Superior oblique myokymia

Superior oblique myokymia (SOM) is an uncommon, monocular movement disorder involving rapid torsional, low-amplitude contractions of the superior oblique muscle that causes monocular oscillopsia and diplopia. Ocular and neurologic examination in these patients is usually normal, and the clinical course is characterized by exacerbation remissions with good response to medical treatment. In this review, we present recent advances in the pathogenesis of SOM and provide an algorithm for the investigation and management of these patients.     

Superior oblique myokymia: magnetic resonance imaging support for the neurovascular compression hypothesis

Superior oblique myokymia is a rare movement disorder thought to be caused by vascular compression of the trochlear nerve. Direct display of such neurovascular compression by magnetic resonance imaging has been lacking. The goal of this study was to assess the presence of neurovascular contacts in patients with superior oblique myokymia, using a specific magnetic resonance imaging protocol. A total of 6 patients suffering from right superior oblique myokymia underwent detailed neuro-ophthalmological examination, which showed tonic or phasic eye movement. All patients underwent magnetic resonance imaging, using a magnetic resonance imaging Fourier transform constructive interference in steady-state sequence in combination with magnetic resonance imaging time of flight magnetic resonance arteriography both before and after the administration of Gd-DTPA. With this protocol, the trochlear nerve could be visualized on 11 of 12 sides (92%). Arterial contact was detected at the root exit zone of the symptomatic right trochlear nerve in all 6 patients (100%). No arterial contact was identified at the root exit zone of the asymptomatic left trochlear nerve in any of the 5 left nerves visualized. In conclusion, superior oblique myokymia can result from neurovascular contact at the root exit zone of trochlear nerve, and therefore should be considered among the neurovascular compression syndromes.     

Contrapulsion of saccades and ipsilateral ataxia: a unilateral disorder of the rostral cerebellum

Contralateral pulsion of saccades and ipsilateral limb ataxia were manifestations of unilateral damage to the rostral cerebellum studied in a patient with occlusion of one superior cerebellar artery. The saccadic disorder consisted of three elements: horizontal saccades away from the lesion during attempted vertical saccades, resulting in oblique trajectories; hypermetria of contralateral saccades; and hypometria of ipsilateral saccades. Magnetic search coil oculography showed that durations of the horizontal components of oblique contrapulsive saccades were lengthened toward the durations of the vertical components. Lengthening of horizontal vectors indicated temporal coupling of the orthogonal components, as occurs in normal oblique saccades. The bias of saccades arose proximal to brainstem loci that decompose commands for oblique saccades into their horizontal and vertical vectors. Contrapulsion of saccades may be explained by imbalanced cerebellar outflow.     

Superior oblique myokymia

Superior oblique myokymia is an unusual eye movement phenomenon generally considered to be a uniocular rotary microtremor easily treated with carbamazepine. Eleven new cases are presented, one with a unique combination of motility problems, emphasizing that the disorder may have varying clinical manifestations. Long-term follow-up of the patients revealed a natural history of recurrent spontaneous remissions and relapses. Treatment with carbamazepine was of short-term benefit in most patients but failed to affect the long-term course of the disease. No patient developed other evidence of neurological disease. The condition seems to be a benign, permanent condition limited in its manifestations to the superior oblique muscle.     

Isolated superior oblique palsies with electrophysiologically documented brainstem lesions

Over a 13.5-year period, we observed 10 patients with isolated superior oblique palsies in whom electrophysiological abnormalities indicated brainstem lesions. In 7 patients unilateral masseter reflex abnormalities were seen, and were located on the side of the superior oblique palsy in 2 patients and on the opposite side in 5 patients. Two patients had slowed gain of following eye movements to the side contralateral to the superior oblique palsy. Slowed adduction saccades in the eye contralateral to the superior oblique palsy were seen in 1 patient. Clinical improvement was frequently (in 7 of 10 patients) associated with improvement or normalization of electrophysiologic findings. Magnetic resonance imaging (MRI) was normal, showing no evidence of brainstem lesions in 6 patients. Unilateral superior oblique palsy may be the only clinical sign of a brainstem lesion. Although such a cause may be underdiagnosed if based on MRI-documented lesions only, it remains a rare condition.     

