Etiology and treatment of amyotrophic lateral sclerosis.

BACKGROUND: To date all researchers conclude that the etiology of Amyotrophic lateral sclerosis (ALS) is not known. On the contrary, since August 2009, we believe that disease is of ischemic origin in the anterior surface of the medulla oblongata. MATERIAL AND METHOD: We present our surgical experience into 45 patients with ALS (bulbar form in 36 cases and spinal form in 9). Preoperative MRI scans revealed microinfarcts in the medulla oblongata and/or cervical cord. During surgery we found: 1) poor quality of omentum in most cases; 2) degenerative changes in the cervical spine; 3) anatomical anomalies at the V4 segments of the vertebral arteries; 4) moderate to severe atherosclerosis at both V4 segments; 5) unilateral absence or stenosis in the anterior-ventral spinal arteries (AVSAs). All patients received omentum on the anterior, lateral and posterior surface of the medulla oblongata, and in 9 cases, an additional segment at the C5-C6 level. RESULTS: Neurological improvement was better during the first days or weeks after surgery than in the following months or years, in all patients. However, 13 patients suffered neurological impairment in about 4 months later, due to greater deterioration of the cervical spine, by contrast, 7 patients with mild ALS have experienced neurological improvement by 80 to 100% during a follow-up of 4 and 6 years. CONCLUSIONS: These results confirm that ALS is of ischemic origin in the intraparenchymal territory of the AVSAs and/or in anterior spinal artery caused by atherosclerosis and associated to anatomical variants in the V4 segments of the vertebral arteries. Because in contrast to this, its revascularization by means of omentum can cure (mild degree) or improve this disease.

Esclerosis Lateral Amiotrófica. Experiencia quirúrgica en 13 pacientes / Amyotrophic Lateral Sclerosis. Surgical experience in 13 patients

Introducción: En agosto del 2009 operamos el primer paciente con esclerosis lateral amiotrófica (ELA). Sin embargo, hasta la fecha, todo los investigadores informan que no hay cura para esta enfermedad. Objetivo: Demostrar que la ELA puede ser detenida y mejorada mediante un transplante de epiplón. Material y Método: Presentamos a 13 pacientes con formas bulbar y espinal de ELA. Durante la cirugía encontramos: 1) variantes anatómicas del segmento V4 de las arterias vertebrales, 2) aterosclerosis moderada o severa en ambos segmentos V4, 3) algunas arterias circunflejas originadas desde las arterias espinales anteroventrales (AEAVs) exsangües, 4) hipotrofia de raicillas nerviosas en la hilera de los nervios IX, X y XI, 5) en algunos casos, hipotrofia de la superficie anterior de las pirámides e 6) hipotrofia de raíces anteriores en C5 - C6.Todos ellos recibieron transplante de epiplón a la superficie anterior, lateral y posterior de la medula oblongada y en 5 pacientes, un transplante adicional a nivel C5-C6. Resultados: La mejoría neurológica fue observada desde el primer día de la operación y fue mayor durante los primeros días o semanas de la cirugía que en los siguientes meses. Actualmente, 2 pacientes con 8 y 12 meses de evolución postoperatoria han mejorado en un 90% los síntomas de la forma bulbar de ELA. Conclusión: Estos resultados indican que la forma bulbar de ELA es originada por isquemia progresiva en el territorio intraparenquimatoso de las AEAVs y la forma espinal por isquemia en la arteria espinal anterior, pero a nivel C5 a T1. Eso explicaría porque su revascularización por medio del epiplón produjo mejoría neurológica.

Introduction: In August 2009 we performed surgery for the first time in a patient with amyotrophic lateral sclerosis (ALE). However, every published report about ALE mentions there is no cure for this condition. Objective: To prove that the progression of ALE may be stopped and the condition may be improved using an epiplon transplant. Material and Method: This report presents our experience with 13 ALE patients, affected by the bulbar and spinal forms of the disease. During the surgical procedures, we made the following findings: 1) anatomical variants in the V4 segment of vertebral arteries, 2) moderate or severe atherosclerosis in both V4 segments, 3) some circumflex blood vessels originating from the low-flow anterior and ventral spinal arteries, 4) hypothrophy of small nerve roots in the pathway of IX, X, and XI cranial nerves, 5) in some cases, hypothrophy of the anterior surface of the pyramids, and 6) hypothrophy of the anterior roots in C5 - C6. Every patient underwent an epiplon transplant upon the anterior, lateral, and posterior aspects of the medulla oblongata, and 5 patients underwent an additional transplant performed upon the C5-C6 territory. Results: Neurological improvement was seen from the first postoperative day, and it reached its maximum level during the first few days or weeks after surgery. Nowadays, 2 patients after eight and twelve months after surgery have had a 90% symptomatic improvement of the bulbar form of ALE. Conclusion: These results indicate that the bulbar form of ALE is caused by progressive ischemia in the parenchymal territory irrigated by the anterior and ventral spinal arteries, and that the spinal form is caused by ischemia in the anterior spinal artery, affecting the area between C5 and T1. This may explain why this revascularization procedure using epiplon tissue led to neurological improvement.