Pupil Sparing Research Articles

Idiopathic unilateral complete oculomotor nerve palsy: a case report of diagnostic quandary

Introduction and importance: When compared to other cranial nerve palsies idiopathic unilateral oculomotor nerve palsy with pupillary sparing is one of the least noted neurological conditions. Moreover, there lies a series of diagnostic dilemmas to come into a final diagnosis resulting in several array of clinical investigations. Hence, there is a delay in prompt management. Case summary: An elderly female without any known comorbidities presented with the complaint of headache, dizziness and dropping of left eyelid. Several arrays of diagnostic workups was done to come to a diagnosis, but even with rigorous laboratory investigations and radiological examinations, a common working diagnosis could not be made. Hence with a diagnosis of exclusion after proper neurological and neuro-ophthalmological examination, idiopathic unilateral common oculomotor nerve palsy was identified for which improvement with steroids was noted in the patient. Discussion: Idiopathic unilateral complete oculomotor nerve palsy is considered as a diagnosis of exclusion when all the diagnostic parameters fail to signify and positive results. The vague symptomatic presentation of the disease condition further compels the treating physician to carry out several panels of laboratory to radiological investigations. But if identified in time the treatment modality is straightforward. Conclusion: The diagnostic quandary in timely identification of such disease conditions needs a pertinent diagnostic guideline so as to avoid the unwanted panel of investigations.

Claude’s Syndrome: A Case Report of Rare Midbrain Syndrome from Bangladesh

Claude's syndrome is a rare brainstem syndrome with ipsilateral third nerve palsy and contralateral ataxia. Here we present a case of a 60-year-old male with Claude's syndrome due to cerebral infarct. The patient had left-sided pupil sparing third nerve palsy with right-sided ataxia. MRI of the brain revealed infarct in the left half of the tegmentum of the midbrain. MRA of the brain was unremarkable. The patient improved gradually and was almost asymptomatic after one month. Though the syndrome was first described a century ago, it warrants particular attention from clinicians due to its rarity and atypical features. JAFMC Bangladesh. Vol 18, No 2 (December) 2022: 72-74

Case report: Pupil sparing third nerve palsy in an immunocompetent host – A rare sequelae of congenital ocular toxoplasmosis

We share a case of a 20 yr old female who presented with drooping of left upper eyelid with down and outward deviation of left eye since birth and diminution of vision in left eye noticed since 2 yrs with retinal abnormalities suggestive of congenital ocular toxoplasmosis. The clinical and complementary examinations of the case are discussed. Ocular toxoplasmosis remains to be the most common cause of infectious posterior uveitis in immunocompetent and immunocompromised individuals with highly variable prognosis. A precise diagnosis of the disease is necessary to opt effective and rapid treatment. Hence, It is essential that clinicians be aware of antenatal and postnatal presenting signs and symptoms, as early diagnosis and treatment will minimise the possibility of sequelae as majority of affected infants may develop chorioretinitis, deafness and/or neurological symptoms if left untreated.

Clinical characteristics in patients with oculomotor paralysis caused by isolated midbrain infarction.

The aim of the study was to investigate the clinical characteristics of isolated oculomotor paralysis (OP) cases caused by pure midbrain infarction (MI) with pupil sparing. Patients with pure MI and pontine infarction (PI) at our hospital were included in this study. We compared the blood pressure and lipid levels between the two groups. And the clinical data and imaging features were summarized. In total, 33 and 35 patients were included in the MI and PI groups, respectively. There was no significant difference in the distribution of age (64.9 ±10.0 vs. 65.1 ±10.8 years, p = 0.927) and males (84.8% vs. 74.3%, p = 0.282) between the MI and PI groups, respectively. The pure MI group had a comparable level of serum lipoprotein and cardiovascular risk factors compared with the PI group except for a lower proportion of hypertension (57.6% vs. 85.7%, p = 0.010). The majority (72.7%) of culprit lesions in the pure MI group was located in the paramedian area of the midbrain, and the ocular muscle palsies mostly involved the medial rectus (75.8%). The Chinese patients with OP caused by pure MI were mainly characterized with rapidly progressive symptoms, multiple cerebrovascular risk factors, and typical MRI signs. Further efforts should be made in the differential diagnosis of this atypical midbrain syndrome.

