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Autoimmune nodopathy (AN) is a group of peripheral neuropathies caused by antibodies targeting the nodes of Ranvier or paranodes. It typically presents with sensory ataxia, distal limb weakness, and tremor, and often has a subacute onset, with limited response to immunoglobulin or corticosteroids. We report a case of anti-contactin-1 neuropathy initially manifesting as isolated superior oblique palsy, aiming to broaden the clinical spectrum of the disease. A 68-year-old male with well-controlled diabetes, hypertension, and hyperlipidemia developed acute binocular vertical diplopia, progressing over two months to include distal paresthesia, sensory ataxia, ageusia, and dysarthria. Concurrent nephrotic syndrome was identified. Nerve conduction studies supported demyelination. Despite treatment with intravenous methylprednisolone followed by long-term immunosuppression, some disability persisted. Serum archived during his admission tested positive for anti-contactin-1 IgG, with IgG4 as the predominant subclass, in the flow cytometry assay for AN. This case extends the clinical spectrum of AN. Some cases of isolated cranial nerve palsies, especially in the relevant context like nephrotic syndrome, may be attributed to AN. Prompt initiation of more effective therapies, such as rituximab, could significantly improve outcomes., Competing Interests: Declaration of competing interest The authors report no conflict of interest in connection with this article., (Copyright © 2024 Elsevier B.V. All rights reserved.)

The efficacy of botulinum toxin injection for the treatment of third, fourth, and sixth nerve palsy was evaluated. PubMed, Scopus, EMBASE, Web of Science, and Google Scholar databases were searched. Data about the duration of palsy (acute vs chronic), cause of the palsy, type of toxin used, mean dose, and other background characteristics were collected. Outcome variables were success rate (defined by alleviation of diplopia or reduction in eye deviation) and standardized mean difference of prism diopter and abduction deficit before and after injection. The Joanna Briggs Institute checklist was implemented for the risk of bias assessment. The analysis included 38 articles, comprising 643 patients. The overall treatment success rate in acute and chronic nerve palsy was 79% and 33%, respectively. The success rate was not significantly different between different subgroups of age, type of botulinum toxin, pre-injection prism diopter, etiology of the palsy, duration of follow-up, and mean dose of botulinum toxin injection. However, in both acute and chronic palsy, diabetes etiology was accompanied by the highest success rate. Overall symptomatic response to botulinum injection was 84% (95% CI: 67% to 96%), whereas functional response was observed in 64% (95% CI: 47% to 79%) of the patients. The odds ratio for the success rate of treatment of palsies with botulinum toxin versus expectant management was 2.67 (95% CI: 1.12 to 6.36) for acute palsy and 0.87 (95% CI: 0.17 to 4.42) for chronic palsy. Botulinum toxin can be used for the treatment of acute third, fourth, and sixth nerve palsy, especially in patients with acute palsy and more severe tropia. [ J Pediatr Ophthalmol Strabismus . 2024;61(3):160-171.] .

Competing Interests: The authors report no conflicts of interest.

Since COVID-19 was first reported, different neurological complications have been acknowledged, but their description is constantly evolving. We report a case of concurrent tonic pupil and trochlear nerve palsy in this context. A 62-year-old man reported a 5-day history of binocular vertical diplopia and blurred vision in his left eye, noticing that his left pupil was dilated. He had suffered a flu-like syndrome 2 weeks before. Clinical exam showed a right trochlear nerve palsy and a left mydriatic pupil. MRI, X chest ray, and analytical results were normal. Antibodies for SARS-CoV-2 were positive (low IgM and high IgG titers). Antiganglioside antibodies were negative. A 0.125% pilocarpine test confirmed Adie's pupil diagnosis. The patient was treated with a tapered prednisone dose with resolution of his diplopia but no change in Adie's pupil. This is the first case reporting Adie's pupil as a postinfectious manifestation of COVID-19. An immune-mediated mechanism is presumed.

