Superior Oblique Palsy Research Articles

From WEMINO To WEBINO With Unilateral LMN Facial Palsy (P5-4.002)

<h3>Objective:</h3> To describe a case of progression from apparent trochlear nerve palsy, to wall-eyed monocular internuclear ophthalmoplegia (WEMINO), to wall-eyed bilateral internuclear ophthalmoplegia (WEBINO) with unilateral lower motor neuron (LMN) facial palsy. <h3>Background:</h3> Various descriptions of INO with cranial nerve palsies are in the literature, but we report a patient with a remarkably instructive stepwise evolution. <h3>Design/Methods:</h3> Review of records of a patient with diplopia at our institution. <h3>Results:</h3> A 42-year-old woman presented acutely with oblique binocular diplopia worsening on right gaze and left head tilt, with left hypertropia. Trochlear nerve palsy was suspected but contrasted MRI brain with orbits, and initial CSF testing were unrevealing. On day 3, the hypertropia partly improved but she developed left eye adduction deficit. Given the fluctuation, myasthenia gravis was considered high in the differential diagnosis while test results were awaited. On day 7, she developed LMN left facial palsy and bilateral adduction deficit with abducting eye nystagmus, leading to the realization that a single progressing lesion in the pontine tegmentum could explain her entire sequence of events. Repeat MRI confirmed a minute enhancing lesion in the floor of the fourth ventricle corresponding to the dorsomedial surface of the left facial colliculus and bilateral medial longitudinal fasciculi (MLF), likely of demyelinating etiology. Her pending CSF tests returned with 7 oligoclonal bands. She received intravenous methylprednisolone and her symptoms nearly resolved by day 14. <h3>Conclusions:</h3> This patient presented with evolving symptoms that appeared to localize to the trochlear nerve initially, but lacked imaging correlate. It is clear in retrospect that her initial presentation represented a skew deviation from early INO, progressing to WEMINO and eventually WEBINO with unilateral fascicular facial palsy. The anatomical correlate was confirmed on repeated imaging. Her clinical course beautifully illustrates the importance of lesion localization for etiological diagnosis. <b>Disclosure:</b> Dr. Senthamarai Siddharthan has nothing to disclose. Dr. Witt has nothing to disclose. Dr. Kini has nothing to disclose. Dr. Vasireddy has nothing to disclose. The institution of Dr. Mathur Kumaraswamy has received research support from Woolsey Pharmaceuticals.

Surgical Outcomes of Congenital Superior Oblique Muscle Palsy Operated in Adulthood

Purpose: To investigate the outcomes of patients who underwent surgical correction of congenital superior oblique muscle paralysis in adulthood.Methods: We retrospectively analyzed the medical records of 35 patients who underwent surgical correction of congenital superior oblique muscle paralysis after the age of 18 years. At the final follow-up, success was defined as residual hypertropia &lt; 4 prism diopters (PD), whereas failure was defined as residual hypertropia ≥ 5 PD or overcorrection. Clinical features were compared between the success and failure groups to identify factors affecting the surgical outcome. At the final follow-up, the resolution of symptoms, such as head tilt and diplopia, was also recorded.Results: Of the 35 patients, 24 (68.6%) and 11 (31.4%) were classified into the success and failure groups, respectively. Preoperative vertical deviation was larger in the failure group compared to the success group (23.1 vs. 15.2 PD, respectively; &lt;i&gt;p&lt;/i&gt; &lt; 0.05). Of the 31 patients with preoperative head tilt, head tilt resolved in 29 (93.5%). Of the 19 patients with diplopia, it resolved in all except 1 who was overcorrected.Conclusions: The preoperative vertical angle of the superior oblique muscle may affect the outcome of surgery performed in adulthood for correcting congenital superior oblique paralysis. Head tilt, diplopia, and subjective symptoms are typically resolved postoperatively.

