Cerebellar Dizziness and Vertigo: Etiologies, Diagnostic Assessment, and Treatment

Abstract

Cerebellar dizziness and vertigo account for approximately 10% of diagnoses in a tertiary dizziness center. This term summarizes a large group of disorders with chronic (degenerative, hereditary, acquired cerebellar ataxias), recurrent (episodic ataxias), or acute (stroke, inflammation) presentations. Key to the diagnosis is a comprehensive examination of central ocular motor and vestibular function. Patients with cerebellar dizziness and vertigo usually show a pattern of deficits in smooth pursuit, gaze-holding, saccade accuracy, or fixation-suppression of the vestibulo-ocular reflex. Central fixation nystagmus (e.g., downbeat nystagmus), gaze-evoked nystagmus, central positional nystagmus, or head-shaking nystagmus with cross-coupling (i.e., horizontal head shaking causing inappropriate vertical nystagmus) occurs frequently. Overlap syndromes with peripheral vestibular disorders, such as cerebellar ataxia, neuropathy, and vestibular areflexia, exist rarely. Posturography and gait analysis can contribute to diagnostic differentiation, estimation of the risk of falls, as well as quantification of progression and treatment effects. Patients with cerebellar dizziness and vertigo should receive multimodal treatment, including balance training, occupational therapy, and medication.

Keywords

cerebellar disorders - downbeat nystagmus - ocular motor disorders - aminopyridines - acetyl-leucine

• References

• 1 Neuhauser HK. The epidemiology of dizziness and vertigo. Handb Clin Neurol 2016; 137: 67-82
• 2 Brandt T, Dieterich M, Strupp M. Vertigo and Dizziness: Common Complaints. 2nd ed. London: Springer-Verlag; 2014
• 3 Grimaldi G, Manto M. Topography of cerebellar deficits in humans. Cerebellum 2012; 11 (02) 336-351
• 4 Bodranghien F, Bastian A, Casali C. , et al. Consensus paper: revisiting the symptoms and signs of cerebellar syndrome. Cerebellum 2016; 15 (03) 369-391
• 5 Feil K, Strobl R, Schindler A. , et al. What is behind cerebellar vertigo and dizziness?. Cerebellum 2019; 18 (03) 320-332
• 6 Kim JS, Lee H. Vertigo due to posterior circulation stroke. Semin Neurol 2013; 33 (03) 179-184
• 7 Shaikh AG, Manto M. Vertigo in cerebellar disease- do the eyes have it or it there more to perceive?. Cerebellum 2019; 18 (03) 295-297
• 8 Radtke A, von Brevern M, Neuhauser H, Hottenrott T, Lempert T. Vestibular migraine: long-term follow-up of clinical symptoms and vestibulo-cochlear findings. Neurology 2012; 79 (15) 1607-1614
• 9 Kim SH, Kim HJ, Kim JS. Isolated vestibular syndromes due to brainstem and cerebellar lesions. J Neurol 2017; 264 (Suppl. 01) 63-69
• 10 Newman-Toker DE, Hsieh YH, Camargo Jr CA, Pelletier AJ, Butchy GT, Edlow JA. Spectrum of dizziness visits to US emergency departments: cross-sectional analysis from a nationally representative sample. Mayo Clin Proc 2008; 83 (07) 765-775
• 11 Klockgether T. Sporadic adult-onset ataxia. Handb Clin Neurol 2018; 155: 217-225
• 12 Gilman S, Wenning GK, Low PA. , et al. Second consensus statement on the diagnosis of multiple system atrophy. Neurology 2008; 71 (09) 670-676
