Bradyarrhythmia and hypotension during anesthetic induction—reconsideration of nifedipine: a case report

Cardiac events sometimes occur during anesthesia and surgery and may be severe or even life-threatening. This report describes a case of severe bradyarrhythmia during anesthetic induction with propofol, midazolam, sufentanil, and vecuronium. The patient took nifedipine sustained-release tablets on the morning of surgery as routine treatment for hypertension, and this medication may have contributed to the bradyarrhythmia. Nifedipine is a calcium channel blocker that can dilate blood vessels, depress the activity of the sinoatrial node, and delay the conduction of the atrioventricular node. Although these effects are not usually significant, they may be enhanced by anesthetics or other concomitant drugs. For patients of advanced age, especially those with autonomic disturbance or cardiac abnormalities, these effects can be remarkable, and discontinuation of nifedipine should be considered.

Ganglionic Acetylcholine Receptor Antibodies and Autonomic Dysfunction in Autoimmune Rheumatic Diseases

Autonomic neuropathy has been reported in autoimmune rheumatic diseases (ARD) including Sjögren’s syndrome, systemic sclerosis, rheumatoid arthritis, and systemic lupus erythematosus. However, the pathophysiological mechanism underlying autonomic dysfunction remains unknown to researchers. On the other hand, autoimmune autonomic ganglionopathy (AAG) is an acquired immune-mediated disorder, which causes dysautonomia that is mediated by autoantibodies against ganglionic acetylcholine receptors (gAChRs). The purpose of this review was to describe the characteristics of autonomic disturbance through previous case reports and the functional tests used in these studies and address the importance of anti-gAChR antibodies. We have established luciferase immunoprecipitation systems to detect antibodies against gAChR in the past and determined the prevalence of gAChR antibodies in various autoimmune diseases including AAG and rheumatic diseases. Autonomic dysfunction, which affects lower parasympathetic and higher sympathetic activity, is usually observed in ARD. The anti-gAChR antibodies may play a crucial role in autonomic dysfunction observed in ARD. Further studies are necessary to determine whether anti-gAChR antibody levels are correlated with the severity of autonomic dysfunction in ARD.

P.015 Long-term progression and prognosis in different subtypes of Parkinson’s disease: validation of a new multi-domain subtyping method

Background: Parkinson’s disease (PD) varies in clinical manifestations and course of progression from person to person. Identification of distinct PD subtypes is of great priority to develop personalized care approaches. We aimed to compare long-term progression and prognosis between different PD subtypes. Methods: Data on 421 individuals with de novo early-onset PD was retrieved from Parkinson’s Progression Markers Initiative (PPMI). Using a newly developed multi-domain subtyping method (based on motor phenotype, RBD, autonomic disturbance, early cognitive deficit), we divided PD population into three subtypes at baseline: “mild motor-predominant”, “Diffuse malignant” and “Intermediate”. Rate of global progression (mixed motor and non-motor features) and developing dementia were compared between the subtypes. Results: Patients with “diffuse malignant” PD experienced 0.5 z-score further worsening of global composite outcome (p=0.017) and 2.2 further decline in MOCA score (p=0.001) after 6-years of follow-up. Hazard for MCI/dementia was significantly higher in “diffuse malignant” (HR=3.2, p<0.001) and “intermediate” (HR=1.8, p<0.001) subtypes. Individuals with “diffuse malignant” PD had the lowest level of CSF amyloid-beta (p=0.006) and SPECT striatal binding ratio (p=0.001). Conclusions: This multi-domain subtyping is a valid method to predict subgroups of PD with distinct patterns of long-term progression at drug-naïve early-stage with potential application in real-life clinical practice.