Superior oblique myokymia (SOM) is an uncommon condition of unclear etiology that results in episodes of oscillopsia and diplopia. There is no established treatment protocol for SOM. We present 2 cases of SOM successfully managed with topical levobunolol 0.5%; both patients responded to a short course of medication administration and required minimal ongoing therapy. Case 1 was a 69-year-old woman with left SOM who had previously undergone a left Harada-Ito procedure. Her SOM improved immediately on administration of levobunolol and was maintained at follow-up 1 year later. Case 2 was a 49-year-old man with right SOM that affected his ability to work. After 2 days of topical levobunolol 0.5% nightly in the right eye, SOM episodes ceased; he continues to use drops intermittently for occasional recurrences., (Copyright © 2017 American Association for Pediatric Ophthalmology and Strabismus. Published by Elsevier Inc. All rights reserved.)

Purpose: To evaluate the outcomes of early intra-trochlear steroid injections in patients with acquired Brown syndrome secondary to trochleitis (ABSST)., Methods: Retrospective analysis from medical charts of patients diagnosed as affected by unilateral ABSST from January 2008 to June 2015, and treated according to our Institution protocol: intra-trochlear injection of 1ml of triamcinolone acetonide 40 mg/ml is performed under sterile conditions. In cases of no resolution/improvement within 1 month, further monthly injections are performed up to a maximum number of three. Non-responder patients after three injections undergo recession of the superior oblique muscle., Results: Thirteen patients were diagnosed as affected by unilateral ABSST, and were included in the analysis (seven F, six M; median age at diagnosis 30.38 ± 25.56 years). The mean time interval from ABSST diagnosis to the first steroid injection was 7.84 ± 5.40 days (range 2-17). After a median number of 1.30 injections per patient, 11 patients (84.6% of the total) showed complete remission of symptoms and signs within 22.45 ± 13.85 days after the first injection. None of these responder patients referred to diplopia in primary gaze after injections. The remaining two non-responder patients after three injections underwent superior oblique muscle recession of 8.0 mm., Conclusions: Early intra-trochlear steroid injections are effective in patients with acquired Brown syndrome secondary to trochleitis, leading to a complete recovery of signs and symptoms in the majority of treated patients. Surgical treatment should be limited only to patients non-responding to serial steroid injections.

Herpes zoster ophthalmicus can be associated with a variety of ocular and visual sequelae, including isolated or even multiple cranial neuropathies, potentially affecting the oculomotor, trochlear, or abducens nerves. We report a case of a secondary Brown syndrome following resolution of a unilateral isolated trochlear nerve palsy associated with herpes zoster ophthalmicus in an immunocompetent 57-year-old man., (Published by Elsevier Inc.)

Superior oblique myokymia is a rare condition, characterized by spontaneous rhythmic contractions of the superior oblique muscle and was first described by Duane in 1906. However, the pathophysiology of this condition remains poorly understood even today. A number of medical and surgical treatment modalities have been tried, with variable results. We report a case of superior oblique myokymia in a pregnant female, which could be triggered with flashlight stimulation, and the result of treatment with timolol maleate 0.5% ophthalmic solution., Competing Interests: There are no conflicts of interest.

Background: Tuberculosis is prevalent in China, which is the second greatest contributor to the global tuberculosis burden. Tuberculosis meningitis (TBM) is the most severe disease form but few reports describe long-term clinical outcomes and prognostic factors. Thus, we studied these features in Chinese TBM patients., Methods: A retrospective follow-up study was used to collect clinical features and outcomes of adult TB meningitis at the First Affiliated Hospital of Chongqing Medical University from June 2012 to August 2015. Univariate analysis and multivariate analysis were used to identify predictive factors associated with outcomes at discharge and follow-up., Results: TBM patients (N = 154) were a median age of 41 years (range: 16-82 years). Median time to follow-up was 26.4 months (range: 9.3-46.5 months) and 31% had poor outcomes at follow-up and limb weakness (p = 0.016), lower GCS scores (p < 0.001), cranial-nerve palsy (p = 0.024), and hydrocephalus (p = 0.009) were closely associated with these poor outcomes. Furthermore, a high neutrophil to lymphocytes ratio, high D-dimer, a low albumin to globulin ratio and slow background of EEG associated with poor outcomes as well., Conclusions: Mortality and disability associated with TBM are high in China. Limb weakness, GCS scores, cranial-nerve palsy and hydrocephalus were independent predictors of poor outcomes, and AGR, NLR, D-dimer, and EEG abnormalities may be prognostic factors of TBM.