Clinical Features of Pituitary or Parasellar Tumor Onset with Cranial Nerve Palsy: Surgical Intervention Considerations

This study aimed to clarify the symptoms of pituitary or parasellar tumor onset with cranial nerve palsy (CNP) and to improve our knowledge of this rare symptom and its most appropriate treatment. Among 1281 patients with pituitary or parasellar tumors surgically treated from 2003 to 2020, 30 cases (2.34%; 15 men and 15 women; mean age: 55.6years, range: 6-83years) first presenting with CNP were reviewed to evaluate the neurological symptoms, histological diagnosis, interval from onset to surgery, and time before complete CNP recovery. Pathological diagnoses comprised 17 pituitary adenomas, including 10 pituitary apoplexies and 4 adrenocorticotropic hormone-positive adenomas, and 13 other tumors, including 3 chordomas, 2 xanthogranulomas, 2 malignant lymphomas, 2 metastatic tumors, 1 Rathke cleft cyst, 1 plasmacytoma, 1 craniopharyngioma, and 1 neuroendocrine carcinoma. The mechanisms causing CNP were pituitary apoplexy (n= 10), cranial nerve compression or involvement (n= 17), and inflammatory changes (n= 9). As the first manifestation, 20 (66.7%) patients presented with oculomotor nerve palsy, 2 (6.7%) with trochlear nerve palsy, and 13 (43.3%) with abducens nerve palsy. Full recovery of CNP was obtained in 25 patients (83.3%) after surgery alone and in 2 patients (6.7%) after adjuvant therapy. Early surgery provided no significant difference in full recovery rates although it reduced the time to reach full recovery. It is critical to determine the mechanisms of CNP and intervene surgically to improve symptoms, shorten the duration of the disorder, prevent relapses, and obtain the correct pathological diagnosis to select the proper adjuvant therapy.

COVID-19 and sudden-onset ocular neurogenic palsy in prior healthy patients: a systematic review

ABSTRACT Background The aim of this systematic review is to identify cases of neurogenic ocular palsy in the presence of COVID-19 and to document patient characteristics, type of palsy and possible aetiologies. Methods A systematic search of PubMed, Medline and CINAHL databases was conducted on the 6th of January 2023 to identify cases of neurogenic ocular palsy in patients with current or previous COVID-19 infection. Data were pooled to summarise the neurogenic palsy, patient clinical characteristics and proposed palsy mechanisms. Results The combined database search yielded 1197 articles. Of these, 23 publications consisting of 25 patients met the inclusion criteria. Most patients were male (68%) and ranged in age from 2 to 71 years (median=32.7, SD=21.4). Seven patients (28%) were children aged 2 to 10 years old. Abducens palsies were most common (68%) and the most common ocular presentation was diplopia (76%) with an average time of onset 15 days from testing positive to COVID-19 or having symptoms of the virus. Proposed mechanism of development of a neurogenic palsy secondary to COVID-19 infection was classified into one of three categories: vascular/thrombotic, a viral neuro-invasive or inflammatory virus-mediated immune response. Discussion This study suggests that COVID-19 infection may be linked to oculomotor, trochlear and abducens nerve palsies and the underlying mechanisms may vary but are difficult to definitively establish. Further studies investigating the onset of neurogenic palsy secondary to COVID-19 infection is required.

Correlation of Preoperative High-Resolution Neurovascular Imaging and Surgical Success in Neurovascular Compression Syndromes.

