During the last decade, disorders of the respiratory chain, socalled mitochondrial disorders, have emerged as a major clinical entity. Though Leigh’s disease, also known as Subacute Necrotizing Encephalomyelopathy, has been mostly reported in infancy and childhood, it is described in adults [1]. Leigh's disease is a myopathy causing degradation of motor skills with occasional cardiac involvemen...

The ts1 Moloney murine leukemia virus causes a degenerative neurologic disease in mice characterized by the development of noninflammatory spongiform encephalomyelopathy. To determine whether gag and pol gene products and viral replication are necessary for the ts1-env gene product to cause neurodegeneration, we generated transgenic mice harboring only ts1-env. Neuropathological lesions were ob...

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 which showed no upper motor neurone lesions. The relation of the nine `atypical' cases to the remainder is discussed. It is concluded that motor neurone disease constitutes an ill-defined band in a broad s...

We report the untypical clinical course of a previously healthy two-year-old girl, who died suddenly and unexpectedly after an episode of vomiting. At forensic autopsy no other pathological findings could be diagnosed than multiple reddish, sunken areas in brain stem, mesencephalon, and pons. Histologically they presented as areas of spongiosis of the neuropil with prominent endothelial hyperpl...