CNS developmental defects, 1542 amphiphysin antibodies, 73 connective tissue abnormalities, 1542 GABA-synthesizing enzyme GAD, 73 Arthropods, biological neurotoxins, 1134 potassium channel antibodies, 72–73 Artifacts Stiff person syndrome, 73 nerve biopsy, 239 myositis-overlap antibodies skeletal muscle biopsy, 254 lupus myositis, 76 Asymmetric myopathy, 1199, 1200 mixed connective tissue disease, 75–76 Ataxia-telangiectasia (AT), 568 polymyositis-scleroderma overlap syndrome, 75 ATS. See Andersen-Tawil syndrome (ATS) myositis-specific antibodies Atypical antipsychotics, 1495–1496 anti-Mi-2, 75 Atypical motor neuron disorders anti-p155/140 and anti-MJ (anti-NXP-2), 75 clinical clues, 441–442 anti-signal recognition particle antibodies, 75 definition, 441 antisynthetase autoantibodies, 73–75 HSP neuromuscular junction disorders differential diagnosis, 450–451 AChR antibodies, 69–71 etiology, 450 calcium channel antibodies, 71–72 evaluation, 451 Lambert-Eaton syndrome, 69 genetic subtypes, 451–452 MuSK antibodies, 70–71 pathogenesis, 450 myasthenia gravis, 69 immune-mediated, 442–445 neuronal AChR antibodies, 72 multiple system disorders other autoantibodies, 72 adult-onset hexosaminidase A deficiency, 452–453 striational antibodies, 71 APBD, 454–455 Autogenous nerve grafting, 868 spinocerebellar degenerations, 453–454 Autoimmune neuromuscular diseases, immunotherapy. nonimmune-mediated See Immunotherapy brachial amyotrophic diplegia, 449 Autonomic dysfunction, 589 Fazio-Londe disease and BVVLS, 449–450 Autonomic manifestations, and LEMS, 1091–1092 monomelic amyotrophy, 447–449 Autonomic nervous system (ANS) progressive muscular atrophy and bulbar palsy, 449 anatomy, 202 SMAs, 445–446 bladder, 205 X-linked recessive bulbospinal
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