Abdominal pain related to mitochondrial neurogastrointestinal encephalomyopathy syndrome may benefit from splanchnic nerve blockade.
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.
Celebi
Nalan
N
Department of Anesthesiology and Reanimation, Hacettepe University School of Medicine, Ankara, Turkey.
Sahin
Altan
A
Canbay
Ozgür
O
Uzümcügil
Filiz
F
Aypar
Ulkü
U
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
D000588:Amines; D000700:Analgesics; D000779:Anesthetics, Local; D003509:Cyclohexanecarboxylic Acids; D011318:Prilocaine; D005680:gamma-Aminobutyric Acid; D000077206:Gabapentin