Introduction: The added value of dopamine transporter SPECT (DAT-SPECT) for the diagnosis of "possible" multiple system atrophy of the cerebellar type (MSA-C) remains unknown.
Methods: We reviewed retrospectively the charts of 128 consecutive patients with a clinical diagnosis of MSA-C who were seen between 2007 and 2016 at the French Reference Center for MSA. The main objective was to evaluate the proportion of patients for whom the diagnosis of "possible" MSA-C was made because of a positive DAT-SPECT.
Results: Seventy-eight MSA-C patients had at least one DAT-SPECT. Fifty-nine of them were considered for the final analysis. In these, 22 had "possible" MSA-C and 23 "probable" MSA-C before DAT-SPECT, while 14 did not reach diagnosis criteria at that time. In those with "possible" MSA-C, DAT-SPECT was positive in 64%. In patients with "probable" MSA-C, 83% showed nigrostriatal denervation. Six out of 14 (43%) received a diagnosis of "possible" MSA-C because of positive DAT-SPECT. These patients had mean disease duration of 2.3 years at the time of DAT-SPECT compared to 3.5 years of the entire cohort of MSA-C patients with DAT-SPECT. Of the eight remaining, one had positive DAT-SPECT but also pons atrophy on magnetic resonance imaging, and seven progressed to "probable" MSA based on clinical features.
Conclusion: Our results suggest that DAT-SPECT significantly contributes to the diagnosis of "possible" MSA-C (43% of patients not reaching consensus diagnosis criteria before DAT-SPECT). DAT-SPECT seems especially useful in patients with shorter disease duration, while a negative result does not exclude a diagnosis of MSA.
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http://dx.doi.org/10.1016/j.parkreldis.2019.02.006 | DOI Listing |
Cureus
September 2024
Department of Clinical Neuroscience and Therapeutics, Hiroshima University Graduate School of Biomedical and Health Sciences, Hiroshima, JPN.
Sleep Med
December 2024
Department of Neurophysiology, National Institute of Mental Health and Neuro Sciences (NIMHANS), Bangalore, India. Electronic address:
Clin Nucl Med
October 2024
From the Department of Neurology.
18 F-labeled THK5351 PET can visualize ongoing astrogliosis by estimating monoamine oxidase B levels and can be used as an adjunct for diagnosing neurodegenerative disorders. Little has been reported on multiple system atrophy (MSA) in the differential diagnosis of parkinsonian syndromes. Here, we present 18 F-THK5351 images in typical cases of MSA-P (parkinsonian type) and MSA-C (cerebellar type), showing intense 18 F-THK5351 uptake in the lateral-posterior part of the putamen (MSA-P) and in the pons and middle cerebellar peduncles (MSA-C).
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April 2024
Department of Neurology, Xiangya Hospital, Central South University, Changsha, Hunan, 410008, China; Department of Neurology, The Third Xiangya Hospital of Central South University, Changsha, Hunan, 410008, China; Key Laboratory of Hunan Province in Neurodegenerative Disorders, Central South University, Changsha, Hunan, 410008, China; Bioinformatics Center & National Clinical Research Center for Geriatric Disorders, Xiangya Hospital, Central South University, Changsha, Hunan, 410008, China; Hunan International Scientific and Technological Cooperation Base of Neurodegenerative and Neurogenetic Diseases, Changsha, China; National International Collaborative Research Center for Medical Metabolomics, Central South University, Changsha, Hunan, China; Furong Laboratory, Central South University, Changsha, Hunan, 410008, China; Brain Research Center, Central South University, Changsha, Hunan, 410008, China. Electronic address:
Background: An intronic GAA repeat expansion in FGF14 was recently identified as a cause of GAA-FGF14 ataxia. We aimed to characterise the frequency and phenotypic profile of GAA-FGF14 ataxia in a large Chinese ataxia cohort.
Methods: A total of 1216 patients that included 399 typical late-onset cerebellar ataxia (LOCA), 290 early-onset cerebellar ataxia (EOCA), and 527 multiple system atrophy with predominant cerebellar ataxia (MSA-c) were enrolled.
Brain Commun
February 2024
Ataxia Center, Massachusetts General Hospital and Harvard Medical School, Boston, MA 02114, USA.
Definitive diagnosis of multiple system atrophy of the cerebellar type (MSA-C) is challenging. We hypothesized that rates of change of pons and middle cerebellar peduncle diameters on MRI would be unique to MSA-C and serve as diagnostic biomarkers. We defined the normative data for anterior-posterior pons and transverse middle cerebellar peduncle diameters on brain MRI in healthy controls, performed diameter-volume correlations and measured intra- and inter-rater reliability.
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