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TRANSCRIPT
![Page 1: ç ‰éł»åł³å¡¾2015å¹´12朋è⁄ªçŽºæfl¾éł».pptx [ã…¦ã ... · 2018. 2. 26. · 5*7Á H °Fû FÖ q ·7Á )H H °7Á )H • ö ²Fû G" ¤FÔFï'½Fþ](https://reader035.vdocuments.site/reader035/viewer/2022071420/6119d121e6e3a722bd6d07d3/html5/thumbnails/1.jpg)
自発放電と運動単位電位
神戸市立医療センター 中央市民病院神経内科
幸 原 伸 夫
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(1)自発放電
(2)運動単位電位
針筋電図では具体的に何をみるか?
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• 針電極刺入に伴う活動電位(刺入電位)
• 完全に力を抜いた筋の安静時電位
自発放電の有無
自発放電にはどんなものがあるか?
針が動くことで誘発、持続、あるいは自然に生じる異常興奮
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自発放電の解剖学的起源は?
Fibrillation potentialPositive sharp waveMyotonic dischargeComprex repetitive dischargeFasciculation potentialMyokymic potential etc
![Page 5: ç ‰éł»åł³å¡¾2015å¹´12朋è⁄ªçŽºæfl¾éł».pptx [ã…¦ã ... · 2018. 2. 26. · 5*7Á H °Fû FÖ q ·7Á )H H °7Á )H • ö ²Fû G" ¤FÔFï'½Fþ](https://reader035.vdocuments.site/reader035/viewer/2022071420/6119d121e6e3a722bd6d07d3/html5/thumbnails/5.jpg)
1 筋線維
2 神経末端
![Page 6: ç ‰éł»åł³å¡¾2015å¹´12朋è⁄ªçŽºæfl¾éł».pptx [ã…¦ã ... · 2018. 2. 26. · 5*7Á H °Fû FÖ q ·7Á )H H °7Á )H • ö ²Fû G" ¤FÔFï'½Fþ](https://reader035.vdocuments.site/reader035/viewer/2022071420/6119d121e6e3a722bd6d07d3/html5/thumbnails/6.jpg)
Spontaneous activities (1)
単一筋線維
複数の筋線維
筋線維起源
![Page 7: ç ‰éł»åł³å¡¾2015å¹´12朋è⁄ªçŽºæfl¾éł».pptx [ã…¦ã ... · 2018. 2. 26. · 5*7Á H °Fû FÖ q ·7Á )H H °7Á )H • ö ²Fû G" ¤FÔFï'½Fþ](https://reader035.vdocuments.site/reader035/viewer/2022071420/6119d121e6e3a722bd6d07d3/html5/thumbnails/7.jpg)
Spontaneous activities (1)
単一筋線維Fibrillation potentialPositive sharp waveMyotonic discharge
複数の筋線維Complex repetitive discharge
筋線維起源
![Page 8: ç ‰éł»åł³å¡¾2015å¹´12朋è⁄ªçŽºæfl¾éł».pptx [ã…¦ã ... · 2018. 2. 26. · 5*7Á H °Fû FÖ q ·7Á )H H °7Á )H • ö ²Fû G" ¤FÔFï'½Fþ](https://reader035.vdocuments.site/reader035/viewer/2022071420/6119d121e6e3a722bd6d07d3/html5/thumbnails/8.jpg)
Spontaneous activities (2)
神経末端起源
![Page 9: ç ‰éł»åł³å¡¾2015å¹´12朋è⁄ªçŽºæfl¾éł».pptx [ã…¦ã ... · 2018. 2. 26. · 5*7Á H °Fû FÖ q ·7Á )H H °7Á )H • ö ²Fû G" ¤FÔFï'½Fþ](https://reader035.vdocuments.site/reader035/viewer/2022071420/6119d121e6e3a722bd6d07d3/html5/thumbnails/9.jpg)
Fasciculation potentialMyokymic potential -repetitive fasciculation/grouped fasciculation
Endplate spike/noise
Spontaneous activities (2)
神経末端起源
![Page 10: ç ‰éł»åł³å¡¾2015å¹´12朋è⁄ªçŽºæfl¾éł».pptx [ã…¦ã ... · 2018. 2. 26. · 5*7Á H °Fû FÖ q ·7Á )H H °7Á )H • ö ²Fû G" ¤FÔFï'½Fþ](https://reader035.vdocuments.site/reader035/viewer/2022071420/6119d121e6e3a722bd6d07d3/html5/thumbnails/10.jpg)
VGCC
Synaptic vesicles
AChR
AChE
Mitochondria
Schwann cell
MEPPend-plate noise
At rest
Nerve terminal
Mucle fiber
MEPP:Miniature end plate potential→endplate noise
quantum
Na+ Channel
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100μV
10ms
不規則!トタンに雨だれ
End plate spike
波形
発火パターン
スパイク状(短持続1-2ms)初期陽性の2相性が多い
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Endplate spike
Action potential
初期陰性の3相性
初期陽性の2相性
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chrMyositisFPchangeDeltoidtest.pwvFibComformRegularFas-ALS(TA).pwv
DecreasingIncreasing
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chrMyositisRegularFPDeltoid.pwv
多くは3相性ときに2相性
なぜか?
