315:
spirals are unusual membrane structures that have a spiral pattern. These membrane structures are seen during electron microscopic examination of the affected muscle. These structures merge into or are surrounded by tubular structures that resemble tubular aggregates. Tubular aggregates are abnormal accumulations of membranous tubules and have been observed in a wide variety of muscle diseases and originate from the sarcoplasmic reticulum. Cylindrical spirals were first described in 1979 and were thought to be a non-specific reaction of skeletal muscle secondary to a metabolic disturbance or muscle fibre injury. The molecular basis of cylindrical spiral myopathy is currently unknown, however a genetic mutation affecting the
39:
306:, is associated with small areas of decreased oxidative activities, resulting in areas that appear in this histology as "cores". These appear through microscopy very similar to central core, however the cores are typically smaller in multicore myopathy. As with congenital fiber type disproportion, patients have a greater number of type 1 fibers. Overall, approximately half of diagnosed individuals report no progression of muscle weakness, while half report a very slow progression.
253:, is recognized by pain during exercise and difficulty walking. People affected by this disease typically are wheelchair-reliant by middle adulthood, have weakness in the muscles involved in eye movement, nerve function disorders, and some form of intellectual disability. Myotubular myopathy is very rare, with less than 50 families currently affected. Genetically, myotubular myopathy can have two causes: autosomal dominant and autosomal recessive. When caused by a mutation in the
145:
291:
abundant than type 2 fibers. This leads to affected individuals being able to maintain an active lifestyle, though they usually have lower levels of stamina. Severity with this disease varies greatly, but people typically present symptoms by the age of one. Individuals do not usually worsen with time, and cases have even been reported of improvements.
273:
was first described in 1956 and usually presents in infancy or early childhood as non-progressive mild proximal weakness that persists throughout life. Central core disease is believed to be more prevalent than currently reported, as it is hard to recognize and often misdiagnosed in early childhood.
131:
Myopathies with varying fiber size, such as congenital fiber type disproportion, occurs when type 1 fibers, the slow twitch fibers involved in sustaining activity, are smaller than type 2 fibers, the fast twitch fibers involved in quick activity. Since smaller type 1 fibers is not associated with
51:
is a very broad term for any muscle disorder present at birth. This defect primarily affects skeletal muscle fibres and causes muscular weakness and/or hypotonia. Congenital myopathies account for one of the top neuromuscular disorders in the world today, comprising approximately 6 in 100,000 live
122:
Myopathies with central nuclei, such as myotubular myopathy, involves an error in the gene involved in vesicle movement throughout the cell. This creates problems in vesicles reaching the plasma membrane with the cellular components necessary to fuse myoblast, a major step in the formation of the
314:
Cylindrical spirals myopathy is very rare with only 18 individual cases described as of 2013. The majority of cases are sporadic, and has been observed in only 3 families. It is characterized by the presence of cylindrical spirals as the main pathological finding in muscle biopsies. Cylindrical
224:
Nemaline myopathy was first described in 1963 and is the most common congenital myopathy. It is characterized by generalized muscle weakness and low muscle tone. In its severest form, affected babies often die from respiratory failure. To date, 9 gene mutations have been found to cause nemaline
290:
Congenital fiber type disproportion affects skeletal muscle, typically causing weakness in the shoulders, upper arms, thighs, and hips. Skeletal muscle is made up of two kinds of fiber, type 1 and type 2. In congenital fiber type disproportion, type 1 fibers are not only smaller but often more
187:
can be run to check the electrical activity of the muscle. Diagnosis heavily relies on muscle pathology, where a muscle biopsy is visualised on the cellular level. Diagnosis usually relies on this method, as creatine kinase levels and electromyography can be unreliable and non-specific. Since
386:
Currently, there are no treatments for any of the congenital myopathies. Depending on the severity, there are different therapies available to help alleviate any pain and aid patients in performing varying activities. For example, many congenital myopathy patients are involved in physical or
132:
nemaline myopathy, the most common type of congenital myopathy, it has not been studied in as great detail as many of the others. However, the smaller type 1 fibers explains why patients typically can participate in activities for shorter periods of time, but struggle with extended activity.
1305:
Gang, Qiang; Bettencourt, Conceição; Brady, Stefen; Holton, Janice L.; Healy, Estelle G.; McConville, John; Morrison, Patrick J.; Ripolone, Michela; Violano, Raffaella; Sciacco, Monica; Moggio, Maurizio; Mora, Marina; Mantegazza, Renato; Zanotti, Simona; Wang, Zhaoxia (January 2022).
