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논문 기본 정보
- 자료유형
- 학술저널
- 저자정보
- 발행연도
- 2015.1
- 수록면
- 351 - 357 (7page)
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Most amyotrophic lateral sclerosis (ALS) patients show focal onset of upper and lower motor neuron signs and spread of symptoms to other regions or the other side clinically. Progression patterns of sporadic ALS are unclear. The aim of this study was to evaluate the pattern of respiratory deterioration in sporadic ALS according to the onset site by using respiratory function tests. Study participants included 63 (42 cervical-onset [C-ALS] and 21 lumbosacral-onset [L-ALS]) ALS patients and 31 healthy controls. We compared respiratory function test parameters among the 3 groups. Age was 57.4±9.6 (mean±SD), 60.8±9, and 60.5±7 years, and there were 28, 15, and 20 male participants, in the C-ALS, L-ALS, and control groups, respectively. Disease duration did not differ between C-ALS and L-ALS patients. Sniff nasal inspiratory pressure (SNIP) was significantly low in C-ALS patients compared with controls. Maximal expiratory pressure (MEP) and forced vital capacity percent predicted (FVC% predicted) were significantly low in C-ALS and L-ALS patients compared with controls. Maximal inspiratory pressure to maximal expiratory pressure (MIP:MEP) ratio did not differ among the 3 groups. Eighteen C-ALS and 5 L-ALS patients were followed up. ΔMIP, ΔMEP, ΔSNIP, ΔPEF, and ΔFVC% predicted were higher in C-ALS than L-ALS patients without statistical significance. Fourteen C-ALS (77.8%) and 3 L-ALS (60%) patients showed a constant MIP:MEP ratio above or below 1 from the first to the last evaluation. Our results suggest that vulnerability of motor neurons in sporadic ALS might follow a topographic gradient.
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참고문헌 (25)
참고문헌 신청
Brooks BR / 2000 / El Escorial revisited: revised criteria for the diagnosis of amyotrophic lateral sclerosis / Amyotroph Lateral Scler Other Motor Neuron Disord 1:293~299
Ravits J / 2007 / Focality of upper and lower motor neuron degeneration at the clinical onset of ALS / Neurology 68:1571~1575
Kanouchi T / 2012 / Can regional spreading of amyotrophic lateral sclerosis motor symptoms be explained by prion-like propagation? / J Neurol Neurosurg Psychiatry 83:739~745
Sekiguchi T / 2014 / Spreading of amyotrophic lateral sclerosis lesions--multifocal hits and local propagation? / J Neurol Neurosurg Psychiatry 85:85~91
Mackenzie IR / 2007 / Pathological TDP-43 distinguishes sporadic amyotrophic lateral sclerosis from amyotrophic lateral sclerosis with SOD1mutations / Ann Neurol 61:427~434
Ravits J / 2007 / Focality of upper and lower motor neuron degeneration at the clinical onset of ALS / Neurology 68:1571~1575
Kanouchi T / 2012 / Can regional spreading of amyotrophic lateral sclerosis motor symptoms be explained by prion-like propagation? / J Neurol Neurosurg Psychiatry 83:739~745
Sekiguchi T / 2014 / Spreading of amyotrophic lateral sclerosis lesions--multifocal hits and local propagation? / J Neurol Neurosurg Psychiatry 85:85~91
Mackenzie IR / 2007 / Pathological TDP-43 distinguishes sporadic amyotrophic lateral sclerosis from amyotrophic lateral sclerosis with SOD1mutations / Ann Neurol 61:427~434
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