Reliability of confocal corneal microscopy for measurement of dendritic cell density in suspected small fiber neuropathy.

INTRODUCTION/AIMS: Dendritic cells (DCs) and their contacts with corneal nerves are described in animal models of nerve damage. Dendritic cell density (DCD) is a potential marker of immune activity in suspected small-fiber neuropathy (SFN). Here, we aim to evaluate the intra- and inter-rater reliability of DCD measurements in suspected SFN. METHODS: This retrospective study collected DCD from confocal microscopy images from the corneal sub-basal epithelium of the eye from 48 patients (mean age 49.6 ± 12.1 y, 61% female). Two examiners, each blinded to the other's examinations and measurements, assessed DCD to evaluate inter-rater reliability. For intra-rater reliability, the first examiner performed a second measurement after 14 days. DCs were classified into two cell morphological subtypes: mature and immature. RESULTS: Test-retest reliability for total DCD showed excellent agreement, with an intraclass correlation coefficient of 0.96 and inter-rater reliability intraclass correlation coefficient of 0.77. The immature cell subtype showed excellent intra-rater reliability but lower inter-rater reliability. DISCUSSION: We found that DCD measurements in the corneal sub-basal epithelium are sufficiently reliable for consideration in clinical studies of patients with suspected SFN.

Optimal cut-off value of the modified Toronto Clinical Neuropathy Score in the diagnosis of polyneuropathy.

BACKGROUND AND PURPOSE: The modified Toronto Clinical Neuropathy Score (mTCNS) is a valid and reliable scale for the diagnosis and staging of diabetic sensorimotor polyneuropathy (DSP). The aim of this study was to determine the optimal diagnostic cut-off value of the mTCNS in diverse polyneuropathies (PNPs). METHODS: Demographics and mTCNS values were retrospectively extracted from an electronic database of 190 patients with PNP and 20 normal controls. Sensitivity, specificity, and likelihood ratios and area under the receiver-operating characteristic (ROC) curve were determined for each diagnosis and different cut-off values of the mTCNS. Patients underwent clinical, electrophysiological and functional assessments of their PNP. RESULTS: Forty-three percent of PNP was related to diabetes or impaired glucose tolerance. mTCNS was significantly higher in patients with PNP than in those without (15.27 ± 8 vs. 0.79 ± 1.4; p = 0.001). The cut-off value for diagnosing PNP was ≥3 (sensitivity 98.4%, specificity 85.7%, positive likelihood ratio 6.88). The area under the ROC curve was 0.987. CONCLUSION: A value of 3 or more on the mTCNS is recommended for the diagnosis of PNP.

Epidemiology of Parkinson's Disease in Chile.

INTRODUCTION: Parkinson's disease (PD) is one of the most common neurodegenerative disorders. There is no epidemiological description of PD in Chile and not many descriptions in Latin America. This study aims to describe the incidence and prevalence of PD in Chile. METHODS: The study group was the population on the public health system in Chile between 2010 and 2018 that were registered in the GES system as having PD. Crude and standardized prevalence and incidence were calculated with a 95% confidence interval. RESULTS: 33,345 patients were found in the register as confirmed cases with PD. The crude incidence in 2018 was 23.7/100,000; the crude prevalence in 2018 was 160.7/100,000. The male-to-female ratio was 1.03. CONCLUSION: The prevalence and incidence observed in the Chilean population are consistent with studies from other countries.

Neurogenic Orthostatic Hypotension. Lessons From Synucleinopathies.

Maintenance of upright blood pressure critically depends on the autonomic nervous system and its failure leads to neurogenic orthostatic hypotension (NOH). The most severe cases are seen in neurodegenerative disorders caused by abnormal α-synuclein deposits: multiple system atrophy (MSA), Parkinson's disease, Lewy body dementia, and pure autonomic failure (PAF). The development of novel treatments for NOH derives from research in these disorders. We provide a brief review of their underlying pathophysiology relevant to understand the rationale behind treatment options for NOH. The goal of treatment is not to normalize blood pressure but rather to improve quality of life and prevent syncope and falls by reducing symptoms of cerebral hypoperfusion. Patients not able to recognize NOH symptoms are at a higher risk for falls. The first step in the management of NOH is to educate patients on how to avoid high-risk situations and providers to identify medications that trigger or worsen NOH. Conservative countermeasures, including diet and compression garments, should always precede pharmacologic therapies. Volume expanders (fludrocortisone and desmopressin) should be used with caution. Drugs that enhance residual sympathetic tone (pyridostigmine and atomoxetine) are more effective in patients with mild disease and in MSA patients with spared postganglionic fibers. Norepinephrine replacement therapy (midodrine and droxidopa) is more effective in patients with neurodegeneration of peripheral noradrenergic fibers like PAF. NOH is often associated with other cardiovascular diseases, most notably supine hypertension, and treatment should be adapted to their presence.

