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Sympathovagal imbalance in patient with exercise syncope and Brugada syndrome - case report
Abstracts / Autonomic Neuroscience: Basic and Clinical 192 (2015) 56–141
P21.10 Sympathovagal imbalance in patient with exercise syncope and Brugada syndrome - case report J. Galuszka, K. Vykoupil, M. Taborsky, I. Buriankova Department of Internal Medicine I - Cardiology, University Hospital and Faculty of Medicine and Dentistry Palacký University in Olomouc, Czech Republic Background: Brugada syndrome is rare disorder from the group of socalled channelopathies with autosomal dominant inheritance associated with high risk of malignant arrhythmias and sudden cardiac death, more common in men. The arrhythmias are based on defective myocardial sodium channels causing typical ECG patterns. Autonomic nervous system as well as variety of different factors may participate on triggering arrhythmias. Aim: Sympatovagal balance assessment in 23 year old men without structural heart disease with history of exercise induced syncope and Brugada type I ECG pattern on exercise (normal rest ECG). Methods: Heart rate variability evaluation during supine- standing- supine test (5 minutes in each position). Results: Standing / Supine LF (nu): 93 / 56, HF (nu): 7 /44, LF / HF ratio: 14,1362 / 1,2861. Conclusion: It is possible to suppose that excessive sympathetic tone associated with orthostasis (LF/ HF ratio above 14) can be further potentiated by catecholamines released during exercise. These changes could explain why diagnosis based on typical ECG pattern was unmasked by excercise. The findings suggest participation of autonomic tone imbalance in the pathophysiology of Brugada syndrome. doi:10.1016/j.autneu.2015.07.434
P21.11 Autonomic dysfunction in NMDA receptor encephalitis – Is it central or peripheral in origin? M. Shah, R Bhavaraju-Sanka Department of Neurology, University of Texas Health Science Center at San Antonio, USA Background: Autonomic instability is a common manifestation in anti-NMDAR (N-methyl D-aspartate Receptor) encephalitis, however, exact pathophysiology is poorly understood. Aim: To expose different pathophysiologic mechanisms of autonomic dysfunction in NMDAR encephalitis patients. Methods: Case-reports Results: Case 1: A 23 years old female presented with panic attacks, uncontrolled seizures and orofacial dyskinesias. NMDAR antibodies were elevated and she had a mature ovarian teratoma, which was resected. She progressed to severe encephalopathy with respiratory failure needing intubation. She also exhibited intermittent tachycardia alternating with bradycardia, pauses up to 8 seconds long and significantly elevated blood pressures. MRI brain showed DWI and FLAIR abnormalities in right mesial temporal lobe. She was treated with plasmapheresis, propranolol and clonidine. Case 2: A 22 years old female presented with several months of psychiatric symptoms, hyperhidrosis, postural tachycardia and dizziness. Extensive work up revealed elevated NMDAR antibodies with normal MRI brain. Her symptoms including postural changes improved after plasmapheresis and correlated with reduction in antibody titers. Autonomic testing showed absent sweat production in the leg. Skin biopsy showed reduced intra-epidermal nerve fiber density in her foot suggesting small fiber neuropathy. She also had high titers of Voltage gated potassium channel antibodies. Conclusions: Autonomic instability in both the above cases of NMDAR encephalitis markedly differed in severity and pathophysiology. NMDAR encephalitis is believed to cause dysfunction of central autonomic circuits as in case
135
1, however case 2 showed evidence of post-ganglionic dysfunction. Exact mechanism causing post-ganglionic autonomic dysfunction is unknown and requires further research. doi:10.1016/j.autneu.2015.07.435