• 13 Szmulewicz DJ, Roberts L, McLean CA, MacDougall HG, Halmagyi GM, Storey E. Proposed diagnostic criteria for cerebellar ataxia with neuropathy and vestibular areflexia syndrome (CANVAS). Neurol Clin Pract 2016; 6 (01) 61-68
• 14 Cortese A, Simone R, Sullivan R. , et al. Biallelic expansion of an intronic repeat in RFC1 is a common cause of late-onset ataxia. Nat Genet 2019; 51 (04) 649-658
• 15 Rafehi H, Szmulewicz DJ, Bennett MF. , et al. Bioinformatics-based identification of expanded repeats: a non-reference intronic pentamer expansion in RFC1 causes CANVAS. Am J Hum Genet 2019; 105 (01) 151-165
• 16 Wagner JN, Glaser M, Brandt T, Strupp M. Downbeat nystagmus: aetiology and comorbidity in 117 patients. J Neurol Neurosurg Psychiatry 2008; 79 (06) 672-677
• 17 Feil K, Böttcher N, Guri F. , et al. Long-term course of orthostatic tremor in serial posturographic measurement. Parkinsonism Relat Disord 2015; 21 (08) 905-910
• 18 Klockgether T, Mariotti C, Paulson HL. Spinocerebellar ataxia. Nat Rev Dis Primers 2019; 5 (01) 24
• 19 Choi KD, Choi JH. Episodic ataxias: clinical and genetic features. J Mov Disord 2016; 9 (03) 129-135
• 20 Nachbauer W, Eigentler A, Boesch S. Acquired ataxias: the clinical spectrum, diagnosis and management. J Neurol 2015; 262 (05) 1385-1393
• 21 Lempert T, Olesen J, Furman J. , et al. Vestibular migraine: diagnostic criteria. J Vestib Res 2012; 22 (04) 167-172
• 22 Dieterich M, Obermann M, Celebisoy N. Vestibular migraine: the most frequent entity of episodic vertigo. J Neurol 2016; 263 (Suppl. 01) S82-S89
• 23 von Brevern M, Zeise D, Neuhauser H, Clarke AH, Lempert T. Acute migrainous vertigo: clinical and oculographic findings. Brain 2005; 128 (Pt 2): 365-374
• 24 Strupp M, Zwergal A, Brandt T. Episodic ataxia type 2. Neurotherapeutics 2007; 4 (02) 267-273
• 25 Jen JC, Wan J. Episodic ataxias. Handb Clin Neurol 2018; 155: 205-215
• 26 Lee H, Sohn SI, Cho YW. , et al. Cerebellar infarction presenting isolated vertigo: frequency and vascular topographical patterns. Neurology 2006; 67 (07) 1178-1183
• 27 Choi SY, Kim HJ, Kim JS. Chasing dizzy chimera: diagnosis of combined peripheral and central vestibulopathy. J Neurol Sci 2016; 371: 69-78
• 28 Kuroda R, Nakada T, Ojima T. , et al. The TriAGe+ score for vertigo or dizziness: a diagnostic model for stroke in the emergency department. J Stroke Cerebrovasc Dis 2017; 26 (05) 1144-1153
• 29 Leigh RJ, Zee DS. , eds. The Neurology of Eye Movements. 5th ed. Oxford: Oxford University Press; 2015
• 30 Strupp M, Hüfner K, Sandmann R. , et al. Central oculomotor disturbances and nystagmus: a window into the brainstem and cerebellum. Dtsch Arztebl Int 2011; 108 (12) 197-204
• 31 Choi JY, Kim JH, Kim HJ, Glasauer S, Kim JS. Central paroxysmal positional nystagmus: characteristics and possible mechanisms. Neurology 2015; 84 (22) 2238-2246
• 32 Huh YE, Kim JS. Patterns of spontaneous and head-shaking nystagmus in cerebellar infarction: imaging correlations. Brain 2011; 134 (Pt 12): 3662-3671
• 33 Hüfner K, Frenzel C, Kremmyda O. , et al. Esophoria or esotropia in adulthood: a sign of cerebellar dysfunction?. J Neurol 2015; 262 (03) 585-592
• 34 Baier B, Bense S, Dieterich M. Are signs of ocular tilt reaction in patients with cerebellar lesions mediated by the dentate nucleus?. Brain 2008; 131 (Pt 6): 1445-1454
