J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.35.5.739 on 1 October 1972. Downloaded from Journal ofNeurology, Neurosurgery, and Psychiatry, 1972, 35, 739-742

Ocular bobbing and opsoclonus Two abnormal spontaneous eye movements occurring in the same patient: case report

H. G. BODDIE From the Department of Neurology, North Staffordshire Hospital Centre, Stoke-on-Trent

SUMMARY A case in which two rare abnormal spontaneous eye movements, ocular bobbing and opsoclonus, were observed, is reported. Their pathophysiology and distinction from other abnormal spontaneous eye movements are discussed.

Ocular bobbing is a term first used by Fisher discusses briefly the pathophysiology of ocular (1961) to describe distinctive, abnormal, spon- bobbing and opsoclonus, and their differentiation taneous eye movements occurring in comatose from other abnormal spontaneous eye move- patients with paralysis of horizontal conjugate ments. gaze. The movements consist of an abrupt con- Protected by copyright. jugate downward jerk of the eyes followed by a CASE REPORT slow return to the mid position. The usual cause or infarction. Since A 39 year old female was admitted as an emergency is a pontine haemorrhage on 19 September 1970. Essential hypertension had then, additional reports have further defined the been diagnosed six months previously, and out- clinical spectrum of ocular bobbing (Fisher, patient follow-up showed satisfactory control of 1964; Daroff and Waldman, 1965; Hameroff, blood pressure on propanolol 20 mg t.d.s. For six Garcia-Mullin, and Eckholdt, 1969; Nelson and weeks before admission she had noticed intermittent Johnston, 1970; Susac, Hoyt, Daroff, and headaches of an indeterminate nature. On the day of Lawrence, 1970). admission she had complained of severe frontal head- Opsoclonus is a term first used by Orzechowski aches and had collapsed at work. and Walichiewicz (1913) and Orzechowski (1927) On admission she was unconscious with no re- to describe constant, conjugate, chaotic agitation sponse to painful stimuli; the blood pressure was with a 140/110 mm Hg; respirations were of the Cheyne- of the eyes seen in patients non-epidemic Stokes pattern. General examination was otherwise form of encephalitis. The movements were

normal. There was no neck stiffness and the fundi http://jnnp.bmj.com/ characterized by periods of violent ocular mo- were normal. The pupils were 3 mm in diameter, bility alternating with periods of relative stillness; equal and reacting briskly to direct light, the con- they were present during eye closure and sleep; sensual light reflex being normal. Comeal reflexes they were not rhythmical and occurred in the were absent. There was a left-sided hypertonia and horizontal and vertical planes. Associated abnor- hyperreflexia with bilateral extensor plantar malities were myoclonic movements of the face responses. and limbs, and cerebellar incoordination. Since The most striking abnormality present was the the original description, additional reports have disturbance of ocular movements known as ocular on September 25, 2021 by guest. to be a distinct bobbing, brisk conjugate downward jerks of the eyes further delineated what appears followed by a slow return to the mid position at a entity (Marmion and Sandilands, 1947; Cogan, rate of two to four per minute. No spontaneous hori- 1954, 1968a; Smith and Walsh, 1960). zontal eye movements were present, nor did caloric The occurrence of ocular bobbing and opso- stimulation (ice-water at 00 C) induce reflex eye clonus in the same patient has not been reported movements, or influence the rate of bobbing. The previously. This paper presents such a case, and oculocephalic reflex was absent. 739 J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.35.5.739 on 1 October 1972. Downloaded from 740 H. G. Boddie

