[Strümpell Lorrain's familial spasmodic paraplegia. An anatomical and clinical review and report on a new case (author's transl)].
The authors describe the anatomical and clinical findings in a case of the pure form of Strümpell Lorrain's familial spasmodic paraplegia. This hereditary condition of the dominant autosomic type has very monomorphic pathognomonic features which are found in all patients affected. The slowly progressive isolated spastic hypertonia appears exclusively in the upright position and the extent of the disease can be ascertained by clinical observation during walking. The myelino-axial degeneration which is strictly limited to the spinal cord has an elective localization in the crossed pyramidal tracts, is less evident in the direct tracts and is mainly predominant in the lumbosacral and low dorsal regions of the cord. The extension of the process beyond the limits of the extrapyramidal tracts confirms the generally accepted unity of spinocerebellar degenerations. In this case the spinocerebellar tracts were less involved than the lumbar spinal ganglia, as well as their distant axial continuation to Goll's tracts only at the cervical level and until the bulber nucleus gracilis.
D001921:Brain / Q000473:pathology; D005260:Female; D006801:Humans; D008875:Middle Aged; D009128:Muscle Spasticity / Q000235:genetics / Q000473:pathology; D010264:Paraplegia / Q000235:genetics* / Q000473:pathology; D010375:Pedigree; D013116:Spinal Cord / Q000473:pathology
La paraplégie spasmodique familiale de Strümpell-Lorrain (P.S.F.). Une nouvelle observation anatomo-clinique.