| Identifier |
Upbeat_Nystagmus |
| Title |
Upbeat Nystagmus |
| Creator |
Shirley H. Wray, MD, PhD, FRCP |
| Affiliation |
(SHW) Professor of Neurology, Harvard Medical School; Director, Unit for Neurovisual Disorders, Massachusetts General Hospital, Boston, Massachusetts |
| Subject |
Upbeat Nystagmus; Saccadic Hypermetria; Saccadic Pursuit; Square Wave Jerks; Oscillopsia; Cerebellar Astrocytoma; Primary Position Upbeat Nystagmus; Horizontal Saccadic Dysmetria |
| History |
The patient, a 36 year old Italian, presented in October 1967, at the age of 27, with acute dizziness and ataxia. He was evaluated in Rome. A pneumoencephalogram showed hydrocephalus, attributed to arachnoiditis, and a ventriculo-atrial shunt was placed. Three months post shunt placement he had a return of dizziness and ataxia accompanied by daily bi-occipital headache, diplopia, clumsiness and weakness of the left arm. In January 1968, he was readmitted and a large tumor of the left cerebellar hemisphere with invasion of the vermis was biopsied. A diagnosis of glioblastoma multiforme was made. He was treated with radiation therapy. Post operatively his major deficits were ocular dysmetria, left limb and gait ataxia. In 1970 he immigrated to the US. In 1971 he was referred to Dr. William Sweet, Chief of Neurosurgery at the Massachusetts General Hospital (MGH) with increasing gait ataxia and oscillopsia. Neurological examination: Cranial nerves intact (apart from the eye movements) Motor strength normal Decreased tone in the left extremities Reflexes 2+ throughout with flexor/plantar responses. Coordination: ataxia left extremities Gait ataxia, Romberg negative. Sensory examination normal. Neuro-ophthalmological examination: Visual acuity 20/20 OU Visual fields and pupils normal Fundus exam showed bilateral optic disc drusen Eye movements: • Upbeat nystagmus in primary gaze, increased on up gaze • Square wave jerks • Horizontal gaze evoked nystagmus left > right • Full vertical gaze • Upbeat nystagmus on upgaze • No nystagmus on downgaze • Pursuit, (horizontal and vertical) smooth to a very slow target, markedly saccadic to a fast target • Convergence normal • Marked saccadic hypermetria Right gaze to center overshoot (hypermetria) taking the eyes almost fully to the left Left gaze to center hypermetria taking the eyes almost fully to the right Upgaze to center hypermetria Downgaze to center hypermetria CT scan: In the region of the vermis and the medial portion of the left hemisphere there was an area of high absorption with contrast enhancement involving the cerebellar vermis and medial left cerebellar hemisphere with an apparent associated cystic component consistent with recurrent tumor. Cerebral Arteriogram: A cerebral arteriogram confirmed a highly vascular tumor of the inferior portion of the midline of the cerebellum, bulging into the fourth ventricle. Pathology: The slides from the original biopsy were obtained from Rome and reviewed by Dr E. P. Richardson, Chief of Neuropathology at the MGH. Diagnosis: A high-grade astrocytoma grade 3-4. Surgical procedure: A posterior fossa craniotomy was performed. To access the tumor, the most inferior aspect of the vermis was split to reach the roof of the 4th ventricle. In so doing, a well demarcated tumor nodule was found extending to both sides of the cerebellum, mostly to the left side. It was possible to separate the superior most aspect of the tumor easily from the 4th ventricle. Several small branches of the posterior inferior cerebellar arteries had to be divided to devascularize the tumor which was then gently dissected away from the floor of the 4th ventricle. At the end, the surgeon felt he had as close to a grossly total removal of the tumor as was possible and no visible tumor was left behind. Post operative status: The patient made a good post-operative recovery and was able to walk with an elbow crutch. He continued to be followed by neurosurgery until he was 66 years of age. During that time he had required a shunt revision on two occasions and therapy for an episode of meningitis/cerebritis. |
