Neuropediatrics 2019; 50(03): 204-205
DOI: 10.1055/s-0039-1684030
Images in Neuropediatrics
Georg Thieme Verlag KG Stuttgart · New York

Bright Spotty Lesions in the Myelon: A Hallmark of AQP-4 Positive Neuromyelitis Optica Spectrum Disorders

Emanuela Claudia Turco
1   Child Neuropsychiatric Unit, Maternal and Child Health Department, Parma University-Hospital, Parma, Italy
,
Francesca Greco
2   Child Neuropsychiatric Unit, Department of Medicine and Surgery, Neuroscience Section, University of Parma, Parma, Italy
,
Francesca Ormitti
3   Neurodiagnostic Unit, Diagnostic Department University-Hospital of Parma, Parma, Italy
,
Francesco Pisani
1   Child Neuropsychiatric Unit, Maternal and Child Health Department, Parma University-Hospital, Parma, Italy
2   Child Neuropsychiatric Unit, Department of Medicine and Surgery, Neuroscience Section, University of Parma, Parma, Italy
› Author Affiliations
Further Information

Publication History

23 November 2018

16 February 2019

Publication Date:
09 April 2019 (online)

A 15-year-old girl presented with pain and sensory impairment for temperature and pain of the right arm and later numbness in both lower extremities. Cerebrospinal fluid (CSF) analysis revealed pleocytosis (35 cells/μL; normal range, 0–5/μL), increased total protein (142 mg/dL; normal range, 15–46 mg/dL), while oligoclonal bands were absent. Serum aquaporin-4–immunoglobulin G (AQP4-IgG) was positive. Recent magnetic resonance imaging (MRI) showed increased signal throughout the right optic nerve ([Fig. 1A]), right thalamus ([Fig. 1B]), and longitudinal extensive transverse myelitis (LETM) involving cervical and dorsal cord ([Fig. 1C] and [D]). A LETM MRI pattern may occur in patients with different demyelinating disorders like multiple sclerosis, neuromyelitis optica spectrum disorders, acute disseminated encephalomyelitis (ADEM), or infections, especially by enterovirus-71, neoplastic and paraneoplastic diseases, spinal cord infarction, and traumatic spinal cord injury.[1] [2] [3] [4] Furthermore, a similar MRI pattern in particular in the myelon can be seen in the ADEM or LETM associated with oligodendrocyte glycoprotein antibodies.[5] In conclusion, this is a typical AQP4-IgG positive case of neuromyelitis optica spectrum disorders (NMOSD)[6] in which peculiar bright spotty lesions ([Fig. 1E]), which probably reflect intense damage of the cervical spinal cord,[2] are shown.

Zoom Image
Fig. 1 Hyperintensity throughout the right optic nerve (A) and in the right thalamus (B) in T2-weighted images. Bright spotty lesions (white arrows) as part of LETM in sagittal postgadolinium T1 (C), T2 (D), and axial T2-weighted (E).
 
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