Abstract
OBJECTIVE—To elucidate the character and distribution of the abnormal muscle tonus in the body axis in progressive supranuclear palsy. Although neck hypertonus has been well described in progressive supranuclear palsy, little is known about the involvement of the truncal muscles. METHODS—Muscle tonus of the neck and trunk was separately investigated in 13 patients with progressive supranuclear palsy by clinical examination and surface EMG during passive rotation. Muscle hypertonus was graded according to a four point scale, and subjected to statistical analysis. The results were compared with those from 13 age matched patients with Parkinson's disease and six healthy volunteers. RESULTS—In all but one patient with progressive supranuclear palsy, there was a distinct difference in muscle tonus between the neck and trunk. A tonic shortening reaction characteristic of dystonia and an increased tonic stretch reflex (rigidity) were present in the neck muscles of patients with progressive supranuclear palsy, whereas only normal to moderately increased tonus was noted in the truncal muscles (neck v trunk, shortening reaction p=0.0001; stretch reflex p=0.0241). Follow up studies disclosed an increase in axial muscle tonus with predilection for the neck in three of four patients. In the 13 patients with Parkinson's disease, however, no significant difference was found in muscle rigidity between the neck and trunk. CONCLUSION—Mild changes in truncal muscle tonus with prominent neck dystonia and rigidity are characteristic of progressive supranuclear palsy. It is suggested that separate clinical evaluation of muscle tonus in the neck and trunk may be helpful for distinguishing progressive supranuclear palsy from Parkinson's disease.
Full Text
The Full Text of this article is available as a PDF (182.1 KB).
Selected References
These references are in PubMed. This may not be the complete list of references from this article.
- Behrman S., Carroll J. D., Janota I., Matthews W. B. Progressive supranuclear palsy. Clinico-pathological study of four cases. Brain. 1969;92(3):663–678. doi: 10.1093/brain/92.3.663. [DOI] [PubMed] [Google Scholar]
- FLOYD W. F., SILVER P. H. The function of the erectores spinae muscles in certain movements and postures in man. J Physiol. 1955 Jul 28;129(1):184–203. doi: 10.1113/jphysiol.1955.sp005347. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Fukushima-Kudo J., Fukushima K., Tashiro K. Rigidity and dorsiflexion of the neck in progressive supranuclear palsy and the interstitial nucleus of Cajal. J Neurol Neurosurg Psychiatry. 1987 Sep;50(9):1197–1203. doi: 10.1136/jnnp.50.9.1197. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Fukushima K., Fukushima J., Terashima T. The pathways responsible for the characteristic head posture produced by lesions of the interstitial nucleus of Cajal in the cat. Exp Brain Res. 1987;68(1):88–102. doi: 10.1007/BF00255236. [DOI] [PubMed] [Google Scholar]
- Hoehn M. M., Yahr M. D. Parkinsonism: onset, progression and mortality. Neurology. 1967 May;17(5):427–442. doi: 10.1212/wnl.17.5.427. [DOI] [PubMed] [Google Scholar]
- Jankovic J., Friedman D. I., Pirozzolo F. J., McCrary J. A. Progressive supranuclear palsy: motor, neurobehavioral, and neuro-ophthalmic findings. Adv Neurol. 1990;53:293–304. [PubMed] [Google Scholar]
- Nagumo K., Hirayama K. [A study on truncal rigidity in parkinsonism--evaluation of diagnostic test and electrophysiological study]. Rinsho Shinkeigaku. 1993 Jan;33(1):27–35. [PubMed] [Google Scholar]
- Nagumo K., Hirayama K. [Axial (neck and trunk) rigidity in Parkinson's disease, striatonigral degeneration and progressive supranuclear palsy]. Rinsho Shinkeigaku. 1996 Oct;36(10):1129–1135. [PubMed] [Google Scholar]
- STEELE J. C., RICHARDSON J. C., OLSZEWSKI J. PROGRESSIVE SUPRANUCLEAR PALSY. A HETEROGENEOUS DEGENERATION INVOLVING THE BRAIN STEM, BASAL GANGLIA AND CEREBELLUM WITH VERTICAL GAZE AND PSEUDOBULBAR PALSY, NUCHAL DYSTONIA AND DEMENTIA. Arch Neurol. 1964 Apr;10:333–359. doi: 10.1001/archneur.1964.00460160003001. [DOI] [PubMed] [Google Scholar]
- Waters R. L., Morris J. M. Electrical activity of muscles of the trunk during walking. J Anat. 1972 Feb;111(Pt 2):191–199. [PMC free article] [PubMed] [Google Scholar]