| Anatomy |
Anatomical localization bilateral post-surgical damage to the FOR. |
| Pathology |
Cerebellar astrocytoma |
| Disease/Diagnosis |
Cerebellar astrocytoma. |
| Clinical |
This patient, post-resection of a cerebellar astrocytoma had • Upbeat nystagmus in primary gaze, increased on up gaze • Square wave jerks • Horizontal gaze evoked nystagmus left > right • Full vertical gaze • Upbeat nystagmus on upgaze • No nystagmus on downgaze • Pursuit, (horizontal and vertical) smooth to a very slow target, markedly saccadic to a fast target • Convergence normal • Marked saccadic hypermetria Right gaze to center (hypermetria) taking the eyes almost fully to the left Left gaze to center (hypermetria) taking the eyes almost fully to the right Upgaze to center (hypermetria) Downgaze to center overshoot (hypermetria) Upbeat nystagmus in central position can be seen with tumors of the medulla, cerebellum or midbrain. (See Table 10-2 Etiology of Upbeat Nystagmus Pg 485 (8)) The clinical features of upbeat nystagmus illustrated in this case are 1. Present in central gaze and increased on looking up. 2. Poorly suppressed by visual fixation of a distant target 3. Convergence suppressed the upbeat nystagmus 4. Upbeat nystagmus, in this case, was associated with abnormal vertical vestibular smooth pursuit responses, and saccadic intrusions (square wave jerks), which, on eye movement recording, produce a "bow tie" nystagmus (See Box 10-3 Clinical Features of Upbeat Nystagmus. Pg 487(8)) Lesions of the dorsal vermis and fastigial nuclei: Experimental lesions of the dorsal vermis and fatigial nuclei (fastigial oculomotor region - FOR) cause saccadic dysmetria. Typically hypometria if the vermis alone is involved. Typically hypermetria if the deep nuclei are involved. Dorsal vermis lesions also produce deficits of smooth pursuit, especially at its onset. In this post-operative patient damage to the FOR region is probably bilateral. Leigh and Zee published an MRI of a 50 year old man who had undergone resection of a cystic astrocytoma, with a surgical lesion involving the vestibular nuclei. Similarly in this patient, the main oculomotor deficit was saccadic hypermetria. (8) Square wave jerks are pairs of small horizontal saccades (typically less than 2° ) that take the eye away from the target and then return it within 200 ms. In certain cerebellar syndromes, square wave jerks may occur almost continuously as in this case, and have been called square wave oscillations. These oscillations may be mistaken for nystagmus. |
| Presenting Symptom |
Unsteady gait |
| Ocular Movements |
Upbeat Nystagmus; Saccadic Hypermetria; Saccadic Pursuit; Square Wave Jerks |
| Neuroimaging |
There are no imaging studies available in this patient. |
| Treatment |
Surgical: See above. Vertical smooth pursuit is abnormal in patients with upbeat nystagmus as in this case. Treatment with the potassium channel blocker, 4-aminopyridine, has led to improved pursuit and better fixation suppression of the upbeat nystagmus. ( 7) When saccadic hypermetria is marked, as in this case, with macrosaccadic oscillations (square wave jerks), in patients with deep cerebellar lesions, an attempt has been made to reduce the movements with drugs such as diazepam, clonazepam and barbituates. |
| Etiology |
Cerebellar astrocytoma |
| References |
