Superior oblique myokymia, a review.

PURPOSE OR REVIEW: Superior oblique myokymia is a rare, monocular condition that typically presents in healthy adults. In this article, we review the proposed pathophysiology, describe the typical clinical presentation, and discuss the recommended work up and various treatment options. RECENT FINDINGS: Treatment for this condition ranges from observation and reassurance to various topical and systemic medications. Both strabismus surgery and neurosurgery are reserved for patients who fail medical therapy or are intolerant to medication secondary to side effects. SUMMARY: Due to its episodic nature, SOM is often under-diagnosed. We highlight key features in a patient's clinical history and the examination findings that suggest and support the diagnosis, with review of available treatment options.

Superior oblique myokymia.

Superior oblique myokymia (SOM) is a rare condition of unclear etiology. We discuss the history, etiology, clinical features, differential diagnoses, management, and prognosis of SOM. We conducted a meta-analysis of all 116 cases published since SOM was first described in 1906. The age at examination was 17-72 years (mean: 42 years.) There was a right-sided preponderance in 61% of cases (P < 0.02) that was statistically significant in females (63%, P < 0.04) but not in males (59%, P = 0.18). The pathophysiology of SOM may be neurovascular compression and/or ephaptic transmission. Although various pharmacological and surgical approaches to SOM treatment have been proposed, the rarity of the condition has made it impossible to conduct clinical trials evaluating the safety and efficacy of these approaches. Recently, topical beta blockers have managed SOM symptoms in a number of cases, including the first case treated with levobunolol. Systemic medications, strabismus surgery, and neurosurgery have been used to control symptoms, with strabismus surgery carrying a moderate risk of postoperative diplopia in downgaze. Although there is no established treatment for SOM, we encourage clinicians to attempt topical levobunolol therapy before considering systemic therapy or surgery.

Trochlear pain: clinical characteristics and treatment outcomes.

Trochlear pain is frequently overlooked as published data regarding the clinical characteristic and current treatment are limited. The aim of this study is to evaluate this information from our experiences with trochlear pain. Medical records of 43 patients with trochlear pain from HRH Princess Maha Chakri Sirindhorn Medical Center between November 2010 and April 2017 were reviewed. Most patients were female (88%), with a median age of 51 years. Common characteristic symptoms of trochlear pain were acute, episodic, dull or pressure-like, periorbital pain, often radiating to the forehead, and aggravated by eye movements, especially reading. The causes of trochlear pain were idiopathic or primary trochlear headache (n = 33, 77%) and trochleitis (n = 10, 23%). Treatments included oral NSAIDs or dexamethasone injection into the trochlear region. At a median follow-up of 11 months (range 0-64), 67% of the patients reported complete remission using oral medication. Local steroid injection is useful in non-responding patients to oral therapy with an overall remission of 86%. Successful treatment outcome was achieved in most patients.

Schwannoma of the trochlear nerve-an illustrated case series and a systematic review of management.

Schwannomas of cranial nerves in the absence of systemic neurofibromatosis are relatively rare. Among these, schwannomas of the trochlear nerve are even less common. They can be found incidentally or when they cause diplopia or other significant neurological deficits. Treatment options include observation only, neuro-ophthalmological intervention, and/or neurosurgical management via resection or sterotactic radiosurgery (SRS). In recent years, the latter has become an attractive therapeutic tool for a number of benign skull base neoplasm including a small number of reports on its successful use for trochlear Schwannomas. However, no treatment algorithm for the management of these tumors has been proposed so far. The goal of this manuscript is to illustrate a case series of this rare entity and to suggest a rational treatment algorithm for trochlear schwannomas, based on our institutional experience of recent cases, and a pertinent review of the literature. Including our series of 5 cases, a total of 85 cases reporting on the management of trochlear schwannomas have been published. Of those reported, less than half (40 %) of patients underwent surgical resection, whereas the remainder were managed conservatively or with SRS. Seventy-six percent (65/85) of the entire cohort presented with diplopia, which was the solitary symptom in over half of the cases (n = 39). All patients who presented with symptoms other than diplopia or headaches as solitary symptoms underwent surgical resection. Patients in the non-surgical group were mostly male (M/F = 3.5:1), presented at an older age and had shorter mean diameter (4.6 vs. 30.4 mm, p < 0.0001) when compared to the surgical group. Twelve patients in the entire cohort were treated with SRS, none of whom had undergone surgical resection before or after radiation treatment. Trochlear schwannoma patients without systemic neurofibromatosis are rare and infrequently reported in the literature. Of those, patients harboring symptomatic trochlear Schwannomas do not form a single homogenous group, but fall into two rather distinct subgroups regarding demographics and clinical characteristics. Among those patients in need of intervention, open microsurgical resection as well as less invasive treatment options exist, which all aim at safe relief of symptoms and prevention of progression. Both open microsurgical removal as well as SRS can achieve good long-term local control. Consequently, a tailored multidisciplinary treatment algorithm, based on the individual presentation and tumor configuration, is proposed.

