Interleukin-1 type 1 receptor blockade attenuates the exaggerated exercise pressor reflex in male UC Davis type 2 diabetic mellitus rats.

An exaggerated exercise pressor reflex and peripheral neuropathy are both evoked by the same type of thinly myelinated afferents and are present in patients with type 2 diabetes mellitus (T2DM). Although it is known that the pro-inflammatory cytokine interleukin-1β (IL-1β) contributes to peripheral neuropathy, the effects of IL-1β on the exercise pressor reflex in T2DM are not known. Therefore, we aimed to determine the effect of IL-1 receptors on the exercise pressor reflex in T2DM.

Orthostatic intolerance with tachycardia (postural tachycardia syndrome) and without (hypocapnic cerebral hypoperfusion) represent a spectrum of the same disorder.

Background: Spectrum of chronic orthostatic intolerance without orthostatic hypotension includes postural tachycardia syndrome (POTS), with orthostatic tachycardia and hypocapnic cerebral hypoperfusion (HYCH), without orthostatic tachycardia. This study compared autonomic, cerebrovascular, and neuropathic features of POTS and HYCH.

Methods: This retrospective study evaluated patients with orthostatic intolerance referred for autonomic testing.

Duration of diabetes, glycemic control, and low heart rate variability: The Atherosclerosis Risk in Communities (ARIC) study.

Persons with long-standing, poorly controlled diabetes or recent hyperglycemia had the highest burden of cardiac autonomic neuropathy. Cardiac autonomic neuropathy contributed to elevated long-term incidence of cardiovascular disease and mortality even in persons with well-controlled diabetes.

Middle ear cholesteatoma and facial nerve hypertrophy mimicking schwannoma in Charcot-Marie-Tooth disease: A case report.

Rationale: Charcot-Marie-Tooth (CMT) disease, a hereditary motor and sensory neuropathy, presents with progressive chronic sensory and distal motor polyneuropathy. While sensorineural hearing loss and vestibular impairment have been documented in CMT patients, concurrent middle ear cholesteatoma and persistent direction-changing positional nystagmus have not.

Patient Concerns: This study details a 22-year-old man with CMT1 exhibiting these symptoms.

Spectrum of hereditary transthyretin amyloidosis due to T60A(p.Thr80Ala) variant in an Irish Amyloidosis Network.

Background: Variant transthyretin amyloidosis (ATTRv) is a hereditary multisystem disorder with clinical spectrum ranging from predominant cardiomyopathy to polyneuropathy. In the Irish population, the T60A mutation has been previously recognised as the most common genotype.

Objectives: The aim of this study is to describe the diagnostic and phenotypic spectrum of patients with T60A ATTRv attending an Irish Expert Amyloidosis Network.

The cumulative impact of type 2 diabetes and obstructive sleep apnoea on cardiovascular, liver, diabetes-related and cancer outcomes.

Aim: A bidirectional relationship exists between obstructive sleep apnoea (OSA) and type 2 diabetes (T2D). We aimed to examine the cumulative impact of having both OSA and T2D on patient outcomes, relative to having either condition alone.

Materials And Methods: Using TriNetX, a global federated research network (n = 128 million), we undertook two retrospective cohort studies, using time-to-event analysis.

Small fiber neuropathy associated with COVID-19 infection and vaccination: A prospective case-control study.

Background: Small fiber neuropathy (SFN) after both COVID-19 infection or vaccination has been reported in sporadic cases, but a detailed description and comparison are missing. We aimed to screen a large cohort of patients complaining of pain and autonomic symptoms after COVID-19 natural infection or vaccination to ascertain the presence of SFN and its correlation with autoimmune diseases.

Methods: We prospectively recruited for this case-control study 66 patients: 33 developing sensory and autonomic symptoms after a natural COVID-19 infection (P-COVID) and 33 after a mRNA vaccination against COVID-19 (P-VAC).

A phenotypic comparison of the Romanian and French ATTRv cohorts: Glu54Gln founder pathogenic variant vs the most common variants in Western Europe.

Aim And Methods: We conducted a retrospective observational study of the ATTRv heterozygous mutation frequency, phenotype, and all-cause mortality at two cardiac amyloidosis centers in Romania and France.

Results: 291 patients were included: 26 Glu54Gln (all Romanian), 200 Val122Ile, 47 Val30Met and 18 Ser77Tyr. On diagnosis, Gu54Gln patients were younger than Val122Ile or late-onset Val30Met (median age: 46 [42-50], 76 [71-80] and 70 [61-76], respectively; p < 0.

Clinical Cues for the Early Diagnosis of Transthyretin-Related Polyneuropathy.

Background And Purpose: The estimated prevalence of hereditary transthyretin-related familial amyloid polyneuropathy (TTR-FAP) and the small number of known patients in Germany indicate that many patients with TTR-FAP remain undiagnosed, and may instead be classified as "idiopathic." The aim of this study was to identify biomarkers for detecting TTR-FAP among a cohort of patients with idiopathic polyneuropathy (PNP).

Methods: Clinical evaluations (including the Neuropathy Impairment Score and Neuropathy Disability Score), nerve conduction studies (NCSs), quantitative sensory testing, and autonomic function tests were performed on 23 patients with TTR-FAP and 89 with idiopathic PNP.

Cardiovascular Autonomic Dysfunction Before and After Chemotherapy in Cancer Patients.

