A 1-year-old girl is brought to the clinic for evaluation of poor growth and poor muscle tone. The mother states that the patient “doesn’t seem to be gaining weight,” despite adequate feedings. Temperature is 36.4°C (97.5°F), pulse
is 98/min, blood pressure
is 104/64, and respiratory rate
is 35/min. Motor examination reveals diffuse hypotonia in all 4 limbs. Abdominal examination reveals hepatomegaly. Rapid fingerstick glucose is 40 mg/dL. Echocardiogram reveals left ventricular hypertrophy. Serum lactate levels are within normal limits. A liver biopsy reveals widespread fibrosis with minimal fat infiltration. Further analysis reveals accumulation of limit dextrin-like complexes in the cytosol
. Which of the following is the most likely diagnosis?