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Homozygous prion protein genotype predisposes to sporadic Creutzfeldt–Jakob disease
THE human prion diseases, Creutzfeldt–Jakob disease (CJD) and Gerstmann–Sträussler syndrome (GSS), are neurodegenerative diseases that are unique in being both infectious and genetic. Transmission ofExpand
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The central nervous system in motor neurone disease
Forty-five necropsied cases with primary degeneration of lower motor neurones are described and discussed. Of these, 36 are considered to be `typical' cases of motor neurone disease, eight of whichExpand
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  • Open Access
The distribution of plaques in the cerebrum in multiple sclerosis
One of the fundamental problems posed by multiple sclerosis is concerned with the distribution of the pathological lesions called plaques. The literature concerning the histopathology of this diseaseExpand
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  • Open Access
Cervical spondylosis complieated by anterior spinal artery thrombosis
CERVICAL SPONDYLOSIS is a degenerative disease in which cartilaginous and bony projections arise in association with the affected intervertebral disks.' These projections, when posteriorly situated,Expand
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Aberrant nerve fibres within the spinal cord
Raymond (1893), in a necropsy account of a case of syringomyelia, described in the wall of the syrinx small bundles of fine nerve fibres ensheathed with Schwann cells. He traced the origin of theseExpand
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  • Open Access
Muscular dystrophy in an X; 1 translocation female suggests that Duchenne locus is on X chromosome short arm.
A unique combination of a Duchenne-like muscular dystrophy in a girl with a translocation-inversion rearrangement involving an X chromosome and a no 1 chromosome appeared as a result of both geneExpand
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  • Open Access
Friedreich's Ataxia
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INTENSE RIGIDITY OF THE ARMS DUE TO ISOLATION OF MOTONEURONES BY A SPINAL TUMOUR
In man, persisting muscular rigidity* is usually associated with severe brain damage, as in the decerebrate state and various extrapyramidal disorders. It may also be seen following tetanus. MoreExpand
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Spinal cord involvement by C4–C5 Vertebral subluxation in rheumatoid arthritis: A description of 2 cases examined at necropsy
  • J. Hughes
  • Medicine
  • Annals of neurology
  • 1 June 1977
In 2 cases of severe, longstanding rheumatoid arthritis involving the cervical spine, a progressive spinal cord syndrome developed in which spastic paraparesis was combined with a lower motoneuronExpand
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