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Distinctive Speech Signature in Cerebellar and Parkinsonian Subtypes of Multiple System Atrophy

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Distinctive Speech Signature in Cerebellar and Parkinsonian Subtypes of Multiple System Atrophy Journal of Neurology (2019) 266:1394–1404 https://doi.org/10.1007/s00415-019-09271-7 ORIGINAL COMMUNICATION Distinctive speech signature in cerebellar and parkinsonian subtypes of multiple system atrophy Jan Rusz1,2 · Tereza Tykalová1 · Giulio Salerno3 · Serena Bancone3 · Johara Scarpelli3 · Maria Teresa Pellecchia3 Received: 16 January 2019 / Revised: 28 February 2019 / Accepted: 5 March 2019 / Published online: 11 March 2019 © Springer-Verlag GmbH Germany, part of Springer Nature 2019 Abstract Although motor speech disorders represent an early and prominent clinical feature of multiple system atrophy (MSA), the potential usefulness of speech assessment as a diagnostic tool has not yet been explored. This cross-sectional study aimed to provide a comprehensive, objective description of motor speech function in the parkinsonian (MSA-P) and cerebellar (MSA- C) variants of MSA. Speech samples were acquired from 80 participants including 18 MSA-P, 22 MSA-C, 20 Parkinson’s disease (PD), and 20 healthy controls. The accurate differential diagnosis of dysarthria subtypes was based on quantitative acoustic analysis of 14 speech dimensions. A mixed type of dysarthria involving hypokinetic, ataxic and spastic components was found in the majority of MSA patients independent of phenotype. MSA-P showed significantly greater speech impair- ment than PD, and predominantly exhibited harsh voice, imprecise consonants, articulatory decay, monopitch, excess pitch fluctuation and pitch breaks. MSA-C was dominated by prolonged phonemes, audible inspirations and voice stoppages. Inappropriate silences, irregular motion rates and overall slowness of speech were present in both MSA phenotypes. Speech features allowed discrimination between MSA-P and PD as well as between both MSA phenotypes with an area under curve up to 0.86. Hypokinetic, ataxic and spastic dysarthria components in MSA were correlated to the clinical evaluation of rigidity, cerebellar and bulbar/pseudobulbar manifestations, respectively. Distinctive speech alterations reflect underlying pathophysiology in MSA. Objective speech assessment may provide an inexpensive and widely applicable screening instru- ment for differentiation of MSA and PD from controls and among subtypes of MSA. Keywords Multiple system atrophy · Parkinson’s disease · Atypical parkinsonism · Dysarthria · Speech disorder · Acoustic analyses Introduction MSA are clinically classified as parkinsonian variant (MSA- P) if parkinsonism is the predominant feature and as cer- Multiple system atrophy (MSA) is a sporadic, adult-onset, ebellar variant (MSA-C) if cerebellar features predominate fatal neurodegenerative disease characterized by progressive [2]. Due to variable clinical manifestations, MSA presents a autonomic failure, parkinsonian features, and any combina- major diagnostic challenge as it may masquerade as Parkin- tion of cerebellar and pyramidal features [1]. Patients with son’s disease (PD) or idiopathic late-onset cerebellar ataxia until advanced stages of the disease. Therefore, reliable screening instruments are pivotal as an accurate diagnosis of * Jan Rusz MSA has important prognostic and therapeutic implications. [email protected] As the most complex human motor skill, speech is a 1 Department of Circuit Theory, Faculty of Electrical sensitive marker of damage to neural structures engaged in Engineering, Czech Technical University in Prague, Prague, motor system control [3]. Speech abnormalities develop in Czech Republic the majority of patients with parkinsonism [4, 5], and rep- 2 Department of Neurology and Centre of Clinical resent one of the first prodromal markers of motor dysfunc- Neuroscience, First Faculty of Medicine, Charles University, tion [6, 7]. MSA patients typically develop mixed dysar- Prague, Czech Republic thria with various combinations of hypokinetic, spastic and 3 Center for Neurodegenerative Diseases (CEMAND), ataxic components [5]. Unfortunately, complex investigation Department of Medicine, Surgery and Dentistry “Scuola of speech disorder in MSA has rarely been performed and Medica Salernitana”, University of Salerno, Salerno, Italy Vol:.