Use of a Microelectromechanical Systems Sensor for Objective Measurements of Abnormal Head Posture in Congenital Superior Oblique Palsy Patients.

Purpose: The purpose of this study was to design an objective method for measurement of head positions as achieved with use of a microelectromechanical systems (MEMS) sensor. In addition, to use this system to observe the abnormal head position (AHP) in patients with congenital superior oblique palsy (SOP) before and after their surgery. Methods: An MEMS sensor was designed for recording of the pitch, roll, and yaw values of the head position in real time. The MEMS sensor was then fixed on the synoptophore from -30 degrees to +30 degrees positions horizontally and vertically to test the accuracy of these measurements. Then, we tested 13 participants with AHP using the MEMS method and the photographic method and compared their correlations. Finally, the pitch, roll, and yaw values of head positions were measured using this MEMS sensor in 31 patients with congenital SOP as performed before and after their surgery. Results: The MEMS sensor (LPMS-B2; Alubi, Guangzhou, China; 400 hertz [Hz]), as based on the theory of a gyroscope, was designed and connected to a smartphone via Bluetooth. It was able to conveniently record the patient's pitch, roll, and yaw head positions in real time, recordings which were consistent with the scales of the synoptophore (P > 0.05) and good correlations with the photographic method (P < 0.001). The main preoperative AHP in patients with SOP was roll (22/31, 71%). Pre- and postoperative vertical deviations were 16.4 ± 7.3 prism diopters (PD) and 4.1 ± 4.2 PD, respectively (P = 0.001). The AHP in patients with SOP was positively correlated with the angle of extorsion in the dominant eye (P = 0.01), rather than that of the vertical deviation. Conclusions: The MEMS sensor described in this report is a simple, practical, and accurate objective device for use in head position measurements. In patients with SOP, the AHP is related to the angle of extorsion in the dominant eye. Translational Relevance: The MEMS sensor was designed as a micro-wireless dynamic high-precision device for AHP measurement, which has the potential for use in a clinic.

Bilateral Congenital Trochlear-Oculomotor Synkinesis in a 5-Month-Old Infant.

Oculomotor synkinesis is an unusual phenomenon that encompasses various presentations with few associations reported in the literature. It can occur with or without a previous setting of oculomotor nerve injury. The authors present the first reported case of bilateral congenital trochlear-oculomotor synkinesis in a 5-month-old infant. [J Pediatr Ophthalmol Strabismus. 2024;61(5):e50-e53.].

Surgical treatment of unilateral acquired superior oblique muscle palsy in adults by inferior oblique belly transposition: a retrospective analysis.

PURPOSE: Evaluate and analyze the efficacy of inferior oblique belly transposition (IOBT) in treating adult patients with diplopia and small-angle hypertropia caused by mild to moderate inferior oblique overaction (IOOA) secondary to acquired superior oblique palsy (SOP). METHODS: Nine adult patients with diplopia and small-angle hypertropia associated with mild to moderate IOOA secondary to unilateral acquired SOP were included in the current retrospective study. All patients received the IOBT procedure between February 2019 and May 2023 at The Second People's Hospital of Jinan and were followed up for more than 6 months after the surgery. During the procedure, the belly of the inferior oblique muscle was fixed to the sclera at 5 mm posterior to the temporal insertion of the inferior rectus muscle. The following indicators were reviewed pre- and post-surgery: the vertical deviation (VD) in the primary position and in the Bielschowsky test, the fovea disc angle (FDA) of the affected eye, changes in IOOA, and diplopia. RESULTS: After IOBT, the VD in the primary position decreased from 7.22△ ± 1.72△ (range 4△-10△) to 1.22△ ± 1.30△ (range 0△-3△). The VD in the Bielschowsky test decreased from 13.00△ ± 1.80△ to 3.22△ ± 1.09△. The FDA decreased from 10.02° ± 3.34° to 6.26° ± 1.91°. The grade of IOOA was reduced from 2.00 (1.00, 2.00) to 0.00 (0.00, 1.00). All changes were statistically significant (P < 0.001 or P = 0.006). Diplopia was resolved completely for all patients. CONCLUSIONS: IOBT can effectively treat adults with diplopia and small-angle hypertropia caused by mild to moderate IOOA secondary to acquired SOP.

