Okulomotorikstörungen und Nystagmus

Zusammenfassung

Der Diagnose von Augenbewegungsstörungen und der Nystagmusformen beruht auf einer systematischen klinischen Untersuchung aller Arten von Augenbewegungen. Diese Untersuchung umfasst: Augenposition, Untersuchung auf einen Spontannystagmus, Motilität, Blickfolge, Blickhaltefunktion, Sakkaden, Vergenzreaktion, optokinetischer Nystagmus, Funktion des vestibulookulären Reflexes (VOR) sowie die Fixationssuppression des VOR. Anatomisch relevante Strukturen sind Mesenzephalon, Pons, Medulla oblongata, Zerebellum und Kortex. Topografisch anatomisch gelten die einfachen klinischen Regeln: Vertikale und torsionale Augenbewegungen werden vorwiegend im Mesenzephalon und horizontale Augenbewegungen in dem Pons generiert. Typische Zeichen einer Mittelhirnläsion sind vertikale Sakkaden- oder Blickparese, ein isolierter vertikaler Blickrichtungsnystagmus und einer Ponsläsion entsprechende horizontale Störungen. Das Zerebellum spielt eine Rolle bei praktisch allen Augenbewegungen; typische klinische Zeichen sind eine allseitige Blickfolgesakkadierung, Blickrichtungsnystagmus oder dysmetrische Sakkaden. Unter einem Nystagmus versteht man rhythmische Augenbewegungen, die in der Regel aus einem langsamen (ursächlichen bzw. pathologischen) Augendrift und einer schnellen kompensatorischen Rückstellbewegung (Rückstellsakkade) bestehen. Es lassen sich 3 einfache Kategorien unterscheiden: Spontannystagmus, d. h. ein Nystagmus, der bei Fixation in Geradeaus-Blickposition auftritt, Nystagmusformen, die nur in Abhängigkeit von der Blickrichtung auftreten und Nystagmen, die nur durch bestimmte Manöver ausgelöst werden: Kopfschütteln, Lagerung, Hyperventilation oder physikalischen Druck (z. B. Pressen). Letztere sind oft durch peripher-vestibuläre Läsionen ausgelöst, können aber auch zentralen Ursprungs sein. Viele zentrale Nystagmusformen erlauben eine genaue anatomische Lokalisation, z. B. der Downbeat-Nystagmus (DBN), der meistens auf einer Flocculus-Läsion beruht oder der Upbeat-Nystagmus (UBN) auf einer Läsion im Mesencephalon oder der Medulla oblongata. Beispiele einer Pharmakotherapie sind die Gabe von 4-Aminopyridin beim DBN und UBN, Memantin oder Gabapentin beim Fixationspendelnystagmus oder Baclofen beim periodisch-alternierenden Nystagmus.

Abstract

The diagnosis of ocular motor disorders and the different forms of a nystagmus is based on a systematic clinical examination of all types of eye movements: eye position, spontaneous nystagmus, range of eye movements, smooth pursuit, saccades, gaze-holding function, vergence, optokinetic nystagmus, as well as testing of the function of the vestibulo-ocular reflex (VOR) and visual fixation suppression of the VOR. Relevant anatomical structures are the midbrain, pons, medulla, cerebellum, and cortex. There is a simple clinical rule: vertical and torsional eye movements are generated in the midbrain, horizontal in the pons. The cerebellum is relevant for almost all types of eye movements; typical pathological findings are saccadic smooth pursuit, gaze-evoked nystagmus or dysmetric saccades.

