Leigh syndrome, also referred to as subacute necrotizing encephalomyelopathy, is a severe, early-onset neurodegenerative disorder that is relentlessly progressive and devastating to both the patient and the patient's family. Attributed to the ultimate failure of the mitochondrial respiratory chain, once it starts, the disease often results in the regression of both mental and motor skills, lead...

Like so many complex and incurable neurological disorders, the disease first described in this journal in 1951 by (Archibald) Denis Leigh (1915–1998; figure 1) as ‘Subacute Necrotising Encephalomyelopathy’ (SNE) remained for many years a rare and intriguing enigma, of interest mainly to paediatric neurologists and neuropathologists. The more recent history of this disorder, now designated Leigh...

A 21 year old male, well-nourished and non-alcoholic, died after five weeks illness. He had suffered epileptic fits, bilateral internuclear ophthalmoplegia, bulbar and pontine paralysis, tetraparesia, ataxia and dystonia. A CT brain scan showed low density lesions of the striatum bilaterally. Post-mortem studies revealed pathological anomalies compatible with Leigh's disease, although the prese...

A 4-month-old female Holstein Friesian calf was referred to the Veterinary Teaching Hospital, University of Berne, Switzerland for evaluation of ataxia, weakness, apathy and stunted growth. Clinical examination revealed generalized ataxia, propioceptive deficits, decreased menace response and sensibility. Postmortem examination did not reveal macroscopic changes of major organs. Histologically,...

Various neurological forms of decompression sickness are classified and an inquiry two and a half to five and a half years after the acute illness is reported. One hundred subjects with decompression sickness of the central nervous system were examined. In more than half of the cases the clinical picture was that of chronic encephalomyelopathy, vegetative neurosis, or psychosomatic symptoms. Sy...

Clinical comparisons were made between Japanese patients with multiple sclerosis (66 cases) and neuro-Behçet's syndrome (23 cases). Those with neuro-Behçet showed marked male predominance, while those with multiple sclerosis showed slight female preponderance. Both showed encephalomyelopathy disseminated in time and space. Patients with multiple sclerosis, however, showed a more polyphasic cour...