Proceedings of the first global meeting of the Posterior Fossa Society: state of the art in cerebellar mutism syndrome.

PURPOSE: The Posterior Fossa Society, an international multidisciplinary group, hosted its first global meeting designed to share the current state of the evidence across the multidisciplinary elements of pediatric post-operative cerebellar mutism syndrome (pCMS). The agenda included keynote talks from world-leading speakers, compelling abstract presentations and engaging discussions led by members of the PFS special interest groups. METHODS: This paper is a synopsis of the first global meeting, a 3-day program held in Liverpool, England, UK, in September 2022. RESULTS: Topics included nosology, patient and family experience, cerebellar modulation of cognition, and cerebellar cognitive affective syndrome. In addition, updates from large-scale studies were shared as well as abstracts across neuroradiology, neurosurgery, diagnosis/scoring, ataxia, and rehabilitation. CONCLUSIONS: Based on data-driven evidence and discussions, each special interest group created research priorities to target before the second global meeting, in the spring of 2024.

REhabilitation Approaches in CHildren with cerebellar mutism syndrome (REACH): An international cross-disciplinary survey study.

OBJECTIVE: Pediatric cerebellar mutism syndrome (pCMS) can occur following resection of a posterior fossa tumor and, although some symptoms are transient, many result in long-lasting neurological deficits. A multi-disciplinary rehabilitation approach is often used in cases of pCMS; however, there have been no clinical trials to determine gold standards in rehabilitation practice in this population, which remains a research priority. The purpose of this study was to identify and compare intervention practices used in pCMS throughout the disciplines of occupational and physical therapy, speech-language pathology, and neuropsychology across geographic regions. METHODS: A 55-question e-survey was created by an international multidisciplinary research group made up of members of the Posterior Fossa Society and sent to rehabilitation professionals in pediatric neuro-oncology centers in the US, Canada, and Europe. RESULTS: Although some differences in the type of intervention used in pCMS were identified within each discipline, many of the targeted interventions including dose, frequency, and intensity were similar within disciplines across geographic regions. In addition, there were common themes identified across disciplines regarding challenges in the rehabilitation of this population. CONCLUSION: These results provide a foundation of current practices on which to build future intervention-based clinical trials.

Effects of low-frequency repetitive transcranial magnetic stimulation in adductor laryngeal dystonia: a safety, feasibility, and pilot study.

PURPOSE: The effects of neuromodulation are virtually unexplored in adductor laryngeal dystonia (AdLD), a disorder characterized by involuntary contraction of intrinsic laryngeal muscles. Recent findings indicated that intracortical inhibition is reduced in people with AdLD. Low-frequency repetitive transcranial magnetic stimulation (rTMS) induces prolonged intracortical inhibition, but the effects in AdLD are unexplored. This pilot and feasibility study aimed to examine the safety, feasibility, and effects of a single session 1 Hz rTMS over the laryngeal motor cortex (LMC) in people with AdLD and healthy individuals. METHODS: The stimulation location was individualized and determined through TMS-evoked responses in the thyroarytenoid muscles using fine-wire electrodes. 1200 pulses of 1 Hz rTMS were delivered to the left LMC in two groups: Control (n = 6) and AdLD (n = 7). Tolerance, adverse effects, intracortical inhibition, and voice recordings were collected immediately before and after rTMS. Voice quality was assessed with acoustic-based and auditory-perceptual measures. RESULTS: All participants tolerated the procedures, with no unexpected adverse events or worsening of symptoms. No significant effects on intracortical inhibition were observed. In the AdLD group, there was a large-effect size after rTMS in vocal perturbation measures and a small-effect size in decreased phonatory breaks. CONCLUSIONS: One rTMS session over the LMC is safe and feasible, and demonstrated trends of beneficial effects on voice quality and phonatory function in AdLD. These preliminary findings support further investigation to assess clinical benefits in a future randomized sham-controlled trial. CLINICALTRIALS.GOV: NCT02957942, registered on November 8, 2016.

Transcranial magnetic stimulation and functional magnet resonance imaging evaluation of adductor spasmodic dysphonia during phonation.

BACKGROUND: Reduced intracortical inhibition is a neurophysiologic finding in focal dystonia that suggests a broader problem of impaired cortical excitability within the brain. A robust understanding of the neurophysiology in dystonia is essential to elucidate the pathophysiology of the disorder and develop new treatments. The cortical silent period (cSP) is a reliable, non-invasive method to measure intracortical inhibition in the primary motor cortex associated with a muscle of interest. In adductor spasmodic dysphonia (AdSD), cSP of the laryngeal motor cortex (LMC) which directly corresponds to the affected musculature, the thyroarytenoid (TA), has not been examined. OBJECTIVE: This work evaluated the cSP of the LMC and the relationship between cSP and functional magnetic resonance imaging (fMRI) blood-oxygen-level dependent (BOLD) activation in people with AdSD (n = 12) compared to healthy controls (CTL, n = 14). RESULTS: Shortened LMC cSP were observed bilaterally in people with AdSD vs CTL (F(1, 99) = 19.5226, p < 0.0001), with a large effect size (η2 = 0.1834). Between-group fMRI analysis revealed greater activation in bilateral LMC in the AdSD > CTL contrast as compared to CTL > AdSD contrast. Correlation analysis showed that people with AdSD have positive correlation of left LMC BOLD activation and the cSP. Further, the right LMC cSP lacks either positive or negative associations with BOLD activation. CTL individuals displayed both positive and negative correlations between cSP and BOLD activation in the left LMC. In CTL, the LMC cSP and BOLD activation showed exclusively negative correlations in both hemispheres. CONCLUSION: In AdSD, the cortical activation during phonation may not be efficiently or effectively associated with inhibitory processes, leading to muscular dysfunction. These findings may give insight into the maladaptive cortical control during phonation in people with AdSD.