This article presents the revised consensus criteria for the diagnosis of frontotemporal dysfunction in amyotrophic lateral sclerosis (ALS) based on an international research workshop on frontotemporal dementia (FTD) and ALS held in London, Canada in June 2015. Since the publication of the Strong criteria, there have been considerable advances in the understanding of the neuropsychological profile of patients with ALS. Not only is the breadth and depth of neuropsychological findings broader than previously recognised – – including deficits in social cognition and language – but mixed deficits may also occur. Evidence now shows that the neuropsychological deficits in ALS are extremely heterogeneous, affecting over 50% of persons with ALS. When present, these deficits significantly and adversely impact patient survival. It is the recognition of this clinical heterogeneity in association with neuroimaging, genetic and neuropathological advances that has led to the current re-conceptualisation that neuropsychological deficits in ALS fall along a spectrum. These revised consensus criteria expand upon those of 2009 and embrace the concept of the frontotemporal spectrum disorder of ALS (ALS-FTSD).

Spoken discourse is a fundamental form of communication, yet often disrupted in persons with aphasia and other neurogenic communication disorders. It follows that discourse analysis is important fo...

Introduction: Speech impairment in Parkinson's disease (PD) is pervasive, with life‐impacting consequences. Yet, little is known about how functional connections between the basal ganglia and cortex relate to PD speech impairment (PDSI). Whole‐brain resting‐state connectivity analyses of basal ganglia nuclei can expand the understanding of PDSI pathophysiology.Methods: Resting‐state data from 89 right‐handed subjects were downloaded from the Parkinson's Progression Markers Initiative database. Subjects included 12 older healthy controls (“OHC”), 42 PD patients without speech impairment (“PDN”), and 35 PD subjects with speech impairment (“PDSI”). Subjects were assigned to PDN and PDSI groups based on the Movement Disorders Society Unified Parkinson's Disease Rating Scale (MDS‐UPDRS) Part III speech item scores (“0” vs. “1–4”). Whole‐brain functional connectivity was calculated for four basal ganglia seeds in each hemisphere: putamen, caudate, external globus pallidus (GPe), and internal globus pallidus (GPi). For each seed region, group‐averaged connectivity maps were compared among OHC, PDN, and PDSI groups using a multivariate ANCOVA controlling for the effects of age and sex. Subsequent planned pairwise t‐tests were performed to determine differences between the three groups using a voxel‐wise threshold of p < 0.001 and cluster‐extent threshold of 272 mm³ (FWE<0.05).Results: In comparison with OHCs, both PDN and PDSI groups demonstrated significant differences in cortical connectivity with bilateral putamen, bilateral GPe, and right caudate. Compared to the PDN group, the PDSI subjects demonstrated significant differences in cortical connectivity with left putamen and left GPi. PDSI subjects had lower connectivity between the left putamen and left superior temporal gyrus compared to PDN. In addition, PDSI subjects had greater connectivity between left GPi and three cortical regions: left dorsal premotor/laryngeal motor cortex, left angular gyrus, and right angular gyrus.Conclusions: The present findings suggest that speech impairment in PD is associated with altered cortical connectivity with left putamen and left GPi.

Background: Reduced swallowing frequency affects secretion management in Parkinson disease (PD). Gum chewing increases saliva flow and swallow frequency. This study uses chewing gum to modify swallow frequency and latency between swallows in patients with PD. Objectives: 1) Assess the frequency and latency of swallow at baseline (BL), during gum chewing (GC), and post gum chewing (PGC) for participants with PD (stage 2–4) nonsymptomatic for prandial dysphagia; and 2) assess carryover after gum is expectorated. Methods: Twenty participants were studied across 3 tasks, each of 5 minutes in duration: BL, GC, and PGC. Respiratory and laryngeal signals were continuously recorded using PowerLab (version 5.5.5; ADI Instruments, Castle Hill, Australia). Frequency and latency of swallow events were calculated. Results: Differences (analysis of variance) are reported for frequency (p < 0.000001) and latency (p < 0.000001). Swallow frequency (mean ± SD) increased during GC (14.95 ± 3.02) compared with BL (3.1 ± 2.85) and PGC (7.0 ± 2.57). Latency in seconds (mean ± SD) decreased during GC (24.1 ± 4.174) and increased with BL (131.8 ± 59.52) and PGC (mean = 60.74 ± 25.25). Intertask comparisons (t test) found differences in swallow frequency and latency between tasks: BL vs GC (p < 0.0001, p < 0.0001), BL vs PGC (p < 0.0011, p < 0.0009), and GC vs PGC (p < 0.0001, p < 0.0002), respectively. Post hoc analysis showed carryover to 5.317 minutes. Conclusions: Modifying sensorimotor input by chewing gum alters frequency and latency of swallowing and may be an effective strategy for secretion management in Parkinson disease. Classification of evidence: This study provides Class III evidence that chewing gum increases swallow frequency and decreases latency of swallowing in an experiment in patients with stage 2 to 4 Parkinson disease who are nonsymptomatic for significant prandial dysphagia.