Fibrillation potential
波形
![Page 15: ç ‰éł»åł³å¡¾2015å¹´12朋è⁄ªçŽºæfl¾éł».pptx [ã…¦ã ... · 2018. 2. 26. · 5*7Á H °Fû FÖ q ·7Á )H H °7Á )H • ö ²Fû G" ¤FÔFï'½Fþ](https://reader035.vdocuments.site/reader035/viewer/2022071420/6119d121e6e3a722bd6d07d3/html5/thumbnails/15.jpg)
Action potential
Fibrillation potential
Triphasic Biphasic
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Action potentialInjury
PSW
ALSPSWregular.pwv
規則的,またはスムーズな変化
波形
発火パターン
Positive Sharp Wave
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Fibrillation potential, PSWの臨床的意義
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Chronic/Inactive
Active
正常筋線維変性筋線維
活動性の変性過程を反映脱神経でもミオパチーでも出現する
Fibrillation PotentialPositive sharp wave
![Page 19: ç ‰éł»åł³å¡¾2015å¹´12朋è⁄ªçŽºæfl¾éł».pptx [ã…¦ã ... · 2018. 2. 26. · 5*7Á H °Fû FÖ q ·7Á )H H °7Á )H • ö ²Fû G" ¤FÔFï'½Fþ](https://reader035.vdocuments.site/reader035/viewer/2022071420/6119d121e6e3a722bd6d07d3/html5/thumbnails/19.jpg)
ALSRecent denervationActive myositisProgressive muscular dystrophy / myopathy
Fibrillation potential /PSW
Active or progressive degeneration
Why the Fibs/PSW is important for the diagnosis for ALS?
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excitation
Fasciculation potential
起源 : 神経末端発火はランダム波形:不安定これがあるのは末梢神経の病気 (ミオパチーではない!)
Fas-ALS(Deltoid).pwv
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Complex Repetitive Discharge (CRD)
Ephaptic transmission1
2
3
45
6
211 3 45
71 23 4
562 7
#波形が突然変化することがある
慢性のニューロパチー,ミオパチーでみられる
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CRD
Myotonic Discharge
Fundamental frequency is the same (depend on pacemaker fiber)
Could be changed the waveform
Frequency is changing Multiple single fiber discharges
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ミオトニー放電はミオトニアのないミオパチーやニューロパチーでもしばしば記録される
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Myokymic potential
Grouped fasciculation potentials
DemyelinationEphaptic transmission
Fasciculation potentialと同様ミオパチーでは出現しない
![Page 25: ç ‰éł»åł³å¡¾2015å¹´12朋è⁄ªçŽºæfl¾éł».pptx [ã…¦ã ... · 2018. 2. 26. · 5*7Á H °Fû FÖ q ·7Á )H H °7Á )H • ö ²Fû G" ¤FÔFï'½Fþ](https://reader035.vdocuments.site/reader035/viewer/2022071420/6119d121e6e3a722bd6d07d3/html5/thumbnails/25.jpg)
ALSPSWlileMUPwithFasBB.pwv 70ms 190ms
Semi-regular discharge
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Fibrillation potential, positive sharp wave, fasciculation potential はactiveな病態がある証拠
Fibeillation potential, positive sharp waveとその仲間はリズムが規則的、線形であることが最も確実な特徴
Fiberillation potentialは必ずしも「神経原性」ではなく活動性病変の指標
Fasciculation potential, myokymic potentialは末梢神経の異常を示す所見
Myotonic dischargeは単一筋線維由来で臨床的なミオトニアのない病態でも出現する
運動単位電位(MUP)の発火間隔はゆらぐ
![Page 27: ç ‰éł»åł³å¡¾2015å¹´12朋è⁄ªçŽºæfl¾éł».pptx [ã…¦ã ... · 2018. 2. 26. · 5*7Á H °Fû FÖ q ·7Á )H H °7Á )H • ö ²Fû G" ¤FÔFï'½Fþ](https://reader035.vdocuments.site/reader035/viewer/2022071420/6119d121e6e3a722bd6d07d3/html5/thumbnails/27.jpg)
