Claude Syndrome in Childhood Associated with Probable Neuro-Behcet Disease.

Claude syndrome is a rare midbrain stroke syndrome characterized by ipsilateral third cranial nerve palsy and contralateral hemiataxia. So far, only a few cases have been reported in childhood. We present two children with Claude syndrome at 9 and 15 years of age. The typical clinical picture was consistent with brain magnetic resonance imaging findings. A thorough investigation regarding the underlying etiology revealed no definite diagnosis but clues suggestive of probable neuro-Behcet disease. Awareness of pediatric neurologists on arterial ischemic stroke has been increasing over the past decades, enabling timely diagnosis and appropriate management of rare childhood cases with midbrain stroke.

Aseptic meningitis after amenamevir treatment for herpes zoster ophthalmicus with oculomotor nerve palsy in a patient taking immunosuppressant.

A 79-year-old woman presented with vomiting after being prescribed amenamevir by her primary care physician. She had a medical history of rheumatoid arthritis and was administered prednisolone and methotrexate. She was finally diagnosed with herpes zoster ophthalmicus and aseptic meningitis, and intravenous antiviral therapy was initiated. However, the patient developed oculomotor nerve palsy on the 11th day of hospitalization. In this case, there was a time lag between the administration of antiviral drugs and clinical improvement. Our case suggests the necessity of selecting antivirals, especially in high-risk cases of CNS complications, to avoid the low intracerebral transferability of antiviral drugs, including amenamevir.

Split-Tendon Medial Transposition of Lateral Rectus for Pediatric Complete Oculomotor Palsy.

BACKGROUND: Split-tendon medial transposition of lateral rectus (STMTLR) for complete oculomotor palsy can correct large angles of exotropia in adults, but outcomes are variable, and complications are frequent. Only a few pediatric cases have been reported, and further insight is needed to assess the child's alignment outcomes and ability for postsurgical gain of function. The aim of our study is to report the outcomes of this surgical procedure in pediatric cases of complete oculomotor palsy. METHODS: A retrospective review of outcomes was conducted on 5 consecutive patients with complete oculomotor palsy treated with STMTLR by a single surgeon (V.S.S.) between 2015 and 2021 at tertiary referral centers. Primary outcome was postoperative horizontal alignment, and secondary outcome was demonstration of gain-of-function activity in the field of action of the paretic medial rectus muscle. RESULTS: Five cases of pediatric complete oculomotor palsy underwent surgical treatment with STMTLR. Subjects averaged 5.3 years old (range 10 months-16 years). Two were female. Etiologies were heterogeneous, and all presented with unilateral (n = 2) or bilateral complete oculomotor palsy with exodeviations ranging from 45 to >120 prism diopters. Two subjects had bilateral disease secondary to military tuberculosis with CNS involvement. A third subject presented iatrogenically with complete bilateral third nerve palsies secondary to removal of a nongerminomatous germ cell tumor (NGGCT) of the pineal gland. The 2 remaining subjects had monocular involvement in their right eye, 1 from compressive neuropathy after a cavernoma midbrain hemorrhage, and 1 from a congenital right oculomotor palsy. All patients were observed to have stable ocular alignment for a period of at least 6 months before surgery. Unilateral STMTLR was performed in all cases except the subject with NGGCT, in which bilateral STMTLR was performed. Measurement of alignment permanence out to 1-3 years postop resulted in an average correction of 40.83 prism diopters (range 37.5-45 prism diopters) per operated eye. Four of 5 subjects regained limited but active adduction eye movements, and the 2 unilateral cases demonstrated improved convergence. None of the subjects experienced significant complications. CONCLUSIONS: STMTLR was a safe and effective approach for the surgical correction of complete pediatric oculomotor palsy in our case series. In addition, pediatric patients may benefit from STMTLR with immediate gain-of-function activity in the transposed lateral rectus muscle, which supports the hypothesis that children have a dynamic and adaptive neuroplasticity of visual target selection that predominates established agonist/antagonist neural signaling.