Management of Guillain-Barré Syndrome

Guillain-Barré syndrome (GBS) is the most common, immune-mediated acute polyneuropathy characterized by variable degree of motor weakness, often presenting with quadriparesis. GBS with respiratory failure requiring endotracheal intubation with mechanical ventilation is common, affecting approximately 30% of patients. In addition, they present with sensory features, cranial nerve involvement, and autonomic disturbance. The majority of GBS patients have preceding respiratory tract infection or gastroenteritis. The diagnosis of GBS is based on clinical features supported by cerebrospinal fluid (CSF) examination and nerve conduction studies. Early course of the GBS requires meticulous monitoring and early initiation of immunotherapy. Plasma exchange (PE) and intravenous immunoglobulin (IVIg) are the proven therapies, and both have been shown to be equally effective. General supportive care is an important part of management of GBS. A multidisciplinary approach to prevent and manage potential complications in rapidly progressing GBS is important to reduce morbidity and mortality.

Acute Symptomatic Sinus Bradycardia in High-Dose Methylprednisolone Therapy in a Woman With Inflammatory Myelitis: A Case Report and Review of the Literature

High dose corticosteroid therapy is widely used as attack therapy of inflammatory central nervous system disorders and can induce several adverse reactions. Bradycardia is an infrequent event after corticosteroids administration and is often asymptomatic. We report a case of a woman admitted to the neurological department of our hospital for paraesthesias of the lower limbs. She received adiagnosis of inflammatory myelitis and high dose corticosteroid therapy was prescribed. During the therapy she complained of chest tightness, dyspnoea, weakness and malaise. An electrocardiogram revealed sinus bradycardia. A significant increase in body weight, probably due to plasma volume expansion, was detected. Bradycardia and high blood pressure spontaneously resolved in few days. We provide a collection and a statistical analysis of literature data about steroid induced bradycardia. We found that higher total doses are associated with lower pulse rate and symptomatic bradycardia. Bradycardia is more frequent in older patients and those with underlying cardiac disease or with autonomic disturbance. However clinicians must be aware about the occurrence of symptomatic bradycardia in all patients who undergo high dose corticosteroid therapy, not only in those at risk, to early detect and treat this potentially dangerous condition.

Hemodynamic Response to Massive Bleeding in a Patient with Congenital Insensitivity to Pain with Anhidrosis

A patient with congenital insensitivity to pain with anhidrosis (CIPA) underwent revision of total hip arthroplasty under general anesthesia with only propofol. During surgery, neither elevation of stress hormones nor hemodynamic changes associated with pain occurred; however, when blood was rapidly lost, compensatory tachycardia was observed. Although patients with CIPA are complicated with autonomic disturbance due to dysfunction of postganglionic sympathetic fibers, this compensatory response indicated that the adrenal glands in patients with CIPA secrete catecholamine as part of a compensatory response during bleeding under general anesthesia.

GUILLAIN-BARRÉ SYNDROME

Objectives: To determine the outcome of autonomic disturbances in childrenwith Guillain Barre Syndrome. Study Design: Case series study. Setting: Pediatric ICU andNeurology ward of Nishtar Medical University/hospital Multan. Period: 9th June 2014 to 8th June2015. Methods: 48 children having age less than 18 years. Clinical examination of centralnervous system was conducted by single examiner to see presence of acute flaccid paralysisand to detect autonomic dysfunction. Cerebrospinal fluid analysis for albumino-cytologicdissociation, electromyography and nerve conduction studies was done in every patient ofGuillain Barre Syndrome. Outcome was noted as expired, discharged or LAMA. Results: Meanage was 5.4 years and male female ratio was 1.2:1. Total 34 children were found to have clinicalevidence of autonomic instability. Among 48 children, 17 children (35.4%) received no therapy,28 children (58.3%) received Intravenous immunoglobulin (IVIG) therapy, and 3 children (6.3%)received plasmapheresis. In children with autonomic disturbance, 24 children (70.6%) weredischarged, 8 children (23.5%) expired and 2 children (5.9%) LAMA. Of the total 14 childrenwithout autonomic disturbance, 11 children (78.6%) were discharged, 3 children (21.4%)expired and no child LAMA (p-value >0.05). Mean duration of stay for patients with autonomicinstability was 130 days versus 63 days in patients without autonomic instability (p-value<0.005). Demyelination variety was seen in 35 children (72.9%) and axonal degenerationin 13 children (27.1%). Mortality was high in children who had axonal degeneration but withinsignificant p-value of >0.05. Conclusions: Autonomic instability is common in patients ofGBS and is associated with increased risk of morbidity. Prompt recognition and treatment ofautonomic instability can improve the outcomes of children with GBS.