Nystagmus is defined as a rhythmic, most often involuntary eye movement. It normally consists of a slow (pathological) drift of the eyes and a fast central compensatory movement of the eyes back to the primary position (re-fixation saccade). There are three major categories: first, spontaneous nystagmus, i. e. nystagmus which occurs in the gaze straight ahead position as upbeat or downbeat nystagmus; second, nystagmus that becomes visible at eccentric gaze only and third, nystagmus which can be elicited by certain maneuvers, e. g. head-shaking, head positioning, air pressure or hyperventilation, most of which are of peripheral vestibular origin. The most frequent central types of spontaneous nystagmus are downbeat and upbeat, infantile, pure torsional, pendular fixation, periodic alternating, and seesaw nystagmus. Many types of central nystagmus allow a precise neuroanatomical localization: for instance, downbeat nystagmus, which is most often caused by a bilateral floccular lesion or dysfunction, or upbeat nystagmus, which is caused by a lesion in the mesencephalon or medulla oblongata. Examples of pharmacotherapy are the use of 4-aminopyridine for downbeat and upbeat nystagmus, memantine or gabapentin for fixation pendular nystagmus or baclofen for periodic alternating nystagmus.

Schlüsselwörter

Nystagmus - Blickparese - Blickhaltedefekt - Blickfolge

Keywords

Nystagmus - saccade palsy - gaze palsy - gaze-evoked nystagmus - smooth pursuit

Publication History

Article published online:
09 January 2024

• Literatur

• 1 Strupp M, Brandt T, Dieterich M. Vertigo – Leitsymptom Schwindel. Heidelberg: Springer; 2022. 3. Aufl.
• 2 Eggers SDZ, Bisdorff A, von Brevern M. et al. Classification of vestibular signs and examination techniques: Nystagmus and nystagmus-like movements. J Vestib Res 2019; 29: 57-87
• 3 Choi JH, Jung NY, Kim MJ. et al. Pure upbeat nystagmus in association with bilateral internuclear ophthalmoplegia. J Neurol Sci 2012; 317: 148-150
• 4 Lee SH, Kim JM, Kim JS. Update on the medial longitudinal fasciculus syndrome. Neurological Sciences 2022; 43: 3533-3540
• 5 Kattah JC, Tehrani AS, du Lac S. et al. Conversion of upbeat to downbeat nystagmus in Wernicke encephalopathy. Neurology 2018; 91: 790-796
• 6 Kang S, Shaikh AG. Acquired pendular nystagmus. J Neurol Sci 2017; 375: 8-17
• 7 Jung I, Kim SH, Kim HJ. et al. Modulation of acquired monocular pendular nystagmus in multiple sclerosis: A modeling approach. Prog Brain Res 2019; 249: 227-234
• 8 Lopez LI, Gresty MA, Bronstein AM. et al. Acquired pendular nystagmus: oculomotor and MRI findings. Acta Otolaryngol Suppl 1995; 520: 285-287
• 9 Shaikh AG, Thurtell MJ, Optican LM. et al. Pharmacological tests of hypotheses for acquired pendular nystagmus. Ann N Y Acad Sci 2011; 1233: 320-326
• 10 Averbuch-Heller L, Zivotofsky AZ, Das VE. et al. Investigations of the pathogenesis of acquired pendular nystagmus. Brain 1995; 118: 369-378
• 11 von der Gablentz J, Sprenger A, Heldmann M. et al. Acquired pendular nystagmus and its therapy in progressive supranuclear palsy (PSP) due to inferior olivary hypertrophy. J Neurol 2013; 260: 2424-2426
• 12 Starck M, Albrecht H, Pollmann W. et al. Drug therapy for acquired pendular nystagmus in multiple sclerosis. J Neurol 1997; 244: 9-16
• 13 Starck M, Albrecht H, Pollmann W. et al. Acquired pendular nystagmus in multiple sclerosis: an examiner-blind cross-over treatment study of memantine and gabapentin. J Neurol 2010; 257: 322-327
• 14 Thurtell MJ, Joshi AC, Leone AC. et al. Crossover trial of gabapentin and memantine as treatment for acquired nystagmus. Ann Neurol 2010; 67: 676-680