A retrospective five-year study of clinical profile of patients with acute binocular diplopia at a tertiary eye care center in Kerala

To analyse the demography, clinical features and outcome of patients presented with acute binocular diplopia at a tertiary eye care centre of Kerala. A retrospective review of medical records of patients presented with acute (<four weeks) binocular diplopia over the past five years was done. Detailed ophthalmic evaluation was done and risk factors were noted. Patients were followed up until cure or up to six months, whichever was earlier. Patients with restrictive strabismus or lack of follow up were excluded from the study. 161 eyes of 88 (54.65%) males and 73 (43.34%) females with a mean age of 55.71 years (range 6 to 93 years) were included. Isolated cranial nerve palsies were the most common cause for acute diplopia (n=142, 88.2%) and included pupil sparing 3 nerve palsy (n=38), pupil involving 3rd nerve palsy (n=8), 4 nerve palsy (n=17) and 6th nerve palsy (n=79). Microvascular ischemia (n=119), traumatic (n=10), combined cranial nerve palsy (n=6), Idiopathic (n=13), Ocular myasthenia (n=5), Inter nuclear ophthalmoplegia (INO) (n=3), Parkinsonism (n= 1), Progressive Supra Nuclear Palsy (n=1) and decompensated comitant strabismus (n=3) were the other causes. Diabetes mellitus (n=102, 63.3%) was the most common vasculopathic factor followed by hypertension. Most patients (n=144, 89.4%) were relieved of their symptoms within six months. Acute binocular diplopia, though an alarming and distressing condition, had very good prognosis in our patients. Meticulous clinical examination and investigations can establish the diagnosis in most of the cases.

Medication-Induced Oculomotor Dysfunction: a report of two cases

Background: Consideration of current medications and their interactions as a source of new onset oculomotor dysfunction is imperative for providing good eye care. Medication etiology should be particularly suspected when the symptoms begin with the initiation of a new medication and resolved with discontinuation of the same medication. Case Reports: Presented are two cases demonstrating that it is prudent to be aware of medications capable of inducing oculomotor dysfunction. Each case reveals instances of acute oculomotor dysfunction after the initiation of a central nervous system affecting medication. Case 1 details a partial pupil sparing third nerve palsy after initiating the anti-anxiety drug, buspirone. Case 2 accounts the addition of zolpidem (Ambien), a sedative, and the subsequent new onset nystagmus and hypertropia. Conclusion: In each case, no organic etiology was found, and the oculomotor dysfunction resolved following discontinuation of the recently added CNS affecting medication. Specific drug interactions are also considered as contributing factors to the oculomotor dysfunction in both cases.

Pupil sparing Cranial Nerve III Palsy and Hemiparesis, Weber Syndrome: A Case Report and literature review

Midbrain stroke especially Weber syndrome is a rare case seldom reported in the literature. It involves oculomotor nerve palsy and contralateral hemiparesis. A 46-year-old female presented with sudden onset of blurring of vision along with right-sided hemiparesis, right upper motor neuron type cranial nerve (CN) VII palsy, left-sided CN III palsy, and left-sided ptosis. Magnetic resonance imaging (MRI) revealed T2 flair/hyperintensity in the left side of the midbrain, bilateral gangliocapsular regions, and centrum semi vale indicating acute infarct and chronic ischemic changes respectively as well as a high signal area on the T2/FLAIR sequence, indicating sinusitis. Ocular manifestations of a midbrain stroke are highlighted in this case study, particularly Weber syndrome, which also entails contralateral hemiparesis. A better prognosis can be achieved with early diagnosis and treatment. In our case report, a CT scan was found to be normal and the diagnosis of Weber syndrome was made only after MRI confirming its sensitivity to detect intracranial lesions.

