Interpretation of Autonomic Function Test

The autonomic function test consists of the tests that evaluate the sudomotor, cardiovascular adrenergic, and parasympathetic functions. As its importance grows, more and more medical institutions want to evaluate it properly. However, there are many variables to consider and difficulties in interpreting ambiguous results, which may lead to incorrect screening. This paper is intended to help provide an appropriate interpretation of autonomic function tests in clinical settings.

Clinical Scale for Neuropathic Pain

Neuropathic pain caused by a lesion or disease of the somatosensory nervous system is underdiagnosed and difficult to treat. Questionnaires based on self-reported symptoms have improved diagnosis and management of neuropathic pain. Visual analog scale and numeric rating scale are most well-known unidimensional pain questionnaires. Multidimensional questionnaire or specialized questionnaire for neuropathic pain are more useful to diagnose neuropathic pain. Screening questionnaires help to identify neuropathic pain easily, and assessment questionnaires make it possible to create phenotypic profiles of neuropathic pain and determine an efficacy of management.

Validation of the Korean version of the Boston Autonomic Symptom Questionnaire

Background@#and Purpose The Boston Autonomic Symptom Questionnaire (BASQ) is a quantitative tool using a numeric rating scale to assess the symptoms of systemic dysautonomia, including cardiovascular, gastrointestinal, urinary, sudomotor, vasomotor, and sexual functions. The aim of this study was to validate the Korean version of the BASQ (KBASQ). @*Methods@#Prospectively enrolled subjects who submitted to autonomic function tests, including tests for cardiovagal, adrenergic, and sudomotor functions, also completed the KBASQ and the Korean version of the Orthostatic Grading Scale (KOGS), a validated questionnaire for assessing orthostatic symptoms.Twenty-eight subjects completed the KBASQ twice to assess test-retest reliability. We classified the subjects to dysautonomia or normal control group according to dysautonomic symptoms and the results of autonomic function tests. @*Results@#This study enrolled 225 subjects aged 54.0±18.1 years (mean±standard deviation), with a male/female ratio of 1/1.03. The internal validity of the KBASQ was excellent (Cronbach’s α=0.922), and that of each of its subscales ranged from excellent to acceptable (Cronbach’s α=0.709–0.952). The test-retest reliability was good, with correlation coefficients ranging from 0.354 to 0.917. The subcategory scores for the KBASQ were significantly higher in the dysautonomia group than in the normal control group. There were significant correlations among the items in the KBASQ and KOGS. There was also a significant correlation between KBASQ scores and the results of the autonomic function tests. @*Conclusions@#The internal validity and reliability of the KBASQ were good, indicating that it may be a useful screening tool for the systematic evaluation of autonomic symptoms in patients with dysautonomia.

Interpretation of Autonomic Function Test

The autonomic function test consists of the tests that evaluate the sudomotor, cardiovascular adrenergic, and parasympathetic functions. As its importance grows, more and more medical institutions want to evaluate it properly. However, there are many variables to consider and difficulties in interpreting ambiguous results, which may lead to incorrect screening. This paper is intended to help provide an appropriate interpretation of autonomic function tests in clinical settings.

Clinical Scale for Neuropathic Pain

Neuropathic pain caused by a lesion or disease of the somatosensory nervous system is underdiagnosed and difficult to treat. Questionnaires based on self-reported symptoms have improved diagnosis and management of neuropathic pain. Visual analog scale and numeric rating scale are most well-known unidimensional pain questionnaires. Multidimensional questionnaire or specialized questionnaire for neuropathic pain are more useful to diagnose neuropathic pain. Screening questionnaires help to identify neuropathic pain easily, and assessment questionnaires make it possible to create phenotypic profiles of neuropathic pain and determine an efficacy of management.