P21.12 Clinical, electrophysiologic, and autonomic observations in a 11-year old boy with acute autonomic and sensory ganglionopathy and therapeutic response to noradrenergic enhancement with droxidopa Jose-Alberto Palmaa, Joel Gutierrezb, Cristina Fuente-Moraa, Horacio Kaufmanna a Department of Neurology, New York University School of Medicine, NY University Medical Center, USA b Department of Clinical Neurophysiology, Cuban Institute of Neurology and Neurosurgery, La Habana, Cuba Background: Acute autonomic and sensory ganglionopathy (AASG) is a rare disorder that has been only anecdotally reported. We report the clinical, electrophysiological, autonomic and therapeutic features of one patient with AASG. Methods: Case report of a 11-year-old male presenting with progressive imbalance and severe autonomic and sensory deficits after an upper respiratory tract infection. Results: Autonomic disturbances included vomiting, diarrhea, anhidrosis, dry mouth, dry eyes, orthostatic hypotension and supine hypertension. Neurological examination revealed very reduced or absent reflexes with preserved muscle strength. Cerebrospinal fluid analysis and autoimmune panel were normal. Nerve conduction studies showed extremely reduced/absent sensory nerve action potentials. EMG was normal. Cardiovascular autonomic evaluation during tilt test evidenced decreased heart rate variability (E:I ratio: 1.23), orthostatic hypotension without compensatory tachycardia (111/58 mmHg 109 bpm supine and 71/21 mmHg 123 bpm after 3-min head-up tilt) and very low or undetectable levels of plasma norepinephrine (NE supine was undetectable and increased only to 48 pg/ml standing), suggesting impaired baroreflex-mediated sympathetic activation. Spinal MRI showed extensive T2 hyperintensities in the dorsal aspect of the cervical spinal cord. Autonomic and sensory deficits persisted several months after onset. Administration of droxidopa 100 mg lessened orthostatic intolerance and significantly increased blood pressure after 3-min standing (49/34 mmHg to 111/76 mmHg). Conclusions: The presence of sudden-onset rapidly progressive autonomic and sensory deficits suggests an immune-mediated process. Functional recovery of sensory and autonomic deficits is poor. Treatment with droxidopa seems to be a feasible option to increase blood pressure in these patients. doi:10.1016/j.autneu.2015.07.436
P21.13 Differential effects of Diabetes Mellitus on nerve fibers by type of Diabetes AC Peltiera, I.M. Buteraa, J. Lia,b a Vanderbilt University Medical Center, Nashville, TN b Veterans Administration Medical Center, Tennessee Valley Healthcare System, Nashville, TN Type 1 (T1D) and Type 2 diabetes mellitus (T2D) are often assumed to have similar effects on nerve fibers with a common etiology of hyperglycemia. Mounting evidence suggests that T2D preferentially affects non-myelinated C-fibers compared to myelinated fibers. To
P21.10 Sympathovagal imbalance in patient with exercise syncope and Brugada syndrome - case report J. Galuszka, K. Vykoupil, M. Taborsky, I. Buriankova Department of Internal Medicine I - Cardiology, University Hospital and Faculty of Medicine and Dentistry Palacký University in Olomouc, Czech Republic Background: Brugada syndrome is rare disorder from the group of socalled channelopathies with autosomal dominant inheritance associated with high risk of malignant arrhythmias and sudden cardiac death, more common in men. The arrhythmias are based on defective myocardial sodium channels causing typical ECG patterns. Autonomic nervous system as well as variety of different factors may participate on triggering arrhythmias. Aim: Sympatovagal balance assessment in 23 year old men without structural heart disease with history of exercise induced syncope and Brugada type I ECG pattern on exercise (normal rest ECG). Methods: Heart rate variability evaluation during supine- standing- supine test (5 minutes in each position). Results: Standing / Supine LF (nu): 93 / 56, HF (nu): 7 /44, LF / HF ratio: 14,1362 / 1,2861. Conclusion: It is possible to suppose that excessive sympathetic tone associated with orthostasis (LF/ HF ratio above 14) can be further potentiated by catecholamines released during exercise. These changes could explain why diagnosis based on typical ECG pattern was unmasked by excercise. The findings suggest participation of autonomic tone imbalance in the pathophysiology of Brugada syndrome. doi:10.1016/j.autneu.2015.07.434
