Characterization of severe action XXXX in sialidoses.

@entity1 @entity16045 @entity1952 @entity2045 @entity73 @entity4085 @entity305 @entity600 @entity3148
To asses the characteristics of severe action @entity2045 in three @entity1 with @entity3148 ( @entity3148 ) due to @entity4085 . We assessed EEG-EMG coherence, relative power (RP) and bandwidth (BW) of the EMG-peak associated with @entity2045 ; we also evaluated somatosensory evoked potentials and long-loop reflexes (LLRs). We compared the findings with those obtained in ten @entity16045 ( @entity16045 ) @entity1 . The presentation of @entity4085 included macular cherry-red spot, @entity600 and @entity305 in the infantile form and @entity1952 in the juvenile form. From its onset in adolescence @entity2045 rapidly worsened, quickly leading to severe disability. In @entity4085 @entity1 , the EMG-peak was characterised by higher RP (p<0.01) and narrower BW (p<0.02) than in @entity16045 . EEG-EMG coherence values were higher (p<0.05) than in @entity16045 @entity1 . Taking into account both @entity4085 and @entity16045 @entity1 , the coherence values and the RP of the EMG-peak were directly correlated with the severity of the @entity2045 ; while BW values were inversely correlated. All these measures showed extreme values in @entity4085 @entity1 . In the @entity4085 @entity1 , the strongly rhythmic recurrence of the jerks reflected on LLR, which included multiple components. Subtle differences indicate an especially high level of cortical motor synchronization in the @entity4085 @entity1 , which may account for their particularly severe @entity73 . Neurophysiological indexes indicating high EEG-EMG synchronization parallels the severity of the @entity2045 .
