Abdominal pain related to mitochondrial neurogastrointestinal encephalomyopathy syndrome may benefit from splanchnic nerve blockade

Paediatr Anaesth. 2006 Oct;16(10):1073-6. doi: 10.1111/j.1460-9592.2006.01918.x.

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.

Publication types

  • Case Reports

MeSH terms

  • Abdominal Pain / etiology*
  • Abdominal Pain / therapy*
  • Adolescent
  • Amines / therapeutic use
  • Analgesics / therapeutic use
  • Anesthetics, Local
  • Cyclohexanecarboxylic Acids / therapeutic use
  • Electromyography
  • Gabapentin
  • Gastrointestinal Diseases / complications*
  • Gastrointestinal Diseases / diagnostic imaging
  • Humans
  • Magnetic Resonance Imaging
  • Male
  • Mitochondrial Encephalomyopathies / complications*
  • Mitochondrial Encephalomyopathies / diagnostic imaging
  • Nerve Block*
  • Neurologic Examination
  • Paresthesia / etiology
  • Prilocaine
  • Radiography
  • Splanchnic Nerves*
  • gamma-Aminobutyric Acid / therapeutic use

Substances

  • Amines
  • Analgesics
  • Anesthetics, Local
  • Cyclohexanecarboxylic Acids
  • Prilocaine
  • gamma-Aminobutyric Acid
  • Gabapentin