Advertisement

Criteria-unfulfilled multiple system atrophy at an initial stage exhibits laterality of middle cerebellar peduncles

      Highlights

      • The imaging characteristics of the criteria-unfulfilled MSA-C at the initial stage
      • Decreased pontine area and significant laterality of MCP width of cerebral MRI
      • SPECT analysis revealed that the pontine rCBF was reduced.
      • Laterality of cerebellar rCBF and laterality of MCP width were positive correlated.
      • These could be useful to consider the possibility of criteria-unfulfilled MSA-C.

      Abstract

      To elucidate clinically useful imaging characteristics of multiple system atrophy with predominant cerebellar ataxia (MSA-C) at the initial stage showing pure cerebellar ataxia but unfilling consensus criteria (MSA-pc), clinical and neuroradiological analyses on cerebral MRI and single-photon emission computed tomography (SPECT) for measuring regional cerebral blood flow (rCBF) were performed. Seven MSA-pc patients meeting the above condition at an initial evaluation were identified, and all the MSA-pc patients later developed autonomic dysfunction and finally fulfilled the criteria for probable or possible category of MSA-C. For comparison, two patients with spinocerebellar ataxia type 6 and three patients with idiopathic cerebellar ataxia who did not exhibit autonomic dysfunction for more than three years were enrolled in this study (non-MSA-pc). As non-ataxic controls without cerebellar involvement, seven patients with Parkinson's disease were also enrolled. As a result, MRI analysis clarified a smaller pontine area and significant laterality of middle cerebellar peduncle (MCP) width in MSA-pc in comparison to non-MSA-pc and controls. SPECT analysis revealed that pontine rCBF was reduced even at the initial stage of MSA-pc. Moreover, the laterality of cerebellar rCBF values and the laterality of MCP width in MSA-pc patients exhibited a significant positive correlation, indicating anatomical and functional laterality of afferent projections to cerebellum is a characteristic finding for MSA-pc. These neuroimaging characteristics could be clinically useful to consider the possibility of the criteria-unfulfilled MSA and promote an earlier intervention after obtaining a diagnosis of probable MSA-C.

      Keywords

      To read this article in full you will need to make a payment

      Purchase one-time access:

      Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online access
      One-time access price info
      • For academic or personal research use, select 'Academic and Personal'
      • For corporate R&D use, select 'Corporate R&D Professionals'

      Subscribe:

      Subscribe to Journal of the Neurological Sciences
      Already a print subscriber? Claim online access
      Already an online subscriber? Sign in
      Institutional Access: Sign in to ScienceDirect