Background and Purpose: To study the clinical characteristics, treatment options, and outcome of patients with trochleitis in our population., Methods: Retrospective review of 59 patients diagnosed with trochleitis in the Ramon y Cajal Hospital Emergency Service between 2003 and 2010. Demographic data and trochleitis features were described. The relationship between outcome and treatment options was analyzed by SPSS., Results: The estimated prevalence rate of trochleitis in our area was 12 per 100,000. The average age of patients was 43±18 years. The majority of cases were women (86%). One case was bilateral. Patients' chief complaints were continuous pain (66%), pain only with ocular movements (25%), or pain only with palpation (8%). Ocular movement limitations were presented in 14%. Diplopia was observed in 12%, and 19% complained of headache. Oral non-steroidal anti-inflammatory drugs (NSAIDs) were the first option for treatment in 85% of cases, associated with oral steroids in 8% of patients. Oral steroids were the first and only option for treatment in 3%. Symptoms completely resolved in 80%, with the worst responses seen in cases with motility disturbances. Peritrochlear triamcinolone acetonide was injected in 14% of cases, achieving a good response in 62%., Conclusions: The prevalence of trochleitis in our area is low, and this pathology is more frequent in females. Oral NSAIDs are efficient to resolve isolated pain, but the response is partial if diplopia or motility limitations are associated. Some non-responders achieved good results with peritrochlear triamcinolone. Successful management provides a good prognosis for most patients.

Purpose: To evaluate the utility of botulinum toxin injection into the inferior oblique muscle for secondary inferior oblique muscle overaction., Methods: A retrospective review of 18 patients and 23 injections performed over a 9-year period. Indications and deviations in primary position and contralateral gaze before and after injection were recorded. Functional outcomes and further management (conservative vs surgical) were observed., Results: In 14 patients, chemodenervation resulted in a temporary improvement in symptoms. Eleven of these patients went on to have inferior oblique myectomy with resolution of their diplopia. Two patients preferred to receive regular injections of botulinum toxin as a treatment., Conclusions: Botulinum toxin chemodenervation of the inferior oblique muscle in cases of secondary inferior oblique muscle overaction is useful where one needs to establish a risk of overcorrection following planned inferior oblique muscle weakening. This is particularly true in cases where the primary position deviation may be small but symptoms of diplopia exist on contralateral side gaze, giving rise to a narrowed field of binocular single vision., (Copyright 2016, SLACK Incorporated.)

Herpes zoster ophthalmicus is rare in healthy children. It is occasionally associated with extraocular muscle palsies and rarely with isolated trochlear nerve palsy. We report a case of unilateral isolated trochlear nerve palsy associated with herpes zoster ophthalmicus in an immunocompetent 13-year-old girl who presented with diplopia and blurred vision in her right eye. The right cornea had multiple subepithelial opacities. Ocular motility returned to normal and diplopia and corneal opacification resolved with steroid therapy. To our knowledge, this is the first such case involving the troclear nerve in a child., (Copyright © 2014 American Association for Pediatric Ophthalmology and Strabismus. Published by Mosby, Inc. All rights reserved.)

Purpose: To assess the suitability of galantamine for the symptomatic treatment of post-traumatic oculomotor (III) and trochlear (IV) nerve palsy., Material and Methods: The routine ophthalmic and strabological examination was performed in five patients (4 females and 1 male) at the age of 31 to 57 years (mean 40.7) with the post-traumatic ophthalmic complications. Due to the unilateral oculomotor and trochlear nerve palsy, which had not resolved within 2-6 (mean duration of 4 months) months following traffic accident, galantamine was used. Nivalin and Reminyl were administered in iontophoresis and orally, respectively, for 10-18 months (mean duration of 14 months). The ocular muscle motion exercises and prism correction were also used., Results: The increased range of ocular motion (100%), reducing of the angle of strabismus horizontally (40%) and vertically (60%), statistically significant extension of palpebral fissure (60%), and regression of diplopia (80% total without correction) were observed. The binocular vision after treatment in the free- and instrument-space environment were also improved (100% simultaneous perception, fusion 80%, stereopsis 60%)., Conclusions: The early galantamine administration in patient with n. III and n. IV post-traumatic palsy accelerates the resolution of post-traumatic ophthalmic symptoms. It is an effective treatment which offers the elimination of strabismus, diplopia and ptosis, at the same time improvings ocular movements and binocular vision. galantamine, post-traumatic nerve palsy, oculomotor and trochlear nerves.

