Autonomic Nervous System Symptoms Research Articles

Use of Nasopharyngoscopy Severity Classification of Chronic Epipharyngitis and Its Application for Evaluating the Treatment Outcomes of Epipharyngeal Abrasive Therapy.

Background Chronic epipharyngitis causes postnasal drip, pharyngeal pain, pharyngeal discomfort, headache, and shoulder stiffness. Additionally, autonomic nervous system symptoms such as dizziness, general fatigue, and sleeplessness may occur. It can also contribute to the development of focal diseases. Although epipharyngeal abrasive therapy (EAT) is effective for chronic epipharyngitis involving the abrasion of the epipharynx with a zinc chloride solution, there is a lack of clear diagnostic criteria, and treatment outcomes are rarely reported. Methodology A classification of the severity of chronic epipharyngitis was attempted in 154 cases based on nasopharyngeal endoscopic findings, with a subsequent examination of treatment outcomes using EAT. Diagnosis of chronic epipharyngitis involved identifying redness, swelling, postnasal drip, and crusting of the epipharyngeal mucosa. Severity classification relied on a four-point scale measuring the degree of redness and swelling, with additional points assigned for the presence of postnasal drip and crusting. This classification also served as a criterion for judging treatment effectiveness. The prevalence and improvement rate of black spots and granular changes were assessed through nasopharyngeal endoscopy with narrow-band imaging. Subjective symptoms were evaluated using before and after treatment questionnaires, employing a four-point scale for symptoms commonly associated with chronic epipharyngitis (headache, postnasal drip, nasal obstruction, pharyngeal discomfort, pharyngeal pain, shoulder stiffness, tinnitus, ear fullness, dizziness, cough, and sputum). A 10-point numerical rating scale (NRS) was used to assess the physical condition. Results Following EAT, the severity of nasopharyngeal endoscopic findings notably improved, with a 76.0% (117/154) improvement rate (remarkable improvement: 19.5% (30), improvement: 56.5% (87)). The improvement rate for the chief complaint reached 85.7% (132/154), demonstrating significant enhancement in the score for each symptom. NRS scores also improved at a rate of 76.0% (117/154). A significant correlation was observed between the improvement in local findings and chief complaints. The prevalence of black spots and granular changes before EAT was 83.8% (129/154) and 64.3% (99/154), exhibiting improvement rates of 65.9% (87/132) and 54.8% (57/104), respectively. Conclusions Nasopharyngeal endoscopy proves valuable for diagnosing and assessing the severity of chronic epipharyngitis, as well as evaluating treatment effectiveness. The findings indicate that EAT is an effective treatment for chronic epipharyngitis, with improvements in local findings correlating with enhancements in the chief complaint. This underscores the importance of employing aggressive EAT in managing patients with chronic epipharyngitis.

Impact of the autonomic dysfunction on the quality of life in people with NMOSD and MS: An international cross-sectional study.

A substantial autonomic nervous system (ANS) dysfunction has been described in multiple sclerosis (MS) and recently, also in neuromyelitis optica spectrum disorder (NMOSD). The prevalence of ANS symptoms contributes to the chronic symptom burden in both diseases. The aim of our study was to assess ANS dysfunction in people with (pw) NMOSD and MS, using the Composite Autonomic Symptom Score-31 (COMPASS-31), and additionally, to evaluate if ANS dysfunction have impact on the quality of life of these patients. We conducted cross-sectional study at three national referral neurological clinics in Serbia, Croatia, and Montenegro. A total of 180 consecutive subjects, 80 pwNMOSD and 100 pwMS, followed-up at these clinics, were enrolled in the study. Subjects included in the study completed: the validated versions of the COMPASS-31 and the Multiple Sclerosis Quality of Life-54 (MSQoL-54), and the Beck Depression Inventory (BDI). This study demonstrated that the total COMPASS-31 score > 0.0, implicating the presence of ANS dysfunction, was detected in almost all NMOSD and MS study participants tested (80/80, and 97/100, respectively). Our findings showed that autonomic symptom burden was statistically significantly correlated with decreased quality of life, in both NMOSD and MS cohorts. The independent predictors of the better quality of life in pwNMOSD were lower autonomic burden, particularly the absence of the orthostatic intolerance (p=0.005), along with lower EDSS and BDI score (p≤0.001). Similarly, in pwMS, independent predictors were EDSS, BDI, orthostatic intolerance, and the total COMPASS-31 (p≤0.001). Our study demonstrated that a significant proportion of persons with both NMOSD and MS have considerable dysautonomic symptom burden which is correlated with the decreased quality of life. Further investigations are warranted in order to optimize treatment interventions in MS and NMOSD.