Importance of the "Rule of the Pupil" in the Modern Neuroimaging Era.

ABSTRACT: The "Rule of the Pupil" states that when aneurysms compress the oculomotor nerve, a dilated or sluggishly reactive pupil will result. In previous decades, when cerebral angiography was required to detect an intracranial aneurysm, the "Rule of the Pupil" was used to determine the relative risk of angiography and the likelihood of aneurysmal compression in patients with third nerve palsies (3NPs). Noninvasive imaging including computed tomography angiography (CTA) and magnetic resonance angiography has become readily accessible and can detect all aneurysms large enough to cause 3NPs. It is therefore recommended that all patients with 3NP undergo neuroimaging regardless of pupil status because the consequences of missing an aneurysm are high. The question therefore remains as to whether the "Rule of the Pupil" still has relevance in today's era of modern neuroimaging. We describe a 73-year-old man who developed a left complete, pupil-sparing 3NP and was found to have a paraclinoid meningioma in the left cavernous sinus. As compressive lesions are expected to impair the iris sphincter muscle, no intervention was recommended and his 3NP spontaneously improved within 3 months. We also describe a 54-year-old woman with diabetes and a complete 3NP with a dilated, nonreactive pupil. Initial CTA was reported as normal, but re-review of imaging revealed a posterior communicating artery aneurysm and immediate intervention to coil the aneurysm occurred. The "Rule of the Pupil" is still important in the modern neuroimaging era as demonstrated in cases of incidentally found lesions along the course of the oculomotor nerve and missed radiological findings.

Contained Rupture of a Posterior Communicating Artery Aneurysm in a Patient With a Third Nerve Palsy.

ABSTRACT: It is recommended that every patient with a new third nerve palsy undergo urgent neuroimaging (computed tomography angiography or magnetic resonance angiography) to exclude a posterior communicating artery aneurysm. Because of the novel coronavirus (COVID-19) pandemic, our institution noted a significant decline in the number of patients with aneurysmal subarachnoid hemorrhage presenting to the hospital. We report one such example of a patient who developed new-onset severe headache and vomiting and did not seek medical attention because of COVID-19. Two months later, she was noted to have ptosis during a routine follow-up and was found to have a complete, pupil-involving third nerve palsy. Computed tomography angiography was performed and revealed an irregular bilobed saccular aneurysm (7 × 9 × 5 mm) of the right posterior communicating (PComm) artery, but no acute hemorrhage was visible on CT. On MRI, immediately adjacent to the aneurysm, there was a small subacute hematoma in the right medial temporal lobe with surrounding vasogenic edema. This case had a fortunate and unique outcome as she had a contained hematoma adjacent to the ruptured PComm aneurysm and did not experience severe morbidity from the subarachnoid hemorrhage nor did she rebleed in the interval in which she did not seek care. This case highlights the importance of providing neuro-ophthalmic care even during a pandemic.

Four common diseases causing sudden blindness or death in the eye emergency department.

Neuro-ophthalmological emergency disorders typically present with symptoms of visual loss, diplopia, ocular motility impairment or anisocoria. The ocular manifestations of these disorders are sometimes indicative of a more serious global neurology disease rather than an isolated ocular disease. The aim of this review is to highlight four important neuro-ophthalmological emergency disorders that must not be missed by an ophthalmologist. These include acute painful Horner's syndrome, painful cranial nerve III palsy, giant cell arteritis and transient ischaemic attack with amaurosis fugax. The delayed diagnosis of these clinical entities puts the patient at risk of blindness or death. Therefore, prompt diagnosis and management of these conditions are essential. This can be acquired from understanding the main signs and symptoms of the disease presentation together with a high index of suspicion while working at a busy eye emergency department.

Plus-minus lid syndrome with ataxia and severe apathy-A rare manifestation of midbrain infarct.

