Periodic oculomotor palsy

Introduction to periodic oculomotor nerve paralysis Cyclic oculomotor paralysis refers to a rare clinical phenomenon in which the external muscles regularly have numbness or alternating paralysis and paralysis. basic knowledge The proportion of illness: 0.005% Susceptible people: no specific population Mode of infection: non-infectious Complications: ptosis

Periodic oculomotor nerve palsy

(1) Causes of the disease

The cause and damage of this disease have not yet been fully elucidated, so there is a lot of disagreement. Salus speculates that there is a connection between congenital anomalies and the oculomotor nucleus and the periodic motor center, just like the respiratory or vascular motor center. However, it was not confirmed by the autopsy that Axenfeld-Schurenberg believed that it was a periodic vasospasm of the oculomotor nucleus. Kubik believed that periodic movement was an acquired phenomenon, which was the result of residual nucleus hemorrhage and congenital oculomotor nerve paralysis. Duke-Elder believes that the link between neuromuscular nerve paralysis and periodic spasm is that some ganglion cells are still excitatory, and most of the ocular nucleus may be developmental disorders or degeneration (degeneration), and those who help The ganglion cells that participate in the periodic paralysis of the muscles maintain abnormal excitability. Duke-Elder also believes that the cause of sputum seems to be an incomplete disorder of sympathetic nerve fibers from the optic chiasm (optical nerve root) to the sphincter. Extrapyramidal symptoms other than nuclear damage, evidence of nuclear damage in the basal ganglion plane, Lowenstein and Givner used a pupillary to find out that the pupil sphincter had no paralysis, although neither phase responded to light, whereas in both the sacral and cocaine tests, both phases worked.

(two) pathogenesis

In recent years, with the application of electromyography and electron microscopy technology, a new understanding of the pathogenesis of this disease has been made. Loewenfeld and Thompson believe that it is due to the early trauma or infectious disease of the child, affecting the third pair of intracranial Cerebral nerve fibers, caused by degenerative diseases of oculomotor neurons, periodic sputum is caused by the superposition of subthreshold stimuli, resulting in regular discharge, Burian and Van-Allen speculate that the interphalangeal to oculomotor nucleus Due to the regular action of partially denatured ganglion cells, Kommerell et al. used EMG and electron microscopy to observe the upper face of the disease.

The results show that due to the injury of the axons around the oculomotor nerve, the synapses on the normal nucleus are detached from the motor neurons, and there is an abnormal synaptic neuron network with automatic rhythm. This periodic paralysis is congenital. Or the incidence of cerebral palsy in infantile cerebral palsy is higher, probably due to greater plasticity in the synaptic connections during brain development. The test results of Niedman et al suggest that the disease is due to the oculomotor nerve in the subarachnoid space. Peripheral demyelination, a phenomenon of acquired demyelinating damage before or during early childhood, Bateman and Saunders speculate that the lesion of the oculomotor nerve endings caused by an aneurysm on the sphenoid may be the disease Cause.

Periodic oculomotor nerve paralysis prevention

The main purpose is to perform surgery to correct the appearance of the paralyzed phase, but it is impossible to change the periodic changes. Surgery includes eye position correction and ptosis correction.

Cyclic oculomotor palsy complications Complications

It can be accompanied by demyelination changes of other nerves.

Periodic oculomotor nerve paralysis symptoms Common symptoms Disappearance of photodynamic occlusion nerve palsy ptosis

Periodic change

This disease is a group of syndromes in which the ocular and inner muscles of the oculomotor innervation alternate between paralysis and sputum. The periodic changes of paralysis and sputum are mostly at the beginning of the disease, and a few occur after several years of oculomotor nerve palsy., this phenomenon does not change for a lifetime, it can be expressed as the periodic changes of all muscles dictated by the oculomotor nerves, or only the periodic changes of some of the muscles that it dictates. The clinical manifestations can be divided into paralysis and sputum. Generally, one paralysis and sputum cycle occurs every 1 to 4 minutes. The attack time is around the clock and does not stop after sleeping.

