scholarly journals Electrophysiological investigation for autonomic dysfunction in patients with myasthenia gravis : A prospective study

2021 ◽  
Vol 74 (1-2) ◽  
pp. 33-40
Author(s):  
Mecbure Nalbantoglu ◽  
Mehmet Ali Akalin ◽  
Aysegul Gunduz ◽  
Meral Kiziltan

Myasthenia gravis (MG) is an autoimmune disorder of neuromuscular transmission. Autonomic dysfunction is not a commonly known association with MG. We conducted this study to evaluate autonomic functions in MG & subgroups and to investigate the effects of acetylcholinesterase inhibitors. This study comprised 30 autoimmune MG patients and 30 healthy volunteers. Autonomic tests including sympathetic skin response (SSR) and R-R interval variation analysis (RRIV) was carried out. The tests were performed two times for patients who were under acetylcholinesterase inhibitors during the current assessment. The RRIV rise during hyperventilation was better (p=0.006) and Valsalva ratio (p=0.039) was lower in control group. The SSR amplitudes were lower thereafter drug intake (p=0.030). As much as time went by after drug administration prolonged SSR latencies were obtained (p=0.043).Valsalva ratio was lower in the AchR antibody negative group (p=0.033). The findings showed that both ocular/generalized MG patients have a subclinical parasympathetic abnormality prominent in the AchR antibody negative group and pyridostigmine has a peripheral sympathetic cholinergic noncumulative effect.

2021 ◽  
Author(s):  
Hanan Galal Azouz ◽  
Ali M Abdel Mohsen ◽  
Rana M Mohamed ◽  
hayam mostafa abdelghany

Abstract Cerebral palsy (CP) is the most prevalent severe motor disability among children. The aim of this work was to assess autonomic dysfunction in children with cerebral palsy clinically and electrophysiologically .The study was carried out on forty children with cerebral palsy their age ranged from 4-12 years and twenty healthy children with matched age and sex as control group. CP children were subjected to questionnaire for autonomic dysfunction symptoms. Both CP children and Control group were assessed for Sympathetic Skin Response and Heart rate variability. Most of children had quadriplegic spastic cerebral palsy (82.5%). Based on Gross motor function classification system (GMFCS) classification the majority of children were in levels 4 and 5. The prevalence of autonomic dysfunction symptoms was 80% for thermoregulatory abnormalities (cold extremities), chronic constipation 65%, sleep disturbance 52.5%, loss of appetite 47.5%, sweating abnormalities 40% , recurrent nausea and/or vomiting 25%, increased sensitivity to light or dark 22.5% and bloating 15%. The percentage of unelicited Sympathetic skin response in CP children was 47.5% and 60% in upper limbs and lower limbs respectively, all of them were in level 4 and 5 of GMFCS. 20% of CP children had postural hypotension. Mean Heart rate of CP children was significantly increased more than healthy children upon head tilt test. Sympathetic Skin Response and Heart rate variability were proven to be simple and non invasive procedures in investigating autonomic dysfunction in CP children.


2021 ◽  
Vol 26 (4) ◽  
pp. 693-698
Author(s):  
Hüseyin Sicim ◽  
Özgür Boyraz ◽  
Ertan Demirdas ◽  
Hakan Kartal ◽  
Gökhan Erol ◽  
...  

Background: In this study, we aimed to investigate the autonomic dysfunction in patients with primary Raynaud’s phenomenon with using sympathetic skin response (SSR) as a neurophysiologic test, R-R interval variation analysis and composite autonomic symptom score (COMPASS)-31 questionnaire. Methods: Palmar SSR to median nerve electrical stimulation was recorded in 38 patients with 36 healthy age and sex-matched control subjects. The SSR was recorded from the palmar surface of both left and right hands for patients and control groups. The amplitudes and latencies formed as a result of electrical stimulation were calculated and compared between the two groups. Additionally, R-R interval variability was examined during normal breathing, deep breathing, standing up and Valsalva maneuver in both groups. Furthermore, we asked to complete the COMPASS-31 questionnaire, a validated tool to assess symptoms of autonomic dysfunction. And by calculating total COMPASS-31 scores, the relationship between the two groups was investigated. Results: The Raynaud’s phenomenon and control groups were similar in age (37.4 ± 11.6 vs. 34.9 ± 13.0 years), had identical gender ratios and similar body mass index (24.5 ± 6.1 vs. 25.7 ± 4.6%). Palmar SSR to median nerve stimulation of RP patients shows significantly delayed latency (1890 ± 146) (p=0.03). And no difference between amplitudes in comparison to the control group. In the patient and control groups, R-R interval measurements were evaluated during rest and deep breathing, standing up and Valsalva maneuver. When the R-R interval measurements of the patient and control groups at rest and deep breathing were compared, there was no statistically significant difference between the groups. In addition, COMPASS-31 questionnaire scoring system was applied to both groups. The mean COMPASS-31 score was higher in patient group (22.8 ± 13.8), than from healthy controls (8.9 ± 7.8) (p=0.02) Conclusions: Autonomic dysfunction plays a role in the etiology of Raynaud’s phenomenon, due to latency prolongation in the sympathetic skin response and significant difference between COMPASS-31 tests, and these tests can be used in the diagnosis stage of this disease.