Neurovascular compression syndromes (NVCSs) are a group of neurological disorders characterized by compression of a cranial nerve and include trigeminal neuralgia (TN), hemifacial spasm, geniculate neuralgia, glossopharyngeal neuralgia, vestibular paroxysmia, and trochlear palsy. The European Academy of Neurology recommends constructive interference in steady-state/fast imaging employing steady-state (CISS/FIESTA) magnetic resonance imaging (MRI) in the evaluation of medically intractable TN, but similar recommendations do not exist for the remaining NVCSs. We conducted a retrospective chart review of 300 patients with an NVCS who underwent microvascular decompression (MVD) by a single neurosurgeon from 2004 to 2021. Data were collected on preoperative diagnosis, pre- and postoperative symptoms, presence/absence of preoperative high-spatial-resolution CISS/FIESTA MRI, and intraoperative findings. Rates of symptomatic improvement were used as a correlate of surgical success. The rate of symptomatic improvement in the patients with a preoperative CISS/FIESTA MRI was 5.8% greater than those without preoperative high-spatial-resolution neuroimaging (98.8% vs. 93%, respectively; P= 0.008). Stratified by diagnosis, patients with TN had the greatest difference in surgical success between the 2 groups (99.3% vs. 92.9%, n= 268; P= 0.006). No statistically significant differences were observed in the other NVCSs, although positive trends were noted. Preoperative CISS/FIESTA MRI correlated with greater rates of surgical success in cases of medically intractable TN; however, definitive conclusions could not be made regarding the remaining NVCSs. We support the recommendation that this imaging modality be included as part of the standard of practice for the evaluation and management of TN and encourage future studies to further elucidate this relationship for the less common NVCSs using a larger cohort.

Antero-medial Transposition of Inferior Oblique for a Superior Oblique Palsy: A Case Report

A 29-year-old female presented with large-angle hypertropia, and small-angle exotropia associated with inferior oblique over action (IOOA). Orthoptic examination revealed right hypertropia of 35 PD with 12 PD of exotropia by prism cover test. There was no restriction of ocular motility, but we found right inferior oblique over action. She had a head tilt of approximately 30 degrees towards the left side. The Patient underwent "Antero-medial transposition of the inferior oblique" to correct strabismus. After surgery, she was orthotropic in the primary position with an average head-chin posture. Single approach surgery like the antero-medial transposition with an overacting inferior oblique may help to correct the large angle hypertropia associated with superior oblique (SO) palsy.

Effect of inferior oblique myectomy on ocular torsion according to the absence of the trochlear nerve in unilateral congenital superior oblique palsy.

To evaluate the effect of inferior oblique (IO) myectomy on ocular torsion according to the absence of the trochlear nerve in unilateral congenital superior oblique palsy (UCSOP). We retrospectively reviewed the clinical data of patients who had been diagnosed with UCSOP and underwent ipsilateral IO myectomy (n = 43). Patients were classified into the present and absent groups according to the absence of the trochlear nerve and superior oblique hypoplasia on magnetic resonance imaging (MRI). For quantitative analysis of ocular torsion, disc-fovea angles (DFA) were collected in both eyes using fundus photographs taken within three months before surgery and one month after surgery. DFA of the paretic eye did not differ according to the absence of the trochlear nerve (9.4±5.6° in the present group vs. 11.0±5.4° in the absent group, p = 0.508). However, the present group had a larger DFA in the non-paretic eye than the absent group (14.1±6.7° in the present group vs. 8.0±5.0° in the absent group, p = 0.003). The change of ocular torsion after IO myectomy in the paretic eye was -5.3±3.7° in the present group and -4.8±3.5° in the absent group, respectively (p = 0.801). In the non-paretic eye, the change in DFA was -1.5±3.0° in the present group, which was larger than that in the absent group (0.7±2.6°, p = 0.047). In the multivariate analysis, the change in DFA was correlated with only the preoperative DFA (standardized β = -0.617, p<0.001 in the paretic eye, and standardized β = -0.517, p<0.001 in the non-paretic eye). In the paretic eye, there was no significant difference in the change of ocular torsion between both groups, whereas in the non-paretic eye, the present group had a larger change in DFA after IO myectomy than the absent group. However, in the multivariable analysis, the change in ocular torsion was significantly correlated with preoperative excyclotorsion but not with the presence of the trochlear nerve itself.

