关键词： Transient loss of consciousness   Syncope   Tilt table testing   Vasovagal   Reflex syncope   Orthostatic hypotension   Psychogenic pseudosyncope  

摘要： An expert committee was formed to reach consensus on the use of Tilt Table Testing (TTT) in the diagnosis of disorders that may cause transient loss of consciousness (TLOC) and to outline when other provocative cardiovascular autonomic tests are needed. While TTT adds to history taking, it cannot be a substitute for it. An abnormal TTT result is most meaningful if the provoked event is recognised by patients or eyewitnesses as similar to spontaneous ones. The minimum requirements to perform TTT are a tilt table, a continuous beat-to-beat blood pressure monitor, at least one ECG lead, protocols for the indications stated below and trained staff. This basic equipment lends itself to perform (1) additional provocation tests, such as the active standing test carotid sinus massage and autonomic function tests;(2) additional measurements, such as video, EEG, transcranial Doppler, NIRS, end-tidal CO2 or neuro-endocrine tests;(3) tailor-made provocation procedures in those with a specific and consistent trigger of TLOC. TTT and other provocative cardiovascular autonomic tests are indicated if the initial evaluation does not yield a definite or highly likely diagnosis, but raises a suspicion of (1) reflex syncope, (2) the three forms of orthostatic hypotension (OH), i.e. initial, classic and delayed OH, as well as delayed orthostatic blood pressure recovery, (3) postural orthostatic tachycardia syndrome or (4) psychogenic pseudosyncope. A therapeutic indication for TTT is to teach patients with reflex syncope and OH to recognise hypotensive symptoms and to perform physical counter manoeuvres.

关键词： cardiac arrest   neurological outcome   neurological pupil index   prognostication   pupillometry   somatosensory evoked potentials  

摘要： Background Anoxic-ischemic brain injury is the most common cause of death after cardiac arrest (CA). Robust methods to detect severe injury with a low false positive rate (FPR) for poor neurological outcome include the pupillary light reflex (PLR) and somatosensory evoked potentials (SSEP). The PLR can be assessed manually or with automated pupillometry which provides the neurological pupil index (NPi). We aim to describe the interrelation between NPi values and the absence of SSEP cortical response and to evaluate the capacity of NPi to predict the absence of cortical SSEP response in comatose patients after CA. Methods A total of 50 patients will be included in an explorative, prospective, observational study of adult (>18 years) comatose survivors of CA admitted to intensive care in a university hospital. NPi assessed with a hand-held pupillometer will be compared to SSEP signals recorded >48 hours after CA. Primary outcomes are sensitivity, specificity, and odds ratio for NPi to predict bilateral absence of the SSEP N20 signal, with NPi values corresponding to <5% FPRs of SSEP absence. Secondary outcomes are the PLR and SSEP sensitivity, specificity, and odds ratio for poor neurological outcome at hospital discharge and death at 30 days. Discussion The PLR and SSEP may have a systematic interrelation, and a certain NPi threshold could potentially predict the absence of cortical SSEP response. If this can be concluded from the present study, SSEP testing could be excluded in certain patients to save resources in the multimodal prognostication after CA. Editorial comment The interrelation between loss of the pupillary light reflex (PLR) and the loss of cortical response to a somatosensory evoked potential (SSEP) in comatose cardiac arrest patients is not known. This exploratory prospective study is designed to evaluate whether a specific degree of attenuated PLR, as measured by semiautomated pupillometry, can predict the bilateral loss of cortical SSEP response in

关键词： Abbreviations and Acronyms: DNP   Dorsal nerve of the penis   CGN   Cutaneous branch of genitofemoral nerve   FG   Fluorescent gold   ICP   Intracavernosal pressure   DRG   Dorsal root ganglia   RE   Reflective erection   SCI   Spinal cord injury   SB   Spina bifida   PS   Penile sensation   SD   Sprague-Dawley   PBS   Phosphate-buffered saline   AD   Axon density   HE   Hematoxylin-eosin  

