A case of CPT deficiency, homoplasmic mtDNA mutation and ragged red fibers at muscle biopsy

J Neurol Sci. 2005 Dec 15;239(1):21-4. doi: 10.1016/j.jns.2005.07.008. Epub 2005 Sep 15.

Abstract

A 45-year-old male patient had an episode of acute renal failure with myoglobinuria, myalgias, weakness, and markedly increased serum CK levels. Similar episodes had occurred in the past. Carnitine palmitoyl-transferase II (CPT II) deficiency was documented both biochemically and genetically. Interestingly, muscle biopsy also showed some ragged red fibers (RRF) and complete mitochondrial DNA (mtDNA) sequence disclosed a homoplasmic T3394C point mutation. This mutation is described in Leber's hereditary optic neuropathy (LHON) or in patients with diabetes mellitus.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Acute Kidney Injury / etiology
  • Acute Kidney Injury / physiopathology
  • Base Sequence / genetics
  • Biopsy
  • Carnitine O-Palmitoyltransferase / deficiency*
  • Carnitine O-Palmitoyltransferase / genetics
  • Creatine Kinase / blood
  • DNA Mutational Analysis
  • DNA, Mitochondrial / genetics*
  • Humans
  • Male
  • Middle Aged
  • Mitochondrial Diseases / enzymology*
  • Mitochondrial Diseases / genetics*
  • Mitochondrial Diseases / physiopathology
  • Muscle Fibers, Skeletal / enzymology
  • Muscle Fibers, Skeletal / pathology
  • Muscle Weakness / enzymology
  • Muscle Weakness / genetics
  • Muscle Weakness / physiopathology
  • Muscle, Skeletal / enzymology
  • Muscle, Skeletal / pathology
  • Muscle, Skeletal / physiopathology
  • Muscular Diseases / complications
  • Muscular Diseases / enzymology*
  • Muscular Diseases / physiopathology
  • Myoglobinuria / etiology
  • Myoglobinuria / physiopathology
  • Point Mutation / genetics*
  • Syndrome

Substances

  • DNA, Mitochondrial
  • Carnitine O-Palmitoyltransferase
  • Creatine Kinase