McArdle's disease (glycogen storage disease typeV) and anesthesia – a case report and review of the literature

Abstract

McArdles disease (glycogen storage disease type v) is a rare condition in which energy-metabolism in the muscle is hampered. A case report is presented and the possible risk for perioperative complications including malignant hyperthermia is discussed. A checklist for the anesthesiological management of patients with McArdles disease is provided. A short overview of anesthesiological challenges and perioperative complications of other glycogen storage diseases is given.

Enfermedad de McArdle o glucogenosis tipo v: ¿influye en el manejo anestésico?

“…En el periodo postoperatorio debería realizarse un control analítico de electrolitos, CK, LDH, transaminasas y función renal, y considerar vigilancia 24 h en una unidad de monitorización ante el posible riesgo de complicaciones 5 .…”

Malignant Hyperthermia

“…Muscular conditions other than abnormalities of excitation-contraction coupling may be responsible for the adverse reaction to anesthetics experienced by the proband [13,14]. There is continued discussion regarding the MH risk in patients with myopathy or other diseases such as one of the myotonias (see http://www.mhaus.org/healthcare-professionals/be-prepared/associatedconditions).…”

Sports and McArdle Disease (Glycogen Storage Disease Type V): Danger or Therapy?

“…It is known by different synonyms as myophosphorylase insufficiency, glycogen storage disease type V, or myophosphorylase deficiency [1][2][3][4][5]. Table 1 provides an overview of the different types of glycogen storage diseases [6]. McArdle disease is caused by a lack of myophosphorylase (alpha-1,4-glucan orthophosphate glycosyl transferase) that normally initiates muscle glycogen breakdown during exercise by removing 1,4-glycosyl groups from the glycogen molecule leading to the release of glucose-1-phosphate [1][2][3][4][5] clinically appears to be autosomal dominant in some affected families [2][3][4]7].…”

“…Although no case of malignant hyperthermia during anesthesia has been described in McAd so far, it is a potential risk when patients with McAd have to undergo operations with the need for general anesthesia. Therefore, precautions have to be taken by the anesthesiologist, and local or regional anesthesia may be preferred whenever feasible [6,13].…”