Long-Term Efficacy of Inferior Oblique Myectomy Accompanied with Tenon’s Capsule Closure: Objective Analysis Using Nine-Gaze Photographs

Background: The aim is to evaluate the long-term efficacy of inferior oblique (IO) myectomy combined with Tenon's capsule closure to prevent muscle reattachment to the sclera. Methods: We retrospectively reviewed the medical records of 18 patients with primary and secondary IO overaction who underwent IO myectomy accompanied by Tenon's capsule closure. Patients were followed up for at least 1 year after the surgery. The main outcome measures included oblique muscle dysfunction, which was objectively graded through computerized analysis of nine-gaze photographs, and the amount of vertical deviation in the primary position using alternate prism cover testing. Results: After a mean follow up of 2.5 years, the grade of IO overaction decreased from +2.2 ± 1.0 to -0.8 ± 1.0 (p < 0.001). In patients with secondary IO overaction with superior oblique (SO) palsy, SO underaction improved from -2.2 ± 1.5 to -0.2 ± 1.8 (p = 0.006). Successful vertical deviation in the primary position of seven prism diopters or less was achieved in 83.3% of the patients. Underaction of the IO was observed in 11.1% of patients, whereas none of the patients showed antielevation syndrome. Conclusion: IO myectomy combined with Tenon's capsule closure might be safe and effective for the treatment of primary and secondary IO overaction in the long term.

Abstract Number ‐ 70: Combined Trans‐arterial Flow Diversion and Trans‐venous Embolization Management of High‐Flow Traumatic Cavernous‐Carotid Fistula (CCF)

Introduction Carotid‐Cavernous fistulas (CCF) are abnormal vascular shunts between the internal carotid artery (ICA) and the cavernous sinus. Most commonly due to trauma (75%), CCF occurs in 4%(1) of skull‐base fracture patients. Visual impairment, diplopia, and headache are typical presenting symptoms (88%). Proptosis, chemosis, ophthalmoparesis, and orbital bruits are notable exam findings. Cerebral angiography is the gold standard for diagnosis, showing cavernous sinus enlargement and ophthalmic vein or leptomeningeal vessel dilatation. CCF is typically treated endovascularly andballoon embolization has achieved cure rates of 88–99%(2). Successful obliteration using stents, however, has been reported(3, 4). While both trans‐arterial and the trans‐venous approach embolization are feasible depending on local vascular anatomy and accessibility of fistula points, the combination of trans‐arterial and trans‐venous approach is rarely employed.We present a case of traumatic, high‐flow CCF treated with combined trans‐arterial and trans‐venous coiling embolization and telescoped flow‐diverting stents. Methods A case report of successful treatment of traumatic high‐flow CCF with a combined trans‐arterial and trans‐venous coiling embolization with telescoped flow‐diverting stents. Results A 26‐year‐old woman presented with right eye proptosis, periorbital ecchymosis, and chemosistwo weeks after severe closed head injury treated with decompressive hemicraniectomy and subdural hematoma evacuation. Examination was notable for right eye blindness without light perception and right‐sided third, fourth, and sixth cranial nerve palsies and elevated intraocular pressure. CT head showed a skull base fracture involving the right carotid canal and angiography of ICA confirmed a high‐flow multi‐channel right CCF with diminished intracranial arterial flow and engorged bilateral ophthalmic veins. After unsuccessful attempts to catheterize the fistula via arterial approach, superior‐segment coil embolization was performed using an inferior petrosal venous approach; but further embolization of the cavernous sinus was unsuccessful. Using the cavernous‐petrous segment of the right ICA, a flow‐diversion stent was placed that significantly decreased cross‐fistula blood flow, improved intracranial arterial flow, and reduced bilateral ophthalmic vein congestion. After two months, intraocular pressure normalized and right eye vision returned, though moderate exophthalmos persisted. Angiography showed decreased but persistent residual CCF flow. Another flow‐diversion stent was successfully placed via trans‐arterial approach, covering the previous pipeline stent through petrous‐to‐ophthalmic right ICA. Three‐month follow‐up angiogram showed closure of the CCF with complete restoration of right eye vision. She ultimately underwent right cranioplasty and was able to return to work. Conclusions A combined trans‐venous coiling embolization and trans‐arterial flow‐diversion stenting may be effectively employed in the treatment of CCF with reversal of clinical symptoms and restoration of visual loss. Larger studies are needed to explore the role of this combined approach in the management of CCF.