Single Center Experience of RETREG1-related Hereditary Sensory Autonomic Neuropathy in Turkey (P11-8.009)

<h3>Objective:</h3> To describe the clinical characteristics of patients with <i>RETREG1</i> mutations. <h3>Background:</h3> Homozygous loss-of-function mutations in <i>RETREG1</i> result in Hereditary Sensory Autonomic Neuropathy (HSAN) Type 2. Clinical features include variable autonomic disturbances and upper motor neuron features such as brisk tendon reflexes, spasticity, and Babinski sign. <h3>Design/Methods:</h3> Herein, we evaluated the clinical and genetic features of 6 patients from 3 unrelated families with <i>RETREG1</i> mutation at the Neuromuscular Unit of the Istanbul Faculty of Medicine, Istanbul University. <h3>Results:</h3> Four patients were male. The median age of onset was 7.0 ± 2.5 (between 2 and 9 years). Foot ulcerations were the presenting symptom in four patients and walking difficulties in two. All patients were born to consanguineous marriages. In a median disease duration of 23.0 ± 14.4 years, four patients had osteomyelitis, and two had toe amputations. Five patients had a history of painless fractures in different lower extremity bones. Four patients had autonomic nervous system symptoms such as excessive sweating, constipation and orthostatism. Neurological examination of all patients showed spasticity, and brisk tendon reflexes. Muscle weakness in the lower extremities was noted in five patients. Sensory examination showed stocking type hypoesthesia and diminished vibration sensation in all patients. Five patients had pes planus deformity. Nerve conduction studies showed axonal sensory neuropathy in four patients and sensory-motor in two. Two families had homozygous c.433C&gt;T (p.Gln145*), and one had c.826delA (p.S276VfsX8) pathogenic variants in the <i>RETREG1</i> gene. <h3>Conclusions:</h3> In our study, all patients showed signs and symptoms consistent with pain insensitivity. Although shadowed by marked sensory symptoms, motor signs, such as spasticity, and muscle weakness, were also noted in our patients. Considering the autosomal recessive inheritance, new cases with <i>RETREG1</i> mutation are likely to be reported from Turkey, a country with a high rate of consanguineous marriages. <b>Disclosure:</b> Arman Cakar has nothing to disclose. Ms. BAGIROVA has nothing to disclose. Dr. Durmus has nothing to disclose. Prof. UYGUNER has nothing to disclose. Dr. Parman has nothing to disclose.

Neuroleptic malignant syndrome in fields other than psychiatry

AbstractNeuroleptic malignant syndrome (NMS) is an adverse effect of drugs, mainly neuroleptics. It is characterized by problems related to the autonomic nervous system (such as hyperthermia, diaphoresis, and tachycardia) and extrapyramidal symptoms (such as muscle rigidity and tremor). After onset, NMS progresses rapidly and without treatment, becomes life‐threatening. In recent years, NMS has become better recognized, and treatment has improved; most cases are now curable before becoming dangerous. Most NMS reports are from psychiatrists or neurologists; few are from other specialized clinicians. However, nonpsychiatric clinicians, such as surgeons, emergency physicians, orthopedists, and oral surgeons, sometimes must administer neuroleptic drugs. Although NMS is rare, its effects on patients may be debilitating, and this places a heavy burden on clinicians. Thus, not only psychiatrists but also clinicians in various other fields should understand this illness. This article describes NMS in fields other than psychiatry and its diagnosis, risk factors, and treatment strategies.

Clinical characteristics of chronic rhinitis following stroke.

We previously observed that patients with stroke complained of rhinitis symptoms that developed following the occurrence of stroke. To investigate the relationship between chronic rhinitis (CR) and stroke. This retrospective study analyzed the medical records and questionnaires of patients with stroke who visited our outpatient clinic from June to December 2020. Stroke lesions were mainly classified as supratentorial, infratentorial, and supra/infratentorial lesions. Supratentorial lesions were further divided into cortex, subcortex, and mixed. Participants were screened for CR and were subsequently divided into the CR and non-CR groups. The Sino-Nasal Outcome Test questionnaire and a questionnaire on autonomic nervous system symptoms were administered to all patients. Clinically evaluated indicators were not significantly different between the two groups. The number of patients with lesions in both the cortex and subcortex was significantly higher in the CR group than in the non-CR group. The risk of CR was higher in male patients with stroke than their female counterparts; additionally, the risk of CR was higher in patients with stroke who had both cortical and subcortical lesions, as well as autonomic dysfunction. Individuals with subcortical stroke damage had a greater probability of developing CR. The risk was increased in men, as compared with that in women, when autonomic symptoms were present.