[Conservative management of posttraumatic diplopia]. / Leczenie zachowawcze dwojenia u pacjentów po urazach glowy.

PURPOSE: To determine the effect of prism correction with physicotherapeutic procedures on symptomatic improvement in posttraumatic oculomotor, trochlear and abducent nerves palsy. PATIENTS AND METHODS: Twenty eight patients (five women and twenty three men) at the age range from 23 to 50 years (mean age of 36.5 y.o.) with the posttraumatic ophthalmic complications underwent routine ophthalmic and strabologic examination. Prism correction and vitaminum B1 in iontophoresis were used due to unilateral oculomotor, trochlear and abducent nerve palsy, at 3-11 (mean interval between the trauma and treatment commencement of 7 months) months after traffic accident. Additionally, oculomotor muscles motility training and orthoptic exercises were applied. The duration of therapy ranged from 11 to 18 months (mean treatment duration of 14.5 months). RESULTS: All patients were considered cured at the end of treatment. We achieved the increased ocular range of motion (100%), the reduced horizontal and vertical deviation angle (75%), the extension of palpebral fissure (100%) and regression of diplopia (100%). The binocular vision in the free- and instrument-space environment evaluation improved after treatment (100% simultaneous perception, 100% fusion, 96,43% stereopsis) in 75% and 25% of patients without and with prism correction, respectively. CONCLUSIONS: The use of prism correction led to regression of diplopia, ortophoria without compensatory head position and improved binocular vision function. Prism correction and physicotherapeutic procedures resulted in early improvement of ocular range of motion and accommodation.

Treatment and diagnosis of congenital fourth nerve palsies: an update.

PURPOSE OF REVIEW: This review discusses recent advances in our understanding of the pathology, diagnosis, and treatment of congenital fourth nerve palsies. RECENT FINDINGS: High-definition MRI, which can now more accurately image the trochlear nerve, has increased our understanding of the pathology of fourth nerve palsies. A more sensitive two-step test is proposed to replace the three-step Parks-Bielschowsky test. Procedures strengthening the superior oblique tendon can be performed with a low incidence of secondary Brown syndrome. SUMMARY: Recent MRI studies have shown two pathological mechanisms associated with congenital superior oblique palsies. Superior oblique tuck combined with inferior oblique recession is a very effective way of eliminating head tilt secondary to congenital fourth nerve palsies.

Acquired nonparalytic causes of superior oblique dysfunction.

BACKGROUND AND PURPOSE: To conduct a retrospective study to investigate the causes of acquired superior oblique dysfunction, excluding paralysis, in a consecutive series of adult patients and to compare presenting symptoms and clinical findings. METHODS: A retrospective review of all adult patients with superior oblique dysfunction between the ages of 18 and 80 who met the study profile was conducted at Saint Louis University Medical Center between January 2000 and April 2012. The presenting symptoms, clinical findings, and treatment course for each patient was recorded. The study was approved by the Institutional Review Board of our institution. RESULTS: Acquired forms of nonparalytic superior oblique dysfunction were identified in forty-eight patients. These included superior oblique myokymia (twenty-three patients), superior oblique click syndrome or variable Brown syndrome (nine), canine tooth syndrome (five), spontaneous acquired Brown syndrome (four), iatrogenic or traumatic Brown syndrome (four), and ocular neuromyotonia affecting the superior oblique (three). CONCLUSIONS: Several nonparalytic entities were identified that caused superior oblique dysfunction. Clinical findings may be similar despite entirely different mechanisms. Subjective symptoms may be difficult for the patient to describe or for the examiner to elicit on the day of the examination. Specific techniques can be used in eliciting, differentiating, and documenting the conditions. These included trochlear palpation, modified head tilt technique, interpretation of torsion, and Hess charts.