Two Cases Report of Acupuncture Treatment at the Acupoint BL1 (Jingming) of Both Eyes in Treating Traumatic Trochlear Nerve Palsy

Purpose: To explore a safe and effective therapy in treating diplopia due to traumatic trochlear nerve palsy. Subjects: Two cases of traumatic trochlear nerve palsy both induced by motorcycle traffic accidents were presented in this study. Both cases were right superior oblique muscle palsy after an accidental head injury affected for more than 6 months (case 1) and 4 years (case 2). Methods of treatment: Both cases were subjected to acupuncture therapy at the acupoint BL1 (Jingming) of both eyes using disposable stainless steel acupuncture needles and were clinically evaluated by the ophthalmologist after each session. Each session extended to 20 minutes, 2 or 3 sessions per week till fully recovered. Result: Clinical symptoms of both cases were significantly improved after 3 sessions and continued the treatment for two more months to consolidate the curative effect due to fear of recurrence of symptoms. Conclusion: Acupuncture can be considered an effective therapy in the treatment of diplopia due to traumatic trochlear nerve palsy in addition to conservative therapy.

Neuro-ophthalmic Manifestations of Coronavirus Disease 2019 and Its Vaccination: A Narrative Review.

Coronavirus disease 2019 (COVID-19) is a current pandemic caused by SARS-CoV-2 that has vastly affected the whole world. Although respiratory disease is the most common manifestation of COVID-19, the virus can affect multiple organs. Neurotropic aspects of the virus are increasingly unfolding, in so far as some respiratory failures are attributed to brainstem involvement. The neuro-ophthalmic manifestations of COVID-19 and the neuro-ophthalmic side effects of vaccination were reviewed. The major findings are that the SARS-CoV-2 infection commonly causes headaches and ocular pain. It can affect the afferent and efferent visual pathways by ischemic or inflammatory mechanisms. Optic nerve may be the origin of transient or permanent visual loss from papillophlebitis, idiopathic intracranial hypertension, or optic neuritis. Cerebrovascular strokes are not uncommon and may lead to cortical visual impairment or optic nerve infarction. SARS-CoV-2 may affect the pupillomotor pathways, resulting in tonic pupil (Adie's syndrome) or Horner's syndrome. Cranial neuropathies including third, fourth, sixth, and seventh nerve palsies have all been reported. Rhino-orbital mucormycosis superinfections in COVID-19 patients receiving steroids or other immunosuppressive therapies may result in unilateral or bilateral visual loss and ophthalmoplegia. Autoimmune conditions such as Guillain-Barré, Miller-Fisher syndrome, and ocular myasthenia have been reported.

Deep Learning Model for Static Ocular Torsion Detection Using Synthetically Generated Fundus Images.

The objective of the study is to develop deep learning models using synthetic fundus images to assess the direction (intorsion versus extorsion) and amount (physiologic versus pathologic) of static ocular torsion. Static ocular torsion assessment is an important clinical tool for classifying vertical ocular misalignment; however, current methods are time-intensive with steep learning curves for frontline providers. We used a dataset (n = 276) of right eye fundus images. The disc-foveal angle was calculated using ImageJ to generate synthetic images via image rotation. Using synthetic datasets (n = 12,740 images per model) and transfer learning (the reuse of a pretrained deep learning model on a new task), we developed a binary classifier (intorsion versus extorsion) and a multiclass classifier (physiologic versus pathologic intorsion and extorsion). Model performance was evaluated on unseen synthetic and nonsynthetic data. On the synthetic dataset, the binary classifier had an accuracy and area under the receiver operating characteristic curve (AUROC) of 0.92 and 0.98, respectively, whereas the multiclass classifier had an accuracy and AUROC of 0.77 and 0.94, respectively. The binary classifier generalized well on the nonsynthetic data (accuracy = 0.94; AUROC = 1.00). The direction of static ocular torsion can be detected from synthetic fundus images using deep learning methods, which is key to differentiate between vestibular misalignment (skew deviation) and ocular muscle misalignment (superior oblique palsies). Given the robust performance of our models on real fundus images, similar strategies can be adopted for deep learning research in rare neuro-ophthalmologic diseases with limited datasets.

