|Year : 1989 | Volume
| Issue : 1 | Page : 30-31
V Menon, AV Arya, Prem Prakash
Dr. Rajendra Prasad Centre for Ophthalmic Sciences , All India Institute of Medical Sciences, , New Delhi 110 029, India
Dr. Rajendra Prasad Centre for Ophthalmic Sciences , All India Institute of Medical Sciences, , New Delhi 110 029
Source of Support: None, Conflict of Interest: None
An unusual case of cyclic pupillary movements in an otherwise complete oculomotor nerve palsy in a five year-old girl is reported. This is considered to be due to destruction of somatic and visceral nuclei of the oculomotor nerve following injury to its fascicular part. Pupillary automatism has been explained on the basis of the presence of aberrant autonomic cells in the ciliary ganglion which are discharging in a regular rhythm independent of higher control.
Keywords: Oculomotor nerve, cyclic oculomotor paresis, "aberrant" ganglion cells, ciliary ganglion, pupil, pupillary automatism.
|How to cite this article:|
Menon V, Arya A V, Prakash P. Pupillary automatism. Indian J Ophthalmol 1989;37:30-1
| Introduction|| |
Pupillary automatism is a rare clinical entity in which the pupil exhibits a regular cycle of mydriasis and miosis irrespective of various physiological stimuli. Similar cyclic pupillary phenomenon has been observed in the pastas a part of either cyclic ophthalmoparesis ,,,, or following periodic sympathetic spasms in degenerative lesions of the spinal cord ,,,. A rare case of this interesting periodic pupillary phenomenon is being reported with a plausible explanation of its origin.
| Case report|| |
A five year old girl was seen in February 1987 at Dr. Rajendra Prasad Centre for Ophthalmic Sciences, New Delhi. She was the first child of the parents and at 1 month of age she had mild fever followed by drooping of the left upper lid and an outward and upward deviation of the left eye which has since then shown no improvement.
General physical examination was normal. Ocular examination revealed ptosis with no levator function and outward and upward deviation of the left eye [Figure - 1]. Except for abduction and intorsion all other movements were restricted. No Marcus Gunn Jaw-Winking phenomenon was observed.
The right eye was essentially normal with a vision of 6/6. The vision in the left eye was finger counting at 1 meter and the eye was amblyopic. The pupil of the left eye which was 7.0 mm in diameter [Figure - 2] showed constriction to 2.2 mm size [Figure - 3] at regular intervals with a pupil cycle time of 35 seconds. The pupil would dilate gradually to its maximum in 20 seconds and then constrict slowly during the following 15 seconds. These cycles of constriction and relaxation occurred with regular rhythm, independent of all stimuli such as light, near reflex and attempted ocular movements and firm closure of the eye lids. Attempting to maintain fixation with either eye also did not change the pupillary cycle. No segmental palsy or spasm of the sphincter of the iris was observed. There was no tonic pupillary near response or light near dissociation. The near point of accommodation could not be ascertained because of severe amblyopia.
During sleep, the right pupil remained smaller than the left pupil but no cyclic phenomenon was observed and slightly painful stimulus applied around the eye initiated the cyclic phenomenon. There was no other neurological deficit or disturbance of sleep rhythm.
Atropine 1% dilated the pupil and interrupted the pupillary cycle. Similarly pilocarpine 2% produced miosis and abolished the pupillary cycle. However the pupillary cycle was maintained on instillation of.125% pilocarpine two drops 30 seconds apart and observed for 45 minutes, suggesting no cholinergic supersensitivity.
Under general anaesthesia, the pupillary cycle was extinguished and the left pupil was dilated to 7.0mm while the right pupil was about 2.0 mm. Both pupils were fixed and nonreactive to light. The child had strabismus surgery and operative procedures did not have any effect on the pupil cycle time.