Open-label comparative study of the efficacy of sodium valproate and topiramate as prophylactic therapy for migraine in adults

Background: Migraine is a most common and highly prevalent neurologic disorder characterized by recurrent moderate-to-severe headaches often in association with a number of autonomic nervous system symptoms. Sodium valproate and topiramate are the two newer antiepileptic agents which are commonly prescribed for the migraine prophylaxis in India. Aims and Objectives: The aims of the study were as follows: (1) To compare the efficacy of sodium valproate 15–30 mg/kg/day and topiramate 2–3 mg/kg/day as prophylactic therapy for migraine in adults and (2) to study cost-effectiveness and safety profile of sodium valproate and topiramate. Materials and Methods: It is an open-label comparative study; 100 migraine patients of age group between 20 years and 50 years of both sexes were enrolled in the present study according to the inclusion criteria. Patients were diagnosed as migraine as per the International Criteria For Headache Disorders-3. Before starting treatment and after diagnosis, investigations have done, that is, complete blood picture, liver function test, renal function test, random blood sugar, and thyroid profile. Randomization was done by choosing every alternate patient and study drugs were prescribed. One hundred patients were divided into two groups, Group-A: 50 patients administered Tab. sodium valproate 500 mg twice daily orally and Group-B: 50 patients administered Tab. topiramate 150 mg once daily orally for a period of 6 months. Results: In Group-A, the severity of pain value before starting treatment was 6.440 ± 2.130 which is reduced to 1.820 ± 1.024 at the end of the treatment which was statistically significant (P &lt; 0.0001) and the frequency of headache value before starting treatment was 4.220 ± 1.298 which is reduced to 1.320 ± 0.7407 at the end of the treatment, P &lt; 0.0001 which was statistically significant. In Group-B, the severity of pain value before starting treatment was 6.200 ± 2.119 which is reduced to 1.840 ± 0.9765 after starting treatment which was statistically significant (P &lt; 0.0001) and the frequency of headache value before starting treatment was 4.300 ± 1.199 which is reduced to 1.340 ± 0.6884 after starting treatment, P &lt; 0.0001 which was statistically significant. In between the two groups severity of migraine pain and headache frequencies the P value was statistically significant. Conclusion: The results of the present study demonstrated that both sodium valproate and topiramate were well tolerated, having similar efficacy in reducing the severity of the pain, headache frequencies, and improving the quality of life. Sodium valproate is preferred because of cost-effectiveness.

Prevalence of Undiagnosed Acute Hepatic Porphyria in Cyclic Vomiting Syndrome and Overlap in Clinical Symptoms.

Acute hepatic porphyria (AHP) presents with nausea and vomiting and can mimic cyclic vomiting syndrome (CVS). The prevalence of AHP in CVS and overlap in clinical symptomatology is not known. We thus sought to determine the prevalence of pathogenic variants for AHP and characterize symptom overlap between CVS and AHP. We conducted a cross-sectional study of 234 CVS patients using Rome criteria. Patients were eligible for AHP genetic testing if they had recurrent episodes of severe, diffuse abdominal pain with ≥ 2 of the following-peripheral nervous system (muscle weakness/aching, numbness, tingling), central nervous system (confusion, anxiety, seizures, hallucinations), autonomic nervous system (hyponatremia, tachycardia, hypertension, constipation) symptoms, red/brownish urine, or blistering skin lesions on sun-exposed areas. A family history of AHP or elevated urinary porphobilinogen (PBG)/aminolaevulinic acid (ALA) were also criteria for genetic testing and was performed using a 4-gene panel. Mean age was 38.7 ± 14.5years, 180 (76.9%) were female and 200 (85.5%) were Caucasian. During a CVS attack, 173 (92%) reported abdominal pain, 166 (87.2%) had peripheral nervous system, 164 (86.8%) had central nervous system and 173 (92) % had autonomic symptoms. Ninety-one eligible patients completed genetic testing. None were positive for AHP but two had variants of uncertain significance (VUS) in the HMBS gene. There is a high prevalence of non-gastrointestinal symptoms in CVS, like AHP, which is important for clinicians to recognize. AHP was not detected in this study and larger studies are warranted to ascertain its prevalence.

Relationship between heart rate variability and cognitive function in patients with enlarged perivascular space.