• 35 Glasauer S. Cerebellar contribution to saccades and gaze holding: a modeling approach. Ann N Y Acad Sci 2003; 1004: 206-219
• 36 Kremmyda O, Kirchner H, Glasauer S, Brandt T, Jahn K, Strupp M. False-positive head-impulse test in cerebellar ataxia. Front Neurol 2012; 3: 162
• 37 Kirchner H, Kremmyda O, Hüfner K. , et al. Clinical, electrophysiological, and MRI findings in patients with cerebellar ataxia and a bilaterally pathological head-impulse test. Ann N Y Acad Sci 2011; 1233: 127-138
• 38 Lee SH, Park SH, Kim JS, Kim HJ, Yunusov F, Zee DS. Isolated unilateral infarction of the cerebellar tonsil: ocular motor findings. Ann Neurol 2014; 75 (03) 429-434
• 39 Choi JY, Jung I, Jung JM. , et al. Characteristics and mechanism of perverted head-shaking nystagmus in central lesions: video-oculography analysis. Clin Neurophysiol 2016; 127 (09) 2973-2978
• 40 Lee SU, Choi JY, Kim HJ, Park JJ, Zee DS, Kim JS. Impaired tilt suppression of post-rotatory nystagmus and crosscoupled head-shaking nystagmus in cerebellar lesions: image mapping study. Cerebellum 2017; 16 (01) 95-102
• 41 Choi JH, Seo JD, Choi YR. , et al. Inferior cerebellar peduncular lesion causes a distinct vestibular syndrome. Eur J Neurol 2015; 22 (07) 1062-1067
• 42 Kremmyda O, Zwergal A, la Fougère C, Brandt T, Jahn K, Strupp M. 4-Aminopyridine suppresses positional nystagmus caused by cerebellar vermis lesion. J Neurol 2013; 260 (01) 321-323
• 43 Subramony SH. SARA--a new clinical scale for the assessment and rating of ataxia. Nat Clin Pract Neurol 2007; 3 (03) 136-137
• 44 Schlick C, Schniepp R, Loidl V, Wuehr M, Hesselbarth K, Jahn K. Falls and fear of falling in vertigo and balance disorders: a controlled cross-sectional study. J Vestib Res 2016; 25 (5–6): 241-251
• 45 Diener HC, Dichgans J, Bacher M, Gompf B. Quantification of postural sway in normals and patients with cerebellar diseases. Electroencephalogr Clin Neurophysiol 1984; 57 (02) 134-142
• 46 Krafczyk S, Tietze S, Swoboda W, Valkovič P, Brandt T. Artificial neural network: a new diagnostic posturographic tool for disorders of stance. Clin Neurophysiol 2006; 117 (08) 1692-1698
• 47 Ahmadi SA, Vivar G, Frei J. , et al. Towards computerized diagnosis of neurological stance disorders: data mining and machine learning of posturography and sway. J Neurol 2019; 266 (Suppl. 01) 108-117
• 48 Wuehr M, Schniepp R, Ilmberger J, Brandt T, Jahn K. Speed-dependent temporospatial gait variability and long-range correlations in cerebellar ataxia. Gait Posture 2013; 37 (02) 214-218
• 49 Schniepp R, Möhwald K, Wuehr M. Clinical and automated gait analysis in patients with vestibular, cerebellar, and functional gait disorders: perspectives and limitations. J Neurol 2019; 266 (Suppl. 01) 118-122
• 50 Schniepp R, Wuehr M, Neuhaeusser M. , et al. Locomotion speed determines gait variability in cerebellar ataxia and vestibular failure. Mov Disord 2012; 27 (01) 125-131
• 51 Schniepp R, Möhwald K, Wuehr M. Gait ataxia in humans: vestibular and cerebellar control of dynamic stability. J Neurol 2017; 264 (Suppl. 01) 87-92
• 52 Schniepp R, Wuehr M, Huth S. , et al. The gait disorder in downbeat nystagmus syndrome. PLoS One 2014; 9 (08) e105463