Complications that occur during cancer therapy have emerged as a major contributor to the poor quality of life experienced by cancer patients as they live longer due to improved treatments. Many studies have investigated chemotherapy-induced peripheral neuropathy, but few have investigated the autonomic nervous system. Cardiovascular autonomic dysfunction (CAD) contributes to the distressing symptoms experienced by cancer patients, and it is also related to poor treatment outcomes.

Vincristine loaded pegylated liposomal drug delivery for efficient treatment of acute lymphoblastic leukaemia.

Vincristine sulfate (VIN) is commonly employed as a cytotoxic agent in the treatment of hematological malignancies, particularly acute lymphoblastic leukaemia (ALL). However, its maximum therapeutic benefits have been hindered due to the dose-dependent neurotoxic effects it can induce, which traditionally manifest as autonomic and peripheral sensory-motor neuropathy. The innovative approach aimed to address VIN's neurotoxic limitations while preserving its therapeutic efficacy in combating hematological malignancies, including ALL.

Congenital insensitivity to pain with anhidrosis: a literature review and the advocacy for stem cell therapeutic interventions.

Congenital Insensitivity to Pain with Anhidrosis (CIPA) is a rare genetic disorder affecting the autonomic nervous system, leading to an inability to feel pain, temperature, or sweat1. This condition is caused by mutations in the NTRK1 gene, which encodes a receptor for nerve growth factor (NGF). The lack of NGF signaling results in the improper development and function of sensory and sympathetic neurons.

Experimental West Nile virus infection provides lessons for recovery from enteric neuropathies.

Loss of enteric neurons leading to long-term gastrointestinal dysfunction is common to many diseases, and the path to functional recovery is unclear. In this issue of the JCI, Janova et al. report that West Nile virus killed enteric neurons and glia via CD4+ and CD8+ T cells acting through the perforin and Fas ligand pathways.

Diabetic gastroenteropathy: Associations between gastrointestinal symptoms, motility, and extraintestinal autonomic measures.

Background: Diabetic gastroenteropathy can cause significant diagnostic challenges. Still, it remains unknown if measures of extraintestinal autonomic function reflect diabetic gastroenteropathy. We aimed to assess the associations between (1) gastrointestinal symptoms and motility measures and (2) gastrointestinal symptoms/motility measures and extraintestinal autonomic markers.

Association between sebum secretion and cardiac sympathetic dysfunction in Parkinson's disease.

Background: Patients with Parkinson's disease (PD) have α-synuclein (α-Syn) deposition in the skin, and decreased sebum secretion due to epidermal dysfunction. However, the relationship between sebum secretion and autonomic neuropathies is unknown.

Methods: Using the medical records in our facility, we identified patients newly diagnosed with PD on admission from August 2020 to December 2023.

Analysis of ventricular repolarization parameters and heart rate variability in obesity: a comparative study.

Obesity is associated with dysfunctional electrocardiographic and cardiac autonomic parameters, which may lead to increased cardiovascular morbidity. Novel electrocardiographic repolarization markers such as Tpeak-Tend (Tpe) interval have not yet been deeply studied in obese patients. We aimed to investigate the association between ventricular repolarization parameters and heart rate variability (HRV) and how they are affected by changes occurring in the cardiac autonomic nervous system.

Atypical Presentation of Congenital Insensitivity to Pain With Anhidrosis Leading to Diagnostic Odyssey.

Background: Congenital insensitivity to pain with anhidrosis (CIPA) (OMIM 256800) is a rare autosomal-recessive condition, also known as hereditary sensory and autonomic neuropathy type IV (HSAN-IV). The most commonly reported features include anhidrosis, intellectual disability, self-mutilation, febrile episodes, impaired temperature perception, recurrent infections and/or autonomic nervous system impairment. Major joint destruction and joint deformity known as Charcot (neuropathic) joints are also seen in CIPA patients attributed to insensitivity to joint pain.

Autonomic and Immune Stress Response Networks in Patients Living With HIV.

Background And Objectives: Stress response systems are frequently dysregulated in patients with chronic inflammatory disorders. Pre-clinical studies have demonstrated direct influences of the sympathetic and vagal/parasympathetic branches of the autonomic nervous system (ANS) on the immune system. However, these connections have not been examined in humans.

Characteristics of storage and voiding symptoms in adult patients with type 2 diabetes with lower urinary tract symptoms.

Aims: We aimed to assess the relationships between lower urinary tract symptoms (LUTS) and various parameters including diabetes complications in patients with type 2 diabetes.

Methods: In this single-center cross-sectional study, we enrolled 404 patients hospitalized for diabetes. We ultimately analyzed data from 160 patients.

Possible Glycemic Effects of Vagus Nerve Stimulation Evaluated by Continuous Glucose Monitoring in People with Diabetes and Autonomic Neuropathy: A Randomized, Sham-Controlled Trial.

Autonomic neuropathy is associated with dysglycemia that is difficult to control. We investigated if transcutaneous vagus nerve stimulation (tVNS) could improve glycemic levels. We randomized 145 individuals with type 1 diabetes (T1D) ( = 70) or type 2 diabetes (T2D) ( = 75) and diabetic autonomic neuropathy (DAN) to self-administered treatment with active cervical tVNS ( = 68) or sham ( = 77) for 1 week (4 daily stimulations) and 8 weeks (2 daily stimulations), separated by a wash-out period of at least 2 weeks.