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The nicotinic acetylcholine receptor (nAChR) antibody directly contributes to the autonomic dysfunction in Autoimmune Autonomic Ganglionopathy (AAG). The pathological mechanism leading to autonomic dysfunction in seronegative AAG is unclear. We evaluated patients with presumed antibody negative AAG (n=49) to determine whether there was an association with other autoantibodies. Three patients met the clinical criteria and were positive for N-type calcium channel antibodies. All patients had severe autonomic dysfunction characterized by orthostatic hypotension and gastrointestinal involvement. Autonomic testing revealed severe impairment of sudomotor, cardiovagal, and adrenergic domains. These findings raise the possibility that other autoantibodies may contribute to the pathogenesis of AAG.

Objective To test the hypothesis that Joint hypermobility and autonomic dysfunction are over-expressed within neurodevelopmental disorders. Joint hypermobility is a widespread poorly recognized connective tissue condition with affected individuals overrepresented among panic and anxiety disorders, irritable bowel syndrome, fibromyalgia, and chronic fatigue. The relevance of hypermobility to neuropsychiatric disorders of developmental origin is currently unknown, despite anecdotal case reports and clinical suspicion of a link. Autonomic nervous system dysregulation, typically postural tachycardia syndrome is often found in hypermobile individuals. Interestingly, differences in amygdala and superior temporal cortex anatomy have been reported in hypermobile populations and functional abnormalities in patients with autism. Method Thirty-seven adults with neurodevelopmental disorder, 205 patients attending general psychiatric clinics without neurodevelopmental diagnosis and 29 healthy controls were recruited. Hypermobility was assessed using the Beighton scale (BS) and autonomic symptoms using the Autonomic Symptoms and Quality of Life Score (ASQoLS: orthostatic, gastrointestinal, bladder, secretomotor, sudomotor and sleep domains. Results The neurodevelopmental cohort had a mean age of 34.6 years (27 male). Nineteen had Attention Deficit Hyperactivity Disorder (ADHD), 4 Autistic Spectrum Disorder (ASD), 1 Tourette Syndrome (TS) and the remainder combinations of ADHD, ASD and TS. Nine had co-morbid affective disorder. Eighteen patients (48.6%) were classified as hypermobile (BS>=4) compared to 67/204(32.7%) in the general psychiatric group (p=0.048) and 3/29(10.3%) in healthy controls (p=0.007) and this prevalence was also significantly higher that reported in a large general population cohort (1156/6022, 19.19%, p=<0.001). Mean autonomic dysfunction score was significantly higher in the neurodevelopmental cohort compared to controls (mean±SEM: neurodevelopmental disorder patients, 45.8±4.86; controls, 8.5±1.62). This effect was seen across all sub-scales of the ASQoLS. Total autonomic dysfunction score did not differ significantly between neurodevelopmental cohort and the general psychiatric group, however neurodevelopmental disorder patients had significantly higher scores on orthostatic and gastrointestinal disturbance subscales. Conclusion We demonstrate for the first time that rates of hypermobility and symptoms of autonomic dysfunction are particularly high in adults with neurodevelopmental diagnoses. It is likely that the importance of hypermobility and autonomic dysfunction to the generation and maintenance of psychopathology in neurodevelopmental disorders is poorly appreciated. Work underway(autonomic testing, fMRI) will test the hypothesis that autonomic reactivity and interoceptive sensitivity predispose to the expression of psychiatric symptoms, particularly anxiety. It is further hypothesized that inefficient neural co-ordination of efferent autonomic drive with imprecise interoceptive representat...

