Electrocardiographic Evaluation in Patients With Spinal Muscular Atrophy: A Case-Control Study

Background: This study aimed to show the impairment of autonomic cardiac conduction causing bradycardia and/or electrocardiographic alterations in children affected by spinal muscular atrophy type 1 and 2 (SMA 1 and 2). Methods: We included 25 spinal muscular atrophy patients, admitted from November 2016 to May 2017. All patients underwent an electrocardiographic examination and we studied PR and QRS intervals, P-waves and QRS amplitudes, and heart rate in spinal muscular atrophy patients compared to a control group. Results: In all patients, we found longer PRi and QRSi ( P < .05), lower P-wave and QRS complex amplitudes ( P < .01), and a decreased heart rate ( P < .01) with respect to controls. When we divided our patients into SMA1 and SMA2 subgroups, we found that statistical differences were maintained for P-wave and QRS complex amplitudes and heart rate, but not for PRi and QRSi with respect to controls. Conclusion: We suggest the hypothesis of SMN expression on cardiac tissue condition and/or autonomic cardiac conduction.

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Combination Therapy with Nusinersen and Onasemnogene Abeparvovec-xioi in Spinal Muscular Atrophy Type I

Journal of Clinical Medicine ◽

10.3390/jcm10235540 ◽

2021 ◽

Vol 10 (23) ◽

pp. 5540

Author(s):

Andrada Mirea ◽

Elena-Silvia Shelby ◽

Mihaela Axente ◽

Mihaela Badina ◽

Liliana Padure ◽

...

Keyword(s):

Combination Therapy ◽

Spinal Muscular Atrophy ◽

Motor Function ◽

Muscular Atrophy ◽

Combined Therapy ◽

Genetic Defect ◽

Survival Motor Neuron ◽

Control Group ◽

Type I ◽

Spinal Muscular Atrophy Type

Background: Spinal muscular atrophy (SMA) is a neuromuscular progressive disease, characterized by decreased amounts of survival motor neuron (SMN) protein, due to an autosomal recessive genetic defect. Despite recent research, there is still no cure. Nusinersen, an antisense oligonucleotide acting on the SMN2 gene, is intrathecally administered all life long, while onasemnogene abeparvovec-xioi, a gene therapy, is administered intravenously only once. Both therapies have proven efficacy, with best outcomes obtained when administered presymptomatically. In recent years, disease-modifying therapies such as nusinersen and onasemnogene abeparvovec-xioi have changed the natural history of SMA. Methods: We observed seven SMA type I patients, who received both therapies. We compared their motor function trajectories, ventilation hours and cough assist sessions to a control group of patients who received one therapy, in order to investigate whether combination therapy may be more effective than a single intervention alone. Results: Patients who received both therapies, compared to the monotherapy cohort, had the same motor function trajectory. Moreover, it was observed that the evolution of motor function was better in the 6 months following the first therapy than in the first 6 months after adding the second treatment. Conclusions: Our results suggest that early treatment is more important than combined therapy.

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Gabungan Ekstrak Rimpang Temulawak, Daun Tanjung, dan Daun Belimbing Manis Berdasarkan Electrocardiogram Berpotensi Sebagai Antiaritmia pada Kucing

jurnal veteriner ◽

10.19087/jveteriner.2019.20.3.409 ◽

2019 ◽

Vol 20 (3) ◽

pp. 409

Author(s):

Min Rahminiwati ◽

Widia Safitri ◽

Deni Noviana

Keyword(s):

Heart Rate ◽

Cardiac Activity ◽

P Wave ◽

Control Group ◽

Qrs Complex ◽

Averrhoa Carambola ◽

Effect Mechanism ◽

Atrial Contraction ◽

Mimusops Elengi ◽

Contraction And Relaxation

Mimusops elengi L. , Averrhoa carambola L. and Curcuma xanthorrihiza Roxb singly were reported to have hypothensif effect. Mechanism underlying decrease of blood pressure was suggested through modification of cardiac activity. The Effect of Mimusops elengi L., combined with Averrhoa carambola L. and Curcuma xanthorrihiza Roxb extract on cardiac activity were studied using ECG on 12 male cats that were grouped to be a control group administered aquadestilata, and treatment group administered extract of 21 mg and 82 mg/2 kg bw orally respectively. The extracts were given 3 h prior to ECG. The results showed a decrease in P wave, QRS complex and the speed of the heart rate after administration of combination of extract . However QT and PR intervals were increased. This showed that the extract can weaken a contraction of the Atria and ventricles, prolonging the onset of the occurrence of atrial contraction towards ventricular contraction, extending the onset of contraction and relaxation of the ventricles and cause a decrease in heart rate. Based the cardiogram, It was concluded that their combination is valuable for treatment of aritmia.

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