Bilateral superior oblique palsy: etiology and therapeutic options.

PURPOSE: To study the causes of bilateral superior oblique palsy (BSOP), treatment with botulinum toxin and/or surgery, and outcome of treatment. METHODS: This was an 11-year retrospective study of patients with BSOP treated with injections of botulinum toxin (Botox), surgery, or both. Treatment was considered successful when anomalous head turn and diplopia in primary gaze position and downgaze resolved. RESULTS: Bilateral superior oblique palsy was diagnosed in 12 patients (8 male; mean age, 29.5 years). Palsy was secondary to a neoplasm in 3 cases and to head trauma in 2. In 2 cases, it was ischemic, in 2 it was congenital; the remaining cases were iatrogenic (hydrocephalus secondary to meningitis, 1), hemorrhagic (1), and idiopathic (1). The clinical manifestations recorded were diplopia (10), anomalous head posture (9), V pattern (12), subjective excyclotorsion (8), and objective excyclotorsion (6). Recovery was spontaneous in 1 case with neoplastic disease. Botox was injected in 8 cases (inferior oblique and/or inferior rectus muscles [successful in 2]), and subsequent surgery was required in 6. Two patients underwent surgery without prior injection of Botox. The most common surgical technique was recession of the inferior oblique muscle (6 patients), either as the only operation or associated with other procedures. The final result was good in 72.72% (8/11). Mean follow-up was 62.6 months (range 9-99 months). CONCLUSIONS: Causes of BSOP were varied (most frequently neoplastic). Botox was effective as the only treatment in 25% (2/8). Outcome was good in a high percentage of cases with Botox, surgery, or both.

Success of prisms in the management of diplopia due to fourth nerve palsy.

BACKGROUND: To analyze the success of prism use in alleviating diplopia in patients with fourth nerve palsy and to provide recommendations for prism prescription. METHODS: In this retrospective cohort study, the medical records of 83 patients who were prescribed prisms for symptomatic diplopia due to fourth nerve palsy were analyzed. Data on the nature and duration of diplopia, motility and alignment findings, and amount and type of prism prescribed were recorded. The success of prescribed prismatic correction was assessed by the patient's self-reporting of satisfaction with prism use and follow-up records. The main outcome measure was the satisfaction rate associated with the use of prisms (satisfaction score 1 or 2) in patients with fourth nerve palsy. RESULTS: There were 69 patients with congenital fourth nerve palsy and 14 patients with acquired fourth nerve palsy who received prisms. The mean primary position (± SD) deviation in this group was 7.8 (± 4.6) prism diopters (PD). The mean prism prescription was 6 (± 2.9) PD. Overall, 92% of patients were satisfied with the use of prisms. During the length of follow-up, which ranged from 2 months to more than 6 years (median: 18 months), 86% of the cohort continued using prisms while 14% of patients underwent strabismus surgery. Among 15 patients who had primary position deviation greater than 15 PD, 80% of the patients reported satisfaction with prisms, and in the 11 patients who received 10 PD or more of prismatic correction, 82% were satisfied. CONCLUSION: Prisms are an effective modality for the management of patients with symptomatic diplopia due to fourth nerve palsy. Even in patients with larger deviations including those who were prescribed greater than 10 PD of correction, the success rate of prisms in alleviating diplopia was high. Prisms should be considered as initial therapy in symptomatic patients with fourth nerve palsy.

Recovery of ophthalmoplegia after endovascular treatment of intracranial aneurysms.