BackgroundWe aimed to create a multidisciplinary consensus clinical guideline for best practice in the diagnosis, investigation and management of spontaneous intracranial hypotension (SIH) due to cerebrospinal fluid leak based on current evidence and consensus from a multidisciplinary specialist interest group (SIG).MethodsA 29-member SIG was established, with members from neurology, neuroradiology, anaesthetics, neurosurgery and patient representatives. The scope and purpose of the guideline were agreed by the SIG by consensus. The SIG then developed guideline statements for a series of question topics using a modified Delphi process. This process was supported by a systematic literature review, surveys of patients and healthcare professionals and review by several international experts on SIH.ResultsSIH and its differential diagnoses should be considered in any patient presenting with orthostatic headache. First-line imaging should be MRI of the brain with contrast and the whole spine. First-line treatment is non-targeted epidural blood patch (EBP), which should be performed as early as possible. We provide criteria for performing myelography depending on the spine MRI result and response to EBP, and we outline principles of treatments. Recommendations for conservative management, symptomatic treatment of headache and management of complications of SIH are also provided.ConclusionsThis multidisciplinary consensus clinical guideline has the potential to increase awareness of SIH among healthcare professionals, produce greater consistency in care, improve diagnostic accuracy, promote effective investigations and treatments and reduce disability attributable to SIH.

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Background: Fibromyalgia is characterised by chronic widespread pain. Quality of life is further reduced by autonomic and cognitive symptoms, including subjective brain-fog and dissociative experiences. Although an association with joint hypermobility suggests variant connective tissue is a factor in both fibromyalgia and dysautonomia, the mechanisms underlying the neuropsychiatric symptoms are poorly understood. Methods: 21 fibromyalgia patients and 21 healthy controls were assessed for joint hypermobility dissociative experiences, autonomic symptoms and interoceptive sensibility. Mediation analyses were conducted according to the method of Baron and Kenny. Results: Patients with fibromyalgia reported greater dissociative experiences and autonomic symptoms. The relationship between fibromyalgia and dissociative experiences was fully mediated by symptoms of orthostatic intolerance. Fibromyalgia, dissociative experiences and orthostatic intolerance all were associated with joint hypermobility and interoceptive sensibility. Conclusions: This exploratory investigation highlights the relationship between dissociative experiences in the context of fibromyalgia and subjective experience of aberrant physiological responses. These findings can enhance the recognition and management of neuropsychiatric symptoms in patients with fibromyalgia, wherein dissociative experiences reflect disturbance of self-representation that can arise through abnormalities in internal agency, autonomic (dys)control and interoceptive prediction errors.

ObjectiveTo assess the knowledge, attitudes and practices of healthcare professionals regarding the diagnosis and management of spontaneous intracranial hypotension (SIH).MethodsWe performed a cross-sectional, web-based survey of multiple healthcare professional groups in the UK from June to August 2021. There were 227 respondents to the survey, including 62 general practitioners, 39 emergency medicine physicians, 38 neurologists, 35 radiologists, 20 neurosurgeons, 18 anaesthetists and 15 headache nurse specialists. The majority of the respondents were at the consultant level and all worked in the UK National Health Service.ResultsFew general practitioners or emergency medicine physicians had ever been involved in the care of a patient with SIH or received teaching about SIH. Only 3 of 62 (4.8%) general practitioners and 1 of 39 (2.5%) emergency medicine physicians were confident in recognising the symptoms of SIH. Most neurologists were confident in recognising SIH and performed MRI of the brain as a first-line investigation, although there was variability in the urgency of the request, whether contrast was given or MRI of the spine organised at the same time. Most said they never or rarely performed lumbar puncture for diagnosis of SIH. Most neuroradiologists, but few general radiologists, were confident in interpreting imaging of patients with suspected SIH. Lack of access to epidural blood patching, personnel able to perform myelography, and established management pathways were identified by many respondents as barriers to the treatment of SIH.ConclusionsWe have identified a lack of awareness of SIH among non-specialists, several barriers to optimal treatment of SIH and a variation in current management pathways. The results highlight the need for education of healthcare professionals about SIH and the development of clinical practice guidelines to enable delivery of optimal and equitable care for patients with SIH.