Plus-minus lid syndrome is a rare manifestation of midbrain infarct, characterized by ptosis of one eye and lid retraction in the other eye. It has also been described in ocular myasthenia gravis, orbital myositis, or after lesions of the oculomotor nerve. Our patient was a 55-year-old man with hypertension and atrial fibrillation, who presented to us with acute onset left-sided ptosis and right-sided eyelid retraction. He was apathic and had right-sided ataxia. His MRI of the brain showed acute infarct involving the paramedian midbrain. To our knowledge, severe apathy and resultant executive function disorder have not been described previously in a patient having plus-minus lid syndrome with ataxia.

Isolated Oculomotor Nerve Palsy: A Unique Presentation of Haemophilus influenzae Type A Meningitis.

In this case report, we describe a unique case of Haemophilus influenzae type A meningitis in a 7-month-old previously healthy girl that presented with an isolated cranial nerve (oculomotor) palsy without other signs and symptoms classically associated with this entity such as fever, meningismus, or a generally ill appearance. Oculomotor nerve abnormalities are rare in pediatrics. Congenital oculomotor palsy is the most common cause followed by trauma, infection, inflammatory conditions, neoplasm, aneurysm or other vascular events, and ophthalmoplegic migraines, respectively. Therefore, had it not been for the unusual magnetic resonance imaging findings identified in this patient prompting an extensive infectious workup with lumbar puncture, the diagnosis and treatment of meningitis may have been delayed further or missed all together. This fact emphasizes the importance of maintaining a broad differential when children present with neurologic abnormalities such as cranial nerve palsies.

Splitting of the lateral rectus muscle with medial transposition to treat oculomotor palsy: a retrospective analysis of 29 consecutive cases.

PURPOSE: The lateralis splitting technique has been an interesting option for treating large-angle exotropia due to complete 3rd nerve paralysis since its inception in the early 1990s. The purpose of this study is to report on our experience regarding the effectiveness and complications of this method. METHODS: Retrospective analysis of a consecutive series of 29 patients operated by one single experienced surgeon and examined according to a specific operative and perioperative protocol. Patients were examined preoperatively, on the 2nd day and 3rd month after surgery. Outcome measures include strabismus angle, horizontal motility, head turn, binocular function, and incidence and resolution of postoperative serous retinal detachment as seen with infrared imaging and spectral domain optical coherence tomography (SD-OCT). RESULTS: Surgery brought about a large and stable reduction of strabismus angle and head turn. It reduced horizontal motility, but moved the range of monocular excursion much closer to center. Eighty percent of patients with constant diplopia acquired some fields of single binocular vision. A significant number of cases (33.3%) developed transitory serous retinal detachment with varying onset and extent. CONCLUSION: This is by far the largest published study regarding the outcome of lateralis splitting in NIII palsy. The procedure is difficult, yet a very useful option. Serous detachment is a serious complication, but usually transitory. Its cause and mechanisms are not fully understood and warrant further investigation.

Efficacy of endovascular intervention in patients with unruptured posterior communicating artery aneurysm-related oculomotor nerve palsy.

OBJECTIVES: Oculomotor nerve palsy (ONP) is commonly encountered in daily neurosurgical activities. The ONP secondary to un-ruptured PComA aneurysm might be a unique entity that was different in diagnosis, treatment and prognosis from its ruptured counterparts. Perhaps as a result of the limitation in sample size, studies that solely focused on factors affecting recovery of ONP in patients with unruptured corresponding PComA aneurysms were scarce. METHODS: In this study, we would like to report a relatively larger case series of patients with un-ruptured PComA aneurysm-related ONP. A retrospective review of medical records of 39 patients with un-ruptured PComA aneurysm-related ONP was performed with endovascular coiling. RESULTS: All 39 consecutive patients underwent endovascular coiling. Eighteen (46%) patients had a complete resolution of ONP, 14 (36%) patients had a partial resolution. Time interval from onset of ONP to endovascular intervention (P=0.004), degree of ONP (P=0.015) and age (P=0.016) were predictors of ONP recovery with statistical significance. Sex, aneurysm size and risk factor exposure (smoking, alcohol abuse and hypertension) were not associated with ONP outcomes. CONCLUSION: ONP secondary to un-ruptured aneurysm should be treated as a unique entity from its ruptured counterparts. A prospective study that contains surgical clipping and endovascular coiling, and comparison between two treatment modalities would be more convincing and is anticipated.