Reader Response: Pearls & Oy-sters: Isolated Oculomotor Nerve Palsy due to Pituitary Apoplexy Missed on CT Scan

I enjoyed reading this interesting case report by Marzoughi et al.1 As explained by the authors, medial lesions of the cavernous sinus can spare other neighboring cranial nerves and present as isolated oculomotor nerve palsy such as in the reported case. However, the authors do not relate to the innervation of the pupil. Their patient had briskly reactive pupils to light and accommodation, that is, the parasympathetic fibers were spared. Pupillary sparing in third nerve palsy has been described in cavernous sinus lesions that save the superior and lateral aspect of the nerve where pupillary parasympathetic fibers are concentrated. Nevertheless, the right pupil was larger than the left. The anisocoria became worse in bright light vs dim light, which indicates a parasympathetic lesion and is in discrepancy with the finding of brisk reaction to light. Could the mild dilatation of the right pupil be due to an irritative lesion of the sympathetic fibers to the right pupil? If this was the case, signs of lesion of the ophthalmic branch of the trigeminal nerve—which contains sympathetic pupillary fibers—should be present. Finally, a physiologic anisocoria could account for mild pupil differences but should not differ in dim vs bright light. Since this is a teaching article, I would be grateful for an explanation.

AB0853 IGG4-RELATED DISEASE CAUSING OCULAR NERVE PALSIES AND ORBITAL APEX SYNDROME: CASE REPORT AND LITERATURE REVIEW

Background:IgG4-Related Disease (IgG4-RD) is a systemic immune-mediated fibroinflammatory condition. The epidemiology is not well defined: it usually affects adults from middle-age onwards, predominantly male. Both B and T-cells are central in IgG4-RD pathogenesis, as demonstrated by the efficacy of B-cell depletion therapy.IgG4-RD can affect multiple organs including the central and peripheral nervous system, producing a constellation of clinical symptoms and signs, depending on the organ structures involved.IgG4-related orbital disease is relatively rare can implicate all extra-ocular muscles, structures emerging from the Orbital apex, optic canal, or superior and inferior orbital fissure. Depending on the structures involved, it can produce different or sometimes subtle clinical presentations, posing diagnostic challenge. There were case reports of IgG4-related ophthalmic disease misdiagnosed as intraocular tumour.Objectives:IgG4-RD is increasingly recognised as an entity affecting the head and neck region. However, it rarely involves skull base and presents with orbital apex syndrome. In this current case report, we describe an interesting case of IgG-related orbital disease presenting with ocular nerve palsies and orbital apex syndrome.Methods:Case report.Results:A 36-year-old gentleman with cocaine and alcohol misuse presented with a 2-month history of left sided headache, diplopia, recurrent ear infections, otalgia and hearing loss. Initial imaging suggested left otomastoiditis and intravenous antibiotics were commenced. Contralateral partial third nerve palsy with pupil sparing was elicited. 2 months later, there was worsening right eye ptosis, proptosis, right relative afferent pupillary defect, reduced visual acuity and colour vision as well as a near-complete ophthalmoplegia. Subsequent imaging showed worsening soft tissue swelling centred on the upper left parapharyngeal and masticator space, with multiple perineural enhancement and lateral extension to right orbital apex and orbital fissures. Blood tests only revealed raised IgG4 subclass. Infectious aetiology was excluded. Left nasal mass biopsy performed showed no fungal organism or malignancy. There were lymphoplasmacytic proliferation but no storiform fibrosis or obliterative phlebitis. IgG4 immunostaining on two assessable fields revealed 22 and 17 positive plasma cells respectively, and an IgG4: IgG ratio of <10%, and 50% in the other. Significant improvement was seen clinically and radiologically with antibiotics and a tapering regime of oral Prednisolone. Patient was commenced on Azathioprine as long term immunosuppression.Conclusion:A high degree of clinical suspicion is necessary to diagnose IgG4-RD when presenting with orbital apex syndrome and ocular nerve palsies,IgG4-RD can mimic mastoiditis of infectious aetiology. Other differentials may include cocaine-induced midline destructive lesions and granulomatosis with polyangiitis. The diagnosis can be supported by elevated serum IgG, elevated IgG index and pathognomonic histopathological findings. . The diagnosis of IgG4-related orbital disease should be deliberated on by a multidisciplinary group, with every effort being made to exclude an infectious aetiology, before embarking on immunosuppressive therapy.Primary treatment is with steroids. However, immunotherapy using azathioprine can be utilised in recurrent disease or patients with steroid intolerance.