Transsynaptic retrograde transport of fragment C of tetanus toxin demonstrated by immunohistochemical localization

Injection of the non-toxic fragment C of tetanus toxin into the superior oblique muscle of the eye results in strong direct retrograde labelling of the motoneurons in the contralateral trochlear nucleus and clear transsynaptic labelling of neurons in the ipsilateral and contralateral vestibular nuclei. Standard immunohistochemical procedures using a monoclonal antibody localize fragment C in the brain with high sensitivity and excellent resolution. An injection of fragment C into the superior oblique muscle labels the same pool of trochlear motoneurons as an HRP injection into the superior oblique muscle. A comparison of the fragment C labelling of trochlear motoneurons with intracellular injections of HRP into trochlear motoneurons suggests that fragment C stains not just the soma, but also the distal dendrites of motoneurons. Moreover, a fragment C injection into the superior oblique muscle labels transsynaptically more neurons in each vestibular nucleus than an injection of HRP into the trochlear nucleus labels directly.     

Superior oblique palsy in a patient with a history of perineural spread from a periorbital squamous cell carcinoma.

A 74-year-old man experienced vertical diplopia. Two years earlier, he was diagnosed with a squamous cell carcinoma of the periorbital frontal skin, with perineural spread involving the ophthalmic division of the right trigeminal nerve and the right facial nerve. The clinical findings were consistent with a right fourth cranial nerve palsy. Computerized tomography and magnetic resonance imaging demonstrated a discrete mass involving the belly of the right superior oblique muscle. An anterior orbitotomy and biopsy demonstrated a mass extending into the belly of the superior oblique muscle. Histology revealed an infiltrating squamous cell carcinoma. The possibility of perineural, direct, or metastatic spread to the superior oblique muscle should be considered in a patient with a history of squamous cell carcinoma of the head and neck. The authors believe this case to be the first report of superior oblique underaction due to involvement of the muscle by squamous cell carcinoma, presumably because of perineural spread. Diagnosis was made possible by neuroimaging and histopathology. There was good short-term resolution of the patient's diplopia after radiotherapy.     

Quantitative study of muscle spindles in suboccipital muscles of human foetuses.

The proprioceptive inputs from the cervical musculature play an important role in head-eye co-ordination and postural processes. Deep cervical muscles in humans are shown to have high spindle content. The density, distribution and morphology of muscle spindles were studied in superior oblique capitis, inferior oblique capitis and rectus capitis posterior major and minor three small suboccipital muscles. The muscles were obtained, post-mortem from stillborn human foetus. The spindle density was calculated as the ratio of mean spindle content to the mean wet weight of that muscle in grams. The distribution and arrangement of spindles within the muscle and their arrangement was studied. The spindle density of superior oblique muscle was found to be 190, that of inferior oblique was 242 and the rectus capitis posterior contained 98 spindles per gram of muscle. No tendon organs were seen. The serial transverse sections of inferior oblique muscle revealed muscle spindles of varying sizes, length varying between 100-650 microns and, diameter 50-250 microns. A complex parallel arrangements of group of large spindles were seen in the belly of the inferior oblique muscle, while the polar regions contain few small isolated spindles. The relevance of such high spindle receptor content in these tiny muscles is discussed.     

Vertical saccades in superior oblique palsy and Brown's syndrome.

OBJECTIVE: To compare saccadic dynamics in superior oblique palsy and Brown's syndrome. METHODS: Vertical saccades in adduction and in abduction were studied in two subjects with superior oblique palsy and one with Brown's syndrome. Using large numbers of centrifugal saccades over a wide range of amplitudes, we measured peak velocity, duration, and the peak velocity/mean velocity ratio (PV/MV) as a function of saccadic amplitude. We compared vertical saccades in 30 degrees of abduction with those in 30 degrees of adduction. RESULTS: Superior oblique palsy caused a 15-18% reduction in peak velocities in adduction compared with abduction. Saccadic duration was also increased in adduction, with the result that there was no net change in the PV/MV ratio. In the patient with Brown's syndrome, velocities and durations of upward saccades were similar in abduction and adduction, but the PV/MV ratio was significantly elevated in adduction. We also observed an unusual high-speed lateral 'snap' of about 5 degrees that frequently interrupted vertical saccades in the midline but not elsewhere. CONCLUSION: Both paresis and restriction of the superior oblique alter vertical saccades. The effects of restriction on saccadic dynamics are distinct from the effects of paresis.     