Clinical characteristics and surgical outcomes in patients with superior oblique muscle palsy: a retrospective study on 1057 patients.

BACKGROUND: To evaluate the clinical findings of patients with SOP who underwent surgery. METHODS: This historical cohort study was performed on 1057 SOP patients managed with surgery in Farabi Hospital, Iran, from 2011 to 2022. RESULTS: There were 990 (93.7%) patients with unilateral SOP with the mean age of 21.8 ± 14.8 years. Of these, 715 patients (72.2%) were diagnosed with congenital SOP, and 275 patients (27.8%) had acquired SOP (P < 0.001). In contrast, 67 (6.3%) patients were diagnosed with bilateral SOP, with the mean age of 19.4 ± 15.6 years. Among these, 18 cases exhibited the masked type. The mean angle of vertical deviation in primary position at far in unilateral and bilateral cases was 15.6 ± 8.3 and 13.3 ± 9.1 △, respectively (P < 0.001). In unilateral cases, abnormal head posture (AHP) was detected in 847 (85.5%) patients and 12 (1.2%) had paradoxical AHP. Amblyopia was found in 89 (9.9%) unilateral and 7 (10.3%) bilateral cases. Solitary inferior oblique myectomy, was the most common surgery in both unilateral (n = 756, 77.1%) and bilateral (n = 35, 52.2%) patients. The second surgery was performed for 84 (8.6%) unilateral and 33 (49.3%) bilateral cases (P < 0.001). The prevalence of amblyopia and the mean angle of horizontal deviation were significantly higher in patients who needed more than one surgery (all P < 0.05). CONCLUSION: Congenital SOP was more than twice as frequent as acquired SOP and about 90% of unilateral and 50% of bilateral cases were managed with one surgery. Amblyopia and significant horizontal deviation were the most important factors for reoperation. TRIAL REGISTRATION: The Institutional Review Board approval was obtained from the Tehran University of Medical Sciences (IR.TUMS.FNM.REC.1400.012) and this study adhered to the tenets of the Declaration of Helsinki and HIPAA.

Small tuck for superior oblique palsy.

PURPOSE: To investigate the surgical outcomes of small superior oblique (SO) tuck-denoting minimal tendon laxity-in patients with unilateral SO palsy. METHODS: The medical records of consecutive patients treated with ≤6 mm SO tuck from 2000 to 2018 at Kellogg Eye Center, University of Michigan, were reviewed retrospectively. Tendon tucks were performed to a fairly uniform tension in an amount that just eliminated slack in the tendon. Pre- and postoperative motility measurements were compared. Patients were excluded if they had a history of prior strabismus surgery or concurrent vertical rectus or inferior oblique surgery. RESULTS: A total of 27 cases (14 males) met inclusion criteria. The median age at surgery was 47 years (range 3-74 years). The mean SO tuck (total, both arms of tuck) was 4.9 mm (range, 2-6 mm). After surgery, median hypertropia decreased from 9Δ to 1Δ in primary position and from 20Δ to 4Δ in the SO field of action (contralateral downgaze). Lateral incomitance (difference in hypertropia between contralateral and ipsilateral gaze) decreased from 10Δ to 2Δ (P < 0.001 in each case). Six patients had diplopia in upgaze postoperatively that was not symptomatic enough to require reoperation. Six patients had residual hypertropia requiring additional surgery. CONCLUSIONS: Small SO tuck provided disproportionate correction of hypertropia in the SO field of action and nearly eliminated lateral incomitance without producing unacceptable iatrogenic Brown syndrome. Even in the absence of tendon laxity, SO tuck was a good surgical option for SO palsy in our cohort where there was marked lateral incomitance and the greatest deviation was in the SO field of action.

Superior oblique palsy as the initial manifestation of anti-contactin-1 IgG4 autoimmune nodopathy: A case report.

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.

Torsion and clinical features in patients with acquired fourth cranial nerve palsy.

