Spinal muscular atrophy (SMA) is a group of neurodegenerative disorders resulting from the loss of spinal motor neurons. 95% of patients share a pathogenic mechanism of loss of survival motor neuron (SMN) 1 protein expression due to homozygous deletions or other mutations of the SMN1 gene, with the different phenotypes influenced by variable copy numbers of the SMN2 gene. Advances in supportive care, disease modifying treatment and novel gene therapies have led to an increase in the prevalence of SMA, with a third of SMA patients now represented by adults.Despite the growing number of adult patients, consensus on the management of SMA has focused primarily on the pediatric population. As the disease burden is vastly different in adult SMA, an approach to treatment must be tailored to their unique needs. This review will focus on the management of the adult SMA patient as they age and will discuss proper transition of care from a pediatric to adult center, including the need for continued monitoring for osteoporosis, scoliosis, malnutrition, and declining mobility and functioning. As in the pediatric population, multidisciplinary care remains the best approach to the management of adult SMA.Novel and emerging therapies such as nusinersen and risdiplam provide hope for these patients, though these medications are of uncertain efficacy in this population and require additional study.

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Objective Motor unit number index is a quantitative electrophysiological measure that provides an index of the number of motor neurons supplying a muscle. The aim of this exploratory study was to assess the utility of motor unit number index in the evaluation of patients with lumbar spinal stenosis. Design Participants were assigned to three groups: clinical and radiological lumbar stenosis (lumbar spinal stenosis with neurogenic intermittent claudication), group A; radiological lumbar spinal stenosis without neurogenic intermittent claudication, group B; and a control group, group C. Patients self-rated their pain and functional disability using the numerical rating scale and a series of functional questionnaires. An electromyographer performed nerve conduction tests, electromyography, and motor unit number index testing. Results Seventeen patients completed the study. There were 9, 5, and 3 patients in groups A, B, and C, respectively. There were no discernable differences in motor unit number index values of any individual muscle or combined motor unit number index score between the three groups. Motor unit number index values did not correlate to pain/functional measures. Conclusions In this exploratory study, motor unit number index did not demonstrate discriminatory ability between patients with lumbar spinal stenosis and was not correlated with pain and functional measures. Further study is needed to explore motor unit number index’s role in longitudinal evaluation of patients with the clinical syndrome of lumbar spinal stenosis.