Simplified approach of Gokyigit's technique for complete cranial nerve third palsy.

PURPOSE: To evaluate a simpler approach of the medial transposition of split lateral rectus technique in patients with complete third nerve palsy. METHODS: All eyes with complet third nerve palsy were followed in our Strabismus Department between 2014 and 2016. All patients had complete oculamotor nerve palsy. All patients assed routine ophthalmologic examination. Also the ocular deviation, horizontal and vertical ocular alignments were measured at 6 m and at 1/3 m using the Krimsky corneal reflection test and alternate prism cover test with best optical correction. Same surgeon (BG) performed all procedures in general anesthesia. In this procedure, same Gokyigit's technique except upper and lower part of lateral rectus muscle was passed under the superior oblique tendon and inferior oblique tendon. Final deviation from 0 to 14 PD was considered a successful result. RESULTS: Eight patients were included in the study. The average ages were 39.4 years and male to female ratio 5:3. Patients had a preoperative horizontal deviation - 42.5 ± 2.7 PD and postoperative horizontal deviation - 1.7 ± 2.6 PD. All patients follow-up time were at least 6 months. CONCLUSIONS: Achieved to acceptable alignment in primary position, manage to diplopia and cosmetical appearance are the main aims of patients with third nerve palsy.

Approach to a Patient with Diplopia in the Emergency Department.

BACKGROUND: Diplopia can be the result of benign or life-threatening etiologies. It is imperative for the emergency physician to be proficient at assessing diplopia and recognize when urgent referral or neuroimaging is required. OBJECTIVE: The first part of this review highlights a simple framework to arrive at the appropriate disposition of diplopic patients presenting to the emergency department (ED). The second part of this review provides more detail and further management strategies. DISCUSSION: ED strategies for assessment of diplopia are discussed. Management strategies, such as when to image, what modality of imaging to use, and urgency of referral, are discussed in detail. CONCLUSIONS: Unenhanced plain computed tomography (CT) of the head or orbits is largely not useful in the work-up of diplopia. Magnetic resonance imaging is preferred for ocular motor nerve palsies. Due to limited resources in the ED, patients with isolated fourth and sixth nerve palsies with the absence of other neurological signs on examination should be referred to Neurology or Ophthalmology for further work-up. All patients presenting with an acute isolated third nerve palsy should be imaged with CT and CT angiography of the brain to rule out a compressive aneurysm. Contrast-enhanced CT imaging of the brain and orbits would be indicated in suspected orbital apex syndrome or a retro-orbital mass, thyroid eye disease, or ocular trauma. CT and CT venogram should be considered in cases of suspected cavernous sinus thrombosis. In any patient over the age of 60 years presenting with recent (1 month) history of diplopia, inflammatory markers should be obtained to rule out giant cell arteritis.

Ataxia with oculomotor apraxia type 2: an evolving axonal neuropathy.