This retrospective study aimed to compare objective/subjective torsion and other clinical characteristics of patients with acquired trochlear nerve palsy. This study included 82 consecutive patients who were diagnosed with acquired fourth cranial nerve palsy between 2014 and 2021 and who were followed up for ≥ 6 months. The etiologies, ocular deviation, objective and subjective torsions were reviewed. The etiologies were classified as ischemic, traumatic, brain lesion, idiopathic, or other. The patients were classified into two groups according to the recovery state: full recovery and partial/no-recovery. We compared the torsion and clinical features based on the etiology and recovery state. The average age was 59.1 ± 11.1 years, and 58 (71.0%) of the patients were male. The most common cause was ischemic (n = 49, 59.7%) and other common causes included traumatic (n = 16, 19.5%), brain lesion (n = 8, 9.8%), idiopathic (n = 5, 6.1%) and others (n = 4, 4.9%). Of the 82 patients, 56 (68.3%) were assigned to the full recovery group, and 26 (31.7%) were assigned to the partial/no-recovery group. The average age and number of patients with ischemic causes of palsy were greater in the full recovery group (p = 0.026 and p < 0.000, respectively). The vertical deviation angle, tilted angle on the Lancaster red-green test (LRGT), proportion of patients who experienced subjective torsion on the LRGT, and head tilt were smaller in the full recovery group (p = 0.037, 0.042, 0.045, and 0.006, respectively). Ischemic trochlear nerve palsy, advanced age, a small deviation angle at the primary position, and few cases of excyclotorsion on LRGT were characteristic of the full recovery group of acquired unilateral trochlear nerve palsy patients.

Right-sided Herpes Zoster Ophthalmicus Complicated by Bilateral Third, Fourth, and Sixth Cranial Nerve Palsies and Syndrome of Inappropriate Antidiuretic Hormone Secretion.

Cases of herpes zoster ophthalmicus (HZO) complicated by bilateral ophthalmoplegia are rare, and no cases of bilateral third, fourth, or sixth cranial nerve palsies have been reported. Syndrome of inappropriate antidiuretic hormone secretion (SIADH) is a rare complication of HZO. We herein report an 80-year-old Japanese woman with right-sided HZO complicated by meningoencephalitis and discuss the pathogenesis of this condition. She developed bilateral third, fourth, and sixth cranial nerve palsies and SIADH almost simultaneously during treatment for HZO. The bilateral cranial palsy spontaneously resolved within a few months.

Inferior oblique myectomy for postoperative limitation of elevation in abduction.

Antielevation syndrome is commonly described following anteriorization of the inferior oblique muscle. A similar phenomenon may occur following inferior oblique muscle recession, creating a distinct strabismus pattern in the setting of cranial trochlear nerve palsy. We report 3 adult patients, 69-72 years of age, who presented at the Stanford Byers Eye Institute with a similar strabismus pattern-limited elevation in abduction following previous inferior oblique muscle recession for congenital/long-standing trochlear nerve palsy. All 3 patients had a small hypertropia in primary gaze with ipsilateral inferior oblique overaction, limited elevation in abduction, and inferotemporal conjunctival scars. Two patients had a V-pattern strabismus. Following myectomy of the previously recessed inferior oblique muscle, elevation in abduction improved, and symptoms resolved in all 3 patients.

Teaching Video NeuroImage: Monocular Rotational Nystagmus Caused by Superior Oblique Myokymia.

A 61-year-old man presented with 2 days of "flickering" vision. The symptom resolved with closure of the left eye. Examination demonstrated involuntary high-frequency, low-amplitude intorting movements of the left eye, consistent with superior oblique myokymia (Video 1). Ocular ductions were full, and there were no abnormal movements of the right eye.

[Significance of accurately interpreting the Bielschowsky tilt test in the differential diagnosis of superior oblique muscle paralysis].

Superior oblique muscle paralysis is a common type of vertical rotatory strabismus with various subtypes. Regardless of the subtype, the Bielschowsky tilt test plays a crucial role in the diagnosis of superior oblique muscle paralysis and is often considered a significant criterion for diagnosis and differential diagnosis. However, the sensitivity and specificity of the Bielschowsky tilt test for diagnosing superior oblique muscle paralysis are not 100% due to the mechanism involved. The test is not solely based on extraocular muscle imbalance but also involves reflex pathways of the vestibular system and central nervous system. Consequently, lesions affecting corresponding areas may yield positive results in the Bielschowsky tilt test. Additionally, vestibular and central nervous system lesions can also cause strabismus, leading to a lack of one-to-one correspondence between a positive Bielschowsky tilt test and superior oblique muscle paralysis. Therefore, correctly interpreting the role of the Bielschowsky tilt test in superior oblique muscle paralysis is of paramount importance for the effective clinical management and treatment of associated conditions.