Superior oblique myokymia caused by vascular compression.

A 49-year-old man had left superior oblique myokymia for eight years. Magnetic resonance images with enhanced spoiled gradient recalled acquisition in the steady state (SPGR) and flow imaging using steady acquisition (FIESTA) disclosed a branch of the superior cerebellar artery lying on the root exit zone of the left trochlear nerve. Posterior fossa craniotomy confirmed the imaging findings. A Teflon pad was placed between the compressing artery and the trochlear nerve. The patient's superior oblique myokymia has completely resolved with a one-year follow-up. Only one such case has been previously reported. This is the first report to display the imaging findings.     

Horizontal canal input to cat extraocular motoneurons

Synaptic potentials were recorded in identified extraocular motoneurons in the anesthetized cat, following stimulation of the horizontal canal nerve (HCN). Weak stimulation of the HCN evoked disynaptic EPSPs in ipsilateral medial rectus (i-MR), contralateral lateral rectus (c-LR) and disynaptic IPSPs in i-LR motoneurons. Weak stimulation of the HCN produced longer latency IPSPs (probably trisynaptic) in c-MR motoneurons. It is suggested that the HCN projects to an excitatory interneuron in the vestibular nucleus whose axon in turn projects to a third order inhibitory interneuron in the IIIrd nucleus which finally projects to c-MR motoneurons. Essentially there is no influence of the HCN stimulation on bilateral superior rectus (SR), inferior rectus (IR), superior oblique (SO) and inferior oblique (IO) motoneurons.     

Reinnervation of the extraocular muscles in goldfish is nonselective

The selectivity of axonal regeneration to the extraocular muscles in teleosts has been reinvestigated by mapping, with retrogradely transported HRP, the motor pools of the muscles innervated by the oculomotor nerve. In normal goldfish, the motoneurons of the superior rectus, inferior rectus, and inferior oblique muscles formed discrete, nonoverlapping motor pools; the motor pool of the medial rectus muscle overlapped with those of the inferior oblique and inferior rectus muscles. In fish whose oculomotor nerve had regenerated (after intracranial transection), in contrast, many motoneurons in other, inappropriate motor pools reinnervated the superior rectus and inferior oblique muscles (the only muscles examined in lesioned animals). Furthermore, these inappropriate motoneurons continued to project to these muscles for at least 1 year. The oculomotor nerve and its molecular branches were examined by light and electron microscopy to determine the pathway by which axons regenerated to their muscles. Axons regenerated within the basal laminae of Schwann cells, which persisted in the distal nerve-stump after a lesion. After labeling the inferior oblique nerve with HRP in regenerated nerves, there were labeled axons in all of the muscular branches; this indicates that regenerating axons branched, which was confirmed by finding an increased number of myelinated axons in other, regenerated inferior oblique nerves. Thus, different branches of the same axons sometimes reinnervated different muscles. These results demonstrate that regenerating axons in the oculomotor nerve are misdirected to inappropriate muscles, and do not selectively reinnervate individual muscles, as had been previously suggested (Sperry and Arora, 1965).     

Inferior oblique muscle paresis as a sign of myasthenia gravis

Myasthenia gravis may affect any of the six extra-ocular muscles, masquerading as any type of ocular motor pathology. The frequency of involvement of each muscle is not well established in the medical literature. This study was designed to determine whether a specific muscle or combination of muscles tends to be predominantly affected. This retrospective review included 30 patients with a clinical diagnosis of myasthenia gravis who had extra-ocular muscle involvement with diplopia at presentation. The diagnosis was confirmed by at least one of the following tests: Tensilon test, acetylcholine receptor antibodies, thymoma on chest CT scan, or suggestive electromyography. Frequency of involvement of each muscle in this cohort was inferior oblique 19 (63.3%), lateral rectus nine (30%), superior rectus four (13.3%), inferior rectus six (20%), medial rectus four (13.3%), and superior oblique three (10%). The inferior oblique was involved more often than any other muscle (p < 0.01). Eighteen (60%) patients had ptosis, six (20%) of whom had bilateral ptosis. Diagnosing myasthenia gravis can be difficult, because the disease may mimic every pupil-sparing pattern of ocular misalignment. In addition diplopia caused by paresis of the inferior oblique muscle is rarely encountered (other than as a part of oculomotor nerve palsy). Hence, when a patient presents with vertical diplopia resulting from an isolated inferior oblique palsy, myasthenic etiology should be highly suspected.     

