Assessing Prevalence and Characteristics of Oro-bulbar Involvement in Children and Adults with SMA Type 2 and 3 Using a Multimodal Approach.
Federica Trucco, Francesca Salmin, Andrea Lizio and 19 others
PMID 37289231WHAT IT FOUND
Oro-bulbar impairment is common in SMA types 2 and 3, particularly reduced mouth opening and tongue strength in children and non-ambulant adults.
Objective tests often reveal problems that patients do not report, so clinicians should assess swallowing function directly rather than relying on patient complaints.
Key findings
01Nearly half of the children tested had reduced tongue strength and active mouth opening, with sitters showing significantly worse mouth opening than walkers.
02There was a disconnect between objective impairment and subjective report; while many patients had abnormal test results, only a minority of treated children and adults reported swallowing as burdensome.
03Tongue strength and mouth opening were statistically associated with the time required to eat a standard cracker, suggesting these physical measures directly impact eating efficiency.
STILL TO COME
How it was doneWhat they foundWhat it means for PTsWhat it means for OTsWhat it means for SLPs
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What it does not show
There was no untreated pediatric control group, so the study cannot determine if nusinersen treatment prevented deterioration in children or if the observed stability was natural. The longitudinal analysis was exploratory and had a short follow-up period (median 8 months), limiting conclusions about long-term progression. Objective tests like IOPI and aMMO require maximum patient effort, which can be influenced by fatigue, motivation, and comprehension, potentially affecting reliability. The study compared results to normative data from different countries and populations, which may not perfectly match the Italian cohort's background.
Declared interests
The study was funded by Biogen, the manufacturer of nusinersen, and the Università degli Studi di Milano.
The easy way to misread this
Do not assume that a lack of patient-reported swallowing difficulty means oro-bulbar function is intact. The study found that objective impairments in mouth opening and tongue strength were common, yet only a minority of treated patients reported these issues as burdensome. Clinical assessment must rely on direct measurement of oral-motor function rather than subjective complaints alone.