Perverted head-shaking and positional downbeat nystagmus in patients with multiple system atrophy

The diagnosis of multiple system atrophy (MSA) is mainly based on the clinical criteria, which are often of little assistance in the early stages of the disease. Positional downbeat nystagmus (pDBN) and perverted head-shaking nystagmus (pHSN), possible signs of cerebellar dysfunction, may be useful in differentiating MSA from other parkinsonian disorders. To investigate the occurrences of pDBN and pHSN in patients with MSA compared with those in patients with Parkinson's disease (PD). A total of 127 consecutive patients with MSA and 274 patients with PD underwent a video-oculographic recording of head-shaking and positional nystagmus over a year. The occurrences of pDBN and pHSN were higher in MSA than in PD. pDBN was more frequently observed in MSA with overt cerebellar signs than in those without, but the occurrence of pHSN did not differ between the MSA groups. pHSN was more frequently observed in MSA-p without overt cerebellar signs than in PD, but there was no difference in the occurrence of pDBN between them. The presence of pHSN and pDBN may be a clue for the diagnosis of MSA, and pHSN may be helpful in differentiating MSA-p from PD when the patients do not have overt cerebellar features.