Neuro-ophthalmologic symptoms after coronavirus disease 2019 vaccination: a retrospective study

BackgroundThere have been several studies on inflammatory ophthalmic diseases; however, few studies have reported neuro-ophthalmological symptoms, such as diplopia and ocular motor nerve palsy, after coronavirus disease 2019 (COVID-19) vaccination. Therefore, this study aimed to report neuro-ophthalmological symptoms in patients after COVID-19 vaccination.MethodsThis was a retrospective study based on the medical records of 10 patients who visited our ophthalmology clinic in 2021 with symptoms, such as diplopia (nine patients) and decreased visual acuity (one patient), and showed findings, such as ocular motor nerve palsy, after vaccination against COVID-19.ResultsOne patient had third nerve palsy, two had sixth nerve palsy, and five had fourth nerve palsy. One patient complained of subjective binocular diplopia but all test results were normal. One patient presented with decreased visual acuity accompanied by a sudden increase in intraocular pressure and orbital cellulitis in the other eye. The symptoms improved gradually in most patients. Compared with previous studies, this study reported three cases of antiplatelet therapy that was initiated due to the older age of the patients and underlying diseases.ConclusionAs COVID-19 vaccines can cause neuro-ophthalmological diseases, such as ocular motor nerve palsy, patients’ age and underlying diseases should be considered while administering them.

Bilateral third and fourth cranial nerve palsy presenting secondary to central nervous system tuberculoma

Central nervous system (CNS) tuberculoma can have variable presentations depending on the site and number of tuberculomas. We report a rare case of a 48-year-old male presenting with ptosis and binocular double vision. Clinical examination revealed bilateral third-nerve palsy and fourth-nerve palsy with nystagmus. Magnetic resonance imaging (MRI) of the brain revealed ring-enhancing lesion in the dorsal midbrain, suggestive of tuberculoma. Radiological search for tuberculous foci in other internal organs proved pulmonary tuberculosis. Mantoux was positive, confirming the exposure to tuberculous bacilli. He was started on low-dose steroids along with antitubercular therapy (ATT). There was no improvement after continuing ATT for one-and-a-half years. Thus, the prognosis of CNS tuberculoma is related to the timing of initiating appropriate therapy. Delays in diagnosis and treatment will result in poor prognoses and severe neurological sequelae.

Trigeminal nerve sheath tumor presenting as trochlear nerve palsy

A 73-year-old woman presented with vertical diplopia which was diagnosed as a trigeminal nerve sheath tumor. This case was unusual because the principal symptom was a vertical diplopia due to involvement of the trochlear nerve. Trigeminal function was preserved on initial presentation. The patient was advised neuroimaging along with baseline blood investigations. On subsequent follow-ups she developed trigeminal symptoms in the form of tingling and numbness along her left cheek and below her left eye. Eventually, radiodiagnosis showed a dumbbell-shaped necrotic lesion originating from the trigeminal nerve sheath suggestive of a schwannoma. The case was then referred to a neurosurgeon for appropriate management.

