Whereas paralysis is the hallmark for paralytic rabies, the precise pathological basis of paralysis is not known. It is unclear whether weakness results from involvement of anterior horn cells or of motor nerve fibers...Whereas paralysis is the hallmark for paralytic rabies, the precise pathological basis of paralysis is not known. It is unclear whether weakness results from involvement of anterior horn cells or of motor nerve fibers. There is also no conclusive data on the cause of the neuropathic pain which occurs at the bitten region, although it has been presumed to be related to sensory ganglionopathy. In this study, six laboratory-proven rabies patients (three paralytic and three furious) were assessed clinically and electrophysiologically. Our data suggests th at peripheral nerve dysfunction, most likely demyelination, contributes to the weakness in paralytic rabies. In furious rabies, progressive focal denervation, starting at the bitten segment, was evident even in the absence of demonstrable weakness and the electrophysiologic study suggested anterior horn cell dysfunctio n. In two paralytic and one furious rabies patients who had severe paresthesias as a prodrome, electrophysiologic studies suggested dorsal root ganglionopathy. Postmortem studies in two paralytic and one furious rabies patients, who had local neuropathic pain, showed severe dorsal root ganglionitis. Intense inflammation of the spinal nerve roots was observed more in paralytic rabies patients. Inflammation was mainly noted in the spinal cord segment corresponding to the bite in all cases; however, central chromatolysis of the anterior horn cells could be demonstrated only in furious rabies patient. We conclude that differential sites of neural involvement and possibly different neuropathogenetic mechanisms may explain the clinical diversity in human rabies.展开更多
Objective: Polyneuropathy, organomegaly, endocrinopathy,M protein, and skin ch anges (POEMS) syndrome is a rare cause of polyneuropathy. Although the polyneuro pathy component is essential for the diagnosis of the dis...Objective: Polyneuropathy, organomegaly, endocrinopathy,M protein, and skin ch anges (POEMS) syndrome is a rare cause of polyneuropathy. Although the polyneuro pathy component is essential for the diagnosis of the disease, the pattern of as sociated electrodiagnostic abnormalities has not been characterized in detail. T he purpose of this study was to elucidate the features of nerve conduction abnor malities in POEMS syndrome.Methods: We reviewed the medical records and nerve co nduction studies(NCS) of 12 consecutive patients with POEMS.Results: A total of 68 motor and 46 sensory nerves were examined.Compound muscle action potentials(C MAPs) and sensory nerve action potentials(SNAPs) were not elicited in lower limb s more frequently compared with in upper limbs (P < 0.05, in both motor and sens ory nerves), and the CMAP amplitude was more attenuated in lower limbs than in u pper limbs (P < 0.05). Abnormal conduction slowing was frequently observed in mo tor (95%) and sensory (76%) nerves. Distal motor latencies were abnormally pro longed in 75%of the motor nerves,and terminal latency indices were significant - ly higher in patients than in normal controls (P < 0.05). Conclusions: NCS in POEMS syndrome showed characteristic patterns, in which conduction abnormalities were more frequently and severely affected in the lower limbs, and more predomi nantly in the intermediate nerve segments than in the distal portions. Significa nce:The recognition of these characteristic patterns may be helpful for the earl y diagnosis of polyneuropathy in POEMS syndrome.展开更多
文摘Whereas paralysis is the hallmark for paralytic rabies, the precise pathological basis of paralysis is not known. It is unclear whether weakness results from involvement of anterior horn cells or of motor nerve fibers. There is also no conclusive data on the cause of the neuropathic pain which occurs at the bitten region, although it has been presumed to be related to sensory ganglionopathy. In this study, six laboratory-proven rabies patients (three paralytic and three furious) were assessed clinically and electrophysiologically. Our data suggests th at peripheral nerve dysfunction, most likely demyelination, contributes to the weakness in paralytic rabies. In furious rabies, progressive focal denervation, starting at the bitten segment, was evident even in the absence of demonstrable weakness and the electrophysiologic study suggested anterior horn cell dysfunctio n. In two paralytic and one furious rabies patients who had severe paresthesias as a prodrome, electrophysiologic studies suggested dorsal root ganglionopathy. Postmortem studies in two paralytic and one furious rabies patients, who had local neuropathic pain, showed severe dorsal root ganglionitis. Intense inflammation of the spinal nerve roots was observed more in paralytic rabies patients. Inflammation was mainly noted in the spinal cord segment corresponding to the bite in all cases; however, central chromatolysis of the anterior horn cells could be demonstrated only in furious rabies patient. We conclude that differential sites of neural involvement and possibly different neuropathogenetic mechanisms may explain the clinical diversity in human rabies.
文摘Objective: Polyneuropathy, organomegaly, endocrinopathy,M protein, and skin ch anges (POEMS) syndrome is a rare cause of polyneuropathy. Although the polyneuro pathy component is essential for the diagnosis of the disease, the pattern of as sociated electrodiagnostic abnormalities has not been characterized in detail. T he purpose of this study was to elucidate the features of nerve conduction abnor malities in POEMS syndrome.Methods: We reviewed the medical records and nerve co nduction studies(NCS) of 12 consecutive patients with POEMS.Results: A total of 68 motor and 46 sensory nerves were examined.Compound muscle action potentials(C MAPs) and sensory nerve action potentials(SNAPs) were not elicited in lower limb s more frequently compared with in upper limbs (P < 0.05, in both motor and sens ory nerves), and the CMAP amplitude was more attenuated in lower limbs than in u pper limbs (P < 0.05). Abnormal conduction slowing was frequently observed in mo tor (95%) and sensory (76%) nerves. Distal motor latencies were abnormally pro longed in 75%of the motor nerves,and terminal latency indices were significant - ly higher in patients than in normal controls (P < 0.05). Conclusions: NCS in POEMS syndrome showed characteristic patterns, in which conduction abnormalities were more frequently and severely affected in the lower limbs, and more predomi nantly in the intermediate nerve segments than in the distal portions. Significa nce:The recognition of these characteristic patterns may be helpful for the earl y diagnosis of polyneuropathy in POEMS syndrome.