Validation of the Korean version of the Boston Autonomic Symptom Questionnaire

Background@#and Purpose The Boston Autonomic Symptom Questionnaire (BASQ) is a quantitative tool using a numeric rating scale to assess the symptoms of systemic dysautonomia, including cardiovascular, gastrointestinal, urinary, sudomotor, vasomotor, and sexual functions. The aim of this study was to validate the Korean version of the BASQ (KBASQ). @*Methods@#Prospectively enrolled subjects who submitted to autonomic function tests, including tests for cardiovagal, adrenergic, and sudomotor functions, also completed the KBASQ and the Korean version of the Orthostatic Grading Scale (KOGS), a validated questionnaire for assessing orthostatic symptoms.Twenty-eight subjects completed the KBASQ twice to assess test-retest reliability. We classified the subjects to dysautonomia or normal control group according to dysautonomic symptoms and the results of autonomic function tests. @*Results@#This study enrolled 225 subjects aged 54.0±18.1 years (mean±standard deviation), with a male/female ratio of 1/1.03. The internal validity of the KBASQ was excellent (Cronbach’s α=0.922), and that of each of its subscales ranged from excellent to acceptable (Cronbach’s α=0.709–0.952). The test-retest reliability was good, with correlation coefficients ranging from 0.354 to 0.917. The subcategory scores for the KBASQ were significantly higher in the dysautonomia group than in the normal control group. There were significant correlations among the items in the KBASQ and KOGS. There was also a significant correlation between KBASQ scores and the results of the autonomic function tests. @*Conclusions@#The internal validity and reliability of the KBASQ were good, indicating that it may be a useful screening tool for the systematic evaluation of autonomic symptoms in patients with dysautonomia.

Hereditary Myopathy with Early Respiratory Failure with a Heterozygous TTN Gene Missense Mutation

Hereditary myopathy with early respiratory failure (HMERF) is characterized by early respiratory insufficiency which is inappropriate to the degree of limb muscle weakness. Recently, mutation in TTN gene was found in HMERF patients with the aid of gene sequencing. We describe the first case presenting with distal leg weakness and early respiratory failure confirmed by TTN gene mutation in Korea.

Usefulness of Squatting Combined with the Blood Pressure Measurement for Orthostatic Intolerance in Dizziness Clinic / 대한신경근육질환학회지

Background@#Orthostatic intolerance (OI) is a common clinical symptom in dizziness clinic. The head-up tilt table test (HUT) is one of the primary clinical examination for evaluating OI. There is no consensus on the optimum method for diagnosis of orthostatic hypotension (OH). Herein, we performed the additional squat combined with blood pressure (BP) monitoring for OI patients with normal HUT. @*Methods@#The study included 32 consecutive patients with orthostatic intolerance for 3 months since April, 2018 (Period I) and 27 patients with orthostatic intolerance for 3 months since April, 2019 (Period II) in dizziness clinic of Chungnam National University Hospital. During Period II, the additional squat combined with BP test was performed for normal HUT results in patients with OI. In squat combined orthostatic BP measurement, the first BP measurement was taken following 3 minutes of rest at the squat position; afterwards the patients were raised upright and the measurement was monitored for 2 minutes, using a continuous beat-to-beat BP monitoring. @*Results@#In this study, there was significant difference in OH diagnosis (p<0.001); 40.6% (13/32) by conventional HUT (Period I) vs. 92.5% (25/33) by conventional HUT and additional squat test for normal HUT (Period II). In patients with normal HUT, the positive OH was 86.7% (13/15) by the additional squat combined BP measurement (Period II). @*Conclusions@#In addition to HUT, squat test combined with BP measurement might be more informative for understanding and diagnosing the OH, particularly in patients with OI and normal HUT in dizziness clinic.

Usefulness of Squatting Combined with the Blood Pressure Measurement for Orthostatic Intolerance in Dizziness Clinic / 대한신경근육질환학회지

Background@#Orthostatic intolerance (OI) is a common clinical symptom in dizziness clinic. The head-up tilt table test (HUT) is one of the primary clinical examination for evaluating OI. There is no consensus on the optimum method for diagnosis of orthostatic hypotension (OH). Herein, we performed the additional squat combined with blood pressure (BP) monitoring for OI patients with normal HUT. @*Methods@#The study included 32 consecutive patients with orthostatic intolerance for 3 months since April, 2018 (Period I) and 27 patients with orthostatic intolerance for 3 months since April, 2019 (Period II) in dizziness clinic of Chungnam National University Hospital. During Period II, the additional squat combined with BP test was performed for normal HUT results in patients with OI. In squat combined orthostatic BP measurement, the first BP measurement was taken following 3 minutes of rest at the squat position; afterwards the patients were raised upright and the measurement was monitored for 2 minutes, using a continuous beat-to-beat BP monitoring. @*Results@#In this study, there was significant difference in OH diagnosis (p<0.001); 40.6% (13/32) by conventional HUT (Period I) vs. 92.5% (25/33) by conventional HUT and additional squat test for normal HUT (Period II). In patients with normal HUT, the positive OH was 86.7% (13/15) by the additional squat combined BP measurement (Period II). @*Conclusions@#In addition to HUT, squat test combined with BP measurement might be more informative for understanding and diagnosing the OH, particularly in patients with OI and normal HUT in dizziness clinic.

