Diurnal Vocalization Patterns of Captive Asiatic Lions (Panthera leo persica) Persist After the Replacement of a Single Dominant Male in a Small Pride Living at High Latitude.
Authors: Walsh C, Fonteneau J, Hodnett J, Leahy J, Walsh F, McKeown S, O'Donovan D, Killeen GF
Journal: Zoo biology
mental health
psychology
open access
Abstract
Spinal muscular atrophy (SMA) is a progressive neuromuscular disease characterized by the degeneration of spinal cord motor neurons and caused by a mutation or deletion of the gene. SMA presents with various phenotypes, depending on the age of onset and the severity of muscle weakness and atrophy. In the past decade, the advances of supportive care and the introduction of disease-modifying therapies, such as nusinersen, onasemnogene abeparvovec, and risdiplam, have significantly improved motor function and survival, especially in patients with infantile-onset SMA. However, these therapeutic gains also expose gaps in outcome measurement approaches for adolescents and adults. Traditional functional motor scales, such as the Hammersmith Functional Motor Scale Expanded (HFMSE), Revised Upper Limb Module (RULM), and Motor Function Measure-32 (MFM-32 or MFM), though commonly used, demonstrate ceiling and floor effects that limit their sensitivity across the full spectrum of disease severity. In particular, their ability to detect meaningful clinical changes in adults with advanced disease is limited. Furthermore, constructs such as participation and role functioning are poorly assessed in these motor-centric tools. Efforts to address these limitations have led to increased interest in patient-reported outcome measures (PROMs), including instruments like the Spinal Muscular Atrophy Independence Scale (SMAIS), the SMA Health Index (SMA-HI), Quality of Life for genetic Neuromuscular disorders (QoL-gNMD), or the Patient Reported Outcomes Measurement Information System (PROMIS). These tools seek to capture more holistic dimensions of health such as fatigue, respiratory capacity, swallowing, and autonomy, which are critical to patients but underrepresented in standard clinical assessments. However, challenges remain around responsiveness, interpretability, real-world applicability, and alignment with patient-defined goals.