To explore the relationship between heart rate variability (HRV), the brain distribution of enlarged perivascular space (EPVS), and cognitive impairment in patients with EPVS. The clinical and imaging data of 199 patients with EPVS were retrospectively analyzed. EPVS load in the basal ganglia (BG) and centrum semiovale (CS) regions were assessed using the Potter's method. Cognitive function was evaluated using the Montreal Cognitive Assessment Scale. A logistic regression model was used to analyze the relationship between HRV, the brain distribution of EPVS and cognitive function in patients with EPVS. A receiver operating characteristic curve was used to assess the predictive value of HRV for cognitive function in patients with EPVS. Of the 199 patients, 27 and 42 presented with severe BG-EPVS and cognitive impairment, respectively. Significant differences were observed in the root mean square of successive differences of normal-normal (NN) intervals for period of interest (rMSSD), the percentage of adjacent NN intervals greater than 50 ms (PNN50), and the ratio of low-frequency power (LF) to high-frequency power (HF) between the mild and severe BG-EPVS groups (P < 0.05). Patients who presented with and without cognitive impairment differed significantly in the standard deviation of NN intervals (SDNN), rMSSD, PNN50, total power, LF, and LF/HF (P < 0.05). rMSSD (odds ratio [OR] 0.871, 95% confidence interval [CI] 0.768-0.988) and LF/HF (OR 3.854, 95% CI 1.196-12.419) were independent influencing factors of BG-EPVS, and rMSSD (OR 0.936, 95% CI 0.898-0.976) was an independent influencing factor of cognitive impairment in patients with EPVS. The optimal cut-off point was 0.312, with an area under the curve of 0.795 (95% CI 0.719-0.872) for predicting cognitive impairment in patients with EPVS by rMSSD. Reduced HRV is involved in the pathophysiological mechanisms of the formation and development of BG-EPVS and is associated with cognitive impairment in patients with EPVS, independent of CS-EPVS. For patients with HRV changes but without autonomic nervous system symptoms, positive intervention may slow the occurrence or progression of EPVS and cognitive impairment in patients with EPVS.

0397 Autonomic Reflex Testing Confirms Autonomic Disturbances in a Cohort of Patients with Idiopathic Hypersomnia

Abstract Introduction Symptoms suggestive of autonomic nervous system (ANS) dysfunction have been previously described in patients with idiopathic hypersomnia (IH), however, objective ANS reflex testing data has not been reported. We aimed to better quantify symptoms of ANS dysfunction in a cohort of patients with IH through the use of standardized ANS reflex testing. Methods Patients diagnosed with IH based on ICSD-3 criteria using overnight video polysomnography and multiple sleep latency testing (MSLT) were consecutively enrolled in our study, regardless of ANS symptoms. All patients underwent ANS reflex testing, including measures of parasympathetic (heart rate variability with deep breathing and Valsalva ratio) and sympathetic adrenergic function (Valsalva blood pressure response and 10-minute head-up tilt at an angle of 70 degrees) with continuous blood pressure and heart rate monitoring. Eleven patients also underwent measures of sympathetic cholinergic function (quantitative sudomotor axon reflex testing). All medications that affect ANS function were held prior to ANS testing, including wake-promoting medications and sodium oxybate. Results Twenty patients with IH were enrolled. Fifty percent (10/20) were long sleepers (&amp;gt;11hrs). Mean sleep onset latency and number of sleep onset REM periods (SOREMs) on MSLT were 6.9 (± 3.1) mins and 0.2 (± 0.4), respectively. Mean duration of IH symptoms prior to the date of ANS testing was 6.3 (± 8.1) yrs. Eighty-five percent (17/20) of patients had abnormal ANS testing. Of these, 75% (15/20) had sympathetic adrenergic impairment, 64% (7/11) had sympathetic cholinergic impairment, and 5% (1/20) had parasympathetic impairment. Fifty-five percent (11/20) of patients were diagnosed with postural tachycardia syndrome (POTS), 45% (5/11) with small fiber neuropathy, 5% (1/20) with inappropriate sinus tachycardia and 15% (3/20) with neurally-mediated syncope. Seventy percent (14/20) of patients reported orthostatic intolerance regardless of autonomic diagnosis. Conclusion ANS dysfunction was common and severe in our cohort of IH patients, affecting all domains of ANS reflex testing, with more prominent impairment in sympathetic domains. POTS was the most common comorbid diagnosis, and most patients reported orthostatic intolerance. There was no association with IH disease duration, though our sample size was limited. Future studies will focus on ANS testing in larger cohorts of IH patients, specifically on shared pathophysiological mechanisms of hypersomnia and ANS dysfunction. Support (If Any)

