MNGIE neuropathy: five cases mimicking chronic inflammatory demyelinating polyneuropathy.
We report five patients with mitochondrial neurogastrointestinal encephalomyopathy (MNGIE) who had demyelinating peripheral neuropathy. The MNGIE neuropathy had clinical and electrodiagnostic features typical of acquired, rather than inherited, etiologies. In fact, three patients were actually treated for chronic inflammatory demyelinating polyneuropathy (CIDP). We discuss findings that may help distinguish patients with MNGIE from those with CIDP.
Bedlack
Richard S
RS
Division of Neurology, Duke University Medical Center, 932 Morreene Road, Box 3333, Durham, North Carolina 27707, USA. Bedla001@mc.duke.edu
Vu
Tuan
T
Hammans
Simon
S
Sparr
Steven A
SA
Myers
Bennett
B
Morgenlander
Joel
J
Hirano
Michio
M
D000276:Adjuvants, Immunologic / Q000627:therapeutic use; D000293:Adolescent; D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000328:Adult; D001369:Axons / Q000378:metabolism / Q000473:pathology; D003937:Diagnosis, Differential; D003951:Diagnostic Errors / Q000517:prevention & control*; D004568:Electrodiagnosis; D005260:Female; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D008297:Male; D017237:Mitochondrial Encephalomyopathies / Q000175:diagnosis* / Q000235:genetics / Q000503:physiopathology; D009413:Nerve Fibers, Myelinated / Q000378:metabolism / Q000473:pathology; D009431:Neural Conduction / Q000235:genetics; D010525:Peripheral Nerves / Q000378:metabolism / Q000473:pathology / Q000503:physiopathology; D020277:Polyradiculoneuropathy, Chronic Inflammatory Demyelinating / Q000175:diagnosis* / Q000503:physiopathology; D013939:Thymidine Phosphorylase / Q000172:deficiency / Q000235:genetics
D000276:Adjuvants, Immunologic; D000305:Adrenal Cortex Hormones; D016756:Immunoglobulins, Intravenous; D013939:Thymidine Phosphorylase