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Published on: August 21, 2017
Cerebellar Involvement in Attacks of Aquaporin-4-IgG Positive Neuromyelitis Optica Spectrum Disorder
Alessandro Dinoto1, Laura Cacciaguerra1, Karl N Krecke1
1From the Department of Neurology and Center for Multiple Sclerosis and Autoimmune Neurology (A.D., L.C., J.J.-W.C., B.G.W., S.A.B., S.J.P., E.P.F.), Mayo Clinic College of Medicine, Rochester, MN; Department of Neurosciences (A.D.), Biomedicine, and Movement Sciences, University of Verona, Italy; Department of Radiology (K.N.K.), Mayo Clinic; Department of Ophthalmology (J.J.-W.C.), Mayo Clinic College of Medicine, Rochester, MN; Department of Neurology (D.M.W., C.V.-S.), Mayo Clinic, Scottsdale, AZ; Department of Neurology (B.G.W.), University of Virginia, Charlottesville; Department of Neurology (A.S.L.-C.), Mayo Clinic College of Medicine, Jacksonville, FL; Neurology Unit (E.S.), University Hospital of Sassari, Italy; and Department of Laboratory Medicine and Pathology (S.J.P., E.P.F.), Mayo Clinic College of Medicine, Rochester, MN.
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.
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