[Tendon areflexia in congenital myopathies accompanied by atrophy of type I fibers. Electrophysiologic study].
A predominance and/or an atrophy of type I fibers and a loss of deep tendon reflexes are often observed in different types of congenital myopathy. Various data indicate that both findings can be linked: dysfunction of the myotatic reflex can induce predominant involvement of type I fibers. In order to specify the mechanism of the loss of tendon reflex, an investigation of the Hoffmann reflex (H reflex) was performed in one case of centronuclear myopathy and in one case of congenital type fiber disproportion with type I hypertrophy. The Achilles tendon reflex was absent but the H reflex showed normal recruitment amplitude and latency. The Jendrassik maneuver reinforced the H reflex. These results indicate the involvement of muscle spindles or impairment of the fusimotor system. Nuclear bag intrafusal fibers have common characteristics with type I extrafusal fibers. Both types of fibers could be involved simultaneously in congenital myopathies, thus explaining the loss of tendon reflex.
D002648:Child; D004576:Electromyography; D005260:Female; D006801:Humans; D008297:Male; D009047:Motor Neurons, Gamma / Q000502:physiology; D009132:Muscles / Q000473:pathology*; D009135:Muscular Diseases / Q000151:congenital* / Q000473:pathology; D011999:Recruitment, Neurophysiological; D012021:Reflex, Abnormal / Q000150:complications*; D012023:Reflex, Monosynaptic; D012026:Reflex, Stretch; D013577:Syndrome
Aréflexie tendineuse au cours des myopathies congénitales comportant une atrophie des fibres de type I. Etude électrophysiologique.