Abstract
Several abnormalities in the central nervous system were shown in patients with progressive muscular dystrophy using computerised tomography (CT) scans, electroencephalograms, psychometry, and ophthalmological methods. In congenital muscular dystrophy, the most characteristic finding in the CT scan was a low density area in the white matter, seen in 14 (56%) out of 25 cases. In Duchenne dystrophy, slight cerebral atrophy was observed in 20 (67%) out of 30 cases. It was interesting that in the case of Duchenne dystrophy the older the patient, the more severe were the CT findings. In congenital muscular dystrophy half the patients with a low density area showed a spike or a spike-and-wave complex in the electroencephalogram, and optic atrophy was evident in several cases. It is concluded that progressive muscular dystrophy is not only a myogenic disorder but also one which affects the central nervous system.
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- ALLEN J. E., RODGIN D. W. Mental retardation in association with progressive dystrophy. Am J Dis Child. 1960 Aug;100:208–211. doi: 10.1001/archpedi.1960.04020040210008. [DOI] [PubMed] [Google Scholar]
- Dubowitz V., Crome L. The central nervous system in Duchenne muscular dystrophy. Brain. 1969;92(4):805–808. doi: 10.1093/brain/92.4.805. [DOI] [PubMed] [Google Scholar]
- Eiben R. M., Di Chiro G. Computer assisted tomography in adrenoleukodystrophy. J Comput Assist Tomogr. 1977 Jul;1(3):308–314. doi: 10.1097/00004728-197707000-00004. [DOI] [PubMed] [Google Scholar]
- Gyldensted C. Computer tomography of the brain in multiple sclerosis. A radiological study of 110 patients with special reference to demonstration of cerebral plaques. Acta Neurol Scand. 1976 May;53(5):386–389. doi: 10.1111/j.1600-0404.1976.tb04356.x. [DOI] [PubMed] [Google Scholar]
- Honda Y., Yoshioka M. Ophthalmological findings of muscular dystrophies: a survey of 53 cases. J Pediatr Ophthalmol Strabismus. 1978 Jul-Aug;15(4):236–238. doi: 10.3928/0191-3913-19780701-12. [DOI] [PubMed] [Google Scholar]
- Kamoshita S., Konishi Y., Segawa M., Fukuyama Y. Congenital muscular dystrophy as a disease of the central nervous system. Arch Neurol. 1976 Jul;33(7):513–516. doi: 10.1001/archneur.1976.00500070055011. [DOI] [PubMed] [Google Scholar]
- Murakami T., Konishi Y., Rakamiya M., Tsukagoshi H. Congenital muscular dystrophy associated with micropolygyria - report of two cases. Acta Pathol Jpn. 1975 Sep;25(5):599–612. doi: 10.1111/j.1440-1827.1975.tb01994.x. [DOI] [PubMed] [Google Scholar]
- Prosser E. J., Murphy E. G., Thompson M. W. Intelligence and the gene for Duchenne muscular dystrophy. Arch Dis Child. 1969 Apr;44(234):221–230. doi: 10.1136/adc.44.234.221. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Refsum S., Lonnum A., Sjaastad O., Engeset A. Dystrophia myotonica. Repeated pneumoencephalographic studies in ten patients. Neurology. 1967 Apr;17(4):345–348. doi: 10.1212/wnl.17.4.345. [DOI] [PubMed] [Google Scholar]
- Rosman N. P., Kakulas B. A. Mental deficiency associated with muscular dystrophy. A neuropathological study. Brain. 1966 Dec;89(4):769–788. doi: 10.1093/brain/89.4.769. [DOI] [PubMed] [Google Scholar]
- WORDEN D. K., VIGNOS P. J., Jr Intellectual function in childhood progressive muscular dystrophy. Pediatrics. 1962 Jun;29:968–977. [PubMed] [Google Scholar]
- Zellweger H., Hanson J. W. Psychometric studies in muscular dystrophy type 3a (Duchenne). Dev Med Child Neurol. 1967 Oct;9(5):576–581. doi: 10.1111/j.1469-8749.1967.tb02327.x. [DOI] [PubMed] [Google Scholar]