筋萎縮性側索硬化症(Amyotrophic Lateral Sclerosis:ALS)
上位運動ニューロン(UMN)および下位運動ニューロン(LMN)両者が変性・脱落していくことで随意運動を司る運動神経伝導路に障害が起こり、進行性に筋萎縮・筋力低下をきたす神経変性疾患である。
筋萎縮性側索硬化症の症状
脊髄側索の変性によって上位運動ニューロン障害が引き起こされて、脊髄前角の変性によって下位運動ニューロン障害が引き起こされる。
また、脳神経核が障害されることによって球麻痺が生じる。
上位運動ニューロン障害
四肢腱反射亢進とBabinski徴候陽性の所見を認める。
下位運動ニューロン障害
筋線維束攣縮と筋萎縮を認める。特に呼吸筋障害が問題となり、患者は呼吸に多大な体力を使ってしまう。このため、非侵襲的陽圧換気(NPPV)が適応となる。
球麻痺
延髄に存在する迷走神経・舌咽神経・舌下神経などの神経核の麻痺によって、発声・構音・嚥下・咀嚼などの障害を呈するものを球麻痺と総称する。
延髄は球根のように膨らんでいることから球と呼ばれることがあり、延髄障害によって起こるため球麻痺と名付けられた。
舌下神経核が障害されるため舌萎縮となる。
また、呼吸に多大な体力を使うことに加えて、嚥下障害によって栄養が取り込みにくくなるため体重減少が激しい傾向となる。
このため、胃瘻の造設を呼吸障害が前面に出る前に行うことで栄養不良を解消することが体力を保持することに役立ち予後に重要となる。
筋萎縮性側索硬化症の検査
針筋電図が診断に最も有用な検査
ALSは針筋電図で高振幅電位を示して脱神経電位所見を呈する。
実際のところALSでは多様な針筋電図所見を認めることに加えて医師国家試験において筋電図を読み取ることまでは求められないので、「ALSは針筋電図で高振幅電位を示して脱神経電位所見を呈する」と暗記するのが良いと考えられる。
神経伝導検査が除外検査となる
ALSのような運動ニューロン疾患では神経伝導速度は末期まで保たれることが多く、神経伝導検査を行うことで脱髄疾患や神経筋伝達障害疾患を除外する。
ALSの陰性徴候
ALSでみられることが少ない症状を陰性徴候(陰性症状)という。
感覚障害と自律神経障害
ALSでは運動神経のみが選択的に障害されるため、感覚障害や自律神経障害が起こることが少ない。
膀胱直腸障害と眼球運動障害
理由は明らかではないものの膀胱直腸系を支配する神経と眼球運動系を支配する神経が障害されることが少ないことが知られており、ALSでは膀胱直腸障害と眼球運動障害を認めることが少ない。
褥瘡
理由は明らかではないもののALS患者では皮膚の若返り現象が起こるために、寝たきりの状態になるにも関わらず褥瘡を認めることが少なく特徴的な所見である。
脊髄性筋萎縮症(Werdnig-Hoffmann病)
脊髄性筋萎縮症のうち生後6ヶ月までに発症する病型をWerdnig-Hoffmann病と呼ぶ。
常染色体劣性遺伝形式をとる脊髄前角細胞に変性が起こる疾患である。
筋萎縮性側索硬化症は上位運動ニューロンと下位運動ニューロンの両方が障害される疾患であるのに対して、脊髄性筋萎縮症は下位運動ニューロンのみが障害されるという違いがあることに注意する。
下位運動ニューロン症状として、フロッピーインファント:floppy infant(筋緊張低下のために身体がグニャグニャする状態の乳児を総称した言葉)を呈する。
深部腱反射が消失することが特徴的な所見である。
また、似たような疾患として球脊髄性筋萎縮症が挙げられるが、球脊髄性筋萎縮症は血清CK高値となるのに対して脊髄性筋萎縮症では血清CK正常となるという違いがある。
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