There is evidence to suggest that the central nervous system may metabolize methylmalonyl-CoA in a system isolated from the rest of the body. If this is the case, transplantation may not reverse the neurological effects of methylmalonic acid previous to the transplant or prevent further damage to the brain by continued build up. Prognosis
The prognosis will vary depending on the severity of the condition and the individuals response to treatment. Prognosis is typically better for those with cobalamin-responsive variants and not promising in those suffering from noncobalamin-responsive variants. Milder variants have a higher frequency of appearance in the population than the more severe ones. Even with dietary modification and continued medical care, it may not be possible to prevent neurological damage in those with a nonresponsive acidemia. Without proper treatment or diagnosis, it is not uncommon for the first acidemic attack to be fatal.Despite these challenges, since it was first identified in 1967, treatment and understanding of the condition has improved to the point where it is not unheard of for even those with unresponsive forms of methylmalonic acidemia to be able to reach adulthood and even carry and deliver children safely. Research
Nosologic history
MMA was first characterized by Oberholzer et al. in 1967. Neurologic effects
That MMA can have disastrous effects on the nervous system has been long reported; however, the mechanism by which this occurs has never been determined.
Hydrocortisone sodium succinate is a synthetic glucocorticoid corticosteroid and a corticosteroid ester. == References ==