Journal of Movement Disorders

Brief communication

Movement Disorders Resulting From Bilateral Basal Ganglia Lesions in End-Stage Kidney Disease: A Systematic Review: Kah Hui Yap, Nurul Husna Baharudin, Abdul Halim Abdul Gafor, Rabani Remli, Shen-Yang Lim, Wan Asyraf Wan Zaidi, Shahrul Azmin, Shahizon Azura Mohamed Mukari, Raihanah Abdul Khalid, Norlinah Mohamed Ibrahim; J Mov Disord. 2022;15(3):258-263. Published online May 26, 2022; DOI: https://doi.org/10.14802/jmd.21185

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Abstract PDF Supplementary Material: Objective
The basal ganglia (BG) are susceptible to fluctuations in blood urea levels, sometimes resulting in movement disorders. We described patients with end-stage kidney disease (ESKD) presenting with movement disorders associated with bilateral BG lesions on imaging.
Methods
We report four patients and systematically reviewed all published cases of ESKD presenting with movement disorders and bilateral BG lesions (EBSCOhost and Ovid).
Results
Of the 72 patients identified, 55 (76.4%) were on regular dialysis. Parkinsonism was the most common movement disorder (n = 39; 54.2%), followed by chorea (n = 24; 33.3%). Diabetes mellitus (n = 51; 70.8%) and hypertension (n = 16; 22.2%) were the most common risk factors. Forty-three (59.7%) were of Asian ethnicity. Complete clinical resolution was reported in 17 (30.9%) patients, while 38 (69.1%) had incomplete clinical resolution with relapse. Complete radiological resolution occurred in 14 (34.1%) patients.
Conclusion
Movement disorders associated with BG lesions should be recognized as a rare and potentially reversible metabolic movement disorder in patients with ESKD.

Original Articles

Automated Brainstem Segmentation Detects Differential Involvement in Atypical Parkinsonian Syndromes: Martina Bocchetta, Juan Eugenio Iglesias, Viorica Chelban, Edwin Jabbari, Ruth Lamb, Lucy L. Russell, Caroline V. Greaves, Mollie Neason, David M. Cash, David L. Thomas, Jason D. Warren, John Woodside, Henry Houlden, Huw R. Morris, Jonathan D. Rohrer; J Mov Disord. 2020;13(1):39-46. Published online September 26, 2019; DOI: https://doi.org/10.14802/jmd.19030

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Objective
Brainstem segmentation has been useful in identifying potential imaging biomarkers for diagnosis and progression in atypical parkinsonian syndromes (APS). However, the majority of work has been performed using manual segmentation, which is time consuming for large cohorts.
Methods
We investigated brainstem involvement in APS using an automated method. We measured the volume of the medulla, pons, superior cerebellar peduncle (SCP) and midbrain from T1-weighted MRIs in 67 patients and 42 controls. Diagnoses were corticobasal syndrome (CBS, n = 14), multiple system atrophy (MSA, n = 16: 8 with parkinsonian syndrome, MSA-P; 8 with cerebellar syndrome, MSA-C), progressive supranuclear palsy with a Richardson’s syndrome (PSP-RS, n = 12), variant PSP (n = 18), and APS not otherwise specified (APS-NOS, n = 7).
Results
All brainstem regions were smaller in MSA-C (19–42% volume difference, p < 0.0005) and in both PSP groups (18–33%, p < 0.0005) than in controls. MSA-P showed lower volumes in all regions except the SCP (15–26%, p < 0.0005). The most affected region in MSA-C and MSA-P was the pons (42% and 26%, respectively), while the most affected regions in both the PSP-RS and variant PSP groups were the SCP (33% and 23%, respectively) and midbrain (26% and 24%, respectively). The brainstem was less affected in CBS, but nonetheless, the pons (14%, p < 0.0005), midbrain (14%, p < 0.0005) and medulla (10%, p = 0.001) were significantly smaller in CBS than in controls. The brainstem was unaffected in APS-NOS.
Conclusion
Automated methods can accurately quantify the involvement of brainstem structures in APS. This will be important in future trials with large patient numbers where manual segmentation is unfeasible.