Vision loss, third and fourth nerve palsy and non‐responsive midriasis after contralateral retrobulbar anaesthesia

AbstractPurpose: The purpose of this communication is to report a case of vision loss, third, and fourth nerve palsy and midriasis after contralateral retrobulbar anaesthesia. Although contralateral vision loss has been reported after locoregional block, very little evidence has been found about contralateral extraocular movement palsy and midriasis. Mechanism is not well known, though it is believed to be a consequence of the accidental subdural/subarachnoid anaesthetic injection.Methods: A case which took place at our hospital was selected. A retrobulbar block was performed by the service of anesthesiology in an 80‐year‐old patient prior to cataract surgery on the right eye. The block consisted of a 5 ml injection of 0.5% levobupicain and 2% lidocain (1:1) in his right eye under monitorization and sterile conditions. 10 min after the injection the patient presented bradycardia, hypotension and decreased consciousness. He was administered 0.6 mg atropine, 3 ephedrine bolus and 8 migr noradrenaline and his constants came back to normality. He was kept in observation in the recovery room. After consciousness recovery he claimed vision loss in the fellow eye.Results: At the exploration we found complete vision loss with no light perception, ptosis, third and fourth nerve palsy and dilated and non‐responsive pupil in the left eye. Funduscopy was unremarkable. He was kept in observation and 45 min later he was reexplored: ptosis, extraocular movements and pupil size improved; 2 h later exploration was completely normal.Conclusions: To conclude, we wanted to remember some of the possible complications of retrobulbar block and the importance of its conscious selection. Above all, we wanted to highlight this infrequent reaction on the fellow eye about which we have found very little evidence.

Development and 5-year Evaluation of Diagnosis-Specific Protocols for Visual Neuro-Rehabilitation in a Multicenter Inpatient Rehabilitation Network

ObjectiveTo provide a retrospective evaluation of a new eye and vision rehabilitation care pathway in a U.S. multi-site inpatient rehabilitation network involving the occupational therapy (OT) staff and a consulting doctor of optometry (OD) specializing in vision rehabilitation. DesignRetrospective study. SettingTwo Inpatient Rehabilitation Facilities (IRFs) and 1 Long Term Acute Care Hospital (LTACH). ParticipantsThere were 2083 records reviewed (44% women, avg. age 59 years). The most common diagnoses were hemispatial neglect (19.2%), homonymous field defects (18.5%), and oculomotor cranial nerve palsies (16.7%) (N=2083). InterventionsClinical care was reviewed where diagnosis-specific protocols were developed and training was provided to OTs in order to reinforce OD-prescribed interventions during daily treatment sessions, including (1) third, fourth, and sixth ocular cranial nerve palsies (OCNPs) with prisms fitted for full time, postural adaptation training, and oculomotor re-education using pursuits, saccades, head-rotations, and binocular vision exercises including alternate cover and vergence; (2) homonymous hemianopia with training awareness of field loss, eccentric viewing, and fitting of Peli lens for optical field expansion; and (3) prism adaptation therapy (PAT) for left hemispatial neglect. Main Outcome MeasuresFrequency of diagnoses. HypothesisDiagnoses with developed protocols were most common. Secondarily, feasibility and efficacy by anonymous OT survey. Results2083 vision consults were performed over 5 years. The most common diagnoses were hemispatial neglect (n=399, 19.2%), homonymous field defects (n=386, 18.5%), and OCNPs (n=347, 16.7%). None of the OTs reported the protocols were infeasible and 63% (IQR 38%-69%) reported their patients benefited from the interventions. The survey suggested prism for OCNPs helped in 42%, and Peli lens and PAT both helped in 38%. ConclusionsData support the feasibility of this inpatient eye and vision rehabilitation care pathway which may be used as a foundation for creating or refining similar programs elsewhere. Uniform administration of IRF-based visual neuro-rehabilitation care could provide a substrate for future clinical trials to evaluate efficacy.

Can Binocular Alignment Distinguish Hypertropia in Sagging Eye Syndrome From Superior Oblique Palsy?