387:
occupational therapy in an attempt to strengthen their skeletal muscles. Orthopedic surgery is usually necessary to correct skeletal deformities secondary to muscle weakness, such as scoliosis. Survival is typically determined by the level of respiratory muscle insufficiency.
935:
Chevessier, F; Marty, I; Paturneau-Jouas, M; Hantaı̈, D; Verdière-Sahuqué, M (2004). "Tubular aggregates are from whole sarcoplasmic reticulum origin: Alterations in calcium binding protein expression in mouse skeletal muscle during aging".
278:, which is a life-threatening anesthetic reaction that causes a rise in body temperature, muscular rigidity and muscular breakdown, grossly elevated creatine kinase, and acidosis. Central core disease is caused by a mutation in the
1457:
1442:
225:
myopathy. 6 of the identified genes are associated with the actin filament, which is the basis for muscle contraction. Histologically, nemaline rods stain red with Gomori's trichrome and are mostly seen in the
1057:
Rapuzzi, S; Prelle, A; Moggio, M; Rigoletto, C; Ciscato, P; Comi, G; Francesca, F; Scarlato, G (1995). "High serum creatine kinase levels associated with cylindrical spirals at muscle biopsy".
742:. Lister Hill National Center for Biomedical Communications, U.S. National Library of Medicine, National Institutes of Health, U.S. Department of Health & Human Services. November 25, 2012.
1535:
836:
2008:
541:
1528:
514:
2139:
510:
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gene, the disease is instead autosomal recessive, and both genes must be mutated for the disease to be inherited. Autosomal recessive onset is most common.
123:
skeletal muscle. This creates structural problems throughout the skeletal muscle and in the Z line of the sarcomere, creating the weakness in the muscle.
1979:
1521:
226:
1166:
Taratuto, AL; Matteucci, M; Barreiro, C; Saccolitti, M; Sevlever, G (1991). "Autosomal dominant neuromuscular disease with cylindrical spirals".
370:
Although tubular aggregates are seen in a variety of myopathies, both genetic and environmental, the congenital myopathies associated with genes
102:'Core myopathies' such as multicore myopathy and central core disease are characterized by sharply-demarcated areas devoid of oxidative enzymes
2003:
86:
and protein accumulation is a broad category, and some congenital myopathies that fall within this group are well understood, such as
840:
2033:
331:
have been shown to bind to cylindrical spirals. Cylindrical spirals have also been shown to react with the mitochondrial enzyme
257:
gene, the disorder is autosomal dominant, meaning it can be passed on by one mutated gene. When the mutation takes place in the
1692:
1649:
229:
region of muscle fibres. Nemaline rods have also been observed in the intermyofibrillar region of muscle fibres and within the
1741:
1697:
1621:
1585:
1100:
Malfatti, Edoardo; Chaves, Marcelo; Bellance, Remi; Sarrazin, Elizabeth; Fardeau, Michel; Romero, Norma (February 12, 2013).
1123:
Bove, KE; Iannaccone, ST; Hilton, PK; Samaha, F. (June 1980). "Cylindrical spirals in a familial neuromuscular disorder".
209:
90:(see below). Typically, the development error in this category occurs when muscle proteins aggregate and build up in the
65:
Is a congenital disorder, meaning it occurs during development and symptoms present themselves at birth or in early life.
1513:
338:
Phenotypes are quite variable, and manifestations can include weakness, abnormal gait, myotonia, cramps, and scoliosis.
1790:
1644:
979:
Carpenter, S.; Karpati, G.; Robitaille, Y.; Melmed, C. (Jul–Aug 1979). "Cylindrical spirals in human skeletal muscle".
1101:
179:
There are rarely any specific tests for the congenital myopathies except for muscle biopsy. Tests can be run to check
872:
594:
Ryan MM, Schnell C, Strickland CD, et al. (September 2001). "Nemaline myopathy: a clinical study of 143 cases".