Fascia in Myofascial Pain Syndrome

Myofascial pain syndrome (MPS) is described as the muscle, sensory, motor, and autonomic nervous system symptoms caused by stimulation of myofascial trigger points (MTP), but the participation of fascia in this syndrome has often been neglected. This presentation will explore the important contribution of structural and functional alterations of the deep fascia to the pathophysiology and clinical manifestation of MPS. The interaction of the three dimensional fascial system, with the dynamic nature of sensitization, will be presented in the context of the pathophysiology of MTP and the expression of quantitative, reproducible physical findings in the clinical manifestation of chronic myofascial pain. Emphasis will be given to recent developments in the biology of fascia, and in particular, its hyaluronan (HA)‐rich matrix content that provide a viscoelastic property possible cause of MPS. The presentation will translate these findings to highlight novel clinical directions in the diagnosis and non‐pharmaceutical management of MPS.

Cranial Autonomic Symptoms in Migraine: An Observational Study.

Objective:Our aim was to observe frequency of cranial autonomic symptoms (CAS) in migraineurs (primary) and its relation with laterality of headache or other factors, if any.Background:Migraine episodes have headaches with or without aura, and sometimes associated with systemic autonomic nervous system symptoms. Primarily presence of cranial autonomic symptoms suggests diagnosis of TACs. But many studies reported cranial autonomic symptoms (CAS) ranging from 26% to 80% in migraine patients.Material and Methods:Consecutive patients of migraine attending our headache clinic were included in our study. Presence of CAS was recorded with respect to ocular, nasal, facial and aural symptoms along with headache characteristics and laterality information. Detailed clinical examination was performed. We used ICHD 3 (beta version) criteria.Results:Our study cohort comprised of 200 patients having mean (± SD) age 31.12 (± 10.67) years. There were 157 (78.5%), females. Out of 200 patients, 148 (74%) were having at least one CAS, of which 70% were having 2 or more CAS. Frequency of CAS was lacrimation (45.5%), conjunctival injection (34.5%), eyelid edema (34%), aural fullness (27.5%), facial sweating (25%), facial flushing (17.5%), nasal congestion (9%), rhinorrhea (5%) and ptosis (4%). Bilateral CAS was present in 129 (87%) and unilateral CAS in 19 (13%) (OR 35.31; 95% CI 9.19 to 135.7), (P < 0.0001). Sunlight as a trigger was present in all 148 (100%) patients.Conclusion:Our study showed that CASs in migraine is common and bilateral. Sunlight triggers headache in almost all CAS positive patients.

Autonomic dysfunction in post-COVID patients with and witfhout neurological symptoms: a prospective multidomain observational study

The autonomic nervous system (ANS) can be affected by COVID-19, and dysautonomia may be a possible complication in post-COVID individuals. Orthostatic hypotension (OH) and postural tachycardia syndrome (POTS) have been suggested to be common after SARS-CoV-2 infection, but other components of ANS function may be also impaired. The Composite Autonomic Symptom Scale 31 (COMPASS-31) questionnaire is a simple and validated tool to assess dysautonomic symptoms. The aim of the present study was to administer the COMPASS-31 questionnaire to a sample of post-COVID patients with and without neurological complaints. Participants were recruited among the post-COVID ambulatory services for follow-up evaluation between 4 weeks and 9 months from COVID-19 symptoms onset. Participants were asked to complete the COMPASS-31 questionnaire referring to the period after COVID-19 disease. Heart rate and blood pressure were manually taken during an active stand test for OH and POTS diagnosis. One-hundred and eighty participants were included in the analysis (70.6% females, 51 ± 13 years), and OH was found in 13.8% of the subjects. Median COMPASS-31 score was 17.6 (6.9–31.4), with the most affected domains being orthostatic intolerance, sudomotor, gastrointestinal and pupillomotor dysfunction. A higher COMPASS-31 score was found in those with neurological symptoms (p < 0.01), due to more severe orthostatic intolerance symptoms (p < 0.01), although gastrointestinal (p < 0.01), urinary (p < 0.01), and pupillomotor (p < 0.01) domains were more represented in the non-neurological symptoms group. This study confirms the importance of monitoring ANS symptoms as a possible complication of COVID-19 disease that may persist in the post-acute period.

Unveiling the relationship between autonomic involvement, fatigue, and cognitive dysfunction in early relapsing-remitting multiple sclerosis.

