Table 1Clinical data of patients with DBN, arranged according to assumed diagnosis

SexAge, yAdditional neuro-ophthalmologic findingsMRI findingsEtiologyTime since diagnosisΔ PSPV, %Follow-up
The 17 patients included in the study are characterized by their sex, age, additional neuro-ophthalmologic findings, MRI findings, etiology of downbeat nystagmus (DBN), time since diagnosis of DBN, changes of peak slow-phase velocity (PSPV) of DBN by 3,4-diaminopyridine, and the results of the follow-up study.
VOR = vestibulo-ocular reflex; SVV = subjective visual vertical.
F62Fourth nerve palsyDeep-seated tonsils but no prolapse into foramen magnum (cerebellar ectopia?)Arnold–Chiari malformation5 y+3.9No benefit, worsening of symptoms after 1 wk of medication
F62Horizontal hypermetric saccades; disturbed visual fixation suppression of VORDiscrete cerebellar and brain atrophyCerebellar degeneration9 mo−98Benefit, still under treatment (follow-up time: 2 mo)
M61Disturbed visual fixation suppression of VOR; rebound nystagmusCerebellar atrophyCerebellar degeneration3 y−24.5Benefit, still under treatment (follow-up time: 2 mo)
F80Disturbed visual fixation suppression of VOR; incomplete ocular tilt reactionCerebellar atrophyCerebellar degeneration4 y−76.9Benefit, still under treatment (follow-up time: 1 mo)
F72Disturbed visual fixation suppression of VOR; tilt of SVVCerebellar atrophyCerebellar/vermis degeneration6 y−95.0No benefit after 1 mo of treatment, discontinued medication
F63Disturbed visual fixation suppression of VOR; rebound nystagmusCerebellar atrophyIdiopathic cerebellar ataxia/spinocerebellar ataxia type 610 mo−48.0No benefit after 1 mo of treatment, discontinued medication, reported side effects (headache)
M55Disturbed visual fixation suppression of VOR; rebound nystagmusInfarct of right cerebellar hemisphere and vermis; partial resection of lesioned cerebellumInfarction of cerebellum3 y−97Benefit, still under treatment (follow-up time: 2 mo)
F74Disturbed visual fixation suppression of VORUpper right cerebellar peduncleInfarction of cerebellum2 y−20No benefit after 1 mo of treatment, discontinued medication
M62Two ischemic defects in midline of cerebellumInfarction of cerebellum6 mo−100Discontinued treatment after recovery of symptoms
M85Disturbed visual fixation suppression of VOR; rebound nystagmus; hypometric saccadesArachnoid membrane cyst in angleUnknown15 yNo changeBenefit, still under treatment (follow-up time: 3 mo)
M65NormalUnknown6.5 y−64.8Benefit, still under treatment (follow-up time: 2 mo)
F66Disturbed visual fixation suppression of VORNormalUnknown1 y−77.4No benefit after 1 mo of treatment, discontinued medication
M59NormalUnknown4 y−68.1No benefit after 1 mo of treatment, discontinued medication
M76MicroangiopathyUnknown8 mo−41.2No benefit after 1 mo of treatment, discontinued medication
M76Rebound nystagmusUnknown7 y−42.4Benefit, still under treatment (follow-up time: 2 mo)
M50NormalUnknown2 y−40.5Benefit, still under treatment (follow-up time: 1 mo)
F82Disturbed visual fixation suppression of VOR; rebound nystagmusNormalUnknown4 y−15.5Benefit, still under treatment (follow-up time: 1 mo)