Downbeat nystagmus: Aetiology and comorbidity in 117 patients
Open accessAbstract
OBJECTIVES: Downbeat nystagmus (DBN) is the most common form of acquired involuntary ocular oscillation overriding fixation. According to previous studies, the cause of DBN is unsolved in up to 44% of cases. We reviewed 117 patients to establish whether analysis of a large collective and improved diagnostic means would reduce the number of cases with "idiopathic DBN" and thus change the aetiological spectrum. METHODS: The medical records of all patients diagnosed with DBN in our Neurological Dizziness Unit between 1992 and 2006 were reviewed. In the final analysis, only those with documented cranial MRI were included. Their workup comprised a detailed history, standardised neurological, neuro-otological and neuro-ophthalmological examination, and further laboratory tests. RESULTS: In 62% (n = 72) of patients the aetiology was identified ("secondary DBN"), the most frequent causes being cerebellar degeneration (n = 23) and cerebellar ischaemia (n = 10). In 38% (n = 45), no cause was found ("idiopathic DBN"). A major finding was the high comorbidity of both idiopathic and secondary DBN with bilateral vestibulopathy (36%) and the association with polyneuropathy and cerebellar ataxia even without cerebellar pathology on MRI. CONCLUSIONS: Idiopathic DBN remains common despite improved diagnostic techniques. Our findings allow the classification of "idiopathic DBN" into three subgroups: "pure" DBN (n = 17); "cerebellar" DBN (ie, DBN plus further cerebellar signs in the absence of cerebellar pathology on MRI; n = 6); and a "syndromatic" form of DBN associated with at least two of the following: bilateral vestibulopathy, cerebellar signs and peripheral neuropathy (n = 16). The latter may be caused by multisystem neurodegeneration.
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Neurodegenerative disorders that affect posture and gait such as Parkinson's disease, cerebellar degeneration, including the downbeat nystagmus syndrome, and small vessel white matter disease may manifest with dizziness or unsteadiness when standing or walking before motor signs can be detected on physical examination.
“Neurodegenerative disorders that affect posture and gait such as Parkinson’s disease, cerebellar degeneration [61], including the downbeat nystagmus syndrome [89], and small vessel white matter disease [2, 16, 66] may manifest with dizziness or unsteadiness when standing or walking before motor signs can be detected on physical examination.”
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- Authors
- Wagner, J. N., Glaser, M., Brandt, T., & Strupp, M.
- Affiliations
- LMU Klinikum (DE)
- Ludwig-Maximilians-Universität München (DE)
- Corresponding author
- Wagner, J N
- Venue
- Journal of Neurology, Neurosurgery & Psychiatry
- Publisher
- BMJ
- Published
- 2007-10-10
- Volume
- 79
- Issue
- 6
- Pages
- 672-677
- ISSN
- 0022-3050 (ISSN-L)
- OpenAlex
- W2171673303
- Cited by
- 195
- References
- 31
- Citations by year
- 2026: 8 · 2025: 11 · 2024: 9 · 2023: 15 · 2022: 13 · 2021: 10 · 2020: 12 · 2019: 8 · 2018: 8 · 2017: 8 · 2016: 10 · 2015: 10 · 2014: 10 · 2013: 12 · 2012: 13
- FWCI
- 4.89
- Type
- journal article
- Language
- en
- Open access
- bronze
- doi.org
- Locations
- Journal of Neurology Neurosurgery & Psychiatry (publishedVersion)
- PubMed (publishedVersion)
- Open access LMU (Ludwid Maxmilian's Universitat Munchen) (submittedVersion)
- HighWire Press Open Archive (submittedVersion)
- Topics
- Life Sciences › Neuroscience › Neurology › Vestibular and auditory disorders
- Health Sciences › Medicine › Endocrinology, Diabetes and Metabolism › Neurological and metabolic disorders
- Health Sciences › Medicine › Neurology › Neurological disorders and treatments
- Keywords
- downbeat nystagmus, idiopathic nystagmus, cerebellar degeneration, bilateral vestibulopathy, polyneuropathy, cerebellar ataxia, comorbidities, brain MRI
- MeSH terms
- Adolescent, Adult, Aged, Aged, 80 and over, Child, Diagnosis, Differential, Electromyography, Electronystagmography, Female, Humans, Magnetic Resonance Imaging, Male, Middle Aged, Neurologic Examination, Nystagmus, Pathologic, Polyneuropathies, Retrospective Studies, Spinocerebellar Degenerations, Vestibular Diseases, Comorbidity, Multiple System Atrophy
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