Alessandro Dinoto, Laura Cacciaguerra, Karl N Krecke, John Jing-Wei Chen, Dean M Wingerchuk, Brian G Weinshenker, Samantha A Banks, Alfonso Sebastian Lopez-Chiriboga, Cristina Valencia-Sanchez, Elia Sechi, Sean J Pittock, Eoin P Flanagan
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引用次数: 0
Abstract
Objectives: To characterize the frequency and clinicoradiologic phenotype of cerebellar involvement in attacks of aquaporin-4-IgG positive neuromyelitis optica spectrum disorder (AQP4+NMOSD) which are incompletely captured in current diagnostic criteria.
Methods: Brain MRI scans from patients with AQP4+NMOSD in the Mayo Clinic database were reviewed, and those with cerebellar T2-hyperintense lesions ≤30 days from attack onset were included for clinical and radiologic characterization.
Results: From 432 patients with AQP4+NMOSD, we identified 17 (4%) with cerebellar attacks. The median age at attack onset was 47 years (range, 7-74). Cerebellar symptoms and signs were noted in 16 (94%) of 17 and the remaining patient was intubated preventing a detailed cerebellar exam. The median Expanded Disability Status Scale score at nadir was 5 (range, 2-9.5). Sixteen (94%) had other regions involved during the attack, most frequently with brainstem or area postrema involvement. Cerebellar MRI T2-lesions (8 single; 11 contiguous with the brainstem; 6/15 [35%] enhancing) were located in cerebellar peduncles, 15 (inferior, 5; middle, 10; superior, 10), and cerebellar parenchyma, 8 (dentate, 4; medial, 2; lateral, 4). T2-lesions persisted in 9 (82%) of 11 beyond 6 months.
Discussion: Cerebellar involvement during attacks of AQP4+NMOSD is rare but the associated neurologic deficits tend to be severe. Cerebellar peduncle or dentate nucleus T2-lesions are frequent MRI accompaniments. Clinical features and MRI lesion patterns of cerebellar involvement could be incorporated into future iterations of AQP4+NMOSD criteria.
期刊介绍:
Neurology Neuroimmunology & Neuroinflammation is an official journal of the American Academy of Neurology. Neurology: Neuroimmunology & Neuroinflammation will be the premier peer-reviewed journal in neuroimmunology and neuroinflammation. This journal publishes rigorously peer-reviewed open-access reports of original research and in-depth reviews of topics in neuroimmunology & neuroinflammation, affecting the full range of neurologic diseases including (but not limited to) Alzheimer's disease, Parkinson's disease, ALS, tauopathy, and stroke; multiple sclerosis and NMO; inflammatory peripheral nerve and muscle disease, Guillain-Barré and myasthenia gravis; nervous system infection; paraneoplastic syndromes, noninfectious encephalitides and other antibody-mediated disorders; and psychiatric and neurodevelopmental disorders. Clinical trials, instructive case reports, and small case series will also be featured.