TY - JOUR
T1 - Atypical Neuromyelitis Optica Spectrum Disorder With Diffuse Cerebral Abnormalities
T2 - A Case Report
AU - Newton, Braeden D.
AU - Kantarci, Orhun
AU - Okuda, Darin T.
N1 - Publisher Copyright:
© The Author(s) 2020.
PY - 2020
Y1 - 2020
N2 - The recent expansion of the radiological criteria and the use of a highly specific biomarker, anti-aquaporin 4-IgG (AQP4 IgG), has significantly improved the ability of clinicians to provide a timely and accurate diagnosis for neuromyelitis optica spectrum disorder (NMOSD), especially when faced with an abnormal disease presentation. Here, we report on the 5-year clinical experience of a 69-year-old right-handed African American woman who initially presented following symptoms suggestive of transient global amnesia. Her clinical history was only remarkable for a single episode of visual decline with poor recovery experienced 35 years prior, with prior unrevealing serological investigations. Brain MRI features were significant for diffuse, bilateral white matter abnormalities throughout the supratentorial, deep gray matter, and infratentorial regions. Spinal cord imaging studies were within normal limits with no intramedullary high-signal abnormalities identified. Serological studies were significant for the presence of anti-aquaporin 4-IgG. The clinical features were supportive of the diagnosis of NMOSD. The data provided here highlight both the clinical and radiological heterogeneity of NMOSD.
AB - The recent expansion of the radiological criteria and the use of a highly specific biomarker, anti-aquaporin 4-IgG (AQP4 IgG), has significantly improved the ability of clinicians to provide a timely and accurate diagnosis for neuromyelitis optica spectrum disorder (NMOSD), especially when faced with an abnormal disease presentation. Here, we report on the 5-year clinical experience of a 69-year-old right-handed African American woman who initially presented following symptoms suggestive of transient global amnesia. Her clinical history was only remarkable for a single episode of visual decline with poor recovery experienced 35 years prior, with prior unrevealing serological investigations. Brain MRI features were significant for diffuse, bilateral white matter abnormalities throughout the supratentorial, deep gray matter, and infratentorial regions. Spinal cord imaging studies were within normal limits with no intramedullary high-signal abnormalities identified. Serological studies were significant for the presence of anti-aquaporin 4-IgG. The clinical features were supportive of the diagnosis of NMOSD. The data provided here highlight both the clinical and radiological heterogeneity of NMOSD.
KW - MRI
KW - Neuromyelitis optica spectrum disorder
KW - anti-aquaporin 4-IgG
KW - spell
KW - white matter abnormalities
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U2 - 10.1177/1179573520973819
DO - 10.1177/1179573520973819
M3 - Article
C2 - 33281464
AN - SCOPUS:85156151035
SN - 1179-5735
VL - 12
JO - Journal of Central Nervous System Disease
JF - Journal of Central Nervous System Disease
ER -