• 15 Cannon SC, Robinson DA. Loss of the neural integrator of the oculomotor system from brain stem lesions in monkey. J Neurophysiol 1987; 57: 1383-1409
• 16 Helmchen C, Rambold H, Fuhry L. et al. Deficits in vertical and torsional eye movements after uni- and bilateral muscimol inactivation of the interstitial nucleus of Cajal of the alert monkey. Exp Brain Res 1998; 119: 436-452
• 17 Pehere NK, Gofer K. Clinical Reasoning: A 48-year-old man presenting with diplopia. Neurology 2020;
• 18 Zwergal A, Cnyrim C, Arbusow V. et al. Unilateral INO is associated with ocular tilt reaction in pontomesencephalic lesions: INO plus. Neurology 2008; 71: 590-593
• 19 Heide W, Kompf D. Combined deficits of saccades and visuo-spatial orientation after cortical lesions. Exp Brain Res 1998; 123: 164-171
• 20 Averbuch-Heller L, Leigh RJ, Mermelstein V. et al. Ptosis in patients with hemispheric strokes. Neurology 2002; 58: 620-624
• 21 Rambold H, Moser A, Zurowski B. et al. Saccade initiation in ocular motor apraxia. J Neurol 2006; 253: 950-952
• 22 Cazzoli D, Jung S, Nyffeler T. et al. The role of the right frontal eye field in overt visual attention deployment as assessed by free visual exploration. Neuropsychologia 2015; 74: 37-41
• 23 Schweyer K, Busche MA, Hammes J. et al. Pearls & Oy-sters: Ocular motor apraxia as essential differential diagnosis to supranuclear gaze palsy: Eyes up. Neurology 2018; 90: 482-485
• 24 Helmchen C, Koch PJ, Girard G. et al. NPTX1-related oculomotor apraxia: an intra-hemispheric disconnection disorder. J Neurol 2022;
• 25 Kimmig H, Haussmann K, Mergner T. et al. What is pathological with gaze shift fragmentation in Parkinson’s disease?. J Neurol 2002; 249: 683-692
• 26 Pinkhardt EH, Jurgens R, Becker W. et al. Differential diagnostic value of eye movement recording in PSP-parkinsonism, Richardson’s syndrome, and idiopathic Parkinson’s disease. J Neurol 2008; 255: 1916-1925
• 27 Vintonyak O, Gorges M, Muller HP. et al. Patterns of Eye Movement Impairment Correlate with Regional Brain Atrophy in Neurodegenerative Parkinsonism. Neurodegener Dis 2017; 17: 117-126
• 28 Gorges M, Muller HP, Lule D. et al. Functional connectivity within the default mode network is associated with saccadic accuracy in Parkinson’s disease: a resting-state FMRI and videooculographic study. Brain Connect 2013; 3: 265-272
• 29 Rosini F, Federighi P, Pretegiani E. et al. Ocular-Motor Profile and Effects of Memantine in a Familial Form of Adult Cerebellar Ataxia with Slow Saccades and Square Wave Saccadic Intrusions. Plos One 2013;
• 30 Serra A, Liao K, Martinez-Conde S. et al. Suppression of saccadic intrusions in hereditary ataxia by memantine. Neurology 2008; 70: 810-812
• 31 Ashe J, Hain TC, Zee DS. et al. Microsaccadic flutter. Brain 1991; 114: 461-472
• 32 Shaikh AG, Zee DS, Optican LM. et al. The effects of ion channel blockers validate the conductance-based model of saccadic oscillations. Basic and Clinical Ocular Motor and Vestibular Research 2011; 1233: 58-63
• 33 Moretti R, Torre P, Antonello RM. et al. Opsoclonus-myoclonus syndrome: gabapentin as a new therapeutic proposal. Eur J Neurol 2000; 7: 455-456
• 34 Bartos A. Effective high-dose clonazepam treatment in two patients with opsoclonus and myoclonus: GABAergic hypothesis. Eur Neurol 2006; 56: 240-242
• 35 Paliwal VK, Chandra S, Verma R. et al. Clonazepam responsive opsoclonus myoclonus syndrome: additional evidence in favour of fastigial nucleus disinhibition hypothesis?. J Neural Transm 2010; 117: 613-615