1. Abel LA, Traccis S, Dell'Osso LF, Daroff RB, Troost BT. Square wave oscillation: the relationship of saccadic intrusions and oscillations. Neuroophthalmol 1984;4:21-25. 2. Baloh RW, Yee RD. Spontaneous vertical nystagmus. Rev Neurol (Paris). 1989;145(8-9):527-32. http://www.ncbi.nlm.nih.gov/pubmed/2682931 3. Benjamin EE, Zimmerman CF, Troost BT. Lateropulsion and upbeat nystagmus are manifestations of central vestibular dysfunction. Arch Neurol. 1986 Sep;43(9):962-4. http://www.ncbi.nlm.nih.gov/pubmed/3488729 4. Elliott AJ, Simpson EM, Oakhill A, Decock R. Nystagmus after medulloblastoma. Developmental Medicine and Child Neurology 1989;391:43-46. http://www.ncbi.nlm.nih.gov/pubmed/2920872 5. Fisher A, Gresty M, Chambers B, Rudge P. Primary position upbeating nystagmus. A variety of central positional nystagmus. Brain. 1983 Dec;106 ( Pt 4):949-64. http://www.ncbi.nlm.nih.gov/pubmed/6606479 6. Gilman N. Baloh RW. Primary position upbeat nystagmus. Neurology 1977;27:294-297. http://www.ncbi.nlm.nih.gov/pubmed/557768 7. Glasauer S. Kalla R, Büttner U, Strupp M, Brandt T. 4-aminopyridine restores visual ocular motor function in upbeat nystagmus. J Neurol Neurosurg Psychiatry 2005;76:451-453. http://www.ncbi.nlm.nih.gov/pubmed/15716550 8. Leigh RJ, Zee DS. Diagnosis of Nystagmus and Saccadic Intruction. Chp 10:475-558. In: The Neurology of Eye Movements 4th Edition. Oxford University Press, New York 2006. 9. Nakada T, Remler MP. Primary position upbeat nystagmus; another central vestibular nystagmus? J Clin Neuroophthalmol 1981;1:181-185. http://www.ncbi.nlm.nih.gov/pubmed/6213659 10. Rabiah PK, Bateman JB, DemerJL, Perlman S. Ophthalmologic findings in patients with cerebellar ataxia. Am J Ophthalmol0 1997:123:108-117. http://www.ncbi.nlm.nih.gov/pubmed/9186105 11. Robinson FR, Straube A, Fuchs AF. Role of the caudal fastigial nucleus in saccade generation. II Effects of muscimol inactivation. J Neurophysiol 1993;70:1741-1758. http://www.ncbi.nlm.nih.gov/pubmed/8294950 12. Sibony PA, Evinger C, Manning KA. Tobacco-induced primary-position upbeat nystagmus. Ann Neurol. 1987 Jan;21(1):53-8. http://www.ncbi.nlm.nih.gov/pubmed/3827215 13. Takagi M, Zee DS, Tamargo R. Effects of lesions of the oculomotor vermis on eye movements in primate: saccades. J Neurophysiol 1998;80:1911-1930. http://www.ncbi.nlm.nih.gov/pubmed/9772249 14. Takagi M, Zee DS, Tamargo R. Effects of lesions of the oculomotor cerebellar vermis on eye movements in primate: smooth pursuit. J Neurophysiol 2000; 83:2047-2062. http://www.ncbi.nlm.nih.gov/pubmed/10758115 15. Tilikete C, Hermier M, Pelisson D, Vighetto A. Saccadic lateropulsion and upbeat nystagmus: disorders of caudal medulla. Ann Neurol. 2002 Nov;52(5):658-62. http://www.ncbi.nlm.nih.gov/pubmed/12402267 16. Traccis S, rosati G, Aiello Iet al. Upbeat nystagmus as an early sign of cerebellar astrocytoma. J Neurol 1989;236:359-360. http://www.ncbi.nlm.nih.gov/pubmed/2795105 17. Troost BT, Martinez J, Abel LA, Heros RC. Upbeat nystagmus and internuclear ophthalmoplegia with brainstem glioma. Arch Neurol 1980; 37:453-456. http://www.ncbi.nlm.nih.gov/pubmed/7387494 18. Tychsen L, Sitaram N. Catecholamine depletion produces irrepressible saccadic eye movements in normal humans. Ann Neurol 1989:25:444-449. http://www.ncbi.nlm.nih.gov/pubmed/2570547 19. Weissman JD, Seidman SH, Dell'Osso LF, Naheedy MH, Leigh RJ. Torsional see-saw "bow-tie" nystagmus in association with brain stem anomalies. Neuroophthalmol 1990;10:315-318. |
| Language |
eng |
| Format |
application/pdf |
| Format Creation |
Microsoft PowerPoint |
| Type |
Text |
| Relation is Part of |
917-5; 942-3 |
| Collection |
Neuro-Ophthalmology Virtual Education Library: Shirley H. Wray Collection: https://novel.utah.edu/Wray/ |
| Publisher |
North American Neuro-Ophthalmology Society |
| Holding Institution |
Spencer S. Eccles Health Sciences Library, University of Utah |
| Rights Management |
Copyright 2002. For further information regarding the rights to this collection, please visit: https://NOVEL.utah.edu/about/copyright |
| ARK |
ark:/87278/s6saz8ps |
| Setname |
ehsl_novel_shw |
| ID |
2174244 |
| Reference URL |
https://collections.lib.utah.edu/ark:/87278/s6saz8ps |
