Anaesthesia for Charcot-Marie-Tooth disease: a review of 86 cases.

Charcot–Marie–Tooth disease: Genetics, epidemiology and complications

Background and aims: Charcot Marie Tooth disease (CMT) is the most prevalent hereditary neuropathy and its frequency is 1 in 2500. CMT is a heterogeneous disease and has different clinical symptoms. The prevalence of CMT and involved genes differ in different countries. CMT patients experience considerable sleep problems and a higher risk of decreased quality of life. In this w...

Whole Exome Sequencing Revealed a Novel GJB1 Pathogenic Variant and a Rare BSCL2 Mutation in Two Iranian Large Pedigrees with Multiple Affected Cases of Charcot-Marie-Tooth

Charcot-Marie-Tooth disease (CMT) is the most common hereditary neuropathy of the peripheral nervous system with a wide range of severity and age of onset. CMT patients share similar phenotypes which make it often impossible to identify the disease types based on clinical presentation and electrophysiological studies alone. In recent years, novel genetic diagnostic approaches such as whole exom...

Anaesthetic management of a patient with Charcot-Marie-Tooth disease for staged diaphragmatic plication.

Total Knee Arthroplasty in Severe Unstable Knee: Case-Report and Literature Review

  Multiplanar or global laxity in arthritic knee is rare , most of this patients have neuromuscular disorder (post poliomyelitis , spinal dystrophy) or history of knee trauma. Ligament insufficiency and severe bone loss is significant in this patient. The estimated prevalence for the concurrence of charcot marie-tooth (CMT) with myasthenia gravis (MG) suggests an extremely rare event. We have p...

Prolonged vecuronium neuromuscular blockade associated with Charcot Marie Tooth neuropathy.

Charcot Marie Tooth (CMT) disease comprises a group of disorders characterized by progressive distal muscle weakness and wasting. Review of the anaesthetic literature produced conflicting reports concerning the responses to neuromuscular blocking drugs in these patients. We describe a case in which vecuronium 0.11 mg kg(-1) produced prolonged neuromuscular blockade lasting 115 min in a patient ...