Snapshot A 23 year old male with a past medical history of diabetes mellitus presents with muscle weakness, staggering gait with frequent falling, dysarthria and a curvature in his upper spine. On physical exam the patient is noted to have marked muscle weakness (2/5 in upper and lower extremitites), a loss of deep tendon reflexes, nystagmus as well as pes cavus and kyphoscoliosis. Patient had a recent ED visit for pneumonia and a bedside ultrasound demonstrated a hypertrophic cardiomyopathy. Introduction Friedrichs Congenital impairment of mitochondrial function leading to cerebellar and spinal cord degeneration login to view 1 more bullet Presents in childhood Autosomal recessive login to view 1 more bullet Presentation Symptoms login to view 5 more bullets Physical exam login to view 9 more bullets Prognosis and Complications Death in adolescence most commonly due to cardiac arrhythmia login to view 1 more bullet