Measures and markers in amyotrophic lateral sclerosis.
Amyotrophic Lateral Sclerosis (ALS) is a neurodegenerative disorder characterized by loss of spinal and cortical motor neurons, leading to progressive weakness and ultimately, death. Clinically, there appears to be an anatomic focus at disease onset, from which the disease then spreads. Because the focus of initial symptoms and the subsequent direction of spread can vary from patient to patient, disease monitoring is difficult, especially in a clinical trial, in which outcome measures must be identical and able to capture progression of all types. Thus, the search for markers of disease progression is especially important in ALS. Many approaches have been taken, from voluntary strength assessment and functional rating scales to physiological and pathological sampling of affected portions of nervous system. No proposed marker has been demonstrated to meet the desired criteria of biological meaning, sensitivity to disease progression, clear relationship to overall prognosis and survival, and ease of measurement. However, progress is being made in all of these regards.
Cudkowicz
Merit
M
Neurology Clinical Trial Unit, Massachusetts General Hospital, Charlestown, Massachusetts 02129, USA. mcudkowicz@partners.org
Qureshi
Muhammad
M
Shefner
Jeremy
J
D000690:Amyotrophic Lateral Sclerosis / Q000175:diagnosis*; D015415:Biomarkers; D004568:Electrodiagnosis; D006801:Humans; D009682:Magnetic Resonance Spectroscopy; D008280:Magnetics
D015415:Biomarkers
Acta Neuropathol. 2002 Jun;103(6):583-9
12012090
Exp Neurol. 2001 Dec;172(2):377-82
11716561
Muscle Nerve. 1993 Dec;16(12):1326-31
8232388
Clin Neurophysiol. 2001 Dec;112(12):2190-201
11738189
Acta Neurol Scand. 2002 Nov;106(5):299-301
12371924
Neurology. 1994 Nov;44(11):2027-31
7969954
Neurology. 1997 Nov;49(5):1292-8
9371911
Neurology. 1997 Aug;49(2):573-8
9270599
J Neurol Neurosurg Psychiatry. 1973 Apr;36(2):194-8
4708453
Neurology. 2003 Aug 26;61(4):456-64
12939417
Ann Neurol. 1996 Jun;39(6):796-800
8651652
J Neurol Neurosurg Psychiatry. 1971 Apr;34(2):121-31
5571599
Ann Neurol. 1987 Sep;22(3):328-33
3118763
Neurology. 2001 Apr 10;56(7):843-8
11294919
J Anat. 2001 Dec;199(Pt 6):699-708
11787823
Proc Natl Acad Sci U S A. 1998 Dec 22;95(26):15763-8
9861044
Muscle Nerve. 1991 May;14(5):416-21
1870632
J Neurol Neurosurg Psychiatry. 1972 Dec;35(6):845-52
4647858
Brain Pathol. 2003 Jan;13(1):10-22
12580541
Muscle Nerve. 1999 Mar;22(3):299-306
10086890
Brain. 2004 Feb;127(Pt 2):340-50
14607785
Neurology. 1996 Dec;47(6):1383-8
8960715
Muscle Nerve. 1999 Oct;22(10):1457-60
10487915
Muscle Nerve. 1993 Nov;16(11):1213-9
8413373
J Neurol Sci. 1999 Oct 31;169(1-2):13-21
10540002
J Neurol Sci. 1994 Jul;124 Suppl:96-107
7807156
Phys Ther. 1993 Dec;73(12):868-77
8248295
AJNR Am J Neuroradiol. 2003 Mar;24(3):476-80
12637300
J Neurol Neurosurg Psychiatry. 1979 Jan;42(1):42-51
216781
J Clin Neurophysiol. 1995 Nov;12(6):585-94
8600173
Brain. 2001 Oct;124(Pt 10):2000-13
11571218
Amyotroph Lateral Scler Other Motor Neuron Disord. 2003 Apr;4(1):22-6
12745614
J Neurol Sci. 1998 Oct;160 Suppl 1:S6-24
9851643
Radiology. 1999 Sep;212(3):763-9
10478245
Muscle Nerve. 1997 Feb;20(2):179-85
9040656
Arch Neurol. 2001 May;58(5):729-35
11346367
Cell. 2001 Jun 1;105(5):587-97
11389829
Muscle Nerve. 1999 Nov;22(11):1571-5
10514236
Arch Neurol. 2001 May;58(5):714-6
11346364
Science. 1994 Jun 17;264(5166):1772-5
8209258
Nature. 1993 Mar 4;362(6415):59-62
8446170
Ann Neurol. 1990 Jul;28(1):18-25
2375630
Muscle Nerve. 2000 Jun;23(6):874-82
10842262
Neurology. 2003 Apr 22;60(8):1348-50
12707441
Ann Neurol. 1994 Feb;35(2):142-50
8109895
J Neurol. 2001 Oct;248(10):850-5