Fatigue is a common, yet disabling, symptom in patients with multiple sclerosis (PwMS). Fatigue has shown to be associated with self-reported autonomic nervous system (ANS) symptoms, particularly for cognitive fatigue; however, the question whether ANS involvement is related to cognitive impairment has never been addressed. We performed a study to unveil the complex relationship between fatigue, ANS symptoms, and cognitive impairment. We prospectively recruited early PwMS that were tested with Brief International Cognitive Assessment for Multiple Sclerosis (BICAMS), State-Trait Anxiety Inventory (STAI), Beck Depression Inventory (BDI), Modified Fatigue Impact Scale (MFIS) and Composite Autonomic Symptoms Scale-31 (COMPASS-31) scale. We performed a comparison between fatigued and non-fatigued patients and between cognitive unimpaired and impaired patients. We evaluated the association of COMPASS-31, MFIS, BDI, STAI, and BICAMS scores, and the analysis was repeated for each scale's sub-scores. A multivariable analysis was performed to elucidate predictors of fatigue. Forty-four patients were recruited. Fatigued patients had higher COMPASS-31 total, orthostatic intolerance, secretomotor, and pupillomotor scores. No differences in fatigue and ANS symptoms were found between cognitive impaired and unimpaired patients. MFIS total score correlated with STAI state (p = 0.002) and trait (p < 0.001), BDI (p < 0.001), COMPASS-31 total (p < 0.001), orthostatic intolerance (p < 0.001), pupillomotor scores (p = 0.006). Multivariable analysis showed that BDI (p < 0.001) and COMPASS-31 (p = 0.021) predicted MFIS score. Sub-scores analysis showed that orthostatic intolerance has a relevant role in fatigue. ANS symptoms are closely related with fatigue. Orthostatic intolerance may have a predominant role. Cognitive impairment seems not to be associated with ANS symptoms.

Enterovirus-A71 Rhombencephalitis Outbreak in Catalonia: Characteristics, Management and Outcome.

Background:Between April and June 2016, an outbreak of rhombencephalitis (RE) caused by enterovirus (EV) A71 was detected in Catalonia, Spain—the first documented in Western Europe. The clinical characteristics and outcome of patients with this condition differed from those reported in outbreaks occurring in Southeast Asia.Methods:Observational, multicenter study analyzing characteristics, treatment and outcome of patients with EV-A71 rhombencephalitis diagnosed in 6 publicly funded hospitals within the Catalonian Health Institute. A review of clinical characteristics, diagnosis, treatment and outcome of these patients was conducted.Results:Sixty-four patients met the clinical and virologic criteria for rhombencephalitis caused by EV-A71. All patients had symptoms suggesting viral disease, mainly fever, lethargy, ataxia and tremor, with 30% of hand-foot-mouth disease. Intravenous immunoglobulin therapy was given to 44/64 (69%) patients and methylprednisolone to 27/64 (42%). Six patients (9%) required pediatric intensive care unit admission. Three patients had acute flaccid paralysis of 1 limb, and another had autonomic nervous system (ANS) dysfunction with cardiorespiratory arrest. Outcome in all patients (except the patient with hypoxic-ischemic encephalopathy) was good, with complete resolution of the symptoms.Conclusions:During the 2016 outbreak, rhombencephalitis without ANS symptoms was the predominant form of presentation and most patients showed no hand-foot-mouth disease. These findings contrast with those of other patient series reporting associated ANS dysfunction (10%–15%) and hand-foot-mouth disease (60%–80%). Complete recovery occurred in almost all cases. In light of the favorable outcome in untreated mild cases, therapies for this condition should be reserved for patients with moderate-severe infection. The main relevance of this study is to provide useful information for setting priorities, management approaches and adequate use of resources in future EV-A71 associated rhombencephalitis outbreaks.

Analysis of the clinical characteristics of insulin autoimmune syndrome induced by alpha-lipoic acid.

Alpha-lipoic acid (ALA) is widely used as a dietary supplement and antiageing agent. Insulin autoimmune syndrome (IAS) is the most serious adverse reaction reported with the use of ALA. The purpose of this study was to explore the clinical characteristics of ALA-induced IAS and provide a scientific reference for clinical diagnosis, treatment and prevention. We collected literature on IAS cases induced by ALA for retrospective analysis in Chinese and English. The median age of 37 patients (28 females and 9 males) was 61years (range 32-82). The symptoms occurred at night and in the early morning (60.7%), in the late postprandial period (50.0%) or after fasting (35.7%), within hours in some patients and up to 2months in others after stopping ALA or during medication treatment. Autonomic nervous system symptoms (81.1%) and neurological hypoglycaemia (64.9%) are the main clinical manifestations of hypoglycaemia. The blood glucose concentration at the onset of hypoglycaemia was 2.19mmol/L (median, range 1.09-3.52), the insulin concentration was ≥100 μIU/ml (94.6%), and the C-peptide concentration was ≤20ng/ml (83.3%). Testing for IgG insulin autoantibodies (IAAs) was positive in 37 patients. Pancreatic imaging was unremarkable on computed tomography (CT), magnetic resonance imaging (MRI) and abdominal sonography. Hypoglycaemia disappeared within 5days to 8months after withdrawing ALA alone or using corticosteroid treatment. IAA turned negative in 7months (median; range 2-36). Follow-up showed no recurrent hypoglycaemic episodes at 7.25months (median; range 1-36). ALA-induced IAS is a clinically rare autoimmune disease with hypoglycaemia that occurs during medication treatment or after drug withdrawal that should be treated promptly.

