Neuromuscular

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SPORADIC ALS

Sporadic ALS: Common forms
  Clinical features
    Weakness
    Upper motor neuron
    Spontaneous activity
    Behavioral
    Prognosis
    Treatment
  Diagnostic criteria
  Differential diagnosis
  Discussion of diagnosis
  Epidemiology
    Clinical
    Genetic
  History
  Laboratory features
    Susceptibility loci
  Pathology

ALS Variants
  Hereditary ALS
  Primary lateral sclerosis
  Primary muscular atrophy
  Pseudobulbar paralysis
  Flail arm

Multisystem disorders
  ALS + PNL
  + EOM & Extrapyramidal Δ
  Fronto-Temporal Dementia
  Multiple system atrophy
  Polyglucosan body disease
  Western Pacific ALS
    ALS-PD1
    ALS-PD2

MRI: FLAIR
Motor Neuron Disease (Late)
Cerebral Peduncles: Hyperintense

From NLM
Jean Martin Charcot
Gehrig & Ruth
Lou Gehrig &
Babe Ruth

Sporadic ALS: Common forms


ALS-like disorders with Fronto-Temporal Dementia (FTD) 11


Western Pacific ALS-like disorders (ALS-PD) 6


Atypical Motor Neuron Disease with Ophthalmoplegia & Extrapyramidal Disorders 4


Also see Hereditary ALS


Amyotrophic Lateral Sclerosis (ALS): Discussion of the Diagnosis 1

Objectives of initial counseling Steps in the discussion

Motor Neuron Disease: MRI changes

Early: 6 months of symptoms
  T2 signal in corticospinal tracts (Arrows)

Late in course
  Hyperintense signal on MRI (FLAIR)

Medulla

Pons

Midbrain

Internal capsule

ALS: Early history of concepts


Patient information

Muscular Dystrophy Association
3300 East Sunrise Drive
Tucson, AZ 85718
Telephone 520-529-2000
FAX 520-529-5300

ALS Association (National Office)
21021 Ventura Boulevard, Suite 321
Woodland Hills, CA 91364-2206 USA
Telephone 800-782-4747

ALS Society of Canada
220 - 6 Adelaide Street East
Toronto, Ontario, M5C 1H6 CANADA
Telephone 416-362-0269
Telephone 800-267-4257 (toll-free in Canada)
FAX 416-362-0414
e-mail: alssoc@inforamp.net