P21.11 Autonomic dysfunction in NMDA receptor encephalitis – Is it central or peripheral in origin? M. Shah, R Bhavaraju-Sanka Department of Neurology, University of Texas Health Science Center at San Antonio, USA Background: Autonomic instability is a common manifestation in anti-NMDAR (N-methyl D-aspartate Receptor) encephalitis, however, exact pathophysiology is poorly understood. Aim: To expose different pathophysiologic mechanisms of autonomic dysfunction in NMDAR encephalitis patients. Methods: Case-reports Results: Case 1: A 23 years old female presented with panic attacks, uncontrolled seizures and orofacial dyskinesias. NMDAR antibodies were elevated and she had a mature ovarian teratoma, which was resected. She progressed to severe encephalopathy with respiratory failure needing intubation. She also exhibited intermittent tachycardia alternating with bradycardia, pauses up to 8 seconds long and significantly elevated blood pressures. MRI brain showed DWI and FLAIR abnormalities in right mesial temporal lobe. She was treated with plasmapheresis, propranolol and clonidine. Case 2: A 22 years old female presented with several months of psychiatric symptoms, hyperhidrosis, postural tachycardia and dizziness. Extensive work up revealed elevated NMDAR antibodies with normal MRI brain. Her symptoms including postural changes improved after plasmapheresis and correlated with reduction in antibody titers. Autonomic testing showed absent sweat production in the leg. Skin biopsy showed reduced intra-epidermal nerve fiber density in her foot suggesting small fiber neuropathy. She also had high titers of Voltage gated potassium channel antibodies. Conclusions: Autonomic instability in both the above cases of NMDAR encephalitis markedly differed in severity and pathophysiology. NMDAR encephalitis is believed to cause dysfunction of central autonomic circuits as in case
135
1, however case 2 showed evidence of post-ganglionic dysfunction. Exact mechanism causing post-ganglionic autonomic dysfunction is unknown and requires further research. doi:10.1016/j.autneu.2015.07.435
P21.12 Clinical, electrophysiologic, and autonomic observations in a 11-year old boy with acute autonomic and sensory ganglionopathy and therapeutic response to noradrenergic enhancement with droxidopa Jose-Alberto Palmaa, Joel Gutierrezb, Cristina Fuente-Moraa, Horacio Kaufmanna a Department of Neurology, New York University School of Medicine, NY University Medical Center, USA b Department of Clinical Neurophysiology, Cuban Institute of Neurology and Neurosurgery, La Habana, Cuba Background: Acute autonomic and sensory ganglionopathy (AASG) is a rare disorder that has been only anecdotally reported. We report the clinical, electrophysiological, autonomic and therapeutic features of one patient with AASG. Methods: Case report of a 11-year-old male presenting with progressive imbalance and severe autonomic and sensory deficits after an upper respiratory tract infection. Results: Autonomic disturbances included vomiting, diarrhea, anhidrosis, dry mouth, dry eyes, orthostatic hypotension and supine hypertension. Neurological examination revealed very reduced or absent reflexes with preserved muscle strength. Cerebrospinal fluid analysis and autoimmune panel were normal. Nerve conduction studies showed extremely reduced/absent sensory nerve action potentials. EMG was normal. Cardiovascular autonomic evaluation during tilt test evidenced decreased heart rate variability (E:I ratio: 1.23), orthostatic hypotension without compensatory tachycardia (111/58 mmHg 109 bpm supine and 71/21 mmHg 123 bpm after 3-min head-up tilt) and very low or undetectable levels of plasma norepinephrine (NE supine was undetectable and increased only to 48 pg/ml standing), suggesting impaired baroreflex-mediated sympathetic activation. Spinal MRI showed extensive T2 hyperintensities in the dorsal aspect of the cervical spinal cord. Autonomic and sensory deficits persisted several months after onset. Administration of droxidopa 100 mg lessened orthostatic intolerance and significantly increased blood pressure after 3-min standing (49/34 mmHg to 111/76 mmHg). Conclusions: The presence of sudden-onset rapidly progressive autonomic and sensory deficits suggests an immune-mediated process. Functional recovery of sensory and autonomic deficits is poor. Treatment with droxidopa seems to be a feasible option to increase blood pressure in these patients. doi:10.1016/j.autneu.2015.07.436
P21.13 Differential effects of Diabetes Mellitus on nerve fibers by type of Diabetes AC Peltiera, I.M. Buteraa, J. Lia,b a Vanderbilt University Medical Center, Nashville, TN b Veterans Administration Medical Center, Tennessee Valley Healthcare System, Nashville, TN Type 1 (T1D) and Type 2 diabetes mellitus (T2D) are often assumed to have similar effects on nerve fibers with a common etiology of hyperglycemia. Mounting evidence suggests that T2D preferentially affects non-myelinated C-fibers compared to myelinated fibers. To