![]() Data from this investigation support the use of psychological tests to evaluate cognitive and neuropsychological deficits that vary with metabolic changes associated with MSUD. Selected tests of neuropsychological and cognitive functioning, administered before dietary treatment and immediately following metabolic stabilization and at 5-week follow-up, indicated considerable clinical improvement. Hospitalization was required following an episode of disorientation, social withdrawal, and severe cognitive impairment. The study examines a rare case of an adolescent with MSUD whose history of aggression and impulsivity was linked to metabolic instability. A diet which restricts amino acids is necessary in treating the disease. A review of MSUD literature indicates that infants rarely survive the disorder, and those who do manifest profound neurological abnormality and retardation. This case study also emphasizes the importance of cognitive and behavioral monitoring of metabolic changes in MSUD patients.Ībstract = "This case study describes the developmental history and behavioral and neuropsychological sequelae of maple syrup urine disease (MSUD) in an 18-year-old male. This case study describes the developmental history and behavioral and neuropsychological sequelae of maple syrup urine disease (MSUD) in an 18-year-old male.
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