Author(s):  
Mohammad R. Emad ◽  
Hamid R. Farpour ◽  
Faisal Ahmed ◽  
Masoumeh Tayebi ◽  
Mohammadali Nazarinia ◽  
...  

Objectives: Sympathetic skin response (SSR) is a technique for assessment of the damage of peripheral neuropathies and the disorders of the sympathetic system. This study aimed to evaluate SSR among patients with Raynaud phenomenon (RP). Methods: Between January 1, 2015 and December 30, 2018, about 20 patients with RP and 20 healthy subjects as the control group were recruited from patients referring to the Outpatient Clinics of Shiraz medical University. All the participants were clinically examined, and the SSR was performed using a standard protocol. SSR is abnormal when the latency is prolonged and/or the amplitude reduced. Results: Raynaud's group consisted of 19 women (95 %) and 1 male (5%). 3 patients (15 %) with primary Raynaud's phenomenon (PRP) and 17 patients (85%) with secondary Raynaud's phenomenon (SRP). The control group consisted of 16 women (80%) and 4 males (20%). The mean age of the Raynaud's group and control subjects was 43.1±9 and 36.7±8.6 years, respectively. The SSR to the electrical stimulus was absent in 3 patients (PRP patients). The total median nerve mean latencies in the upper limb were 1.9±0.57 and 1.19±0.52 seconds for the Raynaud's group and control groups, respectively (p <0.001). These findings revealed significantly prolonged SSR latencies in the Raynaud's group, while the mean amplitude showed no significant differences in both groups (p =0.756). Conclusion: Absence or prolonged latency of SSR was associated with the disorders of the unmyelinated axons in the sympathetic system. Our findings suggested the disorders of unmyelinated axons in Raynaud's phenomenon. Keywords: Raynaud Disease; Autonomic Nervous System; Electrodiagnosis; Sympathetic Fibers; Nerve Conduction.


2021 ◽  
Vol 48 (1) ◽  
Author(s):  
Hanan G. Azouz ◽  
Ali M. AbdelMohsen ◽  
Hayam M. Abdel Ghany ◽  
Rana M. Mohamed

Abstract Background Cerebral palsy (CP) is the most prevalent severe motor disability among children. The aim of this work was to assess autonomic dysfunction in children with cerebral palsy clinically and electrophysiologically. Results Age of the studied children ranged from 4 to 12 years. Quadriplegic type of spastic cerebral palsy constituted 82.5% of CP children while diplegic type constituted 17.5%. Based on Gross Motor Function Classification System (GMFCS), the majority of children were in levels 4 and 5. The prevalence of autonomic dysfunction symptoms were 80% for thermoregulatory abnormalities (cold extremities), 65% for chronic constipation, 52.5% for sleep disturbance, 47.5% for loss of appetite, 40% for sweating abnormalities, 25% for recurrent nausea and/or vomiting, 22.5% for increased sensitivity to light or dark and 15% for bloating. As regards sympathetic skin response, 19 CP children had unobtainable response in both upper and lower limbs while 5 children had unobtainable response in lower limbs only. All of them were in levels 4 and 5 of GMFCS. Postural hypotension was present in 20% of CP children. Mean Heart rate of CP children was significantly increased more than healthy children upon head tilt test. Conclusions Autonomic dysfunction has been objectively proven in CP children through absent sympathetic skin response, presence of orthostatic tachycardia and postural hypotension.


2005 ◽  
Vol 12 (11) ◽  
pp. 858-861 ◽  
Author(s):  
A. A. Argyriou ◽  
A. Koutras ◽  
P. Polychronopoulos ◽  
S. Papapetropoulos ◽  
G. Iconomou ◽  
...  

2018 ◽  
Vol 79 (3-4) ◽  
pp. 113-117 ◽  
Author(s):  
Florencia Aguirre ◽  
Andres M. Villa

Background: We investigated the prognosis of patients with myasthenia gravis (MG) initially presenting with only ocular symptoms in an Argentinian population. Summary: We performed a retrospective analysis of 61 patients with MG with pure ocular involvement at onset. Generalized MG (gMG) developed in 73.7% of patients, while the rest only exhibited ocular symptoms throughout the course of the disease (ocular MG [oMG]). The AChR antibody (AChR-ab) was found in 81.4% of the gMG and 50% of the oMG cases. AChR-ab titers were also significantly higher in the gMG than in the oMG group. Patients with oMG more frequently required treatment only with acetylcholinesterase inhibitors and showed more remissions. Key Messages: Our study showed high generalization in patients with MG debuting with ocular symptoms. Patients with AChR seropositivity and higher AChR-ab titers had an increased risk for gMG, while patients with oMG showed lower corticosteroid requirements and a higher remission rate.


Sign in / Sign up

Export Citation Format

Share Document