The International Parkinson’s and Movement Disorder Society (MDS) criteria for progressive supranuclear palsy (PSP) have broadened the clinical spectrum of the disease and established phenotypic characterization according to the predominant manifestation at onset. The objective of this study is to describe clinical/cognitive and imaging features of a monocentric cohort of PSP patients, highlighting different patterns of functional disability according to the assigned phenotype. We retrospectively reviewed clinical/imaging data of 53 PSP patients diagnosed with probable PSP according to the MDS criteria and 40 age/sex-matched healthy controls (HCs). Neurological/neuropsychological assessments were performed using standardized scales, as well as comprehensive magnetic resonance imaging (MRI) morphometric measurements. In our cohort, there were 24/53 PSP-RS (Richardson’s syndrome), 13/53 PSP-P (Parkinsonism), 7/53 PSP-PGF (Progressive gait freezing), and 9/53 PSP-Cog (Cognitive impairment). PSP-Cog presented the worst motor profiles, the highest percentages of dementia and impaired functional autonomy; 4/9 PSP-Cog and 2/7 PSP-PGF died. PSP-P had the lowest motor/cognitive burden. All MRI parameters had good discriminative efficacy vs. HCs, with P/M 2.0 discriminating PSP-PGF from PSP-RS and PSP-Cog. We highlighted discrete clinical and imaging patterns that best characterize different PSP phenotypes. PSP-Cog and PSP-PGF/RS manifest greater incidence of dementia and motor disability, respectively, while PSP-P has a more benign course. The identification of different phenotypes may be the expression of different progression patterns requiring tailored approaches in terms of follow-up and treatment. These findings support the concept of discrete patterns of Tau pathology within the PSP spectrum and encourage research for phenotype-specific outcome measures.
In Parkinson's Disease (PD), recent evidence points toward the involvement of the gut-brain axis as one of the primary physio pathological mechanisms underlying α-Synuclein aggregation and propagation to CNS. Furthermore, gastrointestinal dysfunctions represent one of the main non-motor symptoms in PD, often preceding the development of proper motor symptoms. We aimed to investigate the enteric nervous system (ENS) in PD by characterizing α-Syn alterations and glial responses in duodenum biopsies of PD patients. Patients with symptomatic PD which underwent Duodopa Percutaneous Endoscopic Gastrostomy and Jejunal Tube (PEG-J) procedure were included in the study. A mean of 4 wall biopsies were sampled from each patient. Immunohistochemistry was performed with anti-aggregated α-Syn (5G4) and GFAP antibodies. Morphometrical-semi-quantitative analysis was performed to characterize 5G4+ and GFAP+ density and size. Duodenal control biopsies were included from age-and-sex-matched patients undergoing routine diagnostic endoscopy. Elevated immunoreactivity for aggregated α-Syn was identified in all biopsies of PD patients compared to controls. 5G4+ partially colocalized with neuronal marker β-III-tubulin but was found also outside enteric neurons. Evaluation of enteric glia cells revealed an increased size and density when compared with controls suggesting reactive gliosis. The ENS could be one of the earliest implicated structures in the pathophysiology of PD. The analysis of enteric glia could represent a precocious biological marker of the disease, as its responses to pathological α-Syn could unveil a link between gastrointestinal neural and immune systems in PD inflammation.
Background and purpose: The aim was to evaluate the risk of relapse after severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) vaccination, and its safety and tolerability, in patients with chronic inflammatory neuropathies. Methods:In this multicenter, cohort and case-crossover study, the risk of relapse associated with SARS-CoV-2 vaccination was assessed by comparing the frequency of relapse in chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) and multifocal motor neuropathy (MMN) patients who underwent or did not undergo vaccination. Frequency of relapse in the 3 months prior to and after vaccination, and safety and tolerability of SARS-CoV-2 vaccination, were also assessed.Results: In all, 336 patients were included (278 CIDP, 58 MMN). Three hundred and seven (91%) patients underwent SARS-CoV-2 vaccination. Twenty-nine patients (9%) did not undergo vaccination. Mild and transient relapses were observed in 16 (5%) patients ( 13CIDP, 3 MMN) after SARS-CoV-2 vaccination and in none of the patients who did not undergo vaccination (relative risk [RR] 3.21, 95% confidence interval [CI] 0.19-52.25).There was no increase in the specific risk of relapse associated with type of vaccine or diagnosis. Comparison with the 3-month control period preceding vaccination revealed an increased risk of relapse after vaccination (RR 4.00, 95% CI 1.35-11.82), which was restricted to CIDP patients (RR 3.25, 95% CI 1.07-9.84). The safety profile of SARS-CoV-2 vaccination was characterized by short-term, mild-to-moderate local and systemic adverse events. Conclusions:Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) vaccination in CIDP and MMN patients does not seem to be associated with an increased risk of relapse at the primary end-point, although a slightly increased risk in CIDP patients was found compared to the 3 months before vaccination.
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