Muscle fiber conduction velocity in the diagnosis of sporadic hypokalemic periodic paralysis.
A 6-year-old girl presented with episodes of profound muscle weakness since the age of 2 years. On the basis of decreased ictal serum potassium level and lack of metabolic disorder, primary hypokalemic periodic paralysis (HPP) was diagnosed. Both parents and 3 sibs were unaffected clinically. In all of them asymptomatic heterozygosity was very unlikely by the finding of normal muscle fiber conduction velocities, whereas in the patient interictal muscle fiber conduction velocity was lowered. Determination of muscle fiber conduction velocity can be helpful in documenting sporadic occurrence of HPP.
Brouwer
O F
OF
Department of Neurology, University Hospital Leiden, The Netherlands.
Zwarts
M J
MJ
Links
T P
TP
Wintzen
A R
AR
D002648:Child; D004576:Electromyography / Q000295:instrumentation; D005260:Female; D006801:Humans; D007008:Hypokalemia / Q000175:diagnosis* / Q000235:genetics / Q000503:physiopathology; D007537:Isometric Contraction / Q000502:physiology; D009132:Muscles / Q000503:physiopathology*; D010245:Paralyses, Familial Periodic / Q000175:diagnosis* / Q000235:genetics / Q000503:physiopathology; D012815:Signal Processing, Computer-Assisted / Q000295:instrumentation