摘要： OBJECTIVE To explore the feasibility of the penile afferent pathway by the cutaneous branch of the genitofemoral nerve to the dorsal nerve of penile transfer in rats. METHODS A total of 54 male rats were randomly divided into model group (n = 18), resection group (n = 18), and sham group (n = 18). In the model group, the distal stump of bilateral DNP was anastomosed to the proximal stump of the bilateral CGN through end-to-end neurorrhaphy. In the resection group, bilateral DNP was severed and ligated, and no end-to-end anastomosis was performed. Only a surgical incision was made in the sham group, and no nerve injury was caused. After the operation, the feasibility of reconstructing the penile afferent pathway was explored by fluorescent-gold retrograde neural labeling. The intracavernous pressure assessment was then carried out. The morphological examination, histological staining of nerves, and ultrastructural observation were performed accordingly. RESULTS Fluorescent-gold labeled L1 and L2 neurons in the model group were positive. The mean ICP in the model group was (12.02 +/- 2.03 mmHg), which is higher than the mean value in the resection group (0 mmHg, P < .05) but lower than that in the sham group (36.95 +/- 5.33 mmHg;P < .05). The morphological studies, HE, and ultrastructure observation revealed that the regeneration of DNP axons in the model group was significantly better than that in the resection group yet did not reach the level of the sham group. CONCLUSION This experiment preliminarily proved the feasibility of restoration of the penile afferent pathway by CGN to DNP transfer in Rats. (C) 2021 Elsevier Inc.

关键词： functional neurological disorder   conversion disorder   placebo   open‐   label placebo   suggestibility   pain  

摘要： Background and purpose On the basis of occasional strong placebo responses, increased susceptibility to placebo has been proposed as a characteristic of functional neurological disorder (FND). The aim of this study was to clarify whether people with FND have a stronger placebo analgesic response than healthy controls. Methods A study using a classic placebo paradigm, with additional conditioning and open-label components, was performed in 30 patients with FND, and in 30 healthy controls. Ratings of mildly to moderately painful electrotactile stimuli were compared before and after the application of a placebo "anaesthetic" cream versus a control cream, after an additional conditioning exposure, and after full disclosure (open-label component). Results Pain intensity ratings at the placebo compared to the control site were similarly reduced in both groups. The conditioning exposure had no additional effect. After placebo disclosure a residual analgesic effect remained. Conclusion Patients with FND did not have stronger placebo responses than healthy controls. The notion of generally increased suggestibility or increased suggestibility to placebo in FND seems mistaken. Instead, occasional dramatic placebo responses may occur because functional symptoms are inherently more changeable than those due to organic disease.

关键词： Glutamic-acid decarboxylase   Antibodies   Limbic encephalitis   Cerebellar ataxia   Stiff-person syndrome   Genetics  

摘要： The common co-occurrence of autoimmune systemic diseases in patients with neurological disorders and antibodies against glutamic acid decarboxylase 65 (GAD65) suggests a shared genetic predisposition to these disorders. However, the nature and frequency of familial aggregation of autoimmune diseases, which might also support this hypothesis, have been poorly investigated. Herein, an exploratory, interview-based study was conducted with the aim of describing the autoimmune diseases displayed by the relatives of GAD65 neurological patients, their frequency, kinship, and potential patterns of inheritance. Patients were enrolled only if they had GAD65 antibodies in the cerebrospinal fluid and typical clinical phenotypes associated with such antibodies (stiff-person syndrome, cerebellar ataxia, limbic encephalitis, or temporal lobe epilepsy). A total of 65 patients were included in the study, and 44/65 (67.7%) reported family history of autoimmunity, including first-degree relatives in 36/65 (55.4%);the sibling recurrence risk (lambda(S)) was 5.5, reinforcing the hypothesis of an underlying strong genetic predisposition. Most pedigrees with familial autoimmunity (38/44, 86.4%) showed multiple autoimmune diseases, all but 2 of them with diabetes mellitus or autoimmune thyroid disease, therefore resembling autoimmune polyendocrine syndromes. Inheritance patterns were diverse, possibly autosomal dominant in 17/44 (38.6%) pedigrees or autosomal recessive in 5/44 (11.4%), and un-defined or complex in 24/44 (54.5%). However, a total of 21/65 (32.3%) patients had no identified family history of autoimmunity. In conclusion, these results suggest a variable and heterogeneous genetic predisposition to GAD65 neurological disorders, possibly involving multiple loci and modes of inheritance with different contribution in each family.