11697520
Brain. 1995 Jun;118 ( Pt 3):707-19
7600088
Ann Neurol. 1995 Sep;38(3):460-3
7668834
Ann Neurol. 2003 Jul;54(1):130-4
12838531
Neurology. 1998 Aug;51(2):603-5
9710047
Adv Neurol. 2002;88:33-52
11908235
Ann Neurol. 2003 Apr;53(4):437-45
12666111
Amyotroph Lateral Scler Other Motor Neuron Disord. 2000 Jun;1 Suppl 2:S7-16
11464945
Neurology. 1988 Mar;38(3):409-13
3347345
Ann Neurol. 1987 Dec;22(6):744-52
3435084
Muscle Nerve. 2000 Feb;23(2):193-7
10639610
J Neurol Neurosurg Psychiatry. 1978 Sep;41(9):773-83
690647
Proc Natl Acad Sci U S A. 2000 Dec 5;97(25):13901-6
11095709
Neurology. 1998 Jun;50(6):1800-5
9633731
J Neuroimaging. 2001 Jul;11(3):293-7
11462297
Muscle Nerve. 1998 Jun;21(6):762-8
9585330
Lancet. 1996 Aug 3;348(9023):334-5
8709704
J Neurol Sci. 1979 May;41(3):287-97
438856
Ann Neurol. 1997 Oct;42(4):644-54
9382477
Muscle Nerve. 1997 Jun;20(6):691-5
9149075
Muscle Nerve. 2002 May;25(5):709-714
11994965
J Biol Chem. 2002 May 3;277(18):15923-31
11854284
Can J Neurol Sci. 1993 Feb;20(1):11-6
8096792
Acta Neurol Scand. 1993 Jul;88(1):51-5
8372631
Neuroreport. 2000 Nov 9;11(16):3419-22
11095490
Adv Neurol. 1991;56:521-46
1853781
Neurology. 2003 Feb 11;60(3):500-2
12578937
Acta Neuropathol. 2002 Mar;103(3):243-7
11907804
Nat Neurosci. 1999 May;2(5):427-33
10321246
Nat Genet. 1994 May;7(1):79-84
8075647
Am Rev Respir Dis. 1969 May;99(5):696-702
5772056
Adv Neurol. 1982;36:15-22
7180681
J Neurol Sci. 1998 Oct;160 Suppl 1:S37-41
9851647
Neuron. 1998 Mar;20(3):589-602
9539131
N Engl J Med. 1994 Mar 3;330(9):585-91
8302340
Nat Rev Neurosci. 2001 Nov;2(11):806-19
11715057
Muscle Nerve. 1999 Sep;22(9):1265-70
10454724
J Neurol. 2002 Dec;249(12):1723-8
12529797
Qual Life Res. 2001;10(2):149-58
11642685
Clin Neurophysiol. 2001 Jun;112(6):955-64
11377252
Rinsho Shinkeigaku. 1995 Dec;35(12):1543-5
8752458
AJNR Am J Neuroradiol. 2000 Apr;21(4):647-58
10782773
Ann Neurol. 2002 Feb;51(2):243-6
11835381
Neurology. 2001 Jul 24;57(2):357-9
11468332
Am J Hum Genet. 1998 Mar;62(3):633-40
9497266
Neurology. 1998 Jan;50(1):66-72
9443459
Neurology. 2003 Dec 9;61(11):1503-7
14663032
Neuroradiology. 2001 Mar;43(3):189-97
11305749
Am J Hum Genet. 2002 Jan;70(1):251-6
11706389
Phys Ther. 1984 Jan;64(1):41-5
6361809
Ann Neurol. 1998 May;43(5):640-4
9585359
J Neurol Sci. 1996 Aug;139 Suppl:34-7
8899655
Neurology. 1986 Jul;36(7):937-41
3714055
Neurology. 1999 Sep 22;53(5):1051-8
10496265
Nat Genet. 2003 Apr;33(4):455-6
12627231
Muscle Nerve. 1993 Jun;16(6):624-33
8502260
Neurology. 1988 Mar;38(3):405-8
3347344
Lancet. 1996 May 25;347(9013):1425-31
8676624
Neurology. 1977 Oct;27(10):981-4
561913
Muscle Nerve. 2000 Oct;23(10):1525-33
11003787
Amyotroph Lateral Scler Other Motor Neuron Disord. 2003 Apr;4(1):17-21
12745613
Lancet. 1996 Jan 20;347(8995):159-60
8544551
Neurol Clin. 1987 Feb;5(1):125-41
3550414
Acta Neuropathol. 2002 Sep;104(3):249-59
12172910
J Neurol Sci. 1995 Oct;132(2):126-8
8543936
Nat Genet. 2001 Oct;29(2):166-73
11586298
Arch Neurol. 1999 Jun;56(6):710-2
10369311
Ann Neurol. 1998 Oct;44(4):696-9
9778272
Muscle Nerve. 1995 Oct;18(10):1139-46
7659108
Nat Genet. 2001 Oct;29(2):160-5
11586297
Nature. 1993 Sep 2;365(6441):27-32
8361533
Muscle Nerve. 1997 Sep;20(9):1137-41
9270669
Brain. 1997 Aug;120 ( Pt 8):1415-21
9278631
Neurology. 1994 Oct;44(10):1933-8
7936250
Arch Neurol. 1996 Feb;53(2):141-7
8639063
J Neurol Sci. 1996 Aug;139 Suppl:38-42
8899656
Neurology. 1996 May;46(5):1244-9
8628460
Free Radic Biol Med. 2000 Oct 1;29(7):652-8
11033417
Phys Med Rehabil Clin N Am. 2003 May;14(2):243-60
12795515
Neurology. 1999 Jul 13;53(1):71-9
10408539
Ann Neurol. 1996 Feb;39(2):256-60
8967757
Neurology. 2002 Mar 12;58(5):773-9
11889242