Subscribe to RSS
The Non-motor Symptoms, Disability Progression, and Survival Analysis of Atypical Parkinsonism: Case Series from Eastern India and Brief Review of Literature
Objective The objectives of this study are (1) to describe the non-motor profile, the motor disability progression, and survival analysis of atypical parkinsonism in a tertiary care hospital of eastern India and (2) to elucidate the neurocircuitry and the putative substrates responsible for non-motor manifestations.
Methods In this prospective observational study, patients were diagnosed based on Consensus Criteria for Progressive Supranuclear Palsy (PSP), The Fourth Consensus Report of the Dementia with Lewy Body (DLBD) Consortium 2017, The Autonomic Neuroscience 2018 Criteria for Multiple System Atrophy (MSA), and Armstrong 2013 Criteria for Corticobasal Degeneration (CBD). Disease severity was assessed at baseline and 6 months of follow-up using the Unified Parkinson's Disease Rating Scales (UPDRS). For PSP and MSA, the PSP-Clinical Deficits Scale (PSP-CDS) and the Unified MSA Rating Scale (UMSARS), respectively, were used. Cox regression analysis and the hazard ratio were calculated.
Results Out of 27 patients, the diagnosis was probable PSP in 12, probable MSA in 7, probable CBD in 5, and probable DLBD in 3. Non-motor symptoms were highly prevalent across all subtypes. Motor disability progression as assessed by UPDRS parts 2 and 3 showed significant deterioration over 6-month follow-up across all groups (p < 0.05). Disease progression assessed by PSP-CDS and UMSARS over 6 months was significant (p < 0.05). One PSP and two MSA patients died during a 6-month follow-up period. The hazard ratio in MSA was 3.5 (95% confidence interval: 0.31–0.38) with p = 0.306.
Conclusion Atypical parkinsonian disorders are rare, and usually more severe than idiopathic parkinsonism. As no definitive treatment is available, symptomatic management involving a multidisciplinary team approach must be prioritized.
Keywordsatypical parkinsonism - progressive supranuclear palsy - survival - non-motor symptoms - multiple system atrophy - corticobasal degeneration
Article published online:
07 April 2022
© 2022. Association for Helping Neurosurgical Sick People. This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/)
Thieme Medical and Scientific Publishers Pvt. Ltd.
A-12, 2nd Floor, Sector 2, Noida-201301 UP, India
- 1 Colosimo C, Morgante L, Antonini A. et al; PRIAMO Study Group. Non-motor symptoms in atypical and secondary parkinsonism: the PRIAMO study. J Neurol 2010; 257 (01) 5-14
- 2 Dan R, Růžička F, Bezdicek O. et al. Separate neural representations of depression, anxiety and apathy in Parkinson's disease. Sci Rep 2017; 7 (01) 12164
- 3 Espay AJ, LeWitt PA, Kaufmann H. Norepinephrine deficiency in Parkinson's disease: the case for noradrenergic enhancement. Mov Disord 2014; 29 (14) 1710-1719
- 4 Pagonabarraga J, Kulisevsky J, Strafella AP, Krack P. Apathy in Parkinson's disease: clinical features, neural substrates, diagnosis, and treatment. Lancet Neurol 2015; 14 (05) 518-531
- 5 Chaudhuri A, Behan PO. Fatigue and basal ganglia. J Neurol Sci 2000; 179 (S 1-2): 34-42
- 6 Rana AQ, Qureshi AR, Siddiqui O, Sarfraz Z, Rana R, Shtilbans A. Prevalence of pain in atypical parkinsonism: a systematic review and meta-analysis. J Neurol 2019; 266 (09) 2093-2102
- 7 Chelban V, Catereniuc D, Aftene D. et al. An update on MSA: premotor and non-motor features open a window of opportunities for early diagnosis and intervention. J Neurol 2020; 267 (09) 2754-2770
- 8 Cutsforth-Gregory JK, Benarroch EE. Nucleus of the solitary tract, medullary reflexes, and clinical implications. Neurology 2017; 88 (12) 1187-1196