BACKGROUND AND PURPOSE: Recovery of aneurysm induced CNP after endosaccular coiling has been reported in the literature. The aim of this study was to assess in detail the parameters that affect the outcome after endovascular treatment of ophthalmoplegic aneurysms due CNP. MATERIALS AND METHODS: Between November 1999 and March 2008, 30 consecutive patients (8 men, 22 women; mean age, 54.9 years) presenting with CNP underwent endosaccular coiling with or without additional use of stents in the parent artery. Subarachnoid hemorrhage was present in 10 patients, whereas 20 patients had unruptured aneurysms. The mean size of the aneurysms was 10 mm. Initial CNP was complete in 11 patients and partial in 19. Mean follow-up after coiling was 19 months. RESULTS: The mean interval between the onset of CNP and aneurysm embolization was 48 days. Fifteen patients (50%) had complete recovery of oculomotor function, 12 had incomplete recovery (40%), and 3 (10%) remained unchanged after treatment. In 4 aneurysms (13.3%), 1 additional embolization was performed, whereas in 4 other aneurysms, 2 additional embolization procedures were necessary. Procedure-related permanent morbidity occurred in 2 patients (6.6%). CONCLUSIONS: Endosaccular coiling is an effective and safe method for the treatment of ophthalmoplegic aneurysms. Age, neck size, and time of treatment do not seem to constitute prognostic factors with respect to CNP recovery, though patients with small aneurysms, unruptured status, and/or location in the posterior circulation showed a tendency for better outcome. The degree of initial CNP was the only statistically significant prognostic factor concerning the final outcome, resulting in better recovery, in case of incomplete initial CNP.

Paralytic strabismus: third, fourth, and sixth nerve palsy.

Eye movement abnormalities constitute an important clinical sign that can be a manifestation of dysfunction of cranial nerves III, IV, and VI (the 3 ocular motor nerves). Specific motility deficits often have highly localizing value within the neuroaxis, serving to refine a differential diagnosis and guide management. This article reviews the key anatomic concepts, clinical presentation, differential diagnosis, and management of ocular motor nerve palsies. Dysfunction of an ocular motor nerve must be distinguished from other causes of abnormal eye movements, such as myasthenia gravis or thyroid eye disease, which are outside the scope of this article.

Clinical course and prognosis of trochlear nerve schwannomas.

PURPOSE: To delineate the disease course and prognosis of patients with mass lesions of the fourth nerve presumed to be schwannomas. DESIGN: Nonrandomized retrospective case series. PARTICIPANTS: Thirty-seven consecutive cases of presumed trochlear nerve schwannoma from 9 tertiary university neuro-ophthalmology centers. METHODS: Cases were collected, and their clinical characteristics on presentation and follow-up are described. Inclusion criteria were brain magnetic resonance imaging (MRI) with a lesion suggestive of a schwannoma along the course of the fourth nerve. Exclusion criteria were other causes of fourth nerve palsy, such as congenital, traumatic or microvascular; normal (or lack of) initial brain MRI; lack of adequate clinical information; and disappearance of the lesion on subsequent follow-up brain MRI. MAIN OUTCOME MEASURES: Demographics of patients, presence of neurofibromatosis, symptoms on presentation, vertical deviation, lesion size (on presentation and follow-up), length of follow-up, and outcomes of treatment for lesions or diplopia. RESULTS: Seven patients were excluded and of the 30 patients included in our series, patients were predominantly male (77%) with a mean age of 51 years (range 9-102 years). In contrast with prior case reports, almost all of our cases had a fourth nerve palsy on presentation (29/30), often isolated. Mean follow-up was 3.1 years (range 0.2 months to 11.1 years). There was no significant difference between initial and follow-up lesion size (4.4 vs. 5 mm) for patients who did not receive treatment of lesions (P = 0.36). Only 3 patients underwent neurosurgical resection and an additional patient received gamma-knife radiotherapy. The majority of patients (24/30) did not pursue strabismus surgery for vertical diplopia. CONCLUSIONS: Patients with isolated fourth nerve palsy and small lesions of the fourth nerve have a good prognosis and should be followed with serial MRI scans without neurosurgical intervention unless they develop signs of brain stem compression. Most patients with diplopia and benign fourth nerve lesions typical of trochlear nerve schwannoma can adapt with either prism spectacles or no treatment at all, although strabismus surgery can be successful.

