The Myotonias • delayed relaxation of skeletal muscle after voluntary contraction, • myotonic contracture is not relieved by regional, NDMR

, or deep GA. • Relaxation may be induced by infiltration of the muscle with local anesthetic. • caused by genetic abnormalities that produce defects in sodium or chloride channels or proteins which alter ion channel function in muscle cells • giving drugs that ↓ influx into the cell and delay return of membrane Na excitability,like quinine, tocainide, or mexilitene, may relax myotonic contracture. Myotonic Dystrophy (Steinert's Disease) • is the most common form of the myotonias and the most severe. • AD inherited disorder (1 in 8000), S/S during the 2nd and 3rd decades of life. • clinical features associated with myotonic dystrophy: muscle degeneration, cataracts, premature balding, DM, thyroid dysfunction, adrenal insufficiency, gonadal atrophy, cardiac conduction abnormalities. • Cardiac abnormalities→ LV diastolic dysfunction, cardiac failure is rare. o AV block , A.fib, or flutter, ventricular dysrhythmias. o 1st -degree AV block may actually precede the onset of clinical symptoms

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o Sudden death may be a result of the abrupt onset of 3rd -degree AV block. o MVP occurs in 20%, but systemic complication are rare. Pulmonary: Also see below o Myotonia of respiratory muscles can produce intense dyspnea, requiring therapy with procainamide. GI→gastric atony and intestinal hypomotility, Pharyngeal muscle weakness in conjunction with delayed gastric emptying increases the risk for aspiration Pregnancy: o often produces an exacerbation of myotonic dystrophy. o It has been suggested that increased progesterone levels of pregnancy contribute to increased symptoms. o Congestive heart failure is also more likely to occur during pregnancy. o LSCS must often be performed due to uterine smooth muscle dysfunction. Possible risk of MH. Therapy: o mostly palliative and includes respiratory muscle training and the administration of androgenic steroids.

o Patients with cardiac dysrhythmias may require pacemaker implantation, cardioversion, or catheter ablation of ventricular reentry pathways. Congential myotonic • is an early-onset form of myotonic dystrophy. • Some patients have very mild symptoms such as a swallowing dysfunction as a result of the inability to relax the oropharyngeal muscles. • improves with age and does not affect a patient's life expectancy. • Some infants, however, have an early-onset form characterized by hypotonia, respiratory distress, and aspiration pneumonia. Mechanical ventilation is often required and mortality during infancy is high. Paramyotonia • is the rarest of the myotonic diseases. • Myotonic contracture develops when the patient is exposed to cold, and warming relaxes the contracted muscle. • Hypokalemia produce skeletal muscle weakness in patients with paramyotonia. • Possible related to hyperkalemic form of Familial periodic paralysis Management of Anesthesia A→ usually ok, Risk of aspiration

B→ restrictive lung disease, hypoxemia, ↓ ventilatory responses to hypoxia and hypercapnia. o Weakness of respiratory muscles diminishes the effectiveness of cough and may lead to pneumonia. o Sensitive to respiratory depression effect of opioids, and BNZ. C→ conduction abnormalities → dysrhythmias, MVP, Avoid drugs that cause delay conduction in the His-Purkinje system, specifically halothane. D→Sux→ exaggerated contracture (Avoid)→ can make intubation difficult, and ventilation, the response to NDMR is unpredictable, Neo may induce myotonia. • The response to nerve stimulator must be carefully interpreted because muscle stimulation may produce myotonia, which can be misinterpreted as sustained tetanus when significant neuromuscular blockade still exists (personal observation). • pregnancy o exacerbate skeletal muscle weakness and myotonia. o Labor is typically prolonged, o ↑ incidence of postpartum hemorrhage from placenta accreta. • Consider worm the OR→ which may ↓ the severity of the myotonia.

Avoid post-op shivering which may precipitate contraction of skeletal muscle.