The cranial vault in an adult is an enclosed space encased by the rigid skull. The Monro–Kellie doctrine proposed in the early nineteenth century states that the volume inside the cranium is fixed and normally filled with the brain, cerebral spinal fluid, and blood in a state of equilibrium. An increase in the volume of one of these constituents must be compensated by a decrease in another. Intracranial pressure (ICP) is the pressure inside the skull due to the volume within the cranium. Elevated ICP can be severely debilitating and even life-threatening. It is very important to quickly identify signs and symptoms of elevated ICP, diagnose the pathology, and promptly initiate treatment in the emergency setting to prevent irreparable brain damage, significant disability, or death.

Organic acidurias (OAs) belong to the group of inborn errors of metabolism (IEM) caused by enzymatic or transport proteins defects in the metabolic pathways of the amino acids, lipids or carbohydrates, having as consequence an energetic failure due to the insufficient “fuel” for the cycle of Krebs and the accumulation of intermediate metabolites with a deleterious effect on different organs, including the nervous system. Diagnosis is based on detection of intermediate metabolites in body fluids, on genetic analysis and measurement of the enzymatic activity in cultured fibroblasts. Strokes related to OAs belong to metabolic stroke category for glutaric, isovaleric, methylmalonic (MMA) and propionic acidurias (PA) and is related to clot formation in the dilated cardiac chambers and to infections favored by neutropenia, in Barth syndrome. Basal ganglia are frequently affected, leading to various types of movement disorders. Both ischemic and hemorrhagic strokes may occur. Early diagnosis, prompt initiation of specific diets, adequate treatment of acute decompensations and chronic monitoring of the patients can reduce the mortality and disability