Clinical Evaluation for Diabetic Neuropathy / 대한신경근육질환학회지

Diabetic polyneuropathy (DPN) causes neuropathic pain with reduced quality of life as well as diabetic foot ulceration which sometimes resulted in amputation. Early detection and improved knowledge of pathogenic pathways are important to prevent and to manage DPN. The screening methods and several tests to diagnose DPN-quantitative sensory testing, skin biopsy, corneal confocal microscopy, etc.-will be described.

Diagnosis of Multiple Sclerosis: 2017 McDonald Diagnostic Criteria

Multiple sclerosis (MS) is a diagnosis of exclusion and the lesions or objective findings should disseminate in space and time to diagnose MS. The diagnostic criteria of MS have continuously evolved overtime. The McDonald criteria were originally proposed in 2001, and the revised 2010 McDonald criteria have been used widely. Scientific advances in the past 7 years since 2010 induced the revised 2017 McDonald criteria. All revisions relied entirely on the available evidences, and not expert opinion. In this review, we will provide an overview of the way to diagnose MS and the 2017 McDonald criteria.

Orthostatic Hypotension and Cognitive Function in Parkinson's Disease

BACKGROUND: Although orthostatic hypotension (OH) and cognitive impairment (CI) are common non-motor symptoms of Parkinson's disease (PD), the relationship between OH and CI remains to be clarified. This study was aimed to investigate the relationship between OH and CI in PD. METHODS: We recruited 192 patients who were diagnosed as PD based on the UK Brain Bank diagnostic criteria. The Hoehn & Yahr stages were ranged I to III and patients underwent extensive clinical evaluation, including brain magnetic resonance imaging (MRI) for cerebral white matter hyperintensity (WMH), tilt table test, the Korean version of Montreal Cognitive Assessments and the Korean version of Mini-Mental Status Examination in one month from the first clinic visit. The participants were divided into two groups according to the presence of OH (OH+ vs. OH−) and cognitive function (cognitive normal, CN vs. CI), respectively. RESULTS: Significant relationship between OH and cognitive function (p=0.04) was found in our patients. The patients with OH+ had higher risk of CI by 2.6 times than that of OH+ patients. Maximum heart rate change during tilt table test was correlated with cognitive function and white matter changes, whereas blood pressure change during tilt table test showed no correlation with those parameters. CONCLUSIONS: There was significant relationship between OH and CI in PD. Therefore, PD patients with either symptom may need periodic evaluation and proper management for OH and cognitive functions.

Clinical Reasoning: A 56-year-old Man with Wrist Drop

No abstract available.

Correlation of Driving-related Cognition and Cognitive Impairment in Drivers over the Age of 60

BACKGROUND: A rate of traffic accidents by elderly drivers increased by 70% between 2011 and 2015 in Korea. Elderly drivers with cognitive impairment are more susceptible to be involved traffic accidents than cognitively normal individuals. This study aimed to evaluate the correlation of driving-related cognition and cognitive impairment in drivers over the age of 60 using the standardized program developed by the Road Traffic Authority (RTA). METHODS: Current drivers older than 60 years were recruited for the study. All participants were tested using the Mini-Mental Status Examination (MMSE), Rey Complex Figure Test (RCFT)-copy, and Digit Symbol Substitution Test (DSST) for cognitive function assessments. The driving aptitude test (DAT) for the elderly developed by RTA consists of 4 different tasks including speed and distance driving tests (SD), visuospatial memory driving tests (VM), sustained attention driving tests (SA), and divided attention driving tests (DA). The participants were divided into two groups by their MMSE score (normal cognition, CN; cognitive impairment, CI). RESULTS: One hundred fourteen participants were enrolled in the study and 57 of them were assigned to the CI group. In comparison, drivers in CI showed worse scores at DSST, SD, VM, and DA than those in CN. DSST and DA were worsened with increasing age. DSST was the best predictive assessment to be the risk or caution grade in DAT. CONCLUSIONS: We could find a correlation between DAT and cognitive function in drivers over the age 60. These results could be used as the basis of investigating optimal tools for decreasing driving risks in the cognitive impaired elderly.

