Clinical Features and Early Recognition of 242 Cases of Autoimmune Encephalitis

Objective: To analyze the clinical features of common autoimmune encephalitis and evaluate the sensitivity of antibodies contributing to focal epilepsy signs and symptoms (ACES) score.Methods: Collecting and analyzing the data of 242 patients with autoimmune encephalitis (AE) diagnosed in the First Affiliated Hospital of Zhengzhou University from August 2015 to December 2020 in this retrospective study. The six items of the ACES score (cognitive symptoms, behavioral changes, autonomic symptoms, speech problems, autoimmune diseases, temporal MRI hyperintensities) were screened in patients with complete clinical data.Results: (1) In total, 242 patients were included, with 147 cases of anti-N-methyl-D-aspartate receptor encephalitis, 47 cases of anti-γ-aminobutyric acid type B (GABA-B) receptor encephalitis, and 48 cases of anti-leucine-rich glioma inactivating protein 1 (LGI1) encephalitis. The most common clinical symptoms are cognitive impairment (77%), behavioral changes (79%), and seizures (71%). In total, 129 cases (54%) combined with autonomic dysfunction, such as gastrointestinal dysmotility, sinus tachycardia, and central hypoventilation. Twelve patients had autoimmune diseases, most of which were of thyroid diseases. (2) One hundred and twenty-seven patients with complete clinical data evaluated ACES score, 126 cases of whom (126/127, 99.2%) were equal to or >2 points, 1 case (1/127, 0.8%) was of <2 points.Interpretation: (1) Cognitive impairment, abnormal behavior, and seizures are the most common manifestations of AE and autonomic symptoms. Thyroid disease is the most autoimmune disease in AE. Clinically, for patients of suspected AE, increasing the knowledge and testing of thyroid function and rheumatism is necessary. (2) ACES score is a simple, effective, and easy-to-operate score, with a certain screening value for most patients suspected of AE.

Cranial autonomic symptoms in episodic and chronic migraine: a cross sectional study in Iran

Background Cranial autonomic symptoms are common in migraine, with eye redness and tearing being the most common ones. Their identification can help to avoid misdiagnosis, predict the disease course, and select the appropriate treatment. Methods This was a cross-sectional study of 904 patients who presented with migraine to a headache referral clinic. The participants filled out a questionnaire about their headache characteristics, as well as the presence of cranial autonomic symptoms. A total of 904 patients, 698 women (77.2%) and 206 men (22.8%), were included in the study, with a mean (SD) age of 38.05 (11.76) years. Results About 70% of subjects with chronic migraine and 56.2% of those with episodic migraine reported one or more cranial autonomic symptoms. The two most commonly reported autonomic symptoms were eye redness (36.06%) and tearing (21.02%). Chronic migraine (43.4% vs. 29.5%), unilateral headache (56.8% vs. 48.7%), and blurred vision (20% vs. 14.7%) were significantly more frequent in migraineurs with cranial autonomic symptoms. Headache intensity and frequency in subjects with cranial autonomic symptoms were significantly higher than in those without cranial autonomic symptoms. Conclusion We found higher percentages of cranial autonomic symptoms in patients with unilateral headaches, frequent and severe attacks and blurred vision. A diagnosis of cranial autonomic symptoms accompanying migraine may predict more severe disease and the possibility of evolution into chronic migraine.

Validation of the Composite Autonomic Symptom Score 31 in the German language

Background The Composite Autonomic Symptom Score 31 (COMPASS 31) is a validated, 31-item self-assessment questionnaire assessing autonomic symptoms in six domains, orthostatic intolerance, vasomotor, secretomotor, gastrointestinal, bladder, and pupillomotor function. So far, there is no validated German COMPASS 31 version. This study aimed at developing and validating a German COMPASS 31. Methods Two autonomic experts with command of German and English independently translated the English COMPASS 31 into German. One agreed-upon German version was translated back into English to assure conformity with the original version. Twenty patients with possible autonomic symptoms and 20 age- and gender-matched healthy persons completed the English and German COMPASS 31 in a randomized order with a 4-week interval. To evaluate reliability of the German COMPASS 31, total scores and sub-scores of the domains assessed with the German version were correlated with corresponding scores of the English version using Pearson’s or Spearman’s test. The Cronbach alpha-coefficient evaluated the internal consistency of the questions. Total- and sub-scores of both COMPASS 31 versions were compared between patients and controls by analysis of variance with post-hoc analysis (significance: p < 0.05). Results Total scores and sub-scores of the German and English COMPASS 31 correlated significantly (p < 0.001) and closely (correlation coefficients: 0.757–0.934). Cronbach alpha-coefficients were above 0.7 in all domains except for the secretomotor domain. In the German and English COMPASS 31, total scores were significantly higher in patients than controls. Conclusions The German COMPASS 31 is reliable, internally consistent, and valid to detect and quantify autonomic symptoms in patients with neurological disorders.