Heart rate variability and plasma nephrines in the evaluation of heat acclimatisation status

Purpose Heat adaptation (HA) is critical to performance and health in a hot environment. Transition from short-term heat acclimatisation (STHA) to long-term heat acclimatisation (LTHA) is characterised by decreased autonomic disturbance and increased protection from thermal injury. A standard heat tolerance test (HTT) is recommended for validating exercise performance status, but any role in distinguishing STHA from LTHA is unreported. The aims of this study were to (1) define performance status by serial HTT during structured natural HA, (2) evaluate surrogate markers of autonomic activation, including heart rate variability (HRV), in relation to HA status. Methods Participants (n = 13) were assessed by HTT (60-min block-stepping, 50% VO2peak) during STHA (Day 2, 6 and 9) and LTHA (Day 23). Core temperature (Tc) and heart rate (HR) were measured every 5 min. Sampling for HRV indices (RMSSD, LF:HF) and sympathoadrenal blood measures (cortisol, nephrines) was undertaken before and after (POST) each HTT. Results Significant (P < 0.05) interactions existed for Tc, logLF:HF, cortisol and nephrines (two-way ANOVA; HTT by Day). Relative to LTHA, POST results differed significantly for Tc (Day 2, 6 and 9), HR (Day 2), logRMSSD (Day 2 and Day 6), logLF:HF (Day 2 and Day 6), cortisol (Day 2) and nephrines (Day 2 and Day 9). POST differences in HRV (Day 6 vs. 23) were + 9.9% (logRMSSD) and − 18.6% (logLF:HF). Conclusions Early reductions in HR and cortisol characterised STHA, whereas LTHA showed diminished excitability by Tc, HRV and nephrine measures. Measurement of HRV may have potential to aid real-time assessment of readiness for activity in the heat.

Evolution of Orofacial Symptoms and Disease Progression in Idiopathic Parkinson’s Disease: Longitudinal Data from the Jönköping Parkinson Registry

Background. Orofacial symptoms are common in Parkinson’s disease (PD) both as initial manifestations and late markers of disease complications. We aimed to investigate the evolution of orofacial manifestations and their prognostic value throughout PD progression. Methods. Data was obtained from “Jönköping Parkinson Registry” database on routine care visits of 314 people with idiopathic PD in southern Sweden. Information on baseline symptomatology, orofacial features, UPDRS, and medications was recorded at baseline and during each follow-up visit within an average of 4.2 (range: 1–12) years. Results. Hypomimia, affected speech, drooling, and impaired swallowing were present in 37.3%/91.6%, 14.1%/65.5%, 11.7%/55.3%, and 10.2%/34.5% at baseline/follow-up, respectively. Male sex [OR = 2.4 (95% CI: 1.0–5.9)], UPDRS motor scores [OR = 1.2 (95% CI: 1.1–1.3)], dominant rigidity [OR = 5.2 (95% CI: 1.4–19.1)], and autonomic disturbance [OR = 3.4 (95% CI: 1.1–10.9)] were risk factors for drooling. Individuals with more severe orofacial burden at baseline had shorter median time to develop UPDRS-Part III > 28 [3rd tertile = 4.7 yr, 2nd tertile = 6.2 yr, and 1st tertile = 7.8 yr; p = 0.014]. Conclusions. Majority of people with PD manifest orofacial manifestations at either early or late stages of the disease. PD severity, symmetry of motor disturbances, and autonomic disorders correlate with orofacial symptoms. Individuals with more severe orofacial burden at baseline progressed faster to more advanced stages.