Sudden Onset Diplopia Associated With Better Diabetes Control - Too Fast Is Not Too Good

Abstract Introduction: Treatment-induced neuropathy of diabetes (TIND) presenting as a new-onset peripheral neuropathy usually associated with autonomic dysfunction has been documented in literature. It can occur when there is a drop of more than 2% in HbA1C in a three month period. Worsening of existing peripheral neuropathy and retinopathy has also been observed suggesting a common pathophysiological mechanism. However, the onset of a cranial neuropathy temporally linked to intensified glycemic control has not been well reported. Case Report: A 32-year-old male patient presented to the emergency department with double vision, preceded by headache, and increased burning in his feet. No other neurologic symptoms were reported. Past medical history was significant for poorly controlled, labile type 1 diabetes mellitus. His home medications included gabapentin for peripheral neuropathy, long-acting and short-acting meal time insulin regimen. Physical examination was unremarkable except for a neurological exam which showed pupil-sparing third cranial nerve palsy. CT angiography of the head and neck revealed no acute ischemia, hemodynamically significant stenosis, aneurysm or dissection. MRI of the brain also showed no abnormality. Review of his prior HbA1c values showed a decrease of 3.6 % (14% to 10.4%) over 3 months. The patient was diagnosed with TIND presenting with simultaneous worsening of peripheral neuropathy and new onset right oculomotor nerve palsy. He was discharged on reduced doses of insulin, targeting a gradual decline in HbA1c after initial stabilization. The patient was also asked to liberalize his diet and was given an eye patch. While his peripheral neuropathy improved, there was not much improvement in his diplopia at a two week follow-up visit. Discussion: Our patient presented with worsening of peripheral neuropathy and pupil sparing oculomotor palsy. Secondary causes including CVA and aneurysm were excluded with brain imaging. Diabetes related microvascular disease presenting with pupil sparing oculomotor palsy is commonly seen in patients older than 50 years of age with uncontrolled diabetes and other vascular risk factors like hypertension and dyslipidemia. Absence of hypertension and hyperlipidemia, sudden onset of presentation and an intense control of DM as evidenced by rapid decline of A1C make TIND the likely cause for his presentation. The paradoxical worsening of his peripheral neuropathy also supports the diagnosis of TIND. Through this case, we highlight the importance of recognizing this entity as management differs significantly. Quality improvement metrics for practitioners in various institutions often include HbA1c target ranges in diabetic patients. Our case highlights the issues associated with rapid corrections of HbA1c and thus we advocate for a gradual decrease of 2 % every 3 months till target goals are reached.

Acute Third Nerve Palsy with Pupillary Sparing: A Rare Manifestation of Sphenoid Sinus Mucocele

Mucocele of para-nasal sinus is a rare entity resulting from chronic accumulation of mucoid secretion, leading to progressive thinning of sinus bony wall. We are presenting a case report of a middle age female patient with sphenoid mucocele causing sudden third cranial nerve palsy with pupil sparing. She was managed by urgent endoscopic marsupialzation.