- 9 Bailey GA, Hubbard EK, Fasano A. et al. Sleep disturbance in movement disorders: insights, treatments and challenges. J Neurol Neurosurg Psychiatry 2021; 92 (07) 723-736
- 10 Schneider LD. Anatomy and physiology of normal sleep. In: Sleep and Neurologic Disease. Academic Press; 2017: 1-28 , chap. 1 DOI: 10.1016/B978-0-12-804074-4.00001-7
- 11 Kim KJ, Jeong SJ, Kim JM. Neurogenic bladder in progressive supranuclear palsy: a comparison with Parkinson's disease and multiple system atrophy. Neurourol Urodyn 2018; 37 (05) 1724-1730
- 12 Frazzitta G, Ferrazzoli D, Folini A, Palamara G, Maestri R. Severe constipation in Parkinson's disease and in parkinsonisms: prevalence and affecting factors. Front Neurol 2019; 10: 621-2019
- 13 Dommershuijsen LJ, Heshmatollah A, Darweesh SKL, Koudstaal PJ, Ikram MA, Ikram MK. Life expectancy of parkinsonism patients in the general population. Parkinsonism Relat Disord 2020; 77: 94-99
- 14 Höglinger GU, Respondek G, Stamelou M. et al; Movement Disorder Society-endorsed PSP Study Group. Clinical diagnosis of progressive supranuclear palsy: the Movement Disorder Society criteria. Mov Disord 2017; 32 (06) 853-864
- 15 McKeith IG, Boeve BF, Dickson DW. et al. Diagnosis and management of dementia with Lewy bodies: Fourth consensus report of the DLB Consortium. Neurology 2017; 89 (01) 88-100
- 16 Palma JA, Norcliffe-Kaufmann L, Kaufmann H. Diagnosis of multiple system atrophy. Auton Neurosci 2018; 211: 15-25
- 17 Armstrong MJ, Litvan I, Lang AE. et al. Criteria for the diagnosis of corticobasal degeneration. Neurology 2013; 80 (05) 496-503
- 18 Piot I, Schweyer K, Respondek G. et al; DescribePSP Study Group, ProPSP Study Group, MDS-endorsed PSP Study Group. The Progressive Supranuclear Palsy Clinical Deficits Scale. Mov Disord 2020; 35 (04) 650-661
- 19 Wenning GK, Tison F, Seppi K. et al; Multiple System Atrophy Study Group. Development and validation of the Unified Multiple System Atrophy Rating Scale (UMSARS). Mov Disord 2004; 19 (12) 1391-1402
- 20 Mahale RR, Krishnan S, Divya KP, Jisha VT, Kishore A. Subtypes of PSP and prognosis: a retrospective analysis. Ann Indian Acad Neurol 2021; 24 (01) 56-62
- 21 Wenning GK, Geser F, Krismer F. et al; European Multiple System Atrophy Study Group. The natural history of multiple system atrophy: a prospective European cohort study. Lancet Neurol 2013; 12 (03) 264-274
- 22 Papapetropoulos S, Tuchman A, Laufer D, Papatsoris AG, Papapetropoulos N, Mash DC. Causes of death in multiple system atrophy. J Neurol Neurosurg Psychiatry 2007; 78 (03) 327-329
- 23 Isono S, Shiba K, Yamaguchi M. et al. Pathogenesis of laryngeal narrowing in patients with multiple system atrophy. J Physiol 2001; 536 (Pt 1): 237-249
- 24 Todisco M, Alfonsi E, Isaias IU. et al. Vocal cord electromyographic correlates of stridor in multiple system atrophy phenotypes. Parkinsonism Relat Disord 2020; 70: 31-35
- 25 Benarroch EE, Schmeichel AM, Low PA, Parisi JE. Involvement of medullary serotonergic groups in multiple system atrophy. Ann Neurol 2004; 55 (03) 418-422
- 26 Brown FS, Rowe JB, Passamonti L, Rittman T. Falls in progressive supranuclear palsy. Mov Disord Clin Pract (Hoboken) 2019; 7 (01) 16-24
- 27 Tomita S, Oeda T, Umemura A. et al. Impact of aspiration pneumonia on the clinical course of progressive supranuclear palsy: a retrospective cohort study. PLoS One 2015; 10 (08) e0135823
- 28 Jean A. Brain stem control of swallowing: neuronal network and cellular mechanisms. Physiol Rev 2001; 81 (02) 929-969
- 29 Maghbooli M, Mohammadzadeh V, Yousefi M. et al. Update on therapeutic strategies for atypical parkinsonian syndromes. Turkish J Neurol 2020; 26: 111-121