Parkinson病と類似の症状を示すParkinson病関連疾患としては進行性核上性麻痺(Progressive Supranuclear Palsy:PSP)と脊髄小脳変性症(SpinoCerebellarDegeneration:SCD)と大脳皮質基底核変性症(CorticoBasal Degeneration:CBD)が挙げられる。
これらのParkinson病関連疾患は初期においてParkinson病と鑑別することが難しく、Parkinson病と診断されていたものが数年後にそれぞれのParkinson病関連疾患に特有の症状を示すことで診断され直すことが多い。
進行性核上性麻痺では初期からの姿勢反射障害によって歩行障害や易転倒性を認めることが多いのに加えて垂直方向の眼球運動障害を認めるのが特徴的である。
脊髄小脳変性症は初期から自律神経障害を認めて起立性低血圧などを生じるのが特徴的である。
大脳皮質基底部変性症はパーキンソニズムのほかに大脳皮質徴候を認めて「失行」を呈することが特徴的である。
進行性核上性麻痺
進行性核上性麻痺の症状
垂直方向の眼球運動が障害される
眼運動神経核(動眼神経核・滑車神経核・外転神経核)に至るまでの上流の神経伝達機構に障害が起こった結果として生じる核上性眼球運動障害が特徴的であり、医師国家試験問題においてはこれが鑑別に有効となる。
進行性核上性麻痺の「核上性」の名前の由来はここからきている。核上性眼球運動障害では上下方向、つまり垂直方向の眼球運動(注視)に障害が出るので垂直性核上性注視麻痺とも呼ばれる。
頸筋の筋緊張が亢進して頸部後屈を呈する
パーキンソン病では前傾前屈姿勢を示すのに対して、進行性核上性麻痺では筋強剛が四肢というよりは体幹・頸部で強く起こるので頸筋の筋緊張が亢進して頸部後屈を呈することが多い。
初期から易転倒性を認める
初期から姿勢保持障害による歩行障害を認めて易転倒性が目立つ。
進行性核上性麻痺の画像所見
ハチドリ徴候(hummingbird sign)
中脳吻側(口側)被蓋(背側)は上下方向の眼球運動や姿勢保持に重要な役割を果たしているとされている。
中脳吻側被蓋が変性することによって核上性進行性麻痺は引き起こされている。
ハチドリ徴候(humming bird sign)とは進行性核上性麻痺の患者において中脳吻側被蓋の萎縮によって中脳吻側が「ハチドリのくちばし様に尖った所見」を呈するものを指す。
脊髄小脳変性症
主に脊髄と小脳が障害されることで引き起こされる疾患の総称であるが脳幹・大脳基底核・末梢神経まで幅広く障害されることもある。
遺伝様式や障害部位によって分類される。
非遺伝性(孤発性)の脊髄小脳変性症が約70%を占めて、常染色体優性遺伝性の脊髄小脳変性症が約30%を占める。
非遺伝性(孤発性)の多系統萎縮症はα-シヌクレインが神経細胞に蓄積することで引き起こされて、常染色体優性遺伝性の脊髄小脳変性症はポリグルタミン病(トリプレットリピート病)である。
かつて、非遺伝性の脊髄小脳変性症のうち初発症状が小脳性運動失調であるものはオリーブ橋小脳萎縮症、パーキンソニズムであるものは線状体黒質変性症、自律神経障害であるものはShy-Drager症候群と呼ばれていた。
しかし、近年になってこの3つは進行すると重複してくることと神経病理学的には同一のものであることが明らかになり、3つを合わせて多系統萎縮症と総称されるようになった。
脊髄小脳変性症の症状
脊髄小脳変性症の症状は病型によって異なるものの全体的に最終的には多系統に障害されるというイメージを持つことが重要である。小脳・錐体外路・自律神経系・錐体路が障害される。
小脳性運動失調
企図振戦
物に手を伸ばしているときなどの意図的な動作の最中に起こる振戦であり、目標物に近づくほど悪化する。
Romberg徴候は陰性
Romberg徴候(閉足立位の際に開眼時と比べて閉眼時に身体の動揺が大きくなる現象を指す)は陰性となる。
Romberg徴候は脊髄後索性運動失調(脊髄後索-内側毛帯系が障害されたことで生じる深部感覚障害)が引き起こすものであり、小脳性運動失調では起こらないからである。