Mean Change in Refractive Status After Levator Muscle Resection in Patients of Simple Congenital Ptosis

Purpose: To determine the mean change in refractive status after Levator muscle resection in patients of simple congenital ptosis.
 Study Design: Quasi Experimental study.
 Place and Duration of Study: This study was carried out at Institute of Ophthalmology, King Edward Medical University, Mayo Hospital Lahore, from 2014 to 2015.
 Material & Methods: Fifty patients were selected under the age of 12 years with no sex predilection presenting with simple congenital ptosis of pupil sparing type. Patients with neurogenic, aponeurotic ptosis, mechanical ptosis, Blepharophimosis syndrome, patients with previous failed ptosis surgery and patients with corneal anomalies were excluded. After history and examination, Cycloplegic retinoscopy was done, which was repeated after surgical intervention. All interventions were done by a single ophthalmic surgeon. The patients were examined and discharged on the second post operative day. All data including preoperative and postoperative recordings of spherical and cylindrical values were collected on a self-designed proforma. Follow up period was extended to 3 months. At 3rd month cycloplegic retinoscopy was done and change in character of refractive status was analyzed. Mean change in refractive status was calculated by difference of preoperative and postoperative sphere and cylinder values.
 Results: Mean age of patients was 4.26 ± 3.78 years. Mean change in spherical value after treatment was 0.447 ± 0.339. This difference was statistically significant with p-value = 0.000. While mean change in cylindrical value after treatment was 0.640 ± 0.580. This difference in cylinder value after treatment was also statistically significant with p-value = 0.000.
 Conclusion: Levator muscle resection has a significant effect on refractive status of the patients with congenital ptosis.

Incidence and Etiologies of Third, Fourth and Sixth Nerve Palsy with Binocular Diplopia at DR. Wahidin Sudiro Husodo General

The purpose of this study is to determine the incidence and etiology of III, IV and VI nerve paralysis with binocular diplopia in RSUD dr. Wahidin Sudiro Husodo General Hospital Mojokerto. Method of this study was descriptive study using secondary medical record data. Incidence rates are adjusted for age and sex distribution of the population. The mean age of onset was 45.6 years, seven male subjects (58.3%) and five female subjects (41.7%). We identified 12 cases of acquired III, IV and VI nerve palsy over a 4-year period. The most common cause of binocular diplopia was sixth nerve palsy (33.3%), 3 patients experienced partial third nerve palsy (25%), one patient with third nerve palsy with pupil sparing (8.3%). Most common etiology was microvascular (58,3%), neoplasms (16.7%), aneurysms (8.3%) trauma (8.3%), and post meningioma neurosurgery (8.3%). Six patients (50%) with microvascular third nerve palsy had diabetes mellitus, while 1 patient (8.3%) had grade 2 hypertension. The most common cause of binocular diplopia was VI nerve palsy. Risk factors such as hypertension and diabetes mellitus which have a significant effect on diplopia. Patients with N. III, IV and VI palsy need to be done early MRI examination so that complications and progression can be prevented.

Frequency of Neurogenic Strabismus in Al-Ibrahim Eye Hospital, Karachi

Purpose: To determine the frequency of Neurogenic Strabismus presenting at the Orthoptics Clinic in Al-Ibrahim Eye Hospital, Malir Karachi. Study Design: Cross-sectional study. Sampling Technique: Non-probability convenient sampling. Place and Duration of Study: Orthoptics Clinic of Al-Ibrahim Eye Hospital (AIEH) Karachi, Pakistan from May to October, 2018. Material and Methods: This study included 349 subjects age ranged from 5 to 75 years. All ocular examinations for strabismus were performed, including cover test, prism cover test and Hess chart. Demographic features and etiologies were recorded, and the causes of extra ocular muscle palsies were grouped as; trauma, diabetes, hypertension and others. SPSS version 20.0 was used to analyze the data. Results: Frequency of neurogenic strabismus was found to be 6%. Out of 21 subjects, 8 (38.1%) subjects had diabetes, followed by 2 (9.5%) subjects with hypertension, 5 (23.8%) subjects with ocular trauma and 6 (28.6%) subjects with other causes. The most commonly affected side was the right eye seen in 13 (61.9%) subjects. The most common ocular motor nerve involved was abducent (sixth) nerve in 13 (61.9%) subjects, followed by oculomotor (third) nerve in 4 (19%) subjects. Out of the patients with third nerve palsy 3 (14.3%) subjects had pupil sparing and only 1 (4.8%) subject had no pupil sparing. Conclusion: Sixth nerve was the most common nerve involved and most common etiology was uncontrolled diabetes. Keywords: Neurogenic Strabismus, Cranial nerve palsies, Paralytic strabismus