→運動単位の構築と機能の異常
・軽い随意収縮による運動単位電位の形態の観察
・筋収縮を強めて最大収縮する過程での運動単位電位数の増加パターン
(recruitment)の観察
針筋電図のステップ(2)
![Page 28: ç ‰éł»åł³å¡¾2015å¹´12朋è⁄ªçŽºæfl¾éł».pptx [ã…¦ã ... · 2018. 2. 26. · 5*7Á H °Fû FÖ q ·7Á )H H °7Á )H • ö ²Fû G" ¤FÔFï'½Fþ](https://reader035.vdocuments.site/reader035/viewer/2022071420/6119d121e6e3a722bd6d07d3/html5/thumbnails/28.jpg)
正常の運動単位電位
電位のたし算
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単一運動単位と運動単位電位の関係
半径1mm
運動単位の範囲
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正常の運動単位電位BA
A B
EMG
W35.pwv
複数の運動単位がモザイク状に配置
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1 2 3 4 5 6
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1 2 3 4 5 6
少し力を入れる
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1 2 3 4 5 6
力を入れてゆく
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1 2 3 4 5 6
最大収縮
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MU4
MU3
MU2
MU1
力を入れる
1)運動単位の発射頻度増大2)新しい運動単位の動員(recruitment)
運動ニューロンの発火力を入れる
動員
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運動単位の動員(recruitment)
(1)正常
加重(力)筋(運動単位)
力を加えると、それの大きさに比例して運動単位の数が増加する
運動単位の動員 recruitment発火頻度も少し上昇
W35W35.pwv
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運動単位の動員・漸増(recruitment)
(2)筋疾患
加重(力)筋(運動単位)
一つ一つの運動単位の力が弱く同じ力を出すのに必要な運動単位の数が多くなる(early recruitment)
W44W42W42.pwv W44.pwv
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運動単位の動員・漸増(recruitment)
(3)慢性期の末梢神経障害
加重(力)筋(運動単位)
一つ一つの運動単位が大きく、同じ力を出すのに必要な運動単位の数が少ない(late recruitment)
それでも足りない分は発火頻度上昇で代償
W49QuadGiant.pwv
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運動単位電位の形態
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MUP
X
X
W38.pwvW38
軸索変性初期、あるいは再生初期
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ALS Biceps unstable, polyphasic MUP
500μV
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BA
軸索変性→脱神経
A
EMG
maxcontraction.pwvWunitloss
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脱神経後、健常なAの軸索からの再支配*(初期)
unstable
未熟な軸索は髄鞘が不完全一部神経伝導が遅く不安定
A
*
*
UnstableMU-ALS(TA).pwv
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脱神経後、再支配後期の運動単位電位
stablepolyphasic
再支配が安定する末端の伝導時間も短縮
A
W40W40.pwv
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脱神経後、再支配慢性期の運動単位電位
large MUP再支配した筋線維の記録部位での線維密度が高い
A
QuadGiant.pwvpostpolioTA
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消失
密度の低下→小さな運動単位電位となる
ミオパチー 運動単位内の大部分の筋線維の変性(高度な障害のある運動単位)
unstable
W42W42.pwv
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ミオパチー 筋線維の大小不同・変性太い筋線維→大きな活動電位、伝導速度は速い細い筋線維→小さな活動電位、伝導速度は遅い
消失
筋線維ごとの伝導のばらつきが大きくなる軽症では持続時間は決して短くない!
大小不同
変性W44
W44.pwv
軸索の変性・再生
軸索末端の変性・再生
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Mitochondrial myopathy BicepsSmall MUP and polyphasic, unstable MUPs
500μV
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運動単位電位の形
1 筋線維の密度2 筋線維の伝導速度のばらつき(太さの違い)3 軸索末端での伝導時間のばらつき4 軸索末端、神経筋接合部、筋線維の伝導の安定性
運動単位電位の数と動員様式1 機能している運動単位の数2 個々の運動単位の生み出す力(筋線維の変性)
運動単位電位からわかること