title: Abdominal pain related to mitochondrial neurogastrointestinal encephalomyopathy syndrome may benefit from splanchnic nerve blockade.
abstract: Patients diagnosed with abdominal pain related to mitochondrial neurogastrointestinal encephalopathy (MNGIE) may benefit from splanchnic nerve blockade. MNGIE, varying in age of onset and rate of progression, is caused by loss of function mutation in thymidine phosphorylase gene. Gastrointestinal dysmotility, pseudo-obstruction and demyelinating sensorimotor peripheral neuropathy (stocking-glove sensory loss, absent tendon reflexes, distal limb weakness, and wasting) are the most prominent manifestations. Patients usually die in early adulthood (mean 37.6 years; range 26-58 years). We report a case of an 18-year-old patient with MNGIE. Our patient's abdominal pain was relieved after splanchnic nerve blockade.
authors.affiliation: Department of Anesthesiology and Reanimation, Hacettepe University School of Medicine, Ankara, Turkey.
authors.forename: Nalan
authors.initials: N
authors.lastname: Celebi
authors.forename: Altan
authors.initials: A
authors.lastname: Sahin
authors.forename: Ozgür
authors.initials: O
authors.lastname: Canbay
authors.forename: Filiz
authors.initials: F
authors.lastname: Uzümcügil
authors.forename: Ulkü
authors.initials: U
authors.lastname: Aypar
mesh_terms: D015746:Abdominal Pain / Q000209:etiology* / Q000628:therapy*; D000293:Adolescent; D000588:Amines / Q000627:therapeutic use; D000700:Analgesics / Q000627:therapeutic use; D000779:Anesthetics, Local; D003509:Cyclohexanecarboxylic Acids / Q000627:therapeutic use; D004576:Electromyography; D000077206:Gabapentin; D005767:Gastrointestinal Diseases / Q000150:complications* / Q000000981:diagnostic imaging; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D017237:Mitochondrial Encephalomyopathies / Q000150:complications* / Q000000981:diagnostic imaging; D009407:Nerve Block; D009460:Neurologic Examination; D010292:Paresthesia / Q000209:etiology; D011318:Prilocaine; D011859:Radiography; D013153:Splanchnic Nerves; D005680:gamma-Aminobutyric Acid / Q000627:therapeutic use
chemical_list: D000588:Amines; D000700:Analgesics; D000779:Anesthetics, Local; D003509:Cyclohexanecarboxylic Acids; D011318:Prilocaine; D005680:gamma-Aminobutyric Acid; D000077206:Gabapentin