A 23-year-old woman had presented initially to a podiatrist complaining of poorly fitting shoes during her adolescence. After extensive neurological review, she was diagnosed with ataxia with oculomotor apraxia type 2. This is a progressive autosomal recessive ataxia associated with cerebellar atrophy, peripheral neuropathy and an elevated serum α-fetoprotein. Within Europe, it is the most frequent autosomal recessive ataxia after Friedreich's ataxia and is due to mutations in the senataxin (SETX) gene. The age of onset is approximately 15 years.The diagnosis of oculomotor apraxia type 2 is often challenging. We provide a framework for assessing a young ataxic patient with or without oculomotor apraxia and review clues that will aid diagnosis. The prognosis, level of disability, cancer and immunosuppression risk all markedly differ between the conditions. Patients and their families need the correct diagnosis for genetic counselling, management and long-term surveillance with appropriate subspecialty services.

An approach to anisocoria.

PURPOSE OF REVIEW: Anisocoria is a finding seen on a daily basis in nearly every eye clinic. Although often benign, it can also represent the sole sign of a life-threatening disease making an up-to-date understanding of pathophysiology and diagnosis essential for anyone practicing medicine. RECENT FINDINGS: Many aspects of the traditional approach to anisocoria still hold true today, but advancements in imaging technology and changing trends in pharmacologic diagnosis and localization have led many to rethink that approach. In addition, the differential diagnosis for anisocoria continuously expands with identification and improved understanding of causal disease processes. SUMMARY: The present article discusses an approach to the classic anisocoria diagnostic algorithm modified by current knowledge from the most recent literature.

Oculomotor nerve tumors masquerading as recurrent painful ophthalmoplegic neuropathy: report of two cases and review of the literature.

BACKGROUND: In recurrent painful ophthalmoplegic neuropathy (RPON) that was previously termed as ophthalmoplegic migraine, enhancement of the ocular motor cranial nerves could be seen in the cisternal segment during the acute phase. However, various tumors involving the oculomotor nerve may mimic RPON. METHODS: We report two patients with MRI findings of oculomotor nerve schwannoma who initially presented with RPON, and found through the literature review five more patients with oculomotor nerve tumors that masqueraded as RPON. RESULTS: All patients showed an involvement of the oculomotor nerve. The radiological or pathological diagnosis included schwannoma in five, venous angioma in one, and neuromuscular harmatoma in another one. MRIs with gadolinium documented an enhancing nodule involving the cisternal portion of the oculomotor nerve in six of them, which was also observed on follow-up MRIs without an interval change. CONCLUSIONS: It should be recognized that an incomplete recovery may occur during future attacks in patients with otherwise uncomplicated RPON. Follow-up MRIs are required to detect tumors involving the ocular motor cranial nerves, especially in patients with suspected RPON when the recovery is incomplete.

Blepharoptosis Associated With Third Cranial Nerve Palsy.

PURPOSE: To evaluate the demographics and outcomes of management for blepharoptosis associated with third cranial nerve palsy at a tertiary eye center. METHODS: This retrospective cross-sectional and interventional study was performed on hospital records of patients with ptosis associated with third cranial nerve palsy who were referred to Labbafinejad Medical Center from January 1999 to January 2009. The authors evaluated age, sex, laterality, severity of involvement, etiology, clinical findings, and treatment modalities and outcomes in patients with blepharoptosis due to third cranial nerve palsy over a 10-year period. RESULTS: Of a total of 45 subjects, 25 cases (55.6%) were males and 20 subjects (44.4%) were females. Mean age of enrolled subjects was 21.1 ± 15.5 years. Etiologies included trauma in 21 (46.7%), congenital in 16 (35.6%), and other causes in 8 (17.7%) cases. Overall, 18 of 45 patients (40%) underwent ptosis surgery: of these, 15 (83.3%) subjects improved with one procedure, whereas 3 (16.7%) subjects required more than one operation. For initial ptosis surgery, 5 (27.7%) patients underwent levator resection and 13 (72.3%) cases underwent frontalis sling; all second and third procedures were frontalis sling. One-step, 2-step, and 3-step ptosis surgery was accomplished in 15 (83.3%), 1 (5.6%), and 2 (11.1%) patients, respectively. Eventually, an open visual axis was attained in all cases following 1-3 operations. CONCLUSIONS: Trauma was the most common cause of ptosis associated with third cranial nerve palsy in the current series. Surgical management of ptosis in patients with third nerve palsy may be challenging but most subjects achieve good results following appropriate and stepwise surgical plans.

