A Child with Intractable Vomiting: Brainstem Mass or Neuromyelitis Optica Spectrum Disorder

¹Roshanak Mehdipour, ²Vahid Shaygannejad

¹Isfahan Neuroscience Research Center, Isfahan University Of Medical Sciences, Isfahan, Iran; ²Isfahan Neuroscience Research Center, Alzahra Research Institute, Isfahan, University of Medical Science, Isfahan, Iran

Background:

NMOSD is an immune-mediated disorder which is characterized by relapsing episodes of optic neuritis and myelitis. Brain stem related symptoms such as Intractable vomiting are not usually considered as the initial presentation and misdiagnosis has been frequently observed. Almost 4% of NMOSD cases are pediatric. Early differentiation of NMOSD from other childhood disorders including ADEM, MS, infections and mass lesions is critical.

Material(s) and Method(s):

11 years old girl presented with intractable vomiting and received several types of gastrointestinal treatments during one month.After that diplopia occurred and also she suffered vertigo. In this stage Brain MRI showed isolated edematous intramedullary lesion with heterogeneous enhancement. Patient received corticosteroid therapy with diagnosis of brain stem mass and the symptoms improved. She was candidate for biopsy to decide for radiation or chemotherapy but her parents didn’t accept. After three months she developed central facial nerve palsy.the brain MRI showed the same lesion. Anti AQP4 ab was positive,so the appropriate treatment started with final diagnosis of NMOSD.

Discussion:

Only about 30% of patients presents with brainstem involvement. It is difficult to diagnosis of NMOSD with presentation of acute brainstem or cerebral or diencephalic syndromes for physicians who are not familiar with its clinical features and diagnostic criteria. Involvement of the area postreoma can lead to the initial presentation of sometimes intractable nausea and vomiting with associated intramedullary lesions on MRI in 16% to 43% of patients.

Conclusion(s):

NMOSD should be considered in differential diagnosis of isolated brain stem lesions to avoid from invasive surgical interventions. Early diagnosis is critical for proper treatment.