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20 Cards in this Set
- Front
- Back
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What are the three myotonias said to result in predisposition to MH?
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1) Central Core dz
2) Evan's myopathy 3) King-Denborough syndrome |
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What is the inheritance patter of central core disease?
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autosomal dominant
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What are the characteristics of central core disease?
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hypotonia (e.g., floppy baby syndrome), delayed motor development, proximal weakness, baseline increased CK
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What is the title of Azar's 1984 paper?
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The Response of Patients with neuromuscular disease to muscle relaxants.
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What year did Azar write The Response of Patient's with neuromuscular disease to muscle relaxants?
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1984
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How did Azar characterize the difference between masseter spasm in myotonia versus that in MH?
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He said myotonia rigidity is short-lived (lasting no longer than the duration of sux) whereas in MH it lasts longer than the duration of sux.
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What is myotonia congenita?
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an inherited disorder of skeletal muscle excitability
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What is the gene affected in myotonia congenita?
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muscle chloride channel gene (CLCN1)
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What are the characteristics of myotonia congenita?
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muscular hypertrophy due to prolonged involuntary activation
- muscular stiffness is a common complaint - warm-up phenomenon (ameliorates with continuous activity) - |
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How does impairment of the chloride channel in myotonia congenita cause myotonia?
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impaired influx of chloride increases excitability of the muscle fiber plasma membrane causing prolonged contraction.
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What is myotonia?
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Myotonia is prolonged muscular contraction
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What does the use of succinylcholine in someone myotonia congenita look like?
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Generalized muscle spasm: masseter, chest, abdomen, extremities, arching of the cervical and lumbar spines.
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What authors wrote about sux given to a patient with undiagnosed myotonia congenita?
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Farbu et al from Haukeland University in Norway in 2003
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What were thepost-sux CK levels of the undiagnosed myotonia congenita patient Farbu et al wrote about?
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CK 1645 - 1126
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What were thepost-sux myoglobin levels of the undiagnosed myotonia congenita patient Farbu et al wrote about?
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443-190 U/liter
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What did Larach's 1987 Anesthesiology paper address?
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Prediction of MH susceptibility with clinical signs
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What did Larach's 1987 paper say about masseter spasm association with MH?
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48% of all patients with masseter rigidity were MH (+)
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What did Larach's 1987 paper say about masseter spasm and CK?
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Of 5 patients with masseter spasm and post-op CK > 20,000: 4 were MH+ by caffeine-halothane standards and 1 was Becker's myotonia
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What is Becker's myotonia?
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an autosomal recessive form of myotonia congenita
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When does Becker's disease present?
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Becker's presents sometime during the 2nd decade of life, often insidiously.
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