1906:
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1974:
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155:
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A distinctive abnormality in skeletal muscle fibres on the cellular level; observable via light microscope
1734:
1702:
1414:
864:
755:"A New Congenital Non-Progressive Myopathy – Central core disease and nemaline myopathy in one family"
1998:
1659:
1969:
1472:
38:
2081:
332:
299:
107:
1984:
1877:
1796:
1549:
316:
275:
639:"Nemaline myopathy: current concepts. The ENMC International Consortium and Nemaline Myopathy"
2109:
1553:
205:
1102:"Cylindrical Spirals Congenital Myopathy Associated with Epileptic Encephalopathy (P07.050)"
1922:
559:
Shy, G. Milton; Engel, W. King; Somers, J. E.; Wanko, Theodor (1963). "Nemaline
Myopathy".
196:
The conditions included under the term "congenital myopathy" can vary. One source includes
52:
births every year. As a whole, congenital myopathies can be broadly classified as follows:
1501:
735:
8:
2091:
2063:
2051:
1669:
1543:
1461:
238:
201:
1363:
188:
congenital myopathies are genetic, there have been advancements in prenatal screenings.
2058:
1960:
1886:
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1714:
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1340:
1307:
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1213:
1191:
1148:
1082:
1039:
1004:
961:
912:
887:
813:
788:
712:
687:
663:
638:
619:
492:
437:
410:
234:
213:
1466:
2038:
2019:
1810:
1580:
1345:
1327:
1251:
1233:
1183:
1179:
1140:
1074:
996:
953:
917:
818:
754:
717:
668:
611:
576:
484:
442:
216:. The term can also be used more broadly, to describe conditions present from birth.
197:
77:
27:
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1008:
965:
2068:
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1132:
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1031:
988:
945:
907:
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658:
650:
623:
603:
568:
496:
476:
432:
422:
184:
83:
1870:
1853:
1477:
1387:
183:
in the blood, which is often normal or mildly elevated in congenital myopathies.
180:
1993:
1988:
1946:
1858:
1290:
688:"Nemaline myopathy caused by mutations in the muscle alpha-skeletal-actin gene"
1451:
1229:
1035:
949:
903:
480:
2133:
1865:
1827:
1677:
1590:
1331:
1237:
572:
324:
2045:
1804:
1349:
1255:
957:
804:
721:
615:
580:
488:
446:
427:
230:
1187:
1144:
1136:
1078:
992:
921:
822:
672:
2027:
1000:
773:
654:
144:
1815:
1723:
1574:
1323:
1070:
351:
335:, which suggests that cylindrical spirals originate from mitochondria.
91:
1434:
362:
is autosomal dominant myosin storage congenital myopathy-7A (CMYP7A).
1496:
934:
607:
355:
60:
32:
1415:
GeneReviews/NCBI/NIH/UW entry on
Congenital Fiber-Type Disproportion
1841:
1607:
1273:
1165:
703:
358:, forming protein aggregates. A myopathy associated with the gene
1308:"Genetic defects are common in myopathies with tubular aggregates"
753:
Afifi, Adel K.; Smith, James W.; Zellweger, Hans (April 1, 1965).
78:
Myopathies with inclusion bodies and abnormal protein accumulation
978:
1950:
1545:
1446:
467:
North, Kathryn (2008). "What's new in congenital myopathies?".
347:
320:
1099:
636:
378:
are known as tubular aggregate myopathy (TAM) types 1 and 2.
375:
371:
1056:
637:
North KN, Laing NG, Wallgren-Pettersson C (September 1997).
1388:"Phenotypic Series - PS160565 - Tubular aggregate myopathy"
1274:"Therapeutic approaches in different congenital myopathies"
1022:
Yan, C.Z.; Xu, J.W.; Zhao, Y.Y.; Li, W. (2014). "G.p.159".
359:
328:
279:
258:
254:
241:
genetic disorder. Sporadic cases have also been described.
103:
1304:
1122:
786:
686:
Ilkovski B, Cooper ST, Nowak K, et al. (June 2001).
111:
787:
Denborough, M. A.; Dennett, X; Anderson, R. M. (1973).
515:
National
Institute of Neurological Disorders and Stroke
274:
Central core disease has been found to be allelic with
126:
1272:
Gineste, Charlotte; Laporte, Jocelyn (February 2023).
285:
593:
1424:
1364:"Tubular aggregates: Other occasional associations"
839:. Muscular Dystrophy Campaign. 2012. Archived from
752:
685:
558:
1212:Tajsharghi, Homa; Oldfors, Anders (January 2013).