Epidemiology of chronic inflammatory demyelinating polyneuropathy in the South-Eastern area of Santiago, Chile.

Chronic inflammatory demyelinating polyneuropathy (CIDP) is an immune-mediated polyneuropathy. It usually has an insidious onset, progressive course and heterogeneous clinical features. As far as we know, there is no epidemiological information on CIDP in South America and the Caribbean. Our aim was to estimate the frequency of CIDP in the South-Eastern region of Santiago, where our hospital is based and the population number assigned is officially reported every year by the health authorities. Records of 581 patients registered with the diagnosis of neuropathy were found and all patients meeting the diagnostic criteria of the EFNS/PNS for definitive and possible CIDP were included. Data were collected using a data extraction protocol designed by the authors and which included demographic, clinical, laboratory and electrophysiological information. The estimated prevalence and incidence of CIDP were 2.95/100,000 and 0.46/100,000 respectively. Fifteen patients (8 men, 7 women) were classified as definitive or possible CIDP. Nine patients had typical CIDP and three also had diabetes mellitus. The prevalence and incidence rates were similar to those reported in other regions of the world.

Cardiovascular responses to isometric handgrip exercise in young patients with recurrent vasovagal syncope.

OBJECTIVE: Arterial blood pressure (BP) increased in healthy humans in response to isometric handgrip (IHG), but the pattern of the relative contribution of cardiac output (CO) and total peripheral resistance (TPR) changes to the pressor response is different among individuals. We investigated weather patients with recurrent vasovagal syncope (VVS) have a similar pattern of individual CO, TPR and pressor responses to IHG, as healthy subjects. METHODS: 32 patients (27.5 ±â€¯2.6 years), and 30 age-matched controls. Autonomic function was evaluated using finger-photoplethysmography to measure BP and heart rate (HR) response to gravitational stress (ΔBP, ΔHR), Valsalva maneuver (VM) and baroreflex sensitivity (BRS), and BP HR and CO changes during IHG. RESULTS: ΔBP, ΔHR, VM and IHG tests did not show significant difference between VVS patients and controls, although BRS was lower in VVS group (p < 0.05). Pattern of individual pressor, CO and TPR responses to IHG was significantly different between VVS patients and healthy subjects (Chi square, p = 0.0246). In 100% of the healthy subjects BP increased during IHG, but in a 19% of the patients BP (CO and TPR) did not increased during IHG. In VVS patients, the autonomic tests (ΔBP, ΔHR, VM and BRS) showed no significant differences between the group with BP increase and the group without pressor response. CONCLUSION: In VVS patients, the pattern of individual CO and TPR changes to IGH is different from healthy subjects. VVS patients may present an abnormal regulation of cardiovascular responses to IHG, with preserved cardiovagal and cardiac sympathetic function.

Descripción electrofisiològica del síndrome de túnel carpiano según edad en pacientes adultos / Electrophysiological severity of carpal tunnel syndrome according to age in adult patients

Background: Carpal tunnel syndrome (CTS) represents 90% of entrapment neuropathies. Severity may be greater in older patients. Aim: To describe the electrophysiological findings in adult patients with CTS and determine if severity is related to age. Material and Methods: Descriptive and retrospective study of electrophysiological findings in patients over 18 years of age with clinical suspicion of CTS, studied between January 2011 and December 2015. Neurophysiological severity was classified in 3 grades, comparing them by age, gender and laterality. Results: Of 1156 patients subjected to electrophysiological studies due to a clinical suspicion of CTS, 690 (60%) had electrophysiological features of the disease. In 274 patients (24%) the compromise was mild, in 162 (14%) it was moderate and in 254 (22%) it was severe. There was a positive association between age and CTS severity (p < 0.01). Severity was significantly greater in males than females (p < 0.01). Bilateral CTS was present in 471 patients (68%), which was associated with increased age and severity (p < 0.01). Conclusions: Electrophysiological severity in CTS increases with age. Other factors associated with higher severity are male gender and bilateral disease.