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Pathological findings in 165 patients explored for malignant hyperthermia susceptibility
D Figarella-Branger1, G Kozak-Ribbens, L Rodet
1Laboratoire de Biopathologie Nerveuse et Musculaire, Faculté de Medecine Timone, Marseille, France.
Neuromuscular Disorders : NMD
|September 1, 1993
Summary
Malignant hyperthermia (MH) susceptibility was investigated in patients with anesthesia complications and exertional heat stroke. Pathological findings were diverse, with no specific MH or heat stroke myopathy identified, suggesting broader patient screening is needed.
Area of Science:
- Neurology
- Genetics
- Pathology
Background:
- Malignant hyperthermia (MH) is a severe pharmacogenetic disorder of skeletal muscle.
- Exertional heat stroke (EHS) shares some clinical similarities with MH.
- Understanding the underlying myopathies in MH-susceptible (MHS) and EHS patients is crucial.
Purpose of the Study:
- To investigate pathological findings in patients with suspected malignant hyperthermia (MH) susceptibility.
- To determine if specific myopathies are associated with MH or exertional heat stroke (EHS).
- To evaluate the need for systematic screening in EHS patients.
Main Methods:
- In vitro contracture testing using halothane and caffeine on muscle biopsies.
- Analysis of pathological findings in 165 patients (120 MH-suspected, 45 EHS).
- Correlation of myopathy types with MH susceptibility and EHS.
Main Results:
- 25 MHS, 5 MHC, 3 MHH, and 87 MHN subjects identified in the MH-suspected group.
- 11 MHS, 6 MHC, 2 MHH, and 26 MHN subjects identified in the EHS group.
- Pathological findings were heterogeneous; central core myopathy was the only condition significantly associated with MH.
Conclusions:
- This study found no specific myopathy for malignant hyperthermia or exertional heat stroke.
- MHS subjects were identified within the EHS group, indicating a need for systematic screening.
- Pathological findings in MH and EHS patients are diverse, not indicative of a single myopathic disease.