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Background: Progressive weakness can affect bulbar muscles in individuals with moderate to severe forms of spinal muscular atrophy (SMA). The paucity of standardized, valid bulbar assessments capturing clinically significant deficits in SMA impedes the ability to monitor function, facilitate intervention, or detect treatment response. Objective: To fill this void, an international multidisciplinary team gathered to develop an agreed upon consensus-derived assessment of bulbar function in SMA for inter-professional administration to enhance our ability to monitor disease progression, support clinical management, and evaluate treatment effects. Methods: Fifty-six international clinicians experienced in SMA were invited and engaged using the Delphi method over multiple rounds of web-based surveys to establish consensus. Results: Serial virtual meetings occurred with 42 clinicians (21 speech and language therapists, 11 physical therapists, 5 neurologists, 4 occupational therapists, and 1 dentist). Seventy-two validated assessments of bulbar function were identified for potential relevance to individuals with SMA (32 accessible objective, 11 inaccessible objective, 29 patient-reported outcomes). Delphi survey rounds (n = 11, 15, 15) achieved consensus on individual items with relevance and wording discussed. Key aspects of bulbar function identified included: oral intake status, oral facial structure and motor strength, swallowing physiology, voice & speech, and fatigability. Conclusions: Multidisciplinary clinicians with expertise in bulbar function and SMA used Delphi methodology to reach consensus on assessments/items considered relevant for SMA across all age groups. Future steps include piloting the new scale moving towards validation/reliability. This work supports the advancement of assessing bulbar function in children and adults with SMA by a variety of professionals.

Aims A recent double‐blind placebo‐controlled crossover 70‐day trial demonstrated that a fixed combination of dextromethorphan and quinidine (DM/Q) improves speech and swallowing function in most patients with amyotrophic lateral sclerosis. In this study, a subset of participants, many of whom did not substantially improve while on DM/Q, were re‐evaluated using computer‐based speech analyses and expert clinician ratings of the overall severity of speech impairment. Methods Speech samples were recorded from the subset of 10 patients at four visits made at approximately 30‐day intervals. The recordings were analysed by automated computer‐based analysis of speech pausing patterns. Severity of speech impairment was rated by three experienced speech‐language pathologists using direct magnitude estimation. Scores on patient‐reported and clinician‐administered scales of bulbar motor involvement were obtained at each visit. Results The effects of DM/Q were detected on several of the objective speech measures, including total pause duration (s) (Cohen's d = 0.73, 95% confidence interval (CI) –1.70, 0.24), pause time (%) (d = 0.77, 95% CI –1.75, 0.21), and mean speech event duration (s) (d = 0.52, 95% CI –0.44, 1.47), but not on clinician ratings of speech or the speech components of the self‐report or clinician‐administered scales. Conclusions These findings suggest that even patients with modest improvement while on DM/Q may experience quantifiable improvements in speech when assessed using sensitive and objective measures. This study provides additional evidence of the positive impact of DM/Q on one or more of the neural systems that control bulbar motor function and production of speech.

In this study, the effect of typicality on lexical access and semantic feature processing was examined by comparing performance on naming and category verification tasks between feature-based (bird, vegetable) and perceptually-based (color) categories. Results demonstrated faster processing of typical exemplars than atypical exemplars across tasks and category types. Naming latencies were similar between feature-based and perceptually-based categories, however verification times were faster for colors than for bird and vegetable stimuli. This is consistent with the theory that color judgments are made through a separate cognitive route that bypasses the semantic system. Results are discussed in relation to models of typicality and lexical processing