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The Autonomic Nervous System, Dysautonomia and Its Relationship to Cranio-Cervical Instability

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The Autonomic Nervous System, Dysautonomia and Its Relationship to Cranio-Cervical Instability EDS Global Learning Conference (Nashville) July 30-August 1, 2019 The Autonomic Nervous System, Dysautonomia and its Relationship to Cranio-cervical Instability Ehlers Danlos Society Nashville , Tennessee Fraser C. Henderson Sr The autonomic system controls: • Cerebral circulation • Cardiac function • Baroreceptors and blood pressure • Kidney blood flow • Gastrointestinal function • Pulmonary secretions, airway resistance • sudomotor function , temp regulation Henderson, FC: Autonomic Nervous System, Dysautonomia and its Relationship to Cranio- cervical Instability 1 EDS Global Learning Conference (Nashville) July 30-August 1, 2019 Sympathetic Parasympathetic Cranio-Sacral Parasympathetic nerves • Cranial nerves : III, VII, IX, X- the Vagus nerve • Sacral nuclei S2,3,4 in the dorsolateral cell column Henderson, FC: Autonomic Nervous System, Dysautonomia and its Relationship to Cranio- cervical Instability 2 EDS Global Learning Conference (Nashville) July 30-August 1, 2019 Mydriasis due to increased sympathetic influence via superior cervical ganglion Hyperhydrosis / heat intolerance • mental stimuli: sympathetic sudomotor postganglionic cholinergic fibers • Response to cold : sympathetic vasoconstriction of Arteriovenous anastomoses Henderson, FC: Autonomic Nervous System, Dysautonomia and its Relationship to Cranio- cervical Instability 3 EDS Global Learning Conference (Nashville) July 30-August 1, 2019 Autonomic influences on the Cerebral Circulation Autoregulation maintains blood flow: { pH, metabolic needs, altered blood flow } Superior sympathetic ganglia vasoconstriction Via Carotid Aa. – forebrain structures Via Vertebrobasilar Aa- hindbrain Parasympatheics (Sup Salivatory Nuc, CN7, Sphenopalatine ganglion dilate blood vessels via peptidergic transmitters, ACh, NO,VIP Locus Coeruleus • Pontine nucleus • Norepinephrine • All parts • Influenced my mental stress, hypothalamus Henderson, FC: Autonomic Nervous System, Dysautonomia and its Relationship to Cranio- cervical Instability 4 EDS Global Learning Conference (Nashville) July 30-August 1, 2019 Dysautonomia in Locus coeruleus • Panic disorder • Anxiety • Clinical depression • Parkinson’s and Alzheimer’s • PTSD • ADHD Autonomic control of cardiac function • Intrinsic control : Starling mechanism • sympathetics increase CO to 20L/min • Sympathetic ganglia T5T6 , via coronary aa influence α 1, Β1 receptors on SA ,AV nodes • vagus enters AV groove: SA and AV nodes increase sarcolemmal K+ , hyperpolarizing the membrane , decrease HR and excitability of pacemaker cells Henderson, FC: Autonomic Nervous System, Dysautonomia and its Relationship to Cranio- cervical Instability 5 EDS Global Learning Conference (Nashville) July 30-August 1, 2019 Autonomic control of blood pressure: the Baroreceptors • Stretch sensitive mechanoreceptors , split second negative feedback loop to maintain BP • aortic arch, vagus nerve , carotid artery to glossopharyngeal nerve to the Nucleus Solitarius • Increased BP excites - caudal ventrolateral thalamus, inhibits rostral ventrolateral thalamus, • decreases sympathetic activity in the pre-ganglionic neurons intermedio-lateral tract of the spinal cord • decreased cardiac stimulation Autonomic control of the Kidneys Decreased BP , less firing Nucleus Solitarius - decreased inhibition of Rostral VLT, increased tonic excitatory input to sympathetic fibers Exit cord as Splanchnic nn – aortico renal ganglia Stimulate β1 receptors on juxtaglomerular cells increase renin increased Na+ reabsortion Increased BP – nuc sol –increased BF to kidneys and increased glomerular filtration also decreased ADH Henderson, FC: Autonomic Nervous System, Dysautonomia and its Relationship to Cranio- cervical Instability 6 EDS Global Learning Conference (Nashville) July 30-August 1, 2019 Autonomic Control of the Airways • NUCLEUS AMBIGUUS • Vagus Nerve : M cells • Bronhoconstriction, mucosal glanDs • Aδ fiBers – smoke histamine,serotonin • C fibers ADrenergic tone regulateD By circulating epinephrine Afferent C Fibers of Vagus noxious stim activates C-fibers afferent impulses to brain parasympathetic activity –constriction of airway smooth muscle –increased mucous gland secretion –vasodilation –increased vascular permeability – leaky vessels –increased muco-ciliary activity Henderson, FC: Autonomic Nervous System, Dysautonomia and its Relationship to Cranio- cervical Instability 7 EDS Global Learning Conference (Nashville) July 30-August 1, 2019 Parasympathetics control the GI tracts • CN VII and IX : salivary glands • CN X gastric acid and pepsinogen, pancreas acinar cells- trypsin, amylase lipase CCK contracts gallbladder • CN X and S2,S3,S4 - transmitters and peptides to control the intrinsic (enteric) nervous plexi: Meisner submucosal, Auerbach myenteric , deep muscular Cajal fibers • GI muscle, electrical syncytium- sequentially linked transverse and longitudinal muscle ANS Control of the GI Vagus innervates to splenic flexure, Sacral S2,3,4 parasympathetics below Sympathetic n. maintains continence contraction of internal sphincter muscle Henderson, FC: Autonomic Nervous System, Dysautonomia and its Relationship to Cranio- cervical Instability 8 EDS Global Learning Conference (Nashville) July 30-August 1, 2019 Autonomic innervation of the bowel Dysautonomia of the GI system dysphagia gastric reflux gastroparesis malabsorption bloating, constipation Irritable bowel syndrome, colitis incontinence Henderson, FC: Autonomic Nervous System, Dysautonomia and its Relationship to Cranio- cervical Instability 9 EDS Global Learning Conference (Nashville) July 30-August 1, 2019 Raynaud’s • numb and cold in response to cold decreased • vasospasm Chronic regional pain syndromes Henderson, FC: Autonomic Nervous System, Dysautonomia and its Relationship to Cranio- cervical Instability 10 EDS Global Learning Conference (Nashville) July 30-August 1, 2019 Dysautonomia: syncope Framingham study 3 % men 3.5% women • activation cardio-inhibitory vasodepressor reflex • orthostatic hypotension, • acute decrease in C.O. • increase in resistance Syncope is co-morbid with Chiari Henderson, FC: Autonomic Nervous System, Dysautonomia and its Relationship to Cranio- cervical Instability 11 EDS Global Learning Conference (Nashville) July 30-August 1, 2019 POTS • POTS : 74% of the EDS population • most disabling manifestation of dysautonomia • high resting sympathetic tone • impaired ability to respond respond to hypotension (vasoconstriction) • normal parasympathetic regulation. Gait Y, Nahir AM, Grahame R, Jacob G. Dysautonomia in the joint hyper- mobility syndrome. Am J Med;115:33–40, 2003 . Low PA . Laboratory evaluation of the autonomic nervous system. In: Low PA, editor. Clinical Autonomic Disorders. 3rd ed. Rochester: Lippincott Williams & Wiklins; 2010;143. Orthostatic intolerance in CFS and EDS > 30 beats / minute Sudden drop in blood POTS: 30 bpm increase (40 bpm in adolescents)pressure in HR with symptoms, or HR > 120 bpm, in first 10 min of standing or HUT Henderson, FC: Autonomic Nervous System, Dysautonomia and its Relationship to Cranio- cervical Instability 12 EDS Global Learning Conference (Nashville) July 30-August 1, 2019 Pooling of blood in the lower extremities Causes of Dysautonomia • Hereditary conditions: Riley Day Syndrome • Autoimmune injury : Sjogrens, lupus , sarcoidosis, MS, NMDA receptor encephalitis , HIV/AIDS, Guillain Barre • Fibromyalgia, CFS, IBS • Asphyxiation/poisoning • Injuries to the PNS: DM, Lyme’s, TB, botulism, • Mitochondrial disorders • Degenerative conditions- Parkinson’s • Trauma to the CNS • Hypermobility Connective tissue disorders- EDS • Brain stem deformity conditions Henderson, FC: Autonomic Nervous System, Dysautonomia and its Relationship to Cranio- cervical Instability 13 EDS Global Learning Conference (Nashville) July 30-August 1, 2019 Where is dysautonomia ? • autonomic impairment is multifactorial • stratified • Involves brain, brainstem, spinal cord, autonomic plexi, peripheral and the sympathetic nerves • DeWandele I, Rombaut L, Leybaert L, VandeBorne P, DeBacker T, Malfait F, DePaepe A, Calders P. Dysautonomia and its underlying mechanisms in the hypermobility type of Ehlers– Danlos syndrome. Seminars in Arthritis and Rheumatism, 44:93- 100,2014 athetic nerves (21) What are the brainstem nuclei implicated in the causation of dysautonomia ? Henderson, FC: Autonomic Nervous System, Dysautonomia and its Relationship to Cranio- cervical Instability 14 EDS Global Learning Conference (Nashville) July 30-August 1, 2019 Dorsal motor nuclei of vagus- GI function Nucleus solitarius Nucleus ambiggus 29 Hypothesis: Bio-mechanical deformity of the cervico-medullary Junction alters autonomic activity Henderson, FC: Autonomic Nervous System, Dysautonomia and its Relationship to Cranio- cervical Instability 15 EDS Global Learning Conference (Nashville) July 30-August 1, 2019 What causes biomechanical deformity of the brainstem ? Structural : Chiari malformation Basilar invagination Platybasia/ kyphotic CXA Congenital anomalies CMJ Hypoplastic condyles Dynamic : Iatrogenic deformity Craniocervical instability Atlanto-axial instability Chiari malformation I RAYMOND TU MD WASHINGTON IMAGING ASSOCIATES Henderson, FC: Autonomic Nervous System, Dysautonomia and its Relationship to Cranio- cervical Instability 16 EDS Global Learning Conference (Nashville) July 30-August 1, 2019 The Complex Chiari and the importance of brainstem deformity • retrospective study 106 Chiari pts • 19 Complex clival-cervical angle <125 degrees • basilar invagination Doug Brockmeyer
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