1211:
789:"Central-core disease and malignant hyperpyrexia"
117:
2131:
885:
309:
1312:Annals of Clinical and Translational Neurology
1271:
886:Myong, NH; Kang, YK; Chi, JG; Suk, SI (1993).
404:
402:
400:
2140:Myoneural junction and neuromuscular diseases
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1021:
397:
1536:
1522:
1214:"Myosinopathies: pathology and mechanisms"
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97:
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511:"Congenital Myopathy Information Page"
244:
1517:
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1265:
1207:
1205:
865:"Congenital fiber-type disproportion"
539:
466:
294:
94:, which leads to muscle dysfunction.
517:. September 30, 2011. Archived from
503:
453:
219:
139:
127:Myopathies with fiber size variation
888:"Multicore myopathy--a case report"
854:
286:Congenital fiber type disproportion
249:Myotubular myopathy, also known as
210:congenital fiber type disproportion
13:
1797:BIN1-linked centronuclear myopathy
1408:
1262:
1202:
692:American Journal of Human Genetics
319:in some patients seems likely, as
14:
2151:
2078:Testosterone deficiency myopathy
1586:Lambert–Eaton myasthenic syndrome
1420:
892:Journal of Korean Medical Science
873:U.S. National Library of Medicine
415:Orphanet Journal of Rare Diseases
72:
2034:Kocher–Debre–Semelaigne syndrome
1683:Limb-girdle muscular dystrophy 2
1640:Limb-girdle muscular dystrophy 1
1112:. American Academy of Neurology.
143:
59:Symptoms of muscle weakness and
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1356:
1298:
1278:Current Opinion in Pharmacology
1159:
1116:
1093:
1050:
1015:
972:
928:
879:
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548:. Merck Sharp & Dohme Corp.
1975:Fatty-acid metabolism disorder
1598:Congenital myasthenic syndrome
728:
679:
630:
587:
552:
533:
346:In myosin storage myopathies,
118:Myopathies with central nuclei
1:
2087:Hypogonadotropic hypogonadism
390:
1180:10.1016/0960-8966(91)90006-e
381:
310:Cylindrical spirals myopathy
174:
7:
643:Journal of Medical Genetics
82:Congenital myopathies with
10:
2156:
1291:10.1016/j.coph.2022.102328
837:"Fibre-type disproportion"
366:Tubular aggregate myopathy
233:. Nemaline myopathy is an
2102:
2064:Hyperparathyroid myopathy
2018:
1959:
1935:
1915:
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1840:
1826:
1754:
1713:
1668:
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1230:10.1007/s00401-012-1024-2
1036:10.1016/j.nmd.2014.06.189
950:10.1016/j.nmd.2003.11.007
904:10.3346/jkms.1993.8.4.312
540:Rubin, M. (August 2009).
481:10.1016/j.nmd.2008.04.002
409:Jungbluth, Heinz (2007).
26:
21:
2059:Hypoparathyroid myopathy
1970:Glycogen storage disease
736:"Centronuclear myopathy"
269:Central core disease or
191:
135:
2082:Late-onset hypogonadism
2073:Corticosteroid myopathy
1791:adaptor protein disease
1368:neuromuscular.wustl.edu
1168:Neuromuscular Disorders
1024:Neuromuscular Disorders
938:Neuromuscular Disorders
869:Genetics Home Reference
793:British Medical Journal
740:Genetics Home Reference
542:"Congenital Myopathies"
469:Neuromuscular Disorders
342:Myosin storage myopathy
333:succinate dehydrogenase
1985:Mitochondrial myopathy
1878:Paramyotonia congenita
1550:neuromuscular junction
805:10.1136/bmj.1.5848.272
573:10.1093/brain/86.4.793
428:10.1186/1750-1172-2-25
411:"Central core disease"
317:sarcoplasmic reticulum
276:malignant hyperthermia
251:centronuclear myopathy
152:This section is empty.
68:Is a genetic disorder.
2110:Inflammatory myopathy
1554:neuromuscular disease
1218:Acta Neuropathologica
1137:10.1002/ana.410070608
1059:Acta Neuropathologica
993:10.1002/mus.880020407
271:central core myopathy
206:central core myopathy
98:Myopathies with cores
1923:Central core disease
1805:cytoskeleton disease
774:10.1212/WNL.15.4.371
655:10.1136/jmg.34.9.705
521:on February 13, 2007
350:accumulates beneath
302:also referred to as
265:Central core disease
114:, in muscle fibres.