      References

        • Savoiardo M.
        • Strada L.
        • Girotti F.
        • et al.
        Olivopontocerebellar atrophy: MR diagnosis and relationship to multisystem atrophy.
        Radiology. 1990; 174: 693-696https://doi.org/10.1148/radiology.174.3.2305051
        • Counsell C.
        • Hughes A.
        Clinical usefulness of MRI in multisystem atrophy.
        J. Neurol. Neurosurg. Psychiatry. 1999; 66: 694https://doi.org/10.1136/jnnp.66.5.694
        • Lee E.A.
        • Cho H.I.
        • Kim S.S.
        • et al.
        Comparison of magnetic resonance imaging in subtypes of multiple system atrophy.
        Parkinsonism Relat. Disord. 2004; 10: 363-368https://doi.org/10.1016/j.parkreldis.2004.04.008
        • Nicoletti G.
        • Fera F.
        • Condino F.
        • et al.
        MR imaging of middle cerebellar peduncle width: differentiation of multiple system atrophy from Parkinson disease.
        Radiology. 2006; 239: 825-830https://doi.org/10.1148/radiol.2393050459
        • Gilman S.
        • Wenning G.K.
        • Low P.A.
        • et al.
        Second consensus statement on the diagnosis of multiple system atrophy.
        Neurology. 2008; 71: 670-676https://doi.org/10.1212/01.wnl.0000324625.00404.15
        • Yoshida K.
        • Kuwabara S.
        • Nakamura K.
        • et al.
        Idiopathic cerebellar ataxia (IDCA): diagnostic criteria and clinical analyses of 63 Japanese patients.
        J. Neurol. Sci. 2018; 384: 30-35https://doi.org/10.1016/j.jns.2017.11.008
        • Mangesius S.
        • Hussl A.
        • Tagwercher S.
        • et al.
        No effect of age, gender and total intracranial volume on brainstem MR planimetric measurements.
        Eur. Radiol. 2020; 30: 2802-2808https://doi.org/10.1007/s00330-019-06504-1
        • Schneider C.A.
        • Rasband W.S.
        • Eliceiri K.W.
        NIH image to ImageJ: 25 years of image analysis.
        Nat. Methods. 2012; 9: 671-675
        • Hara D.
        • Maki F.
        • Tanaka S.
        • et al.
        MRI-based cerebellar volume measurements correlate with the international cooperative Ataxia rating scale score in patients with spinocerebellar degeneration or multiple system atrophy.
        Cerebellum Ataxias. 2016; 3: 14https://doi.org/10.1186/s40673-016-0052-4
        • Sako W.
        • Abe T.
        • Haji S.
        • et al.
        “One line”: a method for differential diagnosis of parkinsonian syndromes.
        Acta Neurol. Scand. 2019; 140: 229-235https://doi.org/10.1111/ane.13136
        • Takeuchi R.
        • Sengoku T.
        • Matsumura K.
        Usefulness of fully automated constant ROI analysis software for the brain: 3DSRT and FineSRT.
        Radiat. Med. 2006; 24: 538-544https://doi.org/10.1007/s11604-006-0054-x
        • Takeuchi R.
        • Yonekura Y.
        • Matsuda H.
        • et al.
        Usefulness of a three-dimensional stereotaxic ROI template on anatomically standardised 99mTc-ECD SPET.
        Eur. J. Nucl. Med. Mol. Imaging. 2002; 29: 331-341https://doi.org/10.1007/s00259-001-0715-z
        • Kimura N.
        • Kumamoto T.
        • Masuda T.
        • et al.
        Evaluation of regional cerebral blood flow in cerebellar variant of multiple system atrophy using FineSRT.
        Clin. Neurol. Neurosurg. 2009; 111: 829-834https://doi.org/10.1016/j.clineuro.2009.08.014
        • Ryo T.
        • Fine S.R.T.
        Atlas.
        Kyoritsu printing, Japan2005
        • Monin M.L.
        • Tezenas du Montcel S.
        • Marelli C.
        • et al.
        Survival and severity in dominant cerebellar ataxias.
        Ann. Clin. Transl. Neurol. 2015; 2: 202-207https://doi.org/10.1002/acn3.156
        • Watanabe H.
        • Saito Y.
        • Terao S.
        • et al.
        Progression and prognosis in multiple system atrophy: an analysis of 230 Japanese patients.
        Brain. 2002; 125: 1070-1083https://doi.org/10.1093/brain/awf117
        • Bocchetta M.
        • Iglesias J.E.
        • Chelban V.
        • et al.
        Automated brainstem segmentation detects differential involvement in atypical parkinsonian syndromes.
        J. Mov. Disord. 2020; 13: 39-46https://doi.org/10.14802/jmd.19030
        • Gama R.L.
        • Távora D.F.
        • Bomfim R.C.
        • et al.
        Morphometry MRI in the differential diagnosis of parkinsonian syndromes.
        Arq. Neuropsiquiatr. 2010; 68: 333-338https://doi.org/10.1590/s0004-282x2010000300001
        • Bürk K.
        • Bühring U.
        • Schulz J.B.
        • et al.
        Clinical and magnetic resonance imaging characteristics of sporadic cerebellar ataxia.
        Arch. Neurol. 2005; 62: 981-985https://doi.org/10.1001/archneur.62.6.981
        • Lin D.J.
        • Hermann K.L.
        • Schmahmann J.D.
        The diagnosis and natural history of multiple system atrophy, Cerebellar Type.
        Cerebellum. 2016; 15: 663-679https://doi.org/10.1007/s12311-015-0728-y
        • Miyoshi F.
        • Kanasaki Y.
        • Shinohara Y.
        • et al.
        Significance of combined use of MRI and perfusion SPECT for evaluation of multiple system atrophy, cerebellar type.
        Acta Radiol. 2016; 57: 742-749https://doi.org/10.1177/0284185115598810
        • Cilia R.
        • Marotta G.
        • Benti R.
        • et al.
        Brain SPECT imaging in multiple system atrophy.
        J. Neural Transm. (Vienna). 2005; 112: 1635-1645https://doi.org/10.1007/s00702-005-0382-5