Pupil-sparing Isolated Fascicular Third Cranial Nerve Palsy due to Infarction: Report of a Case and Literature Review

Because of their anatomic proximity, pupillary and inferior rectus functions are linked in most cases of fascicular oculomotor palsy, with either sparing or involvement of both. A 40-year-old woman presented with painless binocular diplopia and left ptosis. Examination additionally showed limitations of the adduction and depression in the presence of normal supraduction and intact pupillary function in the left eye. Findings of other neurological neuro-ophthalmologic examination were normal. A magnetic resonance imaging revealed a diffusion restriction in the left dorsomedial midbrain. An ischemic lesion restricted to the oculomotor fascicles can cause inferior rectus palsy in the presence of pupillary sparing, which should be differentiated from a microvasculopathic lesion involving the subarachnoid portion of the oculomotor nerve.

Painful ophthalmoplegia with normal cranial imaging

BackgroundPainful ophthalmoplegia with normal cranial imaging is rare and confined to limited etiologies. In this study, we aimed to elucidate these causes by evaluating clinical presentations and treatment responses.MethodsCases of painful ophthalmoplegia with normal cranial MRI at a single center between January 2001 and June 2011 were retrospectively reviewed. Diagnoses of painful ophthalmoplegia were made according to the recommendations of the International Headache Society.ResultsOf the 58 painful ophthalmoplegia cases (53 patients), 26 (44.8%) were diagnosed as ocular diabetic neuropathy, 27 (46.6%) as benign Tolosa-Hunt syndrome (THS), and 5 (8.6%) as ophthalmoplegic migraine (OM). Patients with ocular diabetic neuropathy were significantly older (62.8 ± 7.8 years) than those with benign THS (56.3 ±12.0 years) or OM (45.8 ± 23.0 years) (p < 0.05). Cranial nerve involvement was similar among groups. Pupil sparing was dominant in each group. Patients with benign THS and OM responded exquisitely to glucocorticoid treatment with resolved diplopia, whereas patients with ocular diabetic neuropathy didn’t (p < 0.05). Patients with OM recovered more rapidly than the other groups did (p < 0.05). Overall, most patients (94.8%) recovered completely during the follow-up period.ConclusionsOcular diabetic neuropathy and benign THS accounted for most of the painful ophthalmoplegias in patients with normal cranial imaging. Patient outcomes were generally good.

Exceptions that Prove the Rules in Neuro-Ophthalmology

Exceptions that Prove the Rules in Neuro-Ophthalmology

Case report with sequential nerve conduction studies: A very old patient with atypical foodborne botulism type a with pupil sparing treated with botulinum antitoxin

Case report with sequential nerve conduction studies: A very old patient with atypical foodborne botulism type a with pupil sparing treated with botulinum antitoxin

Anterior clinoid mucocele presenting with orbital apex syndrome

Background:Pneumatized anterior clinoid process is a common anatomic variant. Mucocele formation is a known complication of clinoid drilling during certain intracranial operations; however, mucoceles of pneumatized anterior clinoid processes have been found to spontaneously occur.Case Description:A 44-year-old male presented with complaints of left-sided retro-orbital pain, double vision, and numbness over the upper face and scalp on the left side of 1-week duration. On examination, he was found to develop cranial nerve III, IV, and VI palsies with pupillary sparing, ophthalmic division cranial nerve V dysfunction, and eventually, the onset of vision loss.Conclusions:We report