Introduction: Superior oblique myokymia (SOM/MOS) is an under-recognised and probably under-diagnosed disorder. We describe the clinical signs of this condition among three patients. Next, from review of the literature, we suggest an algorithm for diagnosis and treatment., Observation: Retrospective study of three patients aged 40 to 55 presenting with brief, intermittent monocular episodes of oscillopsia., Discussion: The acute symptomatology of superior oblique myokymia follows a recognizable pattern: it always presents with brief, intermittent monocular vertical oscillopsia and/or vertical diplopia with torsion. The clinical signs are related to a neurogenic hyperexcitability of the superior oblique muscle. Treatment may be medical (carbamazepine, gabapentin, beta-blocker) or surgical. Recent publications report that superior oblique myokymia may result from vascular compression of the trochlear nerve (fourth cranial nerve), which controls the action of the superior oblique muscle, placing this condition in the category of vasculonervous conflicts., Conclusion: Superior oblique myokymia is a relatively poorly known disorder, despite classic pathognomonic symptoms. It is a benign condition, which can nonetheless become incapacitating. It occasionally portends an intracranial pathologic process, which must then be addressed with specific treatment., (Copyright © 2011 Elsevier Masson SAS. All rights reserved.)

A 67-year-old Japanese woman without contributory medical history developed acute onset of left-sided trochlear nerve palsy (TNP) with persistent and severe periorbital pain. There were no other neurological abnormalities. Funduscopic findings were normal. Cranial and orbital magnetic resonance (MR) imaging, and cranial MR angiography demonstrated no abnormalities. By administration of prednisolone 40 mg/day from the day after onset, periorbital pain was resolved within 24 hours, and TNP within 5 days. Thereafter, prednisolone was gradually tapered off. She remained asymptomatic under no medication. In the English language literature, this is the first reported case of Tolosa-Hunt syndrome presenting with isolated TNP.

A 39-year-old man presented with diplopia 1 day following left orbit trauma. CT scan and ultrasound showed a left trochlear hyperintensity that was interpreted by both the radiologist and echographer as a metallic foreign body. Surgical exploration failed to identify a foreign body. The patient presumably had preexisting trochlear calcification with a post-traumatic nonconcomitant small-angle vertical deviation and diplopia. Calcification should be considered in the differential diagnosis of trochlear hyperintensity noted on imaging studies.

The author reports his experience with his own case of Miller-Fisher syndrome (MFS) and discusses the spectrum of GQ 1b-positive oculomotor nerve diseases, including MFS, Bickerstaff's brainstem encephalitis, Guillain-Barré syndrome, ophthalmoplegia without ataxia and isolated oculomotor palsies., (© Georg Thieme Verlag KG Stuttgart · New York.)

Lyme disease, or borreliosis, is caused by a bacteria (Borrelia burgdorferi sensu lato) and is transmitted to humans by the bite of ticks of the Ixodes genus. The authors describe a 16-year-old girl who presented with extraocular muscle palsies and had serology positive for Lyme disease. The muscle palsies resolved after antibiotic treatment with no residual limitation of ocular movements.

Purpose: Superior oblique myokymia (SOM) is an uncommon disorder characterized by episodic monocular oscillopsia. Several medications have been reported to be of benefit for some patients with this condition, but the efficacy of medical treatment has not been well established and little long-term follow-up data are available. The purpose of this study was to better clarify the role of medical therapy in the management of SOM., Methods: A retrospective review of patients with this disorder seen in an outpatient neuro-ophthalmology clinic. The diagnosis of SOM was based on a history of episodic unilateral oscillopsia with or without torsional diplopia. Twenty-seven patients with SOM were identified. Twenty of these were treated medically and these formed the basis of the study. Follow-up interval ranged from 1 to 12.5 years (mean, 6.5 years). The main outcome measure was relief of oscillopsia., Results: Fifteen of the 18 patients treated with carbamazepine (83%) reported some benefit, 6 of whom continue to do well on medication 9 months to 5 years later. In four patients improvement was only transient and in five others treatment was subsequently discontinued for various reasons. In addition, one patient had sustained benefit from phenytoin, one from propranolol, and one from propranolol plus valproic acid. We found no treatment success with baclofen. Overall, nine patients (45%) enjoy sustained benefit unassociated with adverse side effects., Conclusions: In contrast to previous reports emphasizing the efficacy of surgery for SOM, our data demonstrate the potential benefits of medical treatment for patients with this disorder.