• 53 Schlick C, Rasoul A, Wuehr M. , et al. Gait variability predicts a subset of falls in cerebellar gait disorders. J Neurol 2017; 264 (11) 2322-2324
• 54 Stephen CD, Brizzi KT, Bouffard MA. , et al. The comprehensive management of cerebellar ataxia in adults. Curr Treat Options Neurol 2019; 21 (03) 9
• 55 Strupp M, Teufel J, Zwergal A, Schniepp R, Khodakhah K, Feil K. Aminopyridines for the treatment of neurologic disorders. Neurol Clin Pract 2017; 7 (01) 65-76
• 56 Kalla R, Strupp M. Aminopyridines and acetyl-DL-leucine: new therapies in cerebellar disorders. Curr Neuropharmacol 2019; 17 (01) 7-13
• 57 Schniepp R, Wuehr M, Neuhaeusser M. , et al. 4-aminopyridine and cerebellar gait: a retrospective case series. J Neurol 2012; 259 (11) 2491-2493
• 58 Strupp M, Schüler O, Krafczyk S. , et al. Treatment of downbeat nystagmus with 3,4-diaminopyridine: a placebo-controlled study. Neurology 2003; 61 (02) 165-170
• 59 Kalla R, Spiegel R, Claassen J. , et al. Comparison of 10-mg doses of 4-aminopyridine and 3,4-diaminopyridine for the treatment of downbeat nystagmus. J Neuroophthalmol 2011; 31 (04) 320-325
• 60 Claassen J, Spiegel R, Kalla R. , et al. A randomised double-blind, cross-over trial of 4-aminopyridine for downbeat nystagmus--effects on slow phase eye velocity, postural stability, locomotion and symptoms. J Neurol Neurosurg Psychiatry 2013; 84 (12) 1392-1399
• 61 Claassen J, Feil K, Bardins S. , et al. Dalfampridine in patients with downbeat nystagmus--an observational study. J Neurol 2013; 260 (08) 1992-1996
• 62 Strupp M, Kalla R, Dichgans M, Freilinger T, Glasauer S, Brandt T. Treatment of episodic ataxia type 2 with the potassium channel blocker 4-aminopyridine. Neurology 2004; 62 (09) 1623-1625
• 63 Strupp M, Kalla R, Claassen J. , et al. A randomized trial of 4-aminopyridine in EA2 and related familial episodic ataxias. Neurology 2011; 77 (03) 269-275
• 64 Claassen J, Teufel J, Kalla R, Spiegel R, Strupp M. Effects of dalfampridine on attacks in patients with episodic ataxia type 2: an observational study. J Neurol 2013; 260 (02) 668-669
• 65 Feil K, Claaßen J, Bardins S. , et al. Effect of chlorzoxazone in patients with downbeat nystagmus: a pilot trial. Neurology 2013; 81 (13) 1152-1158
• 66 Strupp M, Teufel J, Habs M. , et al. Effects of acetyl-DL-leucine in patients with cerebellar ataxia: a case series. J Neurol 2013; 260 (10) 2556-2561
• 67 Schniepp R, Strupp M, Wuehr M. , et al. Acetyl-DL-leucine improves gait variability in patients with cerebellar ataxia-a case series. Cerebellum Ataxias 2016; 3: 8
• 68 Bremova T, Malinová V, Amraoui Y. , et al. Acetyl-dl-leucine in Niemann-Pick type C: a case series. Neurology 2015; 85 (16) 1368-1375
• 69 Milne SC, Corben LA, Georgiou-Karistianis N, Delatycki MB, Yiu EM. Rehabilitation for individuals with genetic degenerative ataxia: a systematic review. Neurorehabil Neural Repair 2017; 31 (07) 609-622
• 70 Kundakci B, Sultana A, Taylor AJ, Alshehri MA. The effectiveness of exercise-based vestibular rehabilitation in adult patients with chronic dizziness: a systematic review. F1000 Res 2018; 7: 276
• 71 McDonnell MN, Hillier SL. Vestibular rehabilitation for unilateral peripheral vestibular dysfunction. Cochrane Database Syst Rev 2015; 1: CD005397