(1234567890)1 3 Journal of Neurology (2019) 266:1394–1404 1395 limited to perceptual assessment [5, 8]. Only a few stud- to reveal subperceptual speech deviations [16]. Disease ies have provided objective descriptions of specific dysar- duration was estimated based on the self-reported occur- thria manifestations in MSA [9–14]. Dysarthria in MSA rence of first motor symptoms. MSA patients were rated by has been reported to be more pronounced than in PD and the Natural History of Neuroprotection in Parkinson plus characterized by excess pitch fluctuations, excess loudness syndromes-Parkinson plus scale (NNIPPS) [18], while PD variations, increased voice pitch, slow rate, imprecise con- patients were scored according to the motor subscore of sonant articulation, prolonged phonemes, vocal tremor, and Unified Parkinson’s Disease Rating Scale (UPDRS III). The strained-strangled voice [9–14]. Nothing, however, is known NNIPPS scale was chosen as it allows evaluating all clinical so far about the prevalence and patterns of abnormal speech symptoms encountered in parkinsonism that may potentially features in the different phenotypes of MSA and their rela- influence speech production. Parkinsonian features in both tion to clinical features. Moreover, speech-based differen- MSA and PD patients were treated with levodopa alone or tiation between MSA phenotypes may be challenging as a in combination with dopamine agonists and/or amantadine. substantial overlap of speech abnormalities across different All patients were investigated in the on-drug state. No par- dysarthria subtypes can be expected [5, 15, 16]. ticipants received antipsychotic medication. Therefore, we quantitatively assessed 14 speech dimen- The dysarthria presence, type, and severity estimates sions by objective acoustic analyses to determine specific were based on the auditory-perceptual judgment of a speech- dysarthric patterns and estimate their reliability in differen- language pathologist experienced in movement disorders. tiating among PD and both subtypes of MSA. We hypoth- The judgment was based on offline audio recordings includ- esized that quantitative acoustic measures would be affected ing vowel prolongation, sequential motion rates, and con- differently between PD, MSA-P, and MSA-C. An additional nected speech following the perceptual criteria outlined by aim was to explore the relationship between speech and clin- Darley et al. [19, 20]. In addition, perceptual speech sever- ical motor manifestations to provide more insights into the ity was estimated for all patients using speech item of the pathophysiology of dysarthria in MSA. We hypothesized UPDRS III (item 18). Detailed patient characteristics are that the extent of bradykinesia and rigidity would be related listed in Table 1. to the severity of hypokinetic dysarthria elements, cerebel- lar clinical manifestations to ataxic dysarthria components, Speech examination and bulbar/pseudobulbar clinical signs to spastic dysarthria dimensions. Speech recordings were performed in a quiet room with a low ambient noise level using a head-mounted condenser microphone (Beyerdynamic Opus 55, Heilbronn, Germany) Methods placed approximately 5 cm from the participant’s mouth. Speech signals were sampled at 48 kHz with 16-bit resolu- Participants tion. Each participant was recorded during a single session. All participants were instructed to perform three vocal tasks From 2015 to 2018, 40 consecutive patients with a clinical of (i) sustained phonation of the vowel /a/ per one breath diagnosis of probable MSA were recruited for the current for as long and steadily as possible, (ii) rapid /pa/-/ta/-/ka/ study. In this cohort, 22 patients (12 men) were diagnosed syllable repetition at least seven times per one breath (oral with MSA-C and 18 (9 men) with MSA-P. In addition, 20 diadochokinesis), and (iii) monologue on a given topic for patients with idiopathic PD (11 men) were recruited to approximately 90 s. These three speaking tasks were chosen match the MSA groups according to disease duration, age, as they can provide most of the information necessary for gender, and cognitive status. A healthy control (HC) group the objective description and interpretation of motor speech consisting of 20 participants comparable for gender distribu- disorders [15]. Sustained phonation and syllable repetition tion (11 men) and age (mean 62.3, standard deviation 6.7, paradigms were repeated two times for every participant. range 47–73 years) with no history of neurological or com- munication disorders was also included. All participants Speech analyses were native Italian speakers. The diagnosis of MSA was established by the consensus The primary outcome represented the assessment of the diagnostic criteria for MSA [2], whereas the diagnosis of hypokinetic dysarthria index, ataxic dysarthria index, spastic PD was based on the UK Parkinson’s Disease Society Bank dysarthria index and overall dysarthria index. These dys- Criteria [17]. The diagnosis was confirmed by a neurolo- arthria indexes were based upon the quantitative acoustic gist with experience in movement disorders (M.T.P.). The analysis, which provides objective, sensitive and quantifi- presence of dysarthria was not considered as a part of the able information for the precise assessment of speech per- inclusion criteria as the acoustic analyses have the potential formance. We selected
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