Yoga is effective in treating symptoms of Gulf War illness: A randomized clinical trial

Many Veterans of the 1990–1991 Gulf War report symptoms of Gulf War Illness, a condition involving numerous chronic symptoms including pain, fatigue, and mood/cognition symptoms. Little is known about this condition's etiology and treatment. This study reports outcomes from a randomized controlled single-blind trial comparing yoga to cognitive behavioral therapy for chronic pain and other symptoms of Gulf War Illness. Participants were Veterans with symptoms of GWI: chronic pain, fatigue and cognition-mood symptoms. Seventy-five Veterans were randomized to treatment via selection of envelopes from a bag (39 yoga, 36 cognitive behavioral therapy), which consisted of ten weekly group sessions. The primary outcomes of pain severity and interference (Brief Pain Inventory- Short Form) improved in the yoga condition (Cohen's d = .35, p = 0.002 and d = 0.69, p < 0.001, respectively) but not in the CBT condition (d = 0.10, p = 0.59 and d = 0.25 p = 0.23). However, the differences between groups were not statistically significant (d = 0.25, p = 0.25; d = 0.43, p = 0.076), though the difference in an a-priori-defined experimental outcome variable which combines these two variables into a total pain variable (d = 0.47, p = 0.047) was significant. Fatigue, as indicated by a measure of functional exercise capacity (6-min walk test) was reduced significantly more in the yoga group than in the CBT group (between-group d = .27, p = 0.044). Other secondary outcomes of depression, wellbeing, and self-reported autonomic nervous system symptoms did not differ between groups. No adverse events due to treatment were reported. Yoga may be an effective treatment for core Gulf War Illness symptoms of pain and fatigue, making it one of few treatments with empirical support for GWI. Results support further evaluation of yoga for treating veterans with Gulf War Illness. Clinical trial registryclinicaltrials.gov Registration Number NCT02378025.

Clinical comparative analysis of monophasic and multiphasic acute disseminated encephalomyelitis in adults.

This study aims to explore the clinical features and prognostic factors for relapse of acute disseminated encephalomyelitis (ADEM) in adults. 56 patients with ADEM were retrospectively analyzed. The epidemiological characteristics, clinical manifestations, laboratory features, magnetic resonance imaging (MRI), treatment and prognosis data of these patients were analyzed using the χ2 test for categorical variables and Mann-Whitney U-test for continuous variables. Then, the clinical characteristics and recurrence factors were summarized. 56 patients with ADEM, based on the criteria of the International Pediatric Multiple Sclerosis Study Group, were recruited to the study. Among these patients, 31 were male and 25 were female. Furthermore, 13 patients had multiphasic ADEM, and 29 patients (52%) had definite incentive factors before onset. The commonest presenting symptoms and signs were fever (36%), disturbance of consciousness (52%), mental disorder (38%), seizure (14%), headache and dizziness (43%), optic neuritis (34%), autonomic nervous system symptoms (43%), limb paralysis or abnormal sensation (73%), and unilateral or bilateral pyramidal tract signs (48%). Inflammatory changes in the cerebrospinal fluid were prominent. MRI T2-weighted and fluid-attenuated inversion recovery images displayed multiple or large flaky high signals, and the lesions were usually different in the number and distribution of these lesions. Intravenous corticosteroids and/or immunoglobulin were still important treatments in the acute phase. After treatment, 38 patients completely recovered, 9 patients had neurologic deficits, and 9 patients died. ADEM in adults is not uncommon, its clinical features are complex and varied, and some of these are multiphasic. There may be some potential clinical predictors at first onset.

Iatrogenic Opioid Withdrawal Syndrome in Critically Ill Patients: a Retrospective Cohort Study.

