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An Update on MSA: Premotor and Non-Motor Features Open a Window of Opportunities for Early Diagnosis and Intervention

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An Update on MSA: Premotor and Non-Motor Features Open a Window of Opportunities for Early Diagnosis and Intervention Journal of Neurology https://doi.org/10.1007/s00415-020-09881-6 NEUROLOGICAL UPDATE An update on MSA: premotor and non‑motor features open a window of opportunities for early diagnosis and intervention Viorica Chelban1,2 · Daniela Catereniuc2,3,4 · Daniela Aftene3,4 · Alexandru Gasnas3,4,5 · Ekawat Vichayanrat6 · Valeria Iodice6 · Stanislav Groppa2,3,4 · Henry Houlden1,7 Received: 8 March 2020 / Revised: 29 April 2020 / Accepted: 30 April 2020 © The Author(s) 2020 Abstract In this review, we describe the wide clinical spectrum of features that can be seen in multiple system atrophy (MSA) with a focus on the premotor phase and the non-motor symptoms providing an up-to-date overview of the current understanding in this fast-growing feld. First, we highlight the non-motor features at disease onset when MSA can be indistinguishable from pure autonomic failure or other chronic neurodegenerative conditions. We describe the progression of clinical features to aid the diagnosis of MSA early in the disease course. We go on to describe the levels of diagnostic certainty and we discuss MSA subtypes that do not ft into the current diagnostic criteria, highlighting the complexity of the disease as well as the need for revised diagnostic tools. Second, we describe the pathology, clinical description, and investigations of cardiovascu- lar autonomic failure, urogenital and sexual dysfunction, orthostatic hypotension, and respiratory and REM-sleep behavior disorders, which may precede the motor presentation by months or years. Their presence at presentation, even in the absence of ataxia and parkinsonism, should be regarded as highly suggestive of the premotor phase of MSA. Finally, we discuss how the recognition of the broader spectrum of clinical features of MSA and especially the non-motor features at disease onset represent a window of opportunity for disease-modifying interventions. Keywords Multiple system atrophy · Non-motor features · Premotor phase · MSA * Viorica Chelban Introduction [email protected] 1 Department of Neuromuscular Diseases, Queen Square Multiple system atrophy (MSA) is a sporadic, adult-onset, Institute of Neurology, University College London, progressive, rare neurodegenerative disorder of uncertain London WC1N 3BG, UK etiology. It manifests with diverse clinical features, char- 2 Neurobiology and Medical Genetics Laboratory, “Nicolae acterized mainly by a combination of parkinsonism with a Testemitanu” State University of Medicine and Pharmacy, poor response to levodopa treatment, cerebellar features, and 165, Stefan cel Mare si Sfant Boulevard, 2004 Chişinău, Republic of Moldova autonomic failure. The defnite diagnosis of MSA can still only be established pathologically with the presence of glial 3 Department of Neurology, Epileptology and Internal Diseases, Institute of Emergency Medicine, 1, Toma Ciorba cytoplasmic inclusions (GCI) at postmortem [1–3]. Street, 2004 Chişinău, Republic of Moldova In the past, a variety of terms were used to describe 4 Department of Neurology nr. 2, Nicolae Testemitanu” State MSA. In 1900, Joseph Jules Dejerine and André Thomas University of Medicine and Pharmacy, 165, Stefan cel Mare frst reported two cases presenting with cerebellar ataxia si Sfant Boulevard, 2004 Chişinău, Republic of Moldova and pathological lesions in the cerebellum and brain- 5 Cerebrovascular Diseases and Epilepsy Laboratory, stem, described as olivopontocerebellar atrophy. Sixty Institute of Emergency Medicine, 1, Toma Ciorba Street, years later, Shy and Drager described two patients with 2004 Chişinău, Republic of Moldova parkinsonism and autonomic symptoms including ortho- 6 Autonomic Unit, National Hospital for Neurology static hypotension [4] and Van der Eecken et al. reported and Neurosurgery, UCL NHS Trust, London WC1N 3BG, cases with parkinsonism and striatonigral degeneration at UK autopsy in the same year. The term ‘MSA’ was frst intro- 7 Neurogenetics Laboratory, National Hospital for Neurology duced in 1969 by Graham and Oppenheimer to represent and Neurosurgery, Queen Square, London WC1N 3BG, UK Vol.:(0123456789)1 3 Journal of Neurology all three neurological entities: olivopontocerebellar atro- 20% of cases diagnosed in life as MSA subsequently have a phy (OPCA), the Shy–Drager syndrome, and striatonigral pathology confrming PD or DLB [28]. degeneration (SND) [5]. Papp et al. frst demonstrated MSA is characterized by worsening of motor and non- the presence of argyrophilic glial cytoplasmic inclusions motor features over an average of 10 years, with a fast pro- in the central nervous system of patients diagnosed with gression from