Creatine Protocols: For ALS & Neuromuscular Disorders


Return to Motor syndromes
Go to Hereditary ALS
Return to Neuromuscular home page

References
1. J Neurol Sci 1998;160:S127-S133
2. Neuromuscular Disorders 1999;9:593-597
3. Neurology 1999;52:854, Neurology 2000;54:1848-1850
4. Acta Neuropath 2000;100:342-346 1999;98:512-515
5. Neurology 2001;56:753-757
6. Brain 2001;124:2215-2222, Proc Natl Acad Sci U S A 2023;120:e2220984120
7. J Neurol Sci 2001;191:3-9, Neurology 2002;59:280–282
8. Am J Hum Genet 2002;May
9. Neurology 2002;59:99-103, Lancet Neurol 2018;17:423-433
10. Neurology 2002;59:773-775, Am J Hum Genet 2004;75 On-line October, J Neurol 2014 Jul 25, Brain 2023 Apr 12
11. Neurology 2002;59:1077–1079, Acta Neuropathol 2002; July On-Line, Acta Neuropathol 2002; October On-Line
12. Ann Neurol 2002;52:448-457
13. Am J Epidemiol 2003;157:149-157
14. Neurology 2003;60:1348–1350
15. J Neurol 2003;250:473–479
16. J NeurolSci 2003;209:1-4, Muscle Nerve 2018 Apr 20, Ann Neurol 2024 Jan 29
17. J Neurol Neurosurg Psychiatry 2003;74:995–997
18. Nature Genet 2003; Online July
19. Neurology 2003;61:387–389, Neurology 2003;61:291–292
20. Neurology 2004;62:1753–1757
21. Am J Epidemiol 2004;160:26-33, Neurology 2009;73:1693–1698
22. J Neurol Neurosurg Psychiatry 2006;77;388-389
23. Neurology 2006;67:1833–1836
24. Ann Neurol 2007;61:427-434
25. Neurology 2008; Online January
26. Science 2008;319;20, Hum Mol Genet 2011; Jun 10
27. J Neurol Sci 2008 Jun 11
28. Nat Clin Pract Neurol 2008;4:366-374
29. Amyotroph Lateral Scler 2008 Jun 12:1-6
30. Amyotroph Lateral Scler 2008 May 2:1-9
31. Neurology 2008; Online July, Neurology 2013;80: Online April
32. Am J Hum Genetics 2008; Online December
33. Hum Molec Genet 2009; Online July 23
34. Nature Genet 2009; Online September, Neurobiology of Aging 2012; Online August, Ann Neurol 2014 Jun 16, Amyotroph Lateral Scler Frontotemporal Degener 2016 Sep 1:1-7, Front Aging Neurosci 2023;15:1067954
35. Brain 2010 Oct 19
36. J Neurol Neurosurg Psychiatry 2010 Nov 3
37. Journal of Neurological Sciences 2011; Online January
38. J Neuropathol Exp Neurol 2011;70:349-359
39. Acta Neuropathol 2011 May 28
40. J Neurol Neurosurg Psychiatry 2011;82:740-746
41. Exp Neurol 2011 Aug 30
42. Neurobiology of Aging 2011; Online Sept
43. Arch Neurol 2011;68:1440-1446, Neurology 2012; Online September, Neurology 2013; Online January
44. Brain 2011; On line December
45. Nature Medicine 2012; Online August
46. J Neurol Sci 2012; Online Nov
47. Nature Genet 2013; Online April
48. Neurobiol Dis 2013; Apr 29
49. Neuropathology 2013; Online May
50. JAMA Neurol 2014 Feb 17
51. Ann Neurol 2014 Nov
52. Ann Clin Transl Neurol 2015;2:29-37
53. Ann Neurol 2015; Online Jan
54. JAMA Neurol 2015; Jun 1
55. JAMA Neurol 2015; Jun 1
56. Neurology 2015; Online July, Front Neurol 2023;14:1043136
57. Muscle Nerve 2015 Sep 2
58. Epidemiology 2015;26:831-838
59. JNNP 2016; Online Jan
60. Brain 2016 Mar 5, Nature Genetics 2016; Online July
61. Neurology 2016; Online May
62. Am J Human Genet 2016; Online August
63. J Neurol Neurosurg Psychiatry 2017 Mar 14
64. Clin Neurophysiol 2017;128:823-829
65. Muscle Nerve 2017 Mar 25
66. J Alzheimers Dis 2017 Apr
67. PLoS One 2017;12:e0182638
68. Neurology 2017 Aug 23
69. Neurology 2017; Online October
70. Nat Genet 2016;48:1043-1048
71. Neurology 2018 Mar 30
72. Neurol Neurochir Pol 2018;52:124-128
73. J Neurol Neurosurg Psychiatry 2018 Apr 17
74. Am J Med Sci 2015;350:95-97
75. Lancet Neurol 2018;17:416-422
76. PLoS One 2018;13:e0192243
77. Muscle Nerve 2018 Mar 6
78. J Neurol Neurosurg Psychiatry 2018 Apr 23
79. Aging Dis 2019;10:278-292
80. Front Neurol 2019;9:1167, Neurodegener Dis 2018;18:255-261, Biomed Pap Med Fac Univ Palacky Olomouc Czech Repub 2017;161:286-295
81. J Neurol Sci 2019 Jun 6
82. Nat Neurosci 2019 Nov 25, Neurobiol Aging. 2021;106:314.e1-314.e6, Neurobiol Aging 2021 Dec 11
83. Ann Neurol 2019 Nov 26
84. Ann Neurol 2020 Jan 9
85. Global Spine J 2019;9:104-118
86. Ann Neurol 2020 Jul 6
87. Neurobiol Aging 2020 Jun 25
88. Cell Rep 2020;33:108323
89. Ann Neurol 2021 Jan 2
90. Acta Neurol Belg 2021 Jul 9
91. Amyotroph Lateral Scler Frontotemporal Degener 2021 Aug 14
92. Pulmonology 2020;26:45–48
93. Muscle Nerve 2021 Dec 24
94. Cell Rep 2019;29:1164-1177
95. Nat Neurosci 2022 Mar 31
96. Ann Neurol 2022 Aug 23
97. Nat Commun 2023;14:342
98. Ann Neurol 2023 Jan 10
99. J Neurol 2023 May 6
100. Ann Neurol 2023 Aug 1
101. Brain 2023 Dec 20
102. Eur J Neurol 2023 Nov 17
103. Front Neurol 2018;9:147

1/11/2024