Ocular motor nerve palsies: implications for diagnosis and mechanisms of repair.

Measurements of the dynamics of the eyes in ocular motor nerve palsies may aid diagnosis, characterize peripheral and central palsies, and reveal adaptive properties of the brain. Saccadic and vestibulo-ocular reflex (VOR) functions of patients with peripheral and central sixth, and peripheral third and fourth nerve palsies were studied by three dimensional magnetic field search coil oculography. Combined third and fourth cranial nerve microvascular ischaemic palsy in diabetes mellitus produced low ratios of intorsion to adduction amplitudes. Presumed isolated third nerve palsy caused higher ratios of adduction to intorsion and violations of Listing's law. The VOR in third, fourth, and sixth nerve palsies reveals adaptive equilibration of the action of paretic agonist and their non-paretic antagonist muscles in violation of Hering's law during head motion. Saccadic speeds in the field of paretic agonists are repaired in chronic peripheral palsies despite limited ductions, but remain reduced in central palsies. Limited intorsion with third nerve palsy is attributed to concurrent fourth nerve ischaemia in the distribution of the inferolateral trunk of the intracavernous carotid artery. Adaptive repair of the VOR after ocular motor nerve palsies reduces asymmetric retinal image slip and binocular disparity, and repair of saccadic velocity drives both eyes rapidly and simultaneously into the paretic field of motion.

[Oculomotor, trochlear, and abducens nerve palsies]. / Paresen okulomotorischer Hirnnerven.

Patients with oculomotor, trochlear, or abducens nerve palsies mainly complain of binocular double vision, but sometimes merely of blurred vision or vertigo. The clinical signs comprise strabismus, pathologic head posture, and disturbed saccades. The characteristic motility deficits are picked up by measuring the strabismic angles at different directions of gaze. Documentation of all three spatial strabismic components is advantageous. Nonparetic strabismus and orbital diseases are important differential diagnoses. Conclusions about the cause of a palsy can be drawn from the time course of double vision, the character of associated headaches, the patient's general risk factors, and the examination of vicinal structures. Imaging studies are indicated when the ischemic nature of the palsy is in doubt. Therapeutic strategies include prisms, occlusion, and eye-muscle surgery.

Primary trochlear headache and other trochlear painful disorders.

The trochlear region is a source of distinct pain that may give rise to specific primary pain disorders (primary trochlear headache), but also modulate other pre-existing headache disorders such as migraine. The sensory innervation of this region, by a branch of the ophthalmic division of the trigeminal nerve, may explain the modulatory influence of the nociceptive afferents of this region over migraine headache. We propose the term "trochlear migraine" to refer to the coexistence of strictly unilateral migraine and ipsilateral trochleodynia, with the improvement of migraine being dependent on the resolution of the trochleodynia. Trochleitis is an inflammatory trochleodynia, being frequently idiopathic and rarely secondary to usually immunologic and rheumatologic disorders. We postulate that nociceptive afferents from the inner part of the orbit may sustain the activation of trigeminal neurons, thus sensitizing or exacerbating migraine. Decreasing the possible wind-up induced from this nociceptive afferent stimulation may be effective in controlling headache.

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.

The Triple Forced Duction Test(s) for diagnosis and treatment of superior oblique palsy -- with an updated flow chart for unilateral superior oblique palsy.

PURPOSE: To review and update the management of superior oblique extraocular muscle clinical paresis and palsy, (SOP) employing and applying recent advances in the diagnosis and surgical methods. METHODS: These include three recently introduced forced duction tests, respectively for laxity of the SO tendon, absence of the SO tendon, and contracture of the ipsilateral superior rectus muscle. Also discussed are the pathophysiologic mechanisms behind various modes of clinical presentation of SOP, older concepts requiring scrutiny, and prior surgical methods which should no longer be employed due to advances in our knowledge. RESULTS AND CONCLUSIONS: These newer aspects of SOP management are organized and displayed in a revised Plager flow sheet to facilitate their application.