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Schniepp, Roman; Wuehr, Max; Huth, Sabrina; Pradhan, Cauchy; Schlick, Cornelia; Brandt, Thomas; Jahn, Klaus (2014): The Gait Disorder in Downbeat Nystagmus Syndrome.
In: PLOS ONE 9(8), e105463


Background: Downbeat nystagmus (DBN) is a common form of acquired fixation nystagmus with key symptoms of oscillopsia and gait disturbance. Gait disturbance could be a result of impaired visual feedback due to the involuntary ocular oscillations. Alternatively, a malfunction of cerebellar locomotor control might be involved, since DBN is considered a vestibulocerebellar disorder. Methods: Investigation of walking in 50 DBN patients (age 72 +/- 11 years, 23 females) and 50 healthy controls (HS) (age 70 +/- 11 years, 23 females) using a pressure sensitive carpet (GAITRite). The patient cohort comprised subjects with only ocular motor signs (DBN) and subjects with an additional limb ataxia (DBNCA). Gait investigation comprised different walking speeds and walking with eyes closed. Results: In DBN, gait velocity was reduced (p<0.001) with a reduced stride length (p<0.001),increased base of support (p<0.050),and increased double support (p<0.001). Walking with eyes closed led to significant gait changes in both HS and DBN. These changes were more pronounced in DBN patients (p<0.001). Speed-dependency of gait variability revealed significant differences between the subgroups of DBN and DBNCA (p<0.050). Conclusions: (I) Impaired visual control caused by involuntary ocular oscillations cannot sufficiently explain the gait disorder. (II) The gait of patients with DBN is impaired in a speed dependent manner. (III) Analysis of gait variability allows distinguishing DBN from DBNCA: Patients with pure DBN show a speed dependency of gait variability similar to that of patients with afferent vestibular deficits. In DBNCA, gait variability resembles the pattern found in cerebellar ataxia.