筋萎縮性側索硬化症に頸髄空洞症を合併した1剖検例

AN AUTOPSY CASE OF AMYOTROPHIC LATERAL SCLEROSIS ASSOCIATED WITH CERVICAL SYRINGOMYELIA

　筋萎縮性側索硬化症（以下ALS）に頸髄空洞症を合併した剖検例を報告した。症例は死亡時59歳11カ月男性。54歳より下肢脱力（右＜左）55歳右手の脱力に気付き，諸検査にて頸椎症と頸髄に造影剤の滲み込みが認められ，C₅—C₇の前方固定術が施行された。しかし術後も症状は緩徐進行性に悪化し，59歳11カ月呼吸不全のため死亡。剖検では，C₂—C₇までの複数の独立した脊髄の空洞とALSの所見として脊髄前角細胞の萎縮，消失，Bunina bodyの出現，延髄錐体の萎縮などを認めた。本症例は頸椎症に伴う二次性の脊髄の複数の空洞とALSを合併した症例で，これを剖検にて確認できた。

An autopsied case of amyotrophic lateral sclero-sis complicated by cervical syringomyelia was re-ported. The case was a 59-year-old man, who first noticed weakness of both lower extremities at 54-year-old. The weakness spread to both upper ex-tremities within 2 years. Cervical myelography revealed multi-level cervical spondylosis and anter-ior fusion of C₅-C₇ was done.

But the weakness and atrophy of proximal mu-scle, diminished deep tendon reflex on upper extre-mities, hyperreflexia and pathological reflexes on both legs, tongue fasciculation and respiratory mu-scle weakness developed successively, and the pa-tient died of respiratory distress at 59-year-old.

Autopsy revealed multiple independent four sy-rinxes located at the level between C₂-C₇. One of these syrinxes had ependymal cell lining and tho-ught to be ideopathic syringomyelia. The other three syrinxes were considerd to be the cavitation in association with cervical spondylotic myelopa-thy. Degeneration and decreasing of spinal anter-ior horn cells, atrophy of medullary pyramis and Bunina bodies were observed as features of typical amyotrophic lateral sclerosis.

Cervical spondylosis as causative lesion of mul-tiple syrinxes was discussed, and relationship be-tween ALS and the syrinxes was not indicated clearly.