Postprandial Dizziness/Syncope Relieved by Alfa-Glucosidase Inhibitor: A Case Report / 대한평형의학회지

A 74-year-old man presented with positional vertigo and prandial dizziness and syncope. He had experienced episodes of frequent dizziness and loss of consciousness for several months. He underwent total gastrectomy with esophagojejunostomy and brown anastomosis 30 years ago. Thirteen years ago, subtotal colectomy with ileo-descending colostomy was done due to colon cancer. And he also had mitral valve replacement and maze operation due to severe mitral valve stenosis and atrial fibrillation. After cardiac operation, he has suffered from sudden dizziness with diaphoresis and chalky face, which usually occurs especially within 30 minutes from the onset of eating. Sometimes, this event was followed by several seconds of loss of consciousness, which caused recurrent events of falling. Neurological examination showed positional nystagmus compatible with benign paroxysmal positional vertigo arising from posterior semicircular canal of the right ear. The positional vertigo disappeared immediately after canalith repositioning maneuver. We tried to monitor vital signs and serum level of glucose during eating. Hyperglycemia (range, 210–466 mg/dL) was noted during eating, which was accompanied by postprandial and prandial hypotension, up to 60/40 mmHg. The patient was prescribed 100 mg of the alfa-glucosidase, acarbose to be taken half an hour before each meal. Eventually, the treatment with acarbose ameliorated the prandial dizziness and hypotension associated with hyperglycemia. Our patient suggests the acarbose could prevent postprandial dizziness and hypotension.

Characteristics of Cerebral Microbleeds

Cerebral microbleeds (CMBs) are increasingly recognized neuroimaging findings, occurring with cerebrovascular disease, dementia, and aging. CMBs are associated with subsequent hemorrhagic and ischemic stroke, and also with an increased risk of cognitive deterioration and dementia. They occur in the setting of impaired small vessel integrity due to hypertension or cerebral amyloid angiopathy. This review summarizes the concepts, cause or risk factors, histopathological mechanisms, and clinical consequences of CMBs.

Factors Affecting Cognitive Impairment and Depression in the Elderly Who Live Alone: Cases in Daejeon Metropolitan City

BACKGROUND AND PURPOSE: With the rapid increase in the number of elderly people in Korea, multiple socio-economic problems have emerged. In 2015, 6.4 million people accounting for about 13% of the total population in Korea were aged 65 years and over. As the elderly population continues to grow, the elderly who live alone are also increasing. They have potential risks in medical and neuropsychological aspects. The purpose of this study was to investigate the association between cognition and socio-environmental status in the elderly who live alone. METHODS: This study was conducted on 512 people who live alone (equivalent to 1% of the total elderly people) in Daejeon Metropolitan City between April and November 2015. Structured questionnaires were used to investigate the general characteristics, socio-economic status, physical status, and mood for participants. Simple tests using Mini-Mental Status Examination-Demetia Screening, Geriatric Depression Scale and Korean-instrumental activities of daily living were also performed. RESULTS: Among the 512 participants, 109 participants (21.3%) had cognitive impairment, and 128 participants (25.0%) had depression. The number of daily meals, frequency of meeting with family, and depression were independent risk factors for cognitive impairment. Factors including the duration of living alone, cognitive impairment, poor self-perceived health status, frequency of meeting with family and duration of education were considered an independent risk factor for depression. CONCLUSIONS: This study showed that the elderly who live alone are susceptible to cognitive impairment and depression, and factors including the number of daily meals, social contact, and self-perceived health status may affect cognition and depressive mood. Thus, physicians need to pay attention to management of major factors that may cause cognition impairment and depression in the elderly who live alone; in addition, they require ongoing community interest and support.