INVESTIGATIONS Complete blood counts, urinalysis, synchronous with the associated myoclonic move- serum electrolytes, protein electrophoresis, calcium, ments. Despite the severe neurological deficit, the phosphate, alkaline phosphatase, cholesterol, trans- patient went home in May 1971, and when seen in aminases, WR and Kahn tests, 24 hour urinary urea outpatients in October 1971, the signs were un- and electrolytes, creatinine clearance, skull, and changed. chest radiographs were normal. An electrocardio- gram showed changes consistent with pre-existing hypertension. Lumbar puncture showed a uniformly DISCUSSION bloodstained cerebrospinal fluid under a pressure of In the past 25 years a variety of rare abnormal 150 mm water. Xanthochromia was present; total protein content was 80 mg/100 ml. The CSF con- spontaneous eye movements have been increas- tained 3 nucleated cells/cu. mm with 9,400 red blood ingly reported in disorders of the brain-stem, cells/cu. mm. An electroencephalogram on the day cerebellum, and their connections. In some in- of admission was reported as follows (Dr. Rosemary stances their recognition has been of precise Cooper): there was a symmetrical 8-10 Hz dominant localizing value, but in others has led to semantic post-central rhythmic activity with forward spread. confusion. The spectrum includes, besides ocular There was a moderate amount of background slow bobbing and opsoclonus, down-beat nystagmus, activity in the theta range with some delta compo- ocular myoclonus, 'lightning eye movements', nents seen diffusely but maximally posteriorly. There ocular dysmetria, and ocular flutter. was no evidence of asymmetry of frequency or ampli- tude. The patient was unconscious at the time of Cogan (1968b) defined down-beat nystagmus recording and showed no EEG or behavioural as nystagmus in which the fast component is change to arousal stimuli. Conclusion: this is an downwards. Eight of his 27 cases were associated interesting record with its total disassociation of the with radiological evidence of congenital anoma- clinical and EEG state. The lies at the level of the foramen magnum, with or presence of a symmetri- Protected by copyright. cal dominant post-central rhythm in the alpha range without the Arnold-Chiari malformation. The with this level of consciousness strongly suggests a diagnosis in the rest covered a miscellany of con- pontine (tegmental) disturbance. The presumptive ditions which had in common symptoms and diagnosis was that of a pontine haemorrhage secon- signs suggestive of a lesion in the pons, medulla, dary to hypertension. Contrast radiographic studies or cerebellum. It is from were not done. readily distinguished During the first 72 hours after admission, the ocular bobbing by its regularity, more rapid patient remained comatose and the ocular bobbing rate, and the associated clinical picture. persisted at a rate of two to four per minute. During Ocular myoclonus describes the ocular move- the fourth day after admission, her level of con- ments seen in palato-pharyngo-laryngo-oculo- sciousness lightened, and the ocular bobbing de- diaphragmatic myoclonus. These movements are creased in frequency, and finally disappeared on the pendular, rhythmical, and synchronous with the fifth day. She remained in a steady state up to myoclonic movements of associated structures. December 1970, since when there has been a slow Yap, Mayo, and Barron (1968) using a poly- but steady improvement to reach her present clinical graphic showed this to be state, which was achieved in May 1971. She is men- recording technique tally alert and oriented with a marked cerebellar true but nevertheless described the movements http://jnnp.bmj.com/ dysarthria and ataxia in all four limbs, predomin- as 'ocular bobbing', thus leading, as pointed antly left-sided. The hyperreflexia persists on the out by Susac et al. (1970) to predictable confu- left, but both plantar responses are flexor. Myo- sion. Atkin and Bender (1964) described three clonic movements of the face, palate, platysma, patients in whom abnormal eye movements were sternomastoids, and upper limbs are present. These seen, which they called 'lightning eye move- movements are irregular, not rhythmical and occur ments'. These were characterized by rapid bursts at a rate varying between 80 and 120 per minute. to-and-fro of horizontal eye movements, the on September 25, 2021 by guest. Opsoclonus-that is, constant, conjugate, chaotic bursts consisting of small conjugate saccades. movements of the eyes-is present predominantly in there was no evidence of the vertical plane but also with some movement in Although myoclonic the horizontal plane. Its rate varies from 80 to 180 movements in associated structures, they used per minute; the movements are present with eye the term ocular myoclonus synonymously with closure and persist during sleep, although their fre- 'lightning eye movements'. quency and severity are diminished. They are not In ocular dysmetria, overshooting of the eyes J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.35.5.739 on 1 October 1972. Downloaded from Ocular bobbing and opsoclonus 741 on attempted fixation is followed by several In their clinical experience of nine cases they cycles of diminishing amplitude until precise added a further dimension to the syndrome by fixation is achieved. Both this and ocular flutter, classifying the cases of ocular bobbing as (1) the occurrence of intermittent to-and-fro oscilla- 'typical '-abrupt, spontaneous downward jerks tions of the eyes lasting a few seconds which is of the eyes with a slow return to the mid position often spontaneous and precipitated by changes associated with paralysis of horizontal conjugate in fixation, are seen primarily in cerebellar dis- eye movements; (2) 'monocular' (or paretic) ease. Their differentiation from the abnormal reflecting a coexisting unilateral third nerve spontaneous ocular movements under discussion paresis in addition to the bobbing; and (3) has not given rise to difficulty. '(atypical '-either a variation unexplained by Opsoclonus-that is, constant, conjugate, associated oculomotor palsy, or bobbing with chaotic movement of the eyes, has been con- intact spontaneous or reflex horizontal eye move- sidered by authors to be a distinctive abnormal ments. Since their report, ocular bobbing has spontaneous eye movement seen in encephalitis also been reported in a case of Leigh's necrotizing affecting the brain-stem. The clinical syndrome encephalomyopathy (Borit, 1971). consists of the onset of a mild non-specific infec- The diffuse and varied nature of the underlying tion accompanied by fever and malaise, which pathological processes in the post mortem studies is followed about 10 days later by opsoclonus, of cases of opsoclonus and ocular bobbing have cerebellar signs, and myoclonic movements of effectively rendered any precise assessment of the face, neck, and upper limbs. Sometimes there the pathophysiology uncertain. The patient is a pleocytosis in the cerebrospinal fluid. The described here presented with typical ocular prognosis is good, complete recovery in two bobbing and subsequently developed opsoclonus. months occurring in most cases. However, the The available clinical evidence at the time of Protected by copyright. fact that opsoclonus, although most commonly admission (pre-existing hypertension and hae- seen in the syndrome described, has been re- morrhagic cerebrospinal fluid), suggested that ported in other diseases of the central nervous there had been an intrapontine or cerebellar system (Halliday, 1967) limits its usefulness with haemorrhage. The development in sequence of regard to the underlying pathology. More these abnormal eye movements suggests that recently its association with malignancy has these two, at least, have a common pathological been reported (Ross and Zeman, 1967; Solomon background capable of producing one or other and Chutorian, 1968). of these movements. Typical ocular bobbing-that is, abrupt con- jugate, downward jerking of the eyes followed I am grateful to Dr. E. C. Hutchinson for his helpful by a slow return to the mid position and accom- criticism of this paper. panied by paresis of horizontal conjugate gaze- is a valuable clinical sign which is easy to define REFERENCES and recognize. Its importance lies in the fact that Atkin, A., and Bender, M. B. (1964). 'Lightning eye move- the available pathological evidence indicates that ments' (Ocular myoclonus). Journal of the Neurological http://jnnp.bmj.com/ Sciences, 1, 2-12. the underlying lesion is almost always pontine, Borit, A. (1971). Leigh's necrotizing encephalomyelopathy. and commonly a haemorrhage or infarct. Susac Neuro-ophthalmological abnormalities. Archives of Oph- et al. (1970) reviewed the literature on 25 repor- thalmology, 85, 438-442. Cogan, D. G. (1954). Ocular dysmetria; flutter-like oscilla- ted cases, of whom four survived. Of the 21 who tions of the eyes, and opsoclonus. Archives of Ophthal- died, post mortem studies were performed on 18, mology, 51, 318-335. the cause of death being pontine haemorrhage in Cogan, D. G. (1968a). Opsoclonus, body tremulousness, and benign encephalitis. Archives of Ophthalmology, 79, 545- nine; two cases each of pontine infarction and 551. on September 25, 2021 by guest. cerebellar haemorrhage; one case each of pon- Cogan, D. G. (1968b). Down-beat nystagmus. Archives of Ophthalmology, 80, 757-768. tine glioma, post-traumatic lesions of cortex, Daroff, R. B., and Waldman, A. L. (1965). Ocular bobbing. brain-stem, and cerebellum, haemorrhagic in- Journal of Neurology, Neurosurgery, and Psychiatry, 28, farction of cerebellum, metabolic encephalo- 375-377. Fisher, C. M. (1961). In Pathogenesis and Treatment of Cere- pathy, and a patient with presumptive vertebro- brovascular Disease. Edited by W. S. Fields. Thomas: basilar ischaemia. Springfield, Ill. J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.35.5.739 on 1 October 1972. Downloaded from 742 H. G. Boddie