Are isolated ocular motor nerve palsies in the elderly truly 'low risk' for abnormal neuro-imaging outcomes?

BACKGROUND: Neurologically isolated ocular motor nerve palsies often present a management dilemma. Neuroimaging is more likely to be offered to patients <50 years without coexisting ischaemic risk factors as their risk of sinister underlying causes is thought to be higher. However, populations are rapidly ageing and advanced neuroimaging is now more widely available. We thus investigated the incidence of abnormal neuroimaging outcomes in the traditionally low-risk older patient group. METHODS: This is a retrospective cohort study of 353 patients presenting with isolated ocular motor nerve palsies to a tertiary neuro-ophthalmology service in Singapore over a four-year (2015 to 2019) period. Clinical data was obtained through manual review of case records. Common aetiologies, age-based differences in prevalence of causes and abnormal neuroimaging outcomes were statistically analysed. RESULTS: Abnormal neuroimaging outcomes were significantly greater in the younger cohort only when age segregation was performed at 60 years of age. In a multivariate analysis, acute onset rather than ischaemic risk factors were independently predictive of normal neuroimaging outcomes. After adjusting for prior cancer risk and clinical bias from presumed ischaemic palsies, abnormal neuroimaging outcomes were seen in 14.1% ≥ 50 yrs, 10.9% ≥ 60 yrs and 15.1% ≥ 70 yrs. CONCLUSIONS: In patients presenting with isolated ocular motor nerve palsies, acute onset may be a more reliable indicator of an ischaemic palsy rather than advanced age or presence of ischaemic risk factors. If onset is not acute, neuroimaging should be considered irrespective of age and coexisting ischaemic risk factors.

Postoperative outcomes for unilateral congenital trochlear nerve palsy: A retrospective cohort study.

PURPOSE: Congenital trochlear nerve palsy is the most common cause of vertical strabismus. The goal of this study was to investigate surgical outcomes after superior oblique tendon plication with or without inferior oblique recession in children and adults with unilateral congenital trochlear nerve palsy. METHODS: Data and outcomes were collected in patients with a diagnosis of unilateral congenital superior oblique palsy during a retrospective single-center study conducted at the University Hospital of Tours. A reproducible, standard ophthalmological and oculomotor examination was performed pre- and postoperatively at 1 year, including presence or absence of diplopia, vertical and horizontal deviations, and compensatory head posture. Surgical success, defined as an endpoint including absence of diplopia in primary position, absence of head tilt, and vertical deviation at distance fixation<5 prism diopters (PD), was analyzed. RESULTS: A total of fifty-seven patients (median [IQR] age of 11 years [5-42]) were analyzed. Patients experienced a significant reduction in vertical distance and near deviations (p<0.001), compensatory head tilt (p < 0.001), and diplopia after surgery (p < 0.001). Surgical success was higher in adults (17/24, 70.8%) than in children (15/33, 45.5%), although this did not reach statistical significance (p=0.0657). CONCLUSION: This study suggests that plication of the superior oblique muscle tendon, with or without recession of the inferior oblique muscle, can be effective in treating unilateral congenital trochlear nerve palsy. Further studies are necessary to compare surgical procedures and investigate their efficacy in adults compared to children in the short and long term.

The Frequency and Manifestations of Ocular Causes of Abnormal Head Posture.