(1) Paralysis period:

Or paralyzed phase, the muscles innervated by the affected side of the oculomotor nerve are completely paralyzed, including the ptosis of the upper eyelid, the inner and the lower of the eyeball are restricted, the external rotation is normal or too strong, the eyeball is in an external oblique state, the pupil is dilated, The photoreaction disappears and the regulation of the convergence movement disappears or weakens. The current period lasts for 0.5 to 3 minutes. The paralysis period is often longer than that of the flood season. Especially in the case of long period, the paralysis period can be more than 12 times longer than the flood season, and the period is short. The length is similar, and the EMG examination of the rectus muscle during the paralysis period, the upper diaphragm and the upper and lower rectus muscles have no self-generating position.

(2) Period:

Or called the phlegm phase, after the paralysis period, the drooping upper jaw first twitches, then the eyelids are gradually raised, even the upper side of the diseased side is higher than the healthy side, and when the eye is attempting to look down, the upper jaw remains After the retraction position (ie, the pseudo-Graefe phenomenon), the eye position returns to the ortho or oblique position, the pupil is reduced or even smaller than the healthy side, and the atropine or the posterior masculine eye can be used to enlarge the affected pupil to a great extent, using physostigmine And the pilocarpine acupoints make the pupil shrink to a very small time, the periodic changes of the pupil disappear, cocaine can enlarge the pupil, but the periodic changes still exist, no response to adrenaline, electromyography, visible rectus in the temporal phase Strong action potential, the phase lasts 10~30s, then returns to the paralyzed phase, so the cycle is endless.

2. Visual function change

Most patients suffer from low vision and varying degrees of amblyopia. This type of amblyopia is associated with anisometropia and large astigmatism and early visual deprivation (such as ptosis, hemangioma, etc.).

Periodic oculomotor nerve palsy is not difficult to diagnose because of its typical paralysis and sputum. For patients with complete or partial oculomotor nerve palsy, observe at least 5 minutes to see if there are eyelids, pupils or eyeballs.

Periodic oculomotor nerve palsy

Examination of the nervous system and examination of eye movements.

Vision, eye position, pupillary, and measurement of sputum phase and paralysis phase.

Diagnosis and identification of periodic oculomotor nerve paralysis

Differential diagnosis

1. Oculomotor paralysis regenerating malocclusion syndrome When the oculomotor nerve paralysis is regenerative disorder, the extraocular muscles are dominated by the wrong nerve fibers, which can be accompanied by various eye movements, and there are strange eye movements, which are manifested as eye infections. When the eyelids are retracted or the internal rotation is reversed, the eyelids are retracted or the pupils are reduced. Generally, there is no automatic rhythm change. In some cases, the periodic paralysis and the regenerative misdirection are combined, which is more common in the acute or recovery phase of congenital or traumatic oculomotor nerve paralysis.

2. Marcus-Gunn syndrome, also known as mandibular blink syndrome, was first described by Marcus, Gunn, hence the name, the disease is common in patients with congenital ptosis and superior rectus paralysis, manifested as congenital ptosis Special joint movement with the pterygoid muscle, that is, when the ipsilateral pterygoid muscle is stimulated, the ptosis of the eye and the retraction of the eyelids often occur in the mouth, the mandibular protrusion, the chewing and the extension of the tongue, and the pupil has no abnormal change. The cause may be related to the position of the trigeminal nerve branch to the third cranial nerve innervation.

3.Marin-Amat syndrome, also known as anti-mandibular blink syndrome, is characterized by the appearance of drooping eyelids when opening mouth and jaw movement, and disappearing when the mouth is closed or stopped chewing.

4. ocular myopathy myasthenia gravis This disease is a post-synaptic acetylcholine receptor involved in the neuro-muscle junction, resulting in neuro-muscular excitation transmission disorder, with repeated recurrence and remission tendency Autoimmune diseases, manifested as early ptosis, monocular or binocular, morning light and heavy in the afternoon, late sphincter involvement, numbness of palpebral muscles and strabismus changes, often double vision, injection of neos The above symptoms are alleviated or disappeared after Ming or Teng Xilong.

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