Intermediate:
Severity not as bad as classic MSUD. Increased BCAA levels and neurological impairment present.
Acute metabolic decompensation not present.<--- not as serious as classic
BCKD activity 3% to 30% of normal
Intermittent:
Normal early development, normal intelligence. BCAA levels normal.
However under stress, acute metabolic decompensation might occur.
Initial symptoms of intermittent form appears between 5 months to 2 years in association with infection
BCKD activity 5% to 50% of normal
Thiamine Responsive:
Symptoms similar to intermediate form. BCAA levels are high and alloisoleucine is detectable in the plasma.
However, low protein diet and thiamine results in reduction of BCAA levels. Withdrawal of thiamine treatment cause the BCAA levels to become high again. However, thiamine treatment alone is not enough to lower levels of BCAA. Thiamine affects the BCAA levels in plasma
Maple Syrup Urine Disease Type 2: Dihydrolipoamide branched chain transacylase (E2) subunits are encoded by the DBT gene. Mutation in this gene will lead to dysfunctional E2 subunit, causing MSUD type 2.
E3-deficient Maple Syrup Urine Disease: Dihydrolipoamide dehydrogenase (E3) subunits are encoded by the DLD gene. Mutation in this gene will lead to dysfunctional E3 subunit, causing E-3-deficient MSUD.
References
King, M. W. (2012, February 1). Maple Syrup Urine Disease (MSUD). The Medical Biochemistry. Retrieved June 18, 2012, from http://themedicalbiochemistrypage.org/msud.php
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