Citations

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Agnès Pérez-Millan, Sergi Borrego-Écija, John C. van Swieten, Lize Jiskoot, Fermin Moreno, Robert Laforce, Caroline Graff, Mario Masellis, Maria Carmela Tartaglia, James B. Rowe, Barbara Borroni, Elizabeth Finger, Matthis Synofzik, Daniela Galimberti, Rik
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Comparative validation of AI and non-AI methods in MRI volumetry to diagnose Parkinsonian syndromes
Joomee Song, Juyoung Hahm, Jisoo Lee, Chae Yeon Lim, Myung Jin Chung, Jinyoung Youn, Jin Whan Cho, Jong Hyeon Ahn, Kyungsu Kim
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Identification of the pathogenesis features of various phenotypes of multiple sclerosis based on the study of the morphological functional connectivity of subcortical gray matter structures
A. G. Trufanov, A. Y. Polushin, E. A. Gorbunova, M. V. Lukin
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Structural MRI predicts clinical progression in presymptomatic genetic frontotemporal dementia: findings from the GENetic Frontotemporal dementia Initiative cohort
Martina Bocchetta, Emily G Todd, Arabella Bouzigues, David M Cash, Jennifer M Nicholas, Rhian S Convery, Lucy L Russell, David L Thomas, Ian B Malone, Juan Eugenio Iglesias, John C van Swieten, Lize C Jiskoot, Harro Seelaar, Barbara Borroni, Daniela Galim
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Samuel Shribman, Martina Bocchetta, Carole H Sudre, Julio Acosta-Cabronero, Maggie Burrows, Paul Cook, David L Thomas, Godfrey T Gillett, Emmanuel A Tsochatzis, Oliver Bandmann, Jonathan D Rohrer, Thomas T Warner
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Haim Golan, Olga Volkov, Eli Shalom
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Criteria-unfulfilled multiple system atrophy at an initial stage exhibits laterality of middle cerebellar peduncles
Minori Furuta, Masayuki Sato, Setsuki Tsukagoshi, Yoshito Tsushima, Yoshio Ikeda
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A data-driven model of brain volume changes in progressive supranuclear palsy
W. J. Scotton, M. Bocchetta, E. Todd, D. M. Cash, N. Oxtoby, L. VandeVrede, H. Heuer, D. C. Alexander, J. B. Rowe, H. R. Morris, A. Boxer, J. D. Rohrer, P. A. Wijeratne
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Neuroimaging Correlates of Cognitive Deficits in Wilson's Disease
Samuel Shribman, Maggie Burrows, Rhian Convery, Martina Bocchetta, Carole H. Sudre, Julio Acosta‐Cabronero, David L. Thomas, Godfrey T. Gillett, Emmanuel A. Tsochatzis, Oliver Bandmann, Jonathan D. Rohrer, Thomas T. Warner
Movement Disorders.2022; 37(8): 1728. CrossRef
Corticobasal syndrome and Parkinson’s disease at the beginning: asymmetrical patterns of MRI and Blink Reflex for early diagnosis
Giulia Donzuso, Giorgia Sciacca, Antonina Luca, Calogero E. Cicero, Giovanni Mostile, Alessandra Nicoletti, Mario Zappia
Journal of Neural Transmission.2022; 129(12): 1427. CrossRef
Eye movements and association with regional brain atrophy in clinical subtypes of progressive supranuclear palsy
Ji-Hyun Choi, Heejung Kim, Jung Hwan Shin, Jee-Young Lee, Han-Joon Kim, Jong-Min Kim, Beomseok Jeon
Journal of Neurology.2021; 268(3): 967. CrossRef
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Annalisa Vitale, Rossella Villa, Lorenzo Ugga, Valeria Romeo, Arnaldo Stanzione, Renato Cuocolo
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Differential early subcortical involvement in genetic FTD within the GENFI cohort
Martina Bocchetta, Emily G. Todd, Georgia Peakman, David M. Cash, Rhian S. Convery, Lucy L. Russell, David L. Thomas, Juan Eugenio Iglesias, John C. van Swieten, Lize C. Jiskoot, Harro Seelaar, Barbara Borroni, Daniela Galimberti, Raquel Sanchez-Valle, Ro
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Increased Signal in the Superior Cerebellar Peduncle of Patients with Progressive Supranuclear Palsy: Hiroshi Kataoka, Yukako Nishimori, Takao Kiriyama, Hitoki Nanaura, Tesseki Izumi, Nobuyuki Eura, Naoki Iwasa, Kazuma Sugie; J Mov Disord. 2019;12(3):166-171. Published online August 9, 2019; DOI: https://doi.org/10.14802/jmd.19002