Purpose: To evaluate botulinum toxin injection of the inferior oblique muscle for management of superior oblique (SO) paresis., Methods: We undertook a prospective case series of injections of the ipsilateral inferior oblique (IO) muscle with 10-20 units of botulinum toxin type A in patients with a SO muscle palsy/paresis of less than 2 years' duration., Results: We enrolled 16 patients (18 eyes) with a mean age of 33.7 years. The median time from onset of symptoms until injection was 6 months. The cause of paresis was trauma for 81% of the patients. The mean hypertropia decreased from 6.4(Delta) to 1.9(Delta) at 6 months after treatment. Other clinical measures improved: mean IO overaction from + 1.7 to + 0.6, mean SO underaction from -1.5 to -0.4, mean subjective torsion from 9.3 degrees to 0.4 degrees , and mean head tilt from 8.4 degrees to 1.1 degrees., Conclusions: Botulinum toxin injection to IO muscle reduces the symptoms of SO paresis while patients are waiting for recovery.

A 13-year-old boy had right periocular pain, upgaze vertical diplopia, and lid swelling. Right extorsion and hypodense cystic swelling in the right superior oblique involving the muscle and tendon behind the trochlea existed. Oral treatment was prescribed. MRI revealed a collapsed cyst. Ocular motility improved, but levoelevation diplopia persisted.

Purpose: To study the effectiveness of botulinum toxin in the treatment of bilateral acquired oblique superior palsy., Material and Method: Five patients with bilateral asymmetrical acquired superior oblique palsy were treated with periodical injections of botulinum toxin in the inferior oblique and rectus muscles (2.5-5 U). The average age of the sample was 37.4 years. A trauma etiology was present in four cases. Treatment was applied at two months after the beginning of the illness in two cases, at four months in another two cases, and at one year in the other patient., Results: A good result was achieved in three of the cases with botulinum toxin. Two patients required surgery. All of the patients were injected in the inferior obliques, and two cases were also injected in the inferior rectus. The number of injections ranged between four and six applications. It was considered to be a good result when diplopia was corrected in reading and primary position, and torticollis disappeared. Diplopia was not corrected in extreme lateral gazes and supraversion in two cases., Conclusions: Periodical botulinum toxin injections represent an alternative management to surgery in the treatment of bilateral acquired oblique palsy. In some cases botulinum toxin injections can prevent or decrease the number of surgical interventions

Background: Myokymia of the obliquus superior muscle is a rare episodic microtremor caused by uncontrolled activities of the trochlearis nerve fibres. Epilepsy is also caused by spontaneous discharges of neurons. In our report we present an associated epilepsy which to the best of our knowledge is described for the first time., Patient: An 61-year old man with twitches of the right eye for 6 weeks and a subjective feeling of eye movement was investigated at our hospital. His history was void of any ophthalmologic diseases. However, he suffered from cryptogenetic epilepsy known since childhood. The morphological and orthoptical findings of his eyes were normal. During the slit-lamp investigation a unilateral rotating microtremor of the right eye induced by looking downward was seen. The neurologic investigation, magnetic resonance imaging and assessment of the thyreoid function did not show further pathological results. The patient underwent treatment with carbamazepine. Under this therapy he did not show any symptoms of myokymia during follow-up., Summary: To the best of our knowledge this is the first case of myokymia of the obliquus superior muscle associated to epilepsy. To our opinion, any case of this syndrome should be investigated for epilepsy. A causal relation is unlikely since the most probable etiologies are either spontaneous discharges of trochlear nucleus neurons or a close contact between vessel and nerve analogously to trigeminal neuralgia.

Purpose: To investigate therapeutic effects of oral gabapentin therapy on superior oblique myokymia., Design: Observational case series with measurement of visual acuity and eye movements before, during, and after therapy., Methods: Two adult patients with superior oblique myokymia, refractory to other therapies, were treated with gabapentin orally after informed consent was obtained. Eye movements were measured using the magnetic search coil technique., Results: Superior oblique myokymia completely resolved after starting gabapentin., Conclusion: Gabapentin may be an effective treatment for superior oblique myokymia; a double-blind study seems justified.