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Infraclavicular Catheter in MELAS Syndrome for Analgesic Purposes
M Onay, T Tanyel Kiremitçi, G Erdoğan Kayhan, D İlhan Algın, MS GüleçNeurology India 2023 71(4):764-766 MELAS syndrome is defined as mitochondrial myopathy accompanied by encephalopathy, lactic acidosis, myoclonus, stroke-like episodes. It has a progressive course, multi-systemic effects and severe complications. Myoclonic contractions are unresponsive to many anti-epileptic drugs; these contractions and spasms may lead to severe pain. Systemic analgesic drugs are not sufficient to control pain. Therefore, continuous brachial plexus blockage may be pr...
Source: Neurology India - August 18, 2023 Category: Neurology Authors: M Onay T Tanyel Kiremit & #231;i G Erdo & #287;an Kayhan D & #304;lhan Alg & #305;n MS G & #252;le & #231; Source Type: research

What is Ondine ’ s Curse?
Discussion Congenital central hypoventilation syndrome (CCHS) is an autosomal dominant with variable inheritance genetic disease caused by mutation in the Paired Like Homeobox B2 (PHOX2B) gene on chromosome 4. There are two other genes which may also cause CCHS. CCHS affects the chemoreceptor afferent ventilation pathways and is a neural crest migration problem of the autonomic nervous system. The incidence is unknown but a prevalence of 1 in 200,000 live births has been reported. Obviously it is a rarer phenomenon because of the general lethality of the syndrome. Patients usually present at birth or soon afterwards, but o...
Source: PediatricEducation.org - August 15, 2022 Category: Pediatrics Authors: Pediatric Education Tags: Uncategorized Source Type: news

Successful catheter ablation of a  left posterolateral accessory bypass tract and periinterventional management in a patient with MELAS syndrome
AbstractMELAS syndrome is defined as a  combination of mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes resulting from mutations in mitochondrial DNA. All medical interventions in these patients appear challenging due to a high risk of lactate acidosis or anesthesiological complications. Of note, previou s reports suggest that these patients have a higher incidence of Wolff-Parkinson-White (WPW) syndrome. Here, a case of successful catheter ablation of a posteroseptal bypass tract using analgosedation in a patient with MELAS syndrome combined with WPW syndrome is presented.
Source: Herzschrittmachertherapie und Elektrophysiologie - July 8, 2022 Category: Cardiology Source Type: research

Anesthetic considerations for renal transplant surgery in patients with mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes syndrome: a case report
Mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes syndrome is a progressive syndrome with variable involvement of multiple-organ systems. These patients require special consideration for preoperative optimization, intraoperative management, and postoperative care. The medical literature regarding perioperative management of these patients relies heavily on case reports. Here we present a novel experience providing care for a patient with mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes syndrome who underwent renal transplantation for focal segmental glomerulo...
Source: Journal of Clinical Anesthesia - June 2, 2016 Category: Anesthesiology Authors: Michelle L. Humeidan, Julia Dalia, Wanye D. Traetow Tags: Case report Source Type: research

Characteristics of anesthesia in patients with MELAS syndrome : Case report of anesthesia in video-assisted thoracoscopy.
Abstract The mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes (MELAS) syndrome is a disease triggered by a disorder in energy production within mitochondria. The cause of this syndrome is a mutation in the mitochondrial DNA where in 80 % of cases an A-to-G mutation is present at nucleotide 3243 and with a prevalence of 18.4/100,000 in the population. Predominantly affected are organ systems with a high energy metabolism, such as the heart, brain and musculature. During the premedication visit a thorough patient history and examination with respect to neurological impairments must ...
Source: Der Anaesthesist - August 28, 2015 Category: Anesthesiology Authors: Haas A, Wappler F Tags: Anaesthesist Source Type: research