Chronic Inflammatory Demyelinating Polyradiculoneuropathy Associated with Membranous Glomerulonephritis and Tendinitis

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an immune-mediated progressive or relapsing demyelinating peripheral neuropathy. Other autoimmune diseases may be associated with CIDP. A 38-year-old man developed CIDP, which was subsequently associated with membranous glomerulonephritis (MGN) and tendinitis. He was treated with intravenous immunoglobulin, rituximab, and prednisone, which resulted in improvement of the clinical symptoms. This is a case report of CIDP associated with MGN and tendinitis.

Comparison of Somatic and Sudomotor Nerve Fibers in Type 2 Diabetes Mellitus

BACKGROUND AND PURPOSE: The objective of this study was to find a sensitive method for the early detection of diabetic polyneuropathy (DPN) and determine the relationship between the functions of somatic and autonomic small nerve fibers in DPN. METHODS: Patients with type 2 diabetes mellitus and DPN based on clinical symptoms, signs, intraepidermal nerve fiber density (IENFD), and findings in the quantitative sudomotor axon reflex test (QSART) were enrolled retrospectively. Neurological examinations and nerve conduction studies were performed on all patients. Heart-rate variability during deep breathing (DB ratio) and the Valsalva maneuver (Valsalva ratio) were used to quantify the cardiovagal function. Patients were divided into two groups: 1) normal nerve conduction, defined as small-fiber neuropathy (SFN) and 2) abnormal nerve conduction, defined as mixed-fiber neuropathy. RESULTS: In total, 82 patients were enrolled (age: 60.7±10.7 years, mean±SD). A decreased IENFD was the most frequent abnormality across all of the patients, followed by abnormalities of the QSART and cardiovagal function. A decreased IENFD was more sensitive than the QSART, DB ratio, and Valsalva ratio for detecting diabetic SFN. The DB ratio was significantly correlated with the duration of diabetes mellitus and clinical symptoms and signs. There was no correlation between the IENFD and the findings of the QSART for the distal leg. CONCLUSIONS: Measuring the IENFD was a more sensitive method than the QSART for the early detection of DPN. The degree of involvement of the somatic small nerve fibers and sudomotor nerve fibers was independent in DPN.

Treatment of Alzheimer's Disease with Repetitive Transcranial Magnetic Stimulation Combined with Cognitive Training: A Prospective, Randomized, Double-Blind, Placebo-Controlled Study

BACKGROUND AND PURPOSE: Repetitive transcranial magnetic stimulation (rTMS) has been examined as a potential treatment for many neurological disorders. High-frequency rTMS in particular improves cognitive functions such as verbal fluency and memory. This study explored the effect of rTMS combined with cognitive training (rTMS-COG) on patients with Alzheimer's disease (AD). METHODS: A prospective, randomized, double-blind, placebo-controlled study was performed with 27 AD patients (18 and 8 in the treatment and sham groups, respectively, and 1 drop-out). The participants were categorized into mild [Mini-Mental State Examination (MMSE) score=21-26] and moderate (MMSE score=18-20) AD groups. The rTMS protocols were configured for six cortical areas (both dorsolateral prefrontal and parietal somatosensory associated cortices and Broca's and Wernicke's areas; 10 Hz, 90-110% intensity, and 5 days/week for 6 weeks). Neuropsychological assessments were performed using the AD Assessment Scale-cognitive subscale (ADAS-cog), Clinical Global Impression of Change (CGIC), and MMSE before, immediately after, and 6 weeks after the end of rTMS-COG treatment. RESULTS: Data from 26 AD patients were analyzed in this study. There was no significant interactive effect of time between the groups. The ADAS-cog score in the treatment group was significantly improved compared to the sham group (4.28 and 5.39 in the treatment group vs. 1.75 and 2.88 in the sham group at immediately and 6 weeks after treatment, respectively). The MMSE and CGIC scores were also improved in the treatment group. Based on subgroup analysis, the effect of rTMS-COG was superior for the mild group compared to the total patients, especially in the domains of memory and language. CONCLUSIONS: The present results suggest that rTMS-COG represents a useful adjuvant therapy with cholinesterase inhibitors, particularly during the mild stage of AD. The effect of rTMS-COG was remarkable in the memory and language domains, which are severely affected by AD.