BackgroundOpioid withdrawal syndrome (OWS) may occur following the reduction or discontinuation of opioid analgesics. In critically ill pediatric patients, OWS is a common and clinically significant condition. However, OWS in adult patients has not been assessed in detail. Therefore, we aimed to investigate the incidence, risk factors, and clinical features of OWS in mechanically ventilated patients treated in an adult intensive care unit (ICU).MethodsThis study was a retrospective evaluation of data from patients treated in the medical ICU for > 3 days and who received only one type of opioid analgesic. OWS was assessed over a 24 hours period from discontinuation or reduction (by > 50%) of continuous opioid infusion. OWS was defined as the presence of ≥ 3 central nervous system or autonomic nervous system symptoms.ResultsIn 126 patients treated with remifentanil (n = 58), fentanyl (n = 47), or morphine (n = 21), OWS was seen in 31.0%, 36.2%, and 9.5% of patients, respectively (P = 0.078). The most common symptom was a change in respiratory rate (remifentanil, 94.4%; fentanyl, 76.5%; morphine, 100%). Multivariate Cox-proportional hazards model showed that OWS was negatively associated with morphine treatment (hazard ratio [HR], 0.17; 95% confidence interval [CI], 0.037–0.743) and duration of opioid infusion (HR, 0.566; 95% CI, 0.451–0.712).ConclusionOWS is not uncommon in mechanically ventilated adult patients who received continuous infusion of opioids for > 3 days. The use of morphine may be associated with a decreased risk of OWS.

Longitudinal assessment of autonomic nervous system in patients with first demyelinating event suggestive of multiple sclerosis.

As a high proportion of people with clinically isolated syndrome (pwCIS) exhibit sympathetic adrenergic and sudomotor dysfunction, the aim of this study was to investigate the evolution of autonomic nervous system (ANS) abnormalities in pwCIS over a 2-year follow-up. This was a prospective cohort study in which 121 pwCIS were enrolled and followed for 2years. After 2-year follow-up, data were available for 84 pwCIS. ANS symptoms were evaluated with the Composite Autonomic System Score-31 (COMPASS-31) and results of the ANS tests were expressed using the Composite Autonomic Scoring Scale (CASS) at baseline and visit at month 24. Symptomatic dysautonomia was defined if the patient had a COMPASS-31 value above the median of the whole cohort at baseline evaluation (COMPASS-31>6.79) and CASS score >0. Complete CASS data at baseline and month 24 were available for 62 patients; in 24 (38.7%) patients there was worsening, in 16 (25.8%) there was improvement and in 22 (35.5%) there was no change in CASS score. In 90% of pwCIS (72 of 80) there was no change in parasympathetic nervous system tests, whereas 47.3% (35 of 74) had either worsening or improvement in sympathetic adrenergic and 28.6% (20 of 70) had either worsening or improvement in sudomotor function. A multivariable regression model identified the total number of T2 lesions as an independent predictor for worsening of symptomatic dysautonomia. No predictors for worsening or improving of CASS score were identified. A substantial proportion of pwCIS experienced worsening of ANS abnormalities during the 2-year follow-up and magnetic resonance imaging parameters seemed to predict these abnormalities.

P63-T Longitudinal assessment of autonomic nervous system in patients with first demyelinating event suggestive of MS

Background The aim of this study was to investigate the evolution of autonomic nervous system (ANS) abnormalities in people with clinically isolated syndrome (pwCIS) over a two-year follow-up. Material and methods This was a prospective cohort study in which 121 pwCIS were enrolled and followed for two years. After two-year follow-up, data were available for 84 pwCIS. ANS symptoms were evaluated with the Composite Autonomic System Score-31 (COMPASS-31) and results of the ANS tests were expressed using the Composite Autonomic Scoring Scale (CASS) at baseline and month 24 visits. Symptomatic dysautonomia was defined if the patient had COMPASS-31 value above the median of the whole cohort at baseline evaluation (COMPASS-31 > 6.79) and CASS score greater than 0. Results Complete CASS at baseline and M24 was available for 62 patients, in 24 (38.7%) patients there was worsening, in 16 (25.8%) improvement and in 22 (35.5%) there was no change in CASS. A multivariable regression model identified total number of T2 lesions as independent predictor for worsening of symptomatic dysautonomia. Although not reaching significance there was a trend of cervical spinal cord lesions as independent predictor for CASS worsening and age for CASS improvement (p = 0.058 and p = 0.051, respectively). Conclusion Substantial proportion of pwCIS experience worsening of ANS abnormalities during the two-year follow-up and MRI parameters seems to predict these abnormalities.