2115:Congenital myopathy
2092:Androgen deficiency
2052:Thyrotoxic myopathy
1650:Facioscapulohumeral
1125:Annals of Neurology
875:. December 2, 2012.
596:Annals of Neurology
245:Myotubular myopathy
239:autosomal recessive
202:myotubular myopathy
49:Congenital myopathy
22:Congenital Myopathy
1961:Metabolic myopathy
1887:Periodic paralysis
1849:Myotonia congenita
1617:Muscular dystrophy
1488:External resources
1324:10.1002/acn3.51477
1071:10.1007/bf00318582
300:Multicore myopathy
295:Multicore myopathy
235:autosomal dominant
214:multicore myopathy
2127:
2126:
2123:
2122:
2039:Hoffmann syndrome
1931:
1930:
1811:Nemaline myopathy
1750:
1749:
1581:Myasthenia gravis
1511:
1510:
304:minicore myopathy
237:and sometimes an
220:Nemaline myopathy
198:nemaline myopathy
172:
171:
88:nemaline myopathy
46:
45:
16:Medical condition
2147:
2069:Hypercortisolism
1980:AMPD1 deficiency
1936:ATPase disorder
1838:
1837:
1769:Bethlem myopathy
1763:collagen disease
1755:Other structural
1628:
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1566:junction disease
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185:Electromyography
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154:You can help by
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84:inclusion bodies
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2014:
1955:
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1911:
1881:
1871:Isaacs syndrome
1854:Thomsen disease
1822:
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1645:Oculopharyngeal
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1409:Further reading
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1859:Becker disease
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1562:Neuromuscular-
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1429:Classification
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1421:External links
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927:
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828:
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745:
727:
704:10.1086/320605
698:(6): 1333–43.
678:
629:
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567:(4): 793–810.
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73:Classification
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1971:
1967:
1966:
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1958:
1952:
1948:
1947:Brody disease
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1866:Neuromyotonia
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1832:(ion channel)
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1828:Channelopathy
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1678:Calpainopathy
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1174:(6): 433–41.
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1068:
1064:
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1030:(9–10): 849.
1029:
1025:
1018:
1010:
1006:
1002:
998:
994:
990:
986:
982:
975:
967:
963:
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955:
951:
947:
944:(3): 208–16.
943:
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923:
919:
914:
909:
905:
901:
897:
893:
889:
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843:on 2013-03-29
842:
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824:
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709:
705:
701:
697:
693:
689:
682:
674:
670:
665:
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649:(9): 705–13.
648:
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633:
625:
621:
617:
613:
609:
605:
602:(3): 312–20.
601:
597:
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582:
578:
574:
570:
566:
562:
555:
547:
546:Merck Manuals
543:
536:
520:
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498:
494:
490:
486:
482:
478:
475:(6): 433–42.
474:
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325:calsequestrin
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163:December 2017
157:
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105:
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93:
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85:
67:
64:
62:
58:
55:
54:
53:
50:
40:
34:
31:
29:
25:
20:
2114:
2046:Hyperthyroid
1907:Hyperkalemic
1803:
1789:
1783:X-linked MTM
1775:
1761:
1722:
1573:
1544:Diseases of
1495:
1471:
1456:
1441:
1395:. Retrieved
1392:www.omim.org
1391:
1382:
1371:. Retrieved
1367:
1358:
1315:
1311:
1300:
1281:
1277:
1221:
1217:
1171:
1167:
1161:
1131:(6): 550–6.
1128:
1124:
1118:
1109:
1105:
1095:
1065:(6): 660–4.
1062:
1058:
1052:
1027:
1023:
1017:
987:(4): 282–7.
984:
981:Muscle Nerve
980:
974:
941:
937:
930:
898:(4): 312–7.
895:
891:
881:
868:
845:. Retrieved
841:the original
831:
796:
792:
782:
765:
761:
748:
739:
730:
695:
691:
681:
646:
642:
632:
599:
595:
589:
564:
560:
554:
545:
535:
523:. Retrieved
519:the original
505:
472:
468:
418:
414:
385:
369:
354:and between
345:
337:
313:
303:
298:
289:
270:
268:
250:
248:
223:
195:
178:
160:
156:adding to it
151:
130:
121:
101:
87:
81:
48:
47:
2028:Hypothyroid
1895:Hypokalemic
1777:PTP disease
1318:(1): 4–15.
