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 ↓Na influx into the cell and delay return of membrane
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
 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.