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Abstract PDF

Objective
The provisional diagnosis of progressive supranuclear palsy (PSP) depends on a combination of typical clinical features and specific MRI findings, such as atrophy of the tegmentum in the midbrain. Atrophy of the superior cerebellar peduncle (SCP) distinguishes PSP from other types of parkinsonism. Histological factors affect the conventional fluid-attenuated inversion recovery (FLAIR) signals, such as the extent of neuronal loss and gliosis.
Methods
We investigated patients with PSP to verify the percentage of patients with various PSP phenotypes presenting a high signal intensity in the SCP. Three interviewers, who were not informed about the clinical data, visually inspected the presence or absence of a high signal intensity in the SCP on the FLAIR images. We measured the pixel value in the SCP of each patient. Clinical characteristics were evaluated using the Mann-Whitney test, followed by the χ² test.
Results
Ten of the 51 patients with PSP showed a high signal intensity in the SCP on FLAIR MRI. Higher pixel values were observed within the SCP of patients with a high signal intensity in the SCP than in patients without a high signal intensity (p < 0.001). The sensitivity and specificity of the high signal intensity in the SCP of patients with PSP was 19.6% and 100%, respectively. This finding was more frequently observed in patients with PSP with Richardson’s syndrome (PSP-RS) (25.7%) than other phenotypes (6.2%).
Conclusion
The high signal intensity in the SCP on FLAIR MRI might be an effective diagnostic tool for PSP-RS.

Citations

Citations to this article as recorded by

Diffusion tractography of superior cerebellar peduncle and dentatorubrothalamic tracts in two autopsy confirmed progressive supranuclear palsy variants: Richardson syndrome and the speech-language variant
Rodolfo G. Gatto, Peter R. Martin, Farwa Ali, Heather M. Clark, Joseph R. Duffy, Rene L. Utianski, Hugo Botha, Mary M. Machulda, Dennis W. Dickson, Keith A. Josephs, Jennifer L. Whitwell
NeuroImage: Clinical.2022; 35: 103030. CrossRef
The Role of Magnetic Resonance Imaging for the Diagnosis of Atypical Parkinsonism
Lydia Chougar, Nadya Pyatigorskaya, Bertrand Degos, David Grabli, Stéphane Lehéricy
Frontiers in Neurology.2020;[Epub] CrossRef

The ‘Hot Cross Bun’ Sign Is Not Always Multiple System Atrophy: Etiologies of 11 Cases: Christopher Way, David Pettersson, Amie Hiller; J Mov Disord. 2019;12(1):27-30. Published online December 19, 2018; DOI: https://doi.org/10.14802/jmd.18031

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16 Web of Science
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Abstract PDF

Objective
To clarify the specificity of the ‘hot cross bun’ sign (HCBS) for multiple system atrophy (MSA) in adult cerebellar ataxia or parkinsonism.
Methods
The radiologic information systems at an academic center and affiliated veterans’ hospital were queried using the keywords ‘hot cross bun,’ ‘pontocerebellar,’ ‘cruciate,’ ‘cruciform,’ ‘MSA,’ ‘multiple system atrophy,’ and ‘multisystem atrophy.’ Scans were reviewed by a neurologist and neuroradiologist to identify the HCBS. Subjects with the HCBS were reviewed by 2 neurologists to identify the most likely etiology of the patient’s neurologic symptoms.
Results
Eleven cases were identified. Etiologies included MSA (4 probable, 2 possible), hereditary cerebellar ataxia (3/11), probable dementia with Lewy bodies (1/11), and uncertain despite autopsy (1/11).
Conclusion
MSA was the most common etiology. However, 5 of the 11 patients did not have MSA. The most common alternate etiology was an undefined hereditary cerebellar ataxia (3/11).

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