Endocrinopathies in paediatric-onset neuromyelitis optica spectrum disorder with aquaporin 4 (AQP4) antibody

Mult Scler. 2018 Apr;24(5):679-684. doi: 10.1177/1352458517726593. Epub 2017 Aug 14.

Abstract

The involvement of the diencephalic regions in neuromyelitis optica spectrum disorder (NMOSD) may lead to endocrinopathies. In this study, we identified the following endocrinopathies in 60% (15/25) of young people with paediatric-onset aquaporin 4-Antibody (AQP4-Ab) NMOSD: morbid obesity ( n = 8), hyperinsulinaemia ( n = 5), hyperandrogenism ( n = 5), amenorrhoea ( n = 5), hyponatraemia ( n = 4), short stature ( n = 3) and central hypothyroidism ( n = 2) irrespective of hypothalamic lesions. Morbid obesity was seen in 88% (7/8) of children of Caribbean origin. As endocrinopathies were prevalent in the majority of paediatric-onset AQP4-Ab NMOSD, endocrine surveillance and in particular early aggressive weight management is required for patients with AQP4-Ab NMOSD.

Keywords: Neuromyelitis optica (NMO); Quality of Life; endocrinopathies; hypothalamic involvement; obesity.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Amenorrhea / epidemiology
  • Amenorrhea / etiology
  • Aquaporin 4 / immunology*
  • Autoantibodies*
  • Caribbean Region / epidemiology
  • Child
  • Cohort Studies
  • Endocrine System Diseases / epidemiology*
  • Endocrine System Diseases / etiology
  • Female
  • Humans
  • Hyperandrogenism / epidemiology
  • Hyperandrogenism / etiology
  • Hyperinsulinism / epidemiology
  • Hyperinsulinism / etiology
  • Hyponatremia / epidemiology
  • Hyponatremia / etiology
  • Hypothalamus / diagnostic imaging
  • Hypothalamus / pathology
  • Hypothyroidism / epidemiology
  • Hypothyroidism / etiology
  • Immunologic Factors*
  • Magnetic Resonance Imaging
  • Male
  • Morbidity
  • Neuromyelitis Optica / complications
  • Neuromyelitis Optica / diagnostic imaging
  • Neuromyelitis Optica / epidemiology*
  • Neuromyelitis Optica / immunology*
  • Obesity, Morbid / epidemiology
  • Obesity, Morbid / etiology
  • Prevalence
  • Quality of Life

Substances

  • Aquaporin 4
  • Autoantibodies
  • Immunologic Factors