1224:(1): 3–18.
525:January 23,
1939:(ion pump)
1900:Thyrotoxic
1816:Zaspopathy
1724:dystrophin
1688:Congenital
1575:autoimmune
1397:2023-11-11
1373:2023-11-11
1284:: 102328.
847:2012-11-27
768:(4): 371.
391:References
356:myofibrils
352:sarcolemma
92:sarcoplasm
2048:myopathy
2030:myopathy
1497:eMedicine
1332:2328-9503
1238:1432-0533
1106:Neurology
762:Neurology
382:Treatment
175:Diagnosis
61:hypotonia
33:Neurology
28:Specialty
2134:Category
1842:Myotonia
1735:Duchenne
1730:Becker's
1693:Fukuyama
1655:Myotonic
1608:Myopathy
1350:34908252
1256:22918376
1196:39873188
1153:32326168
1087:12346602
1044:54312844
1009:46064876
966:41282763
958:15036331
722:11333380
616:11558787
581:14090530
489:18482838
447:17504518
2103:General
1968:Muscle
1698:Ullrich
1478:D020914
1341:8791796
1247:3535372
1188:1822355
1145:7436360
1079:8615090
922:8198769
913:3053753
823:4265427
814:1588091
713:1226120
673:9321754
664:1051052
624:8648543
497:5490760
438:1887524
231:nucleus
1951:ATP2A1
1552:, and
1546:muscle
1348:
1338:
1330:
1254:
1244:
1236:
1194:
1186:
1151:
1143:
1085:
1077:
1042:
1007:
1001:492204
999:
964:
956:
920:
910:
821:
811:
720:
710:
671:
661:
622:
614:
579:
495:
487:
445:
435:
421:: 25.
348:myosin
327:, and
321:SERCA1
282:gene.
212:, and
110:, and
35:
2009:MNGIE
1994:MERRF
1989:MELAS
1916:Other
1467:359.0
1452:G71.2
1192:S2CID
1149:S2CID
1083:S2CID
1040:S2CID
1005:S2CID
962:S2CID
758:(PDF)
620:S2CID
561:Brain
493:S2CID
376:ORAI1
372:STIM1
192:Types
136:Cause
1622:DAPC
1473:MeSH
1462:9-CM
1346:PMID
1328:ISSN
1252:PMID
1234:ISSN
1184:PMID
1141:PMID
1075:PMID
997:PMID
954:PMID
918:PMID
819:PMID
718:PMID
669:PMID
612:PMID
577:PMID
527:2007
485:PMID
443:PMID
374:and
360:MYH7
329:RYR1
280:RYR1
259:BIN1
255:DNM2
104:NADH
2004:PEO
1999:KSS
1458:ICD
1443:ICD
1336:PMC
1320:doi
1286:doi
1242:PMC
1226:doi
1222:125
1176:doi
1133:doi
1067:doi
1032:doi
989:doi
946:doi
908:PMC
900:doi
809:PMC
801:doi
770:doi
708:PMC
700:doi
659:PMC
651:doi
604:doi
569:doi
477:doi
433:PMC
423:doi
158:.
112:COX
108:SDH
2136::
1715:XR
1670:AR
1632:AD
1548:,
1500::
1476::
1465::
1450::
1447:10
1390:.
1366:.
1344:.
1334:.
1326:.
1314:.
1310:.
1282:68
1280:.
1276:.
1264:^
1250:.
1240:.
1232:.
1220:.
1216:.
1204:^
1190:.
1182:.
1170:.
1147:.
1139:.
1127:.
1110:80
1108:.
1104:.
1081:.
1073:.
1063:90
1061:.
1038:.
1028:24
1026:.
1003:.
995:.
983:.
960:.
952:.
942:14
940:.
916:.
906:.
894:.
890:.
871:.
867:.
856:^
817:.
807:.
795:.
791:.
766:15
764:.
760:.
738:.
716:.
706:.
696:68
694:.
690:.
667:.
657:.
647:34
645:.
641:.
618:.
610:.
600:50
598:.
575:.
565:86
563:.
544:.
513:.
491:.
483:.
473:18
471:.
455:^
441:.
431:.
417:.
413:.
399:^
323:,
208:,
204:,
200:,
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2011:)
1987:(
1953:)
1949:(
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1288::
1258:.
1228::
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1129:7
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902::
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702::
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479::
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425::
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165:)
161:(
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