自律神経症状
自律神経症状として排尿障害や起立性低血圧といった症状が生じる。
突然死の危険性が高い
自律神経は呼吸や心拍活動に重要な役割を果たしているので、自律神経障害によって突然死をきたす可能性が高いことが脊髄小脳変性症(多系統萎縮症)の特徴となっている。
錐体路徴候
錐体路徴候として四肢腱反射亢進とBabinski反射陽性といった所見が出る。
パーキンソニズム(錐体外路徴候)
パーキンソニズムとして筋強剛・無動・嚥下障害といった症状が起こる。
多系統萎縮症では安静時振戦をパーキンソン病に比べて比較的認めることが少なく、パーキンソン病との鑑別に重要である。安静時振戦はパーキンソン病に比較的特異的な症状とされている。
脊髄小脳変性症の画像所見
脊髄小脳変性症では脳幹部(橋)と小脳の萎縮を認めるため、第4脳室は拡大する。
小脳の萎縮は小脳溝の拡大によって確認しやすい。
多系統萎縮症の画像所見
スリットサイン(slit sign)
多系統萎縮症の頭部MRI水平断では被殻外側の低信号域(被殻内で黒くみえる)と被殻外側に沿った高信号域(被殻外で白くみえる)を認める。
つまり、被殻外側(被殻内)が線状に際立って黒く見える、もしくは被殻外側に沿って(被殻の外側)線状に際立って白く見えるのでこれを鑑別に用いる。
Hot cross bun sign(十字サイン=橋クロスサイン)
Hot cross bun(HCB) signは橋の横走線維の変性を反映したものである。初期には縦に進行して、その後十字様にT2強調画像において高信号域となるものである。
ホットクロスバン(Hot cross bun)とはイギリスの菓子パンの一種である。
大脳皮質基底核変性症
典型的な臨床像は大脳皮質の変性によって生じる大脳皮質徴候と大脳基底核の変性によって生じる
錐体外路徴候を中核とする。
大脳皮質徴候
医師国家試験問題において大脳皮質基底核変性症を他の類似疾患と区別するには大脳皮質徴候に注目することが重要である。
大脳皮質徴候である「失行」や「他人の手徴候」はパーキンソン病や進行性核上性麻痺(PSP)といった類似疾患において起こりにくいので、これに注目することで区別できる。
他人の手徴候
自分の意思に反して片側の手が他人の手かのように勝手に動く現象を指す。
患者は制御できる方の手を用いて勝手に動く方の手を抑えようとする。
肢節運動失行
筋強剛などによる運動機能障害がないのにも関わらず、今までできていた単純な動作ができなくなる。
「ボタンがかけにくい」とか「箸使いが困難」といった形で現れることが多い。
「失行」を検査する方法として、「手でキツネの形を真似させる」といったものがある。
患者は制御できる手では真似をできるが、「失行」が生じている方の手では真似ができない。
参考文献
脊髄小脳変性症の治療の進歩:https://www.jstage.jst.go.jp/article/jsnt/34/5/34_510/_pdf/-char/ja
脊髄小脳変性症・多系統萎縮症ガイドライン2018:https://www.neurology-jp.org/guidelinem/sd_mst/sd_mst_2018.pdf
大脳皮質基底核変性症:https://www.neurology-jp.org/guidelinem/degl/sinkei_degl_c_2012_11.pdf
大脳皮質基底核変性症候群と大脳皮質基底核変性の診断:https://www.neurology-jp.org/Journal/public_pdf/056030149.pdf
進行性核上性麻痺とは:https://www.jstage.jst.go.jp/article/iryo1946/59/9/59_9_467/_pdf/-char/ja
9章 進行性核上性麻痺-日本神経学会:https://www.neurology-jp.org/guidelinem/degl/degl_2017_09.pdf
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