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). LS is a progressive neurodegenerative disorder with an incidence of approximately 1/40,000. The lesion is usually located in the basal ganglia and the brain stem of the patient, showing cavernous degeneration and necrosis. 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.