Autonomic Symptoms in Older Adults Are Common and Associated With Health-Related Quality of Life

Background: Autonomic symptoms are common in older adults, and a large body of literature focusing on age-related diseases shows that autonomic symptoms in these diseases constrain Health-Related Quality of Life (HRQoL). To our best knowledge, the association between autonomic symptoms in older adults, independent of specific diseases, and HRQoL has not yet been assessed.Aim: To assess the frequency and the effect of autonomic symptoms in general, as well as orthostatic intolerance, vasomotor, secretomotor, gastrointestinal, bladder, and pupillomotor symptoms, on HRQoL in older adults.Methods: Cross-sectional data of the fourth visit of the Tübinger evaluation of Risk factors for Early detection of Neurodegeneration (TREND) study were included. Autonomic symptoms, as assessed with the Composite Autonomic Symptom Score 31 (COMPASS 31), were quantified and compared with HRQoL, as assessed with the EuroQol five-level version (EQ-5D-5L). Statistical analyses included Spearman's rank correlation and multiple linear regression analysis.Results: The analysis included 928 participants with a median of 68 years; 47% were women. Of those, 85% reported at least one autonomic symptom. Gastrointestinal and secretomotor symptoms were most common. The COMPASS 31 total score and all subdomains were significantly associated with reduced HRQoL. Among the subdomains, the strongest correlations with HRQoL were found for gastrointestinal and bladder symptoms. Overall, autonomic symptoms alone explained 20% of the variance of HRQoL; when depressive mood was added, the model explained 32%.Conclusion: Autonomic symptoms are associated with HRQoL and depressive symptoms in older adults.

The prevalence and characteristics of visceral autonomic symptoms among migraineurs: A population-based study

Background There is limited evidence about visceral autonomic symptoms in the different phases of migraine attack. We evaluated the prevalence of these symptoms in migraineurs before, during, and after headache attacks. In addition, the association between migraine characteristics and visceral autonomic symptoms was investigated. Methods A total of 605 participants who met the entry criteria were enrolled prospectively in this cross-sectional study. Participants were 18–60 years old and met the ICHD-3 criteria for migraine with or without aura. Information on the migraine symptoms was gathered from all participants in face-to-face interviews. A structured questionnaire was used to evaluate the related symptoms before, during, and after attacks. Migraine features of frequency, duration, and severity were also assessed. Results Considering all phases, the most commonly present visceral symptom was nausea (52.9%). About half of participants reported at least one visceral symptom before the onset of attacks. While, during and after attacks, 71% and 36% of participants reported such characteristics, respectively. Notably, the migraine headache in participants with visceral symptoms was longer during attacks compared to participants without them (24.4 ± 29.6 vs. 16.8 ± 19.8; P = 0.008). Additionally, subjects with positive visceral symptoms experienced more severe migraine attacks than participants without symptoms for both the premonitory (8 ± 1.7 vs. 7.6 ± 1.8; P = 0.02) and during-attack (8.01 ± 1.7 vs. 7.09 ± 1.9; P = 0.001) phases. Conclusion This study demonstrated that visceral symptoms were common in migraine sufferers not only during attacks but also in the premonitory and postdrome phases. Positive visceral symptoms were also associated with more burdensome headache attacks. Therefore, recognizing the phenotypic presentation of associated symptoms of migraine, especially during the pre-attack phase, could aid in early implementation of optimal management.

Validation of the Korean version of the composite autonomic symptom scale 31 in patients with Parkinson’s disease

Purpose The composite autonomic symptom scale-31 (COMPASS-31) is a self-rated questionnaire that evaluates diverse autonomic symptoms. In the present study, we developed the Korean version of the COMPASS-31 (K-COMPASS-31) with appropriate translation, and verified its reliability and internal and external validity in patients with Parkinson’s disease (PD). Methods The original COMPASS-31 was translated independently into Korean by two bilingual neurologists. Test-retest reliability was evaluated at a 2-week interval. We investigated the correlations between the K-COMPASS-31, the scale for outcomes in PD-autonomic (SCOPA-AUT), and the results of an autonomic function test (AFT), respectively. Results A total of 90 patients with PD (47 females; mean age, 63.4 ± 10.8 years) were enrolled. The K-COMPASS-31 showed excellent test-retest reliability (intra-class correlation coefficient = 0.874, p < 0.001) and internal validity (Cronbach’s α-coefficient = 0.878). The COMPASS-31 was positively correlated with SCOPA-AUT (r = 0.609, p < 0.001) and the results of the AFT. Conclusions In conclusion, the K-COMPASS-31 showed excellent reliability and validity for the assessment of autonomic symptoms in PD patients. The K-COMPASS-31 is an easy-to-repeat and widely used tool for investigating autonomic dysfunction in various neurologic disorders and enables comparison of autonomic dysfunction among neurologic disorders. We recommend the K-COMPASS-31 as a valid instrument for use in clinical practice for patients with PD.

A case of cranial autonomic dysfunctions predominant migraine: Migraine sans ache

Pain is the essential part of migraine headaches along with other features, whereas, cranial autonomic symptoms (CAS)/signs are a predominant and essential part of trigeminal autonomic cephalalgias. What if other features of migraine are present but the pain is absent? One such variation in migraine symptomatology is reported in this case, where the pain was not there but CAS/signs were predominant along with other features of migraine. The possible mechanism for such variation, in this case, maybe desensitization of afferent loop (comprised by nociceptors in extracranial and intracranial pain-sensitive structures supplied by the trigeminal nerve) and direct activation of the trigemino-cervical complex and brainstem structures causing dissociation of pain from the rest of the features of migraine. In this case, as the pain part gets completely dissociated from CAS/signs, hence this condition can be termed as “migraine sans ache.” The patient was started on anti-migraine treatment and she responded wonderfully