PURPOSE: To determine the frequency and manifestations of different ocular causes of abnormal head posture (AHP). METHOD: This prospective, consecutive case series study was performed on 149 patients with ocular AHP at Farabi hospital, Iran, from February 2020 to June 2021. All patients underwent routine ophthalmic examinations. The manifestation of AHP was determined by direct observation from three viewing angles, while the patient read the smallest line on the vision chart that they could see. In front, above, and lateral gazes, observations were performed to find head tilt, head turn, and chin abnormal position, respectively. A picture with habitual AHP was taken from all patients. The amount of head tilt was measured by calculating the angle between the line that connects the lips center to the center of the eyebrows and the vertical line using the Corel Draw X7 computer software. RESULTS: The mean age of 149 patients with ocular AHP [101 (67.8%) males and 48 (32.2%) females] was 16.2 ± 12.2 (range, 2-57) years. The most common ocular sources of AHP were found to be superior oblique palsy (SOP) in 66 (44.3%) patients, 54 (36.2%) cases with Duane's retraction syndrome (DRS), and 12 (8.1%) patients with nystagmus. Other frequent causes of ocular AHP were dissociated vertical deviation (DVD) in 5 (3.4%), A and V pattern strabismus in 3 (2.0%), and 2 cases (1.3%) in each of Brown syndrome, inferior rectus (IR) palsy, and congenital fibrosis of the extraocular muscles (CFEOM). The most common manifestations of AHP in all cases were "pure head turn" (48.3%), followed by "pure head tilt" (24.8%), "simultaneous head tilt and head turn" (20.8%), and "chin up" (6.0%). The mean head tilt among all patients with head tilt was 10.4° ± 8.9° (range, 5.0°-31.7°). CONCLUSION: The most frequent ocular sources of AHP were SOP, DRS, and nystagmus, followed by DVD, A and V pattern strabismus, IR palsy, CFEOM, and Brown syndrome. In addition, pure head turn and pure head tilt were the most common manifestations of ocular AHP but were not always seen in the same direction or combination as previously reported with these etiologies.

Comparison of head tilt test between sagging eye syndrome and acquired unilateral trochlear nerve palsy.

PURPOSE: To investigate the distinction between sagging eye syndrome (SES group) and acquired unilateral trochlear nerve palsy (Trochlear group) in the Bielschowsky head tilt test (BHTT). METHODS: Fifteen patients in the SES group (mean age 74.6 ± 5.2 years) and 14 patients in the Trochlear group (55.2 ± 15.9 years) visited the Department of Ophthalmology, Hyogo Medical University Hospital between November 2016 and October 2022 for treatment of their diplopia. Eye position was measured with the alternate prism cover test, and values for fixation of the dominant eye, or unaffected eye, were used. Cyclodeviation was measured with the synoptophore and the Glaucoma Module Premium Edition of the SPECTRALIS optical coherence tomography. In the BHTT, eye position was measured in three head postures: primary position (PP), head tilt to the side with hypertropia (Hyper), and head tilt to the side with hypotropia (Hypo). The differences in vertical deviation between PP and Hyper (Hyper - PP), PP and Hypo (PP - Hypo) and Hyper - Hypo were measured and compared. RESULTS: Vertical deviation in primary position was 7.3 ± 4.5 PD in the SES group and significantly larger (17.1 ± 8.4 PD) in the Trochlear group (p = 0.002). The vertical deviation in Hyper was significantly larger in the Trochlear group with 7.7 ± 4.7 PD and 22.1 ± 9.4 PD, respectively (p < 0.001), whereas the that in Hypo was not significantly different between the two groups with 6.5 ± 3.4 PD and 8.4 ± 6.6 PD, respectively (p = 0.725). The SES group showed no significant difference according to the 3 head postures (p = 0.311), while the Trochlear group showed a significantly different with smaller mean values in vertical deviation in Hypo (p < 0.001). The difference in the vertical deviation for the 3 head postures was the largest in Hyper - Hypo (1.7 ± 2.1 PD and 13.6 ± 7.1 PD, respectively), and the accuracy of SES was at the cutoff value of 6 PD, and it was considered not to be SES if the value was 6PD or higher. The accuracy of SES determination was 100% sensitivity and 100% specificity, and the area under the curve was 1.0. CONCLUSION: The difference in Hyper - Hypo in the BHTT may be the most useful index in differentiating SES from acquired unilateral trochlear nerve palsy; if the difference was more than 6 PD, the probability of SES was very low.

Botulinum Toxin Injection for the Treatment of Third, Fourth, and Sixth Nerve Palsy: A Meta-Analysis.

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.].

[Central trochlear nerve palsy treated with inferior oblique muscle weakening]. / Parálisis central del nervio troclear tratada con debilitamiento del músculo oblicuo inferior.

TITLE: Parálisis central del nervio troclear tratada con debilitamiento del músculo oblicuo inferior.