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Biomedical subjects

I J Hopkins

Publications and source records attributed to I J Hopkins.

At least 37 records · Page 2Linked to original sources

'Juvenile' myasthenia gravis in early infancy.

A previously well infant developed severe muscle weakness and hypotonia at 6 months of age. This was reversed by anticholinesterase medication. However, she had subsequent further weakness and died at 10 months after an acute respiratory arrest. The clinical pattern was that of the 'juvenile' form of myasthenia gravis rather than the 'congenital' forms which have previously been described in early infancy.

Age Factors↗

Spongy glio-neuronal dystrophy: a degenerative disease of the nervous system.

A case of spongy glio-neuronal dystrophy is reported. The clinical features included mental and motor retardation in early infancy after a normal birth, and from the age of 4 years increasingly severe attacks of multifocal epilepsy, with prolonged postictal coma, myoclonic twitching and nystagmus, and at times hemiplegia for one to two weeks. Acidosis was present during the periods of postictal coma. Jaundice occurred several days before death at the age of 5 years. Neuropathological examination revealed severe spongy degeneration in the thalamus and dentate nucleus, and the cerebral cortex to a lesser degree, with sparing of the white matter. There was partial necrosis of liver cells and in some areas regenerative nodules. Possible aetiological factors linking the cerebral and hepatic pathology are discussed.

Acidosis↗