Comparative Clinical And Electrophysiological Study Of LSS、MMN And CIDP

Objective To illustrate the clinical performance, physical examination, especially the electrophysiological characteristics of chronic inflammatory demyelinating polyneuropathy (CIDP), lewis-sumner syndrome (LSS) and multifocal motor neuropathy (MMN) and their responses to the hormones therapy, blood plasma therapy, gamma globulin therapy, immunosuppressive therapy, in order to better perform differential diagnosis and then adopt appropriate treatment measures.Method18cases of CIDP patients,12cases of MMN patients and24cases of LSS patients treated in department of neurology of Huashan Hospital from January,2011to December,2012were selected. The age, gender and height of all the groups mentioned above were matched. The clinical information of the individuals were registered and motor nerve conduction of median nerve, ulnar nerve, common peroneal nerve and tibial nerve was tested, and the conduction of sensory nerve ((median nerve, ulnar nerve, sural nerve, superficial peroneal nerve) of LSS group and MMN group was tested. The clinical data, DML, CMAP, F wave and MNCV were performed comparison, and additionally, the site of motor nerve block and SNAP between SLL and MMN group was compared.Results1. Compared with LSS group and MMN group, clinical features of CIDP group were more obvious, symmetry, diseases mostly occurred in lower extremity, development from distal to proximal lesions, more common limb involvement, significant rising of cerebrospinal fluid protein and abnormality in limb tendon reflex. The performances of LSS and MMN group were:asymmetry, lesion of single nerve distribution, diseases mostly occurred in distal upper extremity and relatively localized, most cerebrospinal fluid normal or slightly elevated, and normality in limb tendon reflex’.. Clinical difference between LSS group and MMN group:LSS group had sensory symptoms, while MMN group did not have; LSS group had cranial nerve involvement, but no for MMN group; anti GM-1antibody in the blood increasing occurred in MMN but without in LSS group.3. On curative effect, CIDP group and LSS group had response to hormone, gamma globulin, plasma exchange and immune inhibitors, while MMN group was sensitive to gamma globulin.4. Compared with normal reference value, DML, CMAP, MNCV and F wave of CIDP group, LLS group and MMN group had abnormal changes of different degree, with the performances including prolonging of DML, decreasing of CMAP, and slowing down of MNCV, prolonging of latent period of F wave or disappearing of F wave. Obvious and continuous motor nerve conduction block occurred in LSS group and MMN group, which showed that the basic nerve pathology of CIDP, LSS and MMN had changed into demyelination.5. The degree of prolonging of DML, slowing down or disappearing of F wave and slowing down of MNCV was higher than that of other two groups, with statistically significant difference, while these changed occurred in LSS group and CIDP group was relatively light due to diffuse demyelination, and obvious electrophysiological change was changed into conduction block as the result of focal demyelination. Compared with LSS and MMN group, the degree of decrease of CMAP of CIDP was obvious because secondary axonal damage caused by diffuse demyelination was more serious.6. The occurrence site of motor nerve conduction block between LSS group and MMN group was not statistically significant, only with the occurrence of conduction block in radial nerve of MMN group higher than that of LSS group. And the conduction block of these two diseases mostly occurred in median nerve and ulnar nerve. SNAP of LSS group in sensory nerve electrophysiological detection was decreased, also mostly occurred in median nerve and ulnar nerve, superficial peroneal nerve and sural nerve with less involvement, which was consistent with that the upper limb involvement was easier in clinic. There was no evidence for damage of sensory nerve in MMN group. Conclusion1. The involvement and site of three demyelinating peripheral nerves are different:①CIDP is diffuse demyelination of motor and sensory nerve;②LSS group is focal demyelination, involving motor and sensory nerve;③MMN group is focal demyelination, only involving motor nerve;④There is no difference in the degree of demyelinating between LSS and MMN.2. There is no significant difference of focal lesion in the distribution and degree between LSS group and MMN group, while LSS group has the damage of sensory nerve, with decrease of SNAP, while MMN group has no damage of sensory.3. There is mainly proximal demyelination in LSS and MMN. Further illustrate the non-diffuse demyelinating in LSS and MMN.4. Secondary axonal damage occurred in three diseases; the degree of seriousness is associated with the degree of demyelination; CIDP is the most serious.5. The curative effect has difference:CIDP and LSS group have response to four therapies, while MMN group has response to gamma globulin therapy.