Leigh S Disease

Leigh S Disease

Leigh disease or Leigh's disease, also known as Subacute Necrotizing Encephalomyelopathy (SNEM), is a rare neurometabolic disorder that affects the central nervous system. It is named after Archibald Denis Leigh (1916-1998), a British psychiatrist who first described the condition in 1951.  The disease is characterized by movement disorders. In one case review, 22 of 34 patients had evidence of a movement disorder. Dystonia, occurring in 19 patients, was the most common movement disorder. The dystonia was usually multifocal at onset and showed progression in six patients. Rigidity, tremor, chorea, hypokinesia, myoclonus, and tics were also noted (Macaya et al.). As it progresses rapidly, the earliest signs may be poor sucking ability and loss of head control and motor skills. Other symptoms include loss of appetite, vomiting, irritability, continuous crying (in infants), and seizures. A later sign can also be episodes of lactic acidosis, which can lead to impairment of respiratory and kidney function. Some children can present with loss of development skills or developmental regression.

Leigh S Disease

Leigh S Disease

Leigh S Disease

Leigh S Disease

Leigh S Disease

Leigh S Disease

Leigh S Disease

Leigh S Disease

Leigh S Disease