关键词： Historical neurology   Myerson's sign   Neuroethics   Neuropsychiatry  

关键词： academic neurology   Accreditation Council for Graduate Medical Education   headache rotation   milestones  

摘要： Objective To quantify the proportion of headache-related grand rounds in academic neurology programs and to compare this with adult neurology residency director views on the need for an increase in headache-related grand rounds. Background Although headache are among the most prevalent and most burdensome neurologic conditions, headache medicine is often considered underrepresented in neurology departments. Additionally, prior studies have shown that many neurology residency directors feel that training programs do not include an adequate amount of exposure to headache clinics or headache-related didactics. One important aspect of didactic education in neurology departments is adult neurology grand rounds. Previous publications have evaluated neither the proportion of headache-related grand rounds in academic neurology departments nor the residency program directors' views on appropriate amount of headache-related grand rounds. Our study has attempted to quantify this information to elucidate opportunities to improve practice educational gaps. Methods In this cross-sectional study, we surveyed adult neurology residency directors (from the Accreditation Council for Graduate Medical Education [ACGME] listing of academic adult neurology residency programs) between October 2018 and September 2019. In addition, we used two methods to obtain the proportion of headache-related grand rounds in neurology: (1) emailing residency directors a questionnaire asking for a list of prior grand rounds topics and (2) an online search for each academic neurology program. Results First, for our grand rounds analysis, headache medicine consisted of 3.7% of the lectures in 2017-2018 and 6.3% of the lectures in 2018-2019 (average of each institution;17 institutions and 411 total lectures in 2017-2018, 21 institutions and 463 total lectures in 2018-2019). The most common number of lectures on headache medicine for each grand rounds series was zero (for 7 of 17 grand rounds series in 2017-20

关键词： biomarker   neurofilament   neurologic disease  

摘要： Neurofilament light chain (NfL) is an incredibly specific marker of neuronal injury that is not specific for cause or location of the neuronal damage. NfL is increasingly considered as possible biomarker of disease activity in neurological conditions. Several works reviewed the utility of NfL in the different diseases. Nonetheless, NfL is a universal marker of neuronal damage, which interpretation spaces beyond the single disease. Because of this, the interpretation of NfL may benefit by also considering how neurological symptoms relate to its blood concentration. Here, we review how different neurological symptoms can be associated with blood NfL levels with a practical interpretation of it.

关键词： Carotid intima-media thickness   Arterial tonometry   Cerebral microbleed   Magnetic resonance imaging  

摘要： Aims To determine if arterial functional and structural changes are associated with underlying cerebral small vessel disease in neurologically asymptomatic individuals with type 1 diabetes. Methods We enrolled 186 individuals (47.8% men;median age 40.0, IQR 33.0-45.0 years) with type 1 diabetes (median diabetes duration of 21.6, IQR 18.2-30.3 years), and 30 age- and sex-matched healthy controls, as part of the Finnish Diabetic Nephropathy (FinnDiane) Study. All individuals underwent a biochemical work-up, brain magnetic resonance imaging (MRI), ultrasound of the common carotid arteries and arterial tonometry. Arterial structural and functional parameters were assessed by carotid intima-media thickness (CIMT), pulse wave velocity and augmentation index. Results Cerebral microbleeds (CMBs) were present in 23.7% and white matter hyperintensities (WMHs) in 16.7% of individuals with type 1 diabetes. Those with type 1 diabetes and CMBs had higher median (IQR) CIMT 583 (525 - 663) mu m than those without 556 (502 - 607) mu m, p = 0.016). Higher CIMT was associated with the presence of CMBs (p = 0.046) independent of age, eGFR, ApoB, systolic blood pressure, albuminuria, history of retinal photocoagulation and HbA(1c). Arterial stiffness and CIMT were increased in individuals with type 1 diabetes and WMHs compared to those without;however, these results were not independent of cardiovascular risk factors. Conclusions Structural, but not functional, arterial changes are associated with underlying CMBs in asymptomatic individuals with type 1 diabetes.

关键词： immune cells   immunometabolism   leukocytes   neurodegeneration   neuroinflammation  

摘要： Neuroinflammatory and neurodegenerative diseases are characterized by the recruitment of circulating blood-borne innate and adaptive immune cells into the central nervous system (CNS). These leukocytes sustain the detrimental response in the CNS by releasing pro-inflammatory mediators that induce activation of local glial cells, blood-brain barrier (BBB) dysfunction, and neural cell death. However, infiltrating peripheral immune cells could also dampen CNS inflammation and support tissue repair. Recent advances in the field of immunometabolism demonstrate the importance of metabolic reprogramming for the activation and functionality of such innate and adaptive immune cell populations. In particular, an increasing body of evidence suggests that the activity of metabolites and metabolic enzymes could influence the pathogenic potential of immune cells during neuroinflammatory and neurodegenerative disorders. In this review, we discuss the role of intracellular metabolic cues in regulating leukocyte-mediated CNS damage in Alzheimer's and Parkinson's disease, multiple sclerosis and stroke, highlighting the therapeutic potential of drugs targeting metabolic pathways for the treatment of neurological diseases.