[Effects of Endovascular Treatment on Cranial Nerve Palsy due to Unruptured Intracranial Aneurysms].

This study aimed to assess the effects of endovascular treatment on cranial nerve palsy due to unruptured intracranial aneurysms. Endovascular coiling was performed in 203 patients with intracranial aneurysms between April 2002 and March 2012 in our hospital. Of these patients, 8(3.9%)presented with cranial nerve palsy due to unruptured intracranial aneurysms. Cranial nerve palsy involved the oculomotor nerve in 8 patients and the optic nerve in 2 patients. Two patients had both optic nerve and oculomotor nerve dysfunction. Patients with incomplete oculomotor nerve palsy at admission were more likely to have full recovery after coiling. Optic nerve dysfunction did not improve after coiling. Incomplete oculomotor nerve palsy, early treatment(≤15 days), and small aneurysms(≤10 mm)were likely to be associated with complete recovery after coiling. This study indicates that endovascular coiling may resolve cranial nerve palsy due to unruptured intracranial aneurysms in patients who have incomplete oculomotor nerve palsy due to small aneurysms and are treated as early as possible after symptom onset.

Cognitive impairment in native Chinese with spinocerebellar ataxia type 3.

BACKGROUND: Previous studies have shown cognitive impairment in patients with spinocerebellar ataxia type 3 (SCA3). However, there is a lack of data on Chinese patients with SCA3. METHOD: We investigated 22 native Chinese with SCA3 and 18 controls matched for age, education as well as mental status. Cognitive assessments were carefully carried out to measure verbal fluency, memory, attention, executive function, visuospatial and visuoconstructive functions. RESULTS: The most common impairments of cognition in native Chinese with SCA3 were disruption of phonemic verbal fluency and frontal executive dysfunction. Deficits in semantic fluency were detected in about 31.8% patients. Impaired visuospatial function and verbal memory were also found in native Chinese with SCA3. The degree of ataxia, CAG repeat length and education were found to correlate with cognitive performance. Multivariate binary logistic regression suggested that an oculomotor disorder and depression are predictors of cognitive impairment. CONCLUSION: Native Chinese with SCA3 had cognitive impairment of frontal executive function, temporal and parietal functions. An oculomotor disorder might be an index of cognitive dysfunction.

Atypical teratoid rhabdoid tumor in the cavernous sinus of a toddler presenting with oculomotor nerve palsy.

INTRODUCTION: Atypical teratoid rhabdoid tumor (ATRT) is a rare, highly malignant, and aggressive tumor of infancy. Although the prognosis of ATRT has been extremely poor, recently, the first prospective study for ATRT demonstrated improvement of prognosis. On the other hands, oculomotor nerve palsy is rare in children and the most frequent etiology is congenital. To our knowledge, only a few ATRT cases presenting with oculomotor nerve palsy have been reported, but ATRT originating from the cavernous sinus (CS) has not yet been reported. CASE REPORT: An 18-month-old girl with right oculomotor nerve palsy was admitted, and a small mass in the right CS was detected with brain MRI. Although she received steroid pulse therapy and antimicrobial therapy, the mass continued to enlarge. One month after admission, the mass was partially resected and diagnosed as ATRT. Multimodal therapy including anthracycline-based chemotherapy, intrathecal therapy, and cranial irradiation was performed. Twenty-nine months after resection, she was alive without tumor relapse, but the oculomotor nerve palsy persisted. CONCLUSION: This is the first reported case of ATRT located in the CS presenting with oculomotor nerve palsy. This case was successfully treated with partial removal of the tumor, a new chemotherapy regimen for ATRT and cranial X-ray irradiation.

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.