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Several drugs have been reported to play an inhibitory role in iron-death-mediated diseases

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Distal legs 3rd & 4th decade: Mild face weakness Pulmonary function: Restrictive deficits Progression: Slow Subjective improvement with methylprednisolone treatment EOM: Unaffected CNS: Normal cognition, sensation, cerebellar Systemic: Short stature Laboratory Serum CK: 287 to 984 Serum lactate: High Serum pyruvate: High EMG: Myopathic Muscle pathology Ragged red fibers Biochemistry Cytochrome c oxidase (Complex IV): Reduced Succinate cytochrome c reductase (Complex II & III): Reduced NADH dehydrogenase (Complex I): Normal CHCHD10 variant syndrome: FTD-ALS + Ataxia & Myopathy (FTDALS2) 175, 351 Epidemiology 0.6% to 3% of FTD-ALS China: 7.7% of FTD Genetics Inheritance: Dominant or Sporadic Mutations General: N-terminal half of protein Common locations: Exon 2 Specific mutations P12S Arg15Leu: ALS phenotype

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