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Lopez LI, Bronstein AM, Gresty MA et al (1996) Clinical and MRI correlates in 27 patients with acquired pendular nystagmus. Brain 119:465–472
Howard KL, Hall DA, Moon M et al (2008) Adult-onset Alexander disease with progressive ataxia and palatal tremor. Mov Disord 23:118–122
Storey E, Knight MA, Forrest SM et al (2005) Spinocerebellar ataxia type 20. Cerebellum 4:55–57
Stricker S, Oberwahrenbrock T, Zimmermann H et al (2011) Temporal retinal nerve fibre loss in patients with spinocerebellar ataxia type 1. PLoS ONE 6:e23024
Morgia C, Achilli A, Iommarini L et al (2008) Rare mtDNA variants in Leber hereditary optic neuropathy families with recurrence of myoclonus. Neurology 70:762–770
Amati-Bonneau P, Valentino ML, Reynier P et al (2008) OPA1 mutations induce mitochondrial DNA instability and optic atrophy ‘plus’ phenotypes. Brain 131:338–351
Naviaux RK, Nguyen KV (2005) POLG mutations associated with Alper’s syndrome and mitochondrial DNA depletion. Ann Neurol 58:491
Johansen KK, Bindoff LA, Rydland J et al (2008) Palatal tremor and facial dyskinesia in a patient with POLG1 mutation. Mov Disord 23:1624–1626
Samuel M, Torun N, Tuite PJ et al (2004) Progressive ataxia and palatal tremor (PAPT): clinical and MRI assessment with review of palatal tremors. Brain 127:1252–1268
Deuschl G, Toro C, Valls-Sole J et al (1994) Symptomatic and essential palatal tremor: clinical, physiological and MRI analysis. Brain 117:775–788
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All authors declare no conflicts of interest.
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Video 1 3D video oculography of the right eye during fixation to a LED target, initially in primary gaze and then during fixation 15° to the right, left and back to centre. Note the large, asymmetric, torsional, ‘rotatory’ nystagmus, which is larger and faster to the left (clockwise from the observer’s point of view). (M4 V 15114 kb)
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Papachatzaki, M.M., Ali, N., Arshad, Q. et al. Progressive ataxia with oculo-palatal tremor and optic atrophy. J Neurol 260, 2903–2905 (2013) doi:10.1007/s00415-013-7136-3
- Multiple System Atrophy
- Retinal Nerve Fibre Layer
- Optic Atrophy
- Spinocerebellar Ataxia
- Peripapillary Retinal Nerve Fibre Layer