JUVENILE LEIGH DISEASE Meaning and
Definition
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Juvenile Leigh Disease, also known as Leigh syndrome or subacute necrotizing encephalopathy, is a rare and severe genetic disorder that primarily affects the central nervous system.
Juvenile Leigh Disease is typically diagnosed in early childhood, between the ages of a few months to a few years. The condition is characterized by the progressive degeneration of the developing brain, leading to a wide range of neurological symptoms that worsen over time. These symptoms may include motor abnormalities such as muscle weakness and difficulty coordinating movements, delayed development, loss of previously acquired skills, seizures, feeding difficulties, and respiratory problems.
The underlying cause of Juvenile Leigh Disease is genetic mutations that disrupt the normal functioning of the mitochondria, which are responsible for producing energy within cells. As a result, affected individuals experience energy deficiency in their brain cells, leading to the neurological symptoms associated with the disorder.
There is currently no cure for Juvenile Leigh Disease, and treatment primarily focuses on managing the symptoms and slowing down disease progression. This may involve the use of medications to control seizures, physical and occupational therapy to improve motor skills, and nutritional support to ensure adequate calorie intake. The prognosis for those with Juvenile Leigh Disease is generally poor, with most individuals experiencing progressive neurological decline leading to severe disability or early death.
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