disease onset, helping to distinguish it from MSA in 1989 [6]. These inclusions were later reported to similar degenerative conditions, described above [29]. be positive for α-synuclein, and nowadays, alpha-synu- Approximately 50% of patients require walking aids within clein inclusions in oligodendroglia are the recognized 3 years from the onset of motor symptoms [30]; 60% require neuropathologic hallmarks of MSA and may even rep- a wheelchair after 5 years [31] with a median time to becom- resent a primary pathologic event [7]. Together, Parkin- ing bedridden of 6–8 years [30]. However, a more benign son’s disease, dementia with Lewy bodies, and MSA are MSA variant with longer survival of over 15 years has been called α-synucleinopathies [8]. The mechanisms by which reported in pathology-confrmed cases [[2, 32], as well as α-synuclein, a neuronal protein, ends up in the oligoden- an aggressive MSA phenotype, with a very short disease droglia causing pathology in MSA remain unclear. There duration of less than 3 years [33]. Older age at onset [30, 31, is growing evidence that oligodendroglial pathology and 34–36], a parkinsonian phenotype [29, 35], early develop- “prion-like” spreading of misfolded α-synuclein may be ment of severe autonomic failure [30, 35, 37, 38], and severe the primary events in MSA leading to neurodegeneration urinary retention and nocturnal stridor [39, 40] are all nega- [7–9]. In addition, proteasomal and mitochondrial dys- tive prognostic factors, whereas a cerebellar phenotype [34] function [10], dysregulation of myelin lipids [11, 12], and later onset of autonomic failure [32] predict slower dis- genetic factors [13], microglial activation [14], neuroin- ease progression. Bronchopneumonia, urosepsis, and sudden fammation [15], proteolytic disturbance, autophagy [16], death are the main causes of death in MSA. Sudden death, and other factors contributing to oxidative stress [17] have which often occurs at night, is thought to result from either been reported as part of the pathogenesis of MSA. acute bilateral vocal-cord paralysis or acute disruption of the MSA is an orphan disease [18] with an estimated inci- brainstem cardiorespiratory drive. dence of 0.6–3 per 100,000 people [19, 20]. The incidence Striatonigral degeneration (SND) and olivopontocer- increases with age reaching 12/100,000 in those aged over ebellar atrophy (OPCA) are pathological subtypes of MSA. 70 [21], with no diference between men and women [22, Four levels of pathological severity have been described on 23]. Prevalence estimates range from 1.9 to 4.9 [24] and autopsy of MSA brains. The typical α-synuclein immunore- may reach up to 7.8 per 100,000 after the age of 40 [20, active inclusion pathology (GCIs) within oligodendrocytes 22, 23]. [41] are at the most severe end and are required for a defnite Given its varied clinical manifestation, MSA is frequently postmortem diagnosis of MSA. Less frequent are neuronal misdiagnosed, especially at disease onset. An autonomic cytoplasmic (NCI) and neuronal nuclear inclusions (NNI), presentation of MSA can be indistinguishable from pure glial nuclear (GNI), astroglial cytoplasmic inclusions, and autonomic failure (PAF). PAF is currently considered an neuronal threads, also composed of α-synuclein. The next idiopathic, sporadic, rare neurodegenerative disorder char- level of severity is characterized by selective neuronal loss acterized by autonomic failure without other neurological and axonal degeneration involving multiple regions of the symptoms or signs [25]. Recently, PAF has received more nervous system with predominant involvement of the stria- attention from researchers, partly due to shared common tonigral and OPC systems, followed by myelin degeneration features with other forms of synucleinopathy. Furthermore, with pallor and reduction in myelin basic protein (MBP), recent studies show that a signifcant proportion of patients with accompanying astrogliosis. The fnal and most mild with PAF eventually develop DLB, PD, or MSA over time. form of pathology is microglial activation alone [17]. There are certain features that could predict these conver- Glial cytoplasmic inclusions (GCIs) and the resulting sions [26, 27] raising the question of whether PAF is indeed neurodegeneration commonly occur across multiple CNS a distinct disease entity or simply a prodromal disease stage sites. In addition to the striatonigral and OPC involvement, of alpha-synucleinopathies. CGIs are found in the autonomic nuclei of the brainstem A patient presenting with parkinsonism with autonomic [locus coeruleus (LC), nucleus raphe, dorsal vagal nuclei, involvement may be misdiagnosed as sufering from Parkin- etc.], spinal cord, sacral visceral pathways [42], and the son’s disease. Ataxias resulting from diverse etiologies such peripheral nervous system [43], defning MSA as a truly as toxins, immune-mediated, or genetic forms—for exam-
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