We report two siblings who presented with non- progressive marked sensory ataxia associated with arthrogryposis multiplex congenita (AMC). Deep tendon reflexes and H reflex were completely absent, but F waves were pre...We report two siblings who presented with non- progressive marked sensory ataxia associated with arthrogryposis multiplex congenita (AMC). Deep tendon reflexes and H reflex were completely absent, but F waves were preserved. The sensory nerve conduction studies indicated the presence of relatively mild sensory polyneuropathy. The conventional somatosensory evoked potentials (SEPs) showed mildly prolonged latency for both the peripheral and cortical responses, suggesting a slowed conduction through the peripheral as well as central pathway. However, the ‘ proprioceptive SEPs’ were absent, in conformity with complete loss of joint sense. Sural nerve biopsy revealed only mild thinning of myelin in the younger sister but was entirely normal in her brother. Taken together with the characteristic electrophysiological findings, the symptoms were considered to be due to predominant involvement of a selective population of somatosensory ganglions. The present cases showed no progression of the neurological deficit what- so- ever since birth, which strongly suggests a developmental anomaly or aplasia of a limited population of peripheral sensory neurons.展开更多
Reported are three patients with ictal monoparesis of an arm. In the hemisphere contralateral to the monoparesis, ictal and interictal epilepti-form discharg es were observed in the centroparietal area, and a well-cir...Reported are three patients with ictal monoparesis of an arm. In the hemisphere contralateral to the monoparesis, ictal and interictal epilepti-form discharg es were observed in the centroparietal area, and a well-circumscribed lesion wa s commonly present in the primary arm somatosensory area (SI). In the presence o f an SI lesion, the epileptic activity at the sensorimotor area could lead to se lective or predominant activation of the inhibitory motor system.展开更多
文摘We report two siblings who presented with non- progressive marked sensory ataxia associated with arthrogryposis multiplex congenita (AMC). Deep tendon reflexes and H reflex were completely absent, but F waves were preserved. The sensory nerve conduction studies indicated the presence of relatively mild sensory polyneuropathy. The conventional somatosensory evoked potentials (SEPs) showed mildly prolonged latency for both the peripheral and cortical responses, suggesting a slowed conduction through the peripheral as well as central pathway. However, the ‘ proprioceptive SEPs’ were absent, in conformity with complete loss of joint sense. Sural nerve biopsy revealed only mild thinning of myelin in the younger sister but was entirely normal in her brother. Taken together with the characteristic electrophysiological findings, the symptoms were considered to be due to predominant involvement of a selective population of somatosensory ganglions. The present cases showed no progression of the neurological deficit what- so- ever since birth, which strongly suggests a developmental anomaly or aplasia of a limited population of peripheral sensory neurons.
文摘Reported are three patients with ictal monoparesis of an arm. In the hemisphere contralateral to the monoparesis, ictal and interictal epilepti-form discharg es were observed in the centroparietal area, and a well-circumscribed lesion wa s commonly present in the primary arm somatosensory area (SI). In the presence o f an SI lesion, the epileptic activity at the sensorimotor area could lead to se lective or predominant activation of the inhibitory motor system.