Location of motoneurons in the oculomotor nucleus and the course of their axons in the oculomotor nerve

Subdivisions of the oculomotor nucleus, and the course of axons in the brainstem and more peripherally in the oculomotor nerve of the cat, were studied by directly applying horseradish peroxidase solution to the transected nerve-branch stump in the orbit. The medial rectus subdivision consisted of two subgroups, and intermingling between subdivisions was found. About 20% of the motoneurons controlling the medial rectus muscle were scattered in the medial longitudinal fasciculus or a more ventrolateral area. A few motoneurons controlling the inferior rectus or inferior oblique muscle were also located in the medial longitudinal fasciculus. Axons to the superior branch that supplied the superior rectus and levator muscle coursed in the dorsolateral half of the oculomotor nerve. In contrast, those to the medial rectus, inferior rectus, and inferior oblique muscles were scattered diffusely in the oculomotor nerve.     

Isolated superior oblique palsies with brainstem lesions.

Isolated unilateral superior oblique palsies resulting from brainstem lesions occurred in three patients. MRI documented contralateral tegmental lesions of the trochlear nucleus and adjacent intraaxial trochlear nerve. Lacunar infarct was the cause in two patients and a small hemorrhage in a third.     

Vertical oscillopsia in bilateral superior canal dehiscence syndrome

A patient sought treatment for vertical oscillopsia and impaired vision during locomotion, and unsteadiness of gait. Positive fistula tests and CT of the temporal bones confirmed a diagnosis of bilateral superior canal dehiscence. An impairment of the superior canal vestibulo-ocular reflex, documented by three-dimensional search coil eye movement recordings for oblique (single) and downward pitch head motion (bilateral canal testing), is proposed to induce vertical rather than torsional-vertical oscillopsia during locomotion.     

Peripheral and central oculomotor organization in the goldfish, Carassius auratus

Peripheral and central oculomotor organization was studied in the goldfish. The sizes of the extraocular muscles were quantified by counting the fibers contained in a given muscle and by area measurements of the cross-sectional surfaces. All the muscles were of approximately similar size. Kinematics were determined by electrical stimulation of a given muscle. The macroscopic appearance and kinematics of the muscles had the characteristics of other lateral-eyed animals (e.g., rabbit). Locations of extraocular motor neurons were found by retrograde transport of horseradish peroxidase (HRP) following injections into individual extraocular muscles. The eye muscles were innervated by four ipsilateral (lateral rectus, medial rectus, inferior oblique, inferior rectus) and two contralateral (superior rectus, superior oblique) motor neuron pools. The oculomotor nucleus was found in the midbrain, at the level of the caudal zone of the inferior lobe of the hypothalamus. Inferior rectus motor neurons were located rostrally in the oculomotor nucleus, whereas medial rectus, superior rectus, and inferior oblique motor neurons were intermingled in its more caudal portions. All labelled cells were located dorsally and medially to the medial longitudinal fasciculus (MLF) in close proximity to either the floor of the ventricle or the midline region. Occasionally, motor neurons were interspersed within the fiber bundles of the MLF or the exiting fibers of the oculomotor nerve. The trochlear nucleus, containing superior oblique motor neurons, was found in the immediate lateral and caudal neighborhood of the oculomotor nucleus, where its rostral border overlapped with the caudal border of the latter. The abducens nucleus, containing lateral rectus motor neurons, was located in the posterior brainstem in the neighborhood of the vestibular nuclear complex. This nucleus was divided into a rostral and a caudal portion. The axons of ipsilaterally projecting motor neurons headed toward their respective nerve roots via the shortest possible route, as did the axons of superior rectus motor neurons, which crossed the midline without detour to enter the contralateral oculomotor nerve. In contrast, trochlear motor neuron axons arched around the dorsal aspect of the ventricle through the cerebellar commissure to reach the contralateral trochlear nerve. The morphology of individual motor neurons was visualized by intrasomatic injection of HRP. Cell somata had oblong shapes, and their large dendrites were oriented laterally and ventrally. The axons did not collateralize within the midbrain region or the oculomotor nerve as far as they could be traced.(ABSTRACT TRUNCATED AT 400 WORDS)     