| Discussion|| |
Pupillary automatism is a rare condition and demands a pathophysiological explanation. The patient who was brought to us by her parents because of the cosmetic blemish of ptosis, squint and immobility of the left eye had fever when she was 1 month old. The fever subsided with systemic antibiotic treatment, however the neural deficit persisted. She was detected to have cyclic dilatation and constriction of the pupil in the affected eye during routine examination. However the extraocular muscles and the lids were never observed to have any rhythmic movements. There was no sign of any injury to other cranial nerves. We are of the view that there was involvement of the fascicular portion of the oculomotor nerve by some infectious process leading to complete permanent nerve damage close to the mid brain with associated retrograde degeneration of neurons of the oculomotor nucleus. There was no sign of any normal ocular movement or "misdirection synkinesia" of any extraocular muscle supplied by the third nerve. Cyclic movements of the pupil in the affected eye were involuntary, not influenced by voluntary gaze efforts and independent of all physiological stimuli. Therefore, it was speculated that cyclic pupillary movements in this case were due to discharges either from normally existing parasympathetic neurons of the ciliary ganglion which had developed their own rhythm following loss of influence of higher centres or from some scattered aberrant neurons present along the course of the oculomotor nerve. Such type of neurons had been demonstrated earlier in the animals . and were known to have the same histochemical characteristics as that of automatic ganglionic neurons. Since such rhythmic pupillary phenomenon has never been observed in otherwise commonly encountered oculornotor dysfunction acquired in the adult life, the regular pupil cycle in this case might be a consequence to rhythmic discharges from some aberrant autonomic neurons.
The other possibility was of rhythmic firing from accessory ganglion cells. The accessory ganglion cells commonly occur along a few short ciliary nerves  and may also be considered aberrant ganglion cells. Due to scattered distribution of the accessory ganglion cells along the short ciliary nerves, autonomy of these neurons will manifest as segmental palsy or spasms of the sphincter muscle. Hence absence of segmental movements of the pupil in this case excluded the possibility of automaticity of the accessory ganglion cells.
Thus we envisaged a collection of aberrant neurons possibly located in the ciliary ganglion. Since structurally the ciliary ganglion is a congregation mainly of parasympathetic neurons which are presumed to have migrated from the mesencephalon during its ontogeny  the aberrant neurons may also be considered as displaced mesencephalic neurons. The axons of these aberrant ganglion cells were innervating the sphincter pupillae and the ciliary muscle. A few impulses from the aberrant neurons are enough to bring about contraction of the sphincter muscle and the ciliary muscle because of smaller mass of these muscles.
It is hence, postulated that in some cases of complete paralysis of the third nerve in early childhood, the effector tissue supplied by the somatic nuclei remains paralysed while the aberrant neurons serve the autonomic functions of the third nerve. The development of automaticity of the aberrant cells may be due to loss of influence of the higher centres and associated denervation supersensitivity. The rhythm of the aberrant cells may be modified during sleep and anaesthesia. The automaticity of these aberrant ganglion cells in ciliary ganglion manifests as the clinical entity of pupillary automatism.
| References|| |
Burian HM & Van Allen MW : Cyclic oculomotor paralysis. Am J Ophthalmol 55 :529-537, 1963.
Bourgan P & Laflamme P : Cited by Thompson HS : 13th pupil colloquium. Am 7 Ophthalmo196 : 100-102,1983.
Lowenfeld IE and Thompson HS : Oculomotor paresis with cyclic spasms. A critical review of the literature and a new case. Surv Ophthalmol 20: 81124, 1975.
Miller NR : Solitary oculomotor nerve palsy in childhood. Am J Ophthalmol 83 :106-111, 1977.
Victor DI : The diagnosis of congenital unilateral third nerve palsy, Brain 99 : 711-718, 1976.
Buscaino GA & Sanna G : Patologica neuromuscolare insolita : IV Su di Un Caso di sindrome irritativa accessuale ntotorio-oculosimpatico (spasmo della mano e mildriasi) omolaterale intermittente da lesione intramidollare. Acta Neurol (Napoli) 27 : 321-339, 1972.
Lowenstein O & Levine AS : Pupillographic studies : V Periodic sympathetic spasm and relaxation and role of sympathetic nervous system in pupillary innervation. Arch Ophthalnol 31 : 74-94, 1944.
Tyrer JH, Sutherland JM & Eadie MJ : Rev Neurol 108 : 329-334, 1963.
Tyrer JH, Sutherland JM & Eadie MS : Rev Neurol 109 : 72-76, 1963.
Carpenter FW : ' The development of the oculomotor nerve, the ciliary ganglion and the abducent nerve in the chick. Bull Comp Zool Harvard Coll 48 : 141-229. 1906.
Stefani FH : Further studies on "aberrant" ganglion cells in the human oculomotor nerve. Ophthal Res 3 : 46-54, 1972.
Wolf GA : The ratio of preganglionic neurons in the visual nervous system. J Comp Neurol 75 235-243, 1941.
Duke-Elder S: Normal and abnormal development in System of Ophthalmology Vol III pt I, 269 CV Mosby Co. St. Louis, 1963.
[Figure - 1], [Figure - 2], [Figure - 3]