Fisher, C. M. (1964). Ocular bobbing. Archives ofNeurology, cyst of the cerebellum.] Livowski Tygodnik Lekarski, 8, 11, 543-546. 219-227. (In Polish.) Halliday, A. M. (1967). The clinical incidence of myoclonus. Ross, A. T., and Zeman, W. (1967). Opsoclonus, occult In Modern Trends in Neurology, 4, pp. 69-105. Edited by carcinoma, and chemical pathology in dentate nuclei. D. Williams. Butterworths: London. Archives of Neurology, 17, 546-551. Hameroff, S. B., Garcia-Mullin, R., and Eckholdt, J. (1969). Smith, J. L., and Walsh, F. B. (1960). Opsoclonus-ataxic Ocular bobbing. Archives of Ophthalmology, 82, 774-780. conjugate movements of the eyes. Archives of Ophthal- Marmion, D. E., and Sandilands, J. (1947). Opsoclonia. A mology, 64, 244-250. rare sign in polioencephalitis. Lancet, 2, 508-509. Solomon, G. E., and Chutorian, A. M. (1968). Opsoclonus and occult neuroblastoma. New England Journal of Medi- Nelson, J. R., and Johnston, C. H. (1970). Ocular bobbing. cine, 279, 475-477. Archives of Neurology, 22, 348-356. Susac, J. O., Hoyt, W. F., Daroff, R. B., and Lawrence, W. Orzechowski, K. (1927). De l'ataxie dysmetrique des yeux: (1970). Clinical spectrum of ocular bobbing. Journal of remarques sur l'ataxie des yeux dite myoclonique (opso- Neurology, Neurosurgery, and Psychiatry, 33, 771-775. clonie, opsochorie). Jolurnalfiir Psychologie uind Neurologie, Yap, C.-B., Mayo, C., and Barron, K. (1968). 'Ocular bob- 35, 1-18. bing' in palatal myoclonus. Archives of Neutrology, 18, Orzechowski, K., and Walichiewicz, T. (1913). [Operated 304-310. Protected by copyright. http://jnnp.bmj.com/ on September 25, 2021 by guest.