Pharmacology of vertigo/nystagmus/oscillopsia

PURPOSE OF REVIEW: To describe recent developments in the pharmacological treatment of vertigo and nystagmus while focusing on vestibular neuritis, Meniere's disease, downbeat nystagmus, periodic alternating nystagmus, acquired pendular nystagmus, and superior oblique myokymia. RECENT FINDINGS: In the last 2 years several studies have been published on possible pharmacological treatment options for nystagmus and oscillopsia. In the treatment of vestibular neuritis two studies showed that cortisone treatment was effective for restoring labyrinthine function. This benefit seems more likely if treatment is started within the first 2 days of onset. For recurrent vertigo attacks due to Meniere's disease, the titration technique with daily or weekly doses of intratympanic gentamicin until onset of vestibular symptoms, change in vertigo or hearing loss rated best for complete vertigo control. A new pharmacological treatment option for downbeat nystagmus is the administration of potassium channel blockers (e.g. 4-aminopyridine). They are thought to reinforce the inhibitory action of cerebellar Purkinje cells. Several case reports have proven the beneficial effect of baclofen on periodic alternating nystagmus, of gabapentin and memantine on acquired pendular nystagmus, and of carbamazepine and gabapentin on superior oblique myokymia. SUMMARY: There have been several new developments in the treatment of nystagmus and vertigo over the last 2 years. These include potassium channel blockers for the treatment of downbeat nystagmus, early cortisone treatment to improve recovery of the labyrinth function in vestibular neuritis, and intratympanic gentamicin treatment for Meniere's disease. Other pharmacological treatment options are baclofen for periodic alternating nystagmus, gabapentin and memantine for acquired pendular nystagmus, and carbamazepine for superior oblique myokymia.     

Role of the trochlear nerve in eye abduction and frontal vision of the red‐eared slider turtle (Trachemys scripta elegans)

Horizontal head rotation evokes significant responses from trochlear motoneurons of turtle that suggests they have a functional role in abduction of the eyes like that in frontal‐eyed mammals. The finding is unexpected given that the turtle is generally considered lateral‐eyed and assumed to have eye movements instead like that of lateral‐eyed mammals, in which innervation of the superior oblique muscle by the trochlear nerve (nIV) produces intorsion, elevation, and adduction (not abduction). Using an isolated turtle head preparation with the brain removed, glass suction electrodes were used to stimulate nIV with trains of current pulses. Eyes were monitored via an infrared camera with the head placed in a gimble to quantify eye rotations and their directions. Stimulations of nIV evoked intorsion, elevation, and abduction. Dissection of the superior oblique muscle identified lines of action and a location of insertion on the eye, which supported kinematics evoked by nIV stimulation. Eye positions in alert behaving turtles with their head extended were compared with that when their heads were retracted in the carapace. When the head was retracted, there was a reduction in interpupillary distance and an increase in binocular overlap. Occlusion of peripheral fields by the carapace forces the turtle to a more frontal‐eyed state, perhaps the reason for the action of abduction by the superior oblique muscle. These findings support why trochlear motoneurons in turtle respond in the same way as abducens motoneurons to horizontal rotations, an unusual characteristic of vestibulo‐ocular physiology in comparison with other mammalian lateral‐eyed species. J. Comp. Neurol. 521:3464‐3477, 2013. © 2013 Wiley Periodicals, Inc.     

Evaluation of hippocampal infolding using magnetic resonance imaging

To investigate developmental morphological variation of the hippocampal formation, we evaluated the degree of hippocampal infolding in cross-sectional oblique coronal images of the cerebral peduncle and the superior cerebellar peduncle. We defined the hippocampal infolding angle as the angle between the vertical midline and the straight line connecting the medial superior margin of the subiculum with the lateral margin of the cornu ammonis. The angle increased slightly with age, and was larger in the superior cerebellar peduncle than in the cerebral peduncle and larger in the right superior cerebellar peduncle than in the left superior cerebellar peduncle. This suggests that this angle and its variation with age and location merit our attention in morphological evaluation of the hippocampal formation.