PurposeAlthough the three-step test (3ST) is typically used to diagnose superior oblique palsy (SOP), sagging eye syndrome (SES) has clinical similarities. We sought to determine if alignment measurements can distinguish unilateral SOP from hypertropia in SES.MethodsWe studied hypertropic subjects who underwent surface-coil magnetic resonance imaging (MRI) demonstrating either SO cross-section reduction indicative of congenital or acquired palsy (SOP group) or lateral rectus muscle sag (SES group). Alignment was measured by Hess screen and prism-cover testing. Multiple supervised machine learning methods were employed to evaluate diagnostic accuracy. Rectus pulley coordinates were determined in SES cases fulfilling the 3ST.ResultsTwenty-three subjects had unilateral SOP manifested by SO atrophy. Eighteen others had normal SO size but MRI findings of SES. Maximum cross-section of the palsied SO was much smaller than contralaterally and in SES (P < 2 × 10−5). Inferior oblique cross-sections were similar in SOP and SES. In both SOP and SES, hypertropia increased in contralateral and decreased in ipsilateral gaze and was greater in ipsilateral than contralateral head tilt. In SES, nine subjects (50%) fulfilled the 3ST and had greater infraplacement of the lateral than medial rectus pulleys in the hypotropic orbit. Supervised machine learning of alignment data distinguished the diagnoses with areas under the receiver operating curves up to 0.93, representing excellent yet imperfect differential diagnosis.ConclusionsBecause the 3ST is often positive in SES, clinical alignment patterns may confound SES with unilateral SOP, particularly acquired SOP. Machine learning substantially but imperfectly improves classification accuracy.

Fourth Nerve Paresis plus Crossed Horner Syndrome in Acute Leukemia

A 29-year-old patient with diplopia showed a unilateral fourth nerve palsy combined with a Horner syndrome on the opposite side. Magnetic resonance images disclosed a focal hemorrhage in the left dorsal midbrain, affecting the nucleus of the fourth nerve and adjacent oculosympathetic fibers. Further evaluations including a bone marrow biopsy confirmed acute lymphoblastic leukemia. The combination of ocular motor and oculosympathetic palsy has a distinct localizing value. Fourth nerve palsy ipsilateral to Horner syndrome locates lesion in the cavernous sinus, while contralateral fourth nerve palsy locates the lesion in the midbrain.

A rare presentation of neurosarcoidosis as the superior orbital fissure syndrome

We are reporting a rare case of neurosarcoidosis that presented with superior orbital fissure syndrome. A 51-year-old Indian woman who suffered from 2 weeks duration, insidious onset gradually progressive left sided headache limited to forehead, left periorbital pain, diplopia on looking toward left, and numbness on the left forehead was referred to our hospital. Ophthalmic findings included left oculomotor nerve, trochlear nerve and abducens nerve palsies, absent corneal reflex, and loss of touch, pain and temperature sensation in V1 distribution. Brain and orbit magnetic resonance imaging (MRI) showed contrast enhancing area at the left orbital apex with normal optic nerve. Normal CSF analysis. High ESR, and chest x-ray showing non-homogenous patchy opacities in bilateral lung fields leaded us for neoplastic screening. Chest computed tomography (CT) revealed multiple ill-defined irregular lung parenchymal nodular opacities of varying sizes involving bilateral lungs with enlarged mediastinal and bilateral axillary lymph nodes. Elevated angiotensin converting enzyme level was found. Biopsy of the axillary lymph node showed non-caseating granulomatous lymphadenitis with giant cells containing schaumann bodies and asteroid bodies in the cytoplasm. These findings indicating an inflammatory response induced by sarcoidosis. After the initiation of steroid treatment, the patient experienced complete remission without any recurrence. When examining a case presenting with superior orbital fissure syndrome with contrast enhancing MRI lesion at the orbital apex, the additional chest CT to exclude neoplastic lesions can detect pulmonary sarcoidosis and extraneural lesions. These may avoid delays in the diagnosis and management of neurosarcoidosis.