In 1951, Archibald Denis Leigh first described a boy with an aggravated clinical manifestation of subacute necrotizing
encephalomyelopathy, which was later named as Leigh syndrome (LS).[1] LS is a progressive neurodegenerative disorder with an incidence of approximately 1/40,000.[2] The lesion is usually located in the basal ganglia and the brain stem of the patient, showing cavernous degeneration and necrosis.[3] LS occurs mainly in the 1st year of birth, especially between 3 months and 12 months.
Experts suggest that the polyneuropathy sometimes present in patients with [B.sub.12] deficiency due to gastrointestinal malabsorption and/or gastric bypass surgery may be secondary to concurrent nutritional deficiencies (e.g., copper, pyridoxine), and pure cobalamin deficiency causes an
encephalomyelopathy but not an encephalomyeloneuropathy (1).
It is characterized by a progressive neurodegenerative course with subacute necrotizing
encephalomyelopathy, and it presents with developmental delay, seizures, dysarthria, ataxia, and myopathy.
Fabry disease and mitochondrial
encephalomyelopathy with lactic acidosis and stroke-like episodes (MELAS) are rare genetical causes.
Leigh syndrome or 'subacute necrotizing
encephalomyelopathy' occurs mostly due to 'cytochrome c oxidase'' deficiency.
LEIGH'S disease, a form of Leigh syndrome, also known as Subacute Necrotizing
Encephalomyelopathy (SNEM), is a rare disorder that affects the central nervous system.
Methylmalonic and malonic aciduria in a dog with progressive
encephalomyelopathy. Metabolic Brain Disease, v.11, n.3, p.239-247, 1996.
The brain MRI results were compatible with a diagnosis of Leigh disease, also known as subacute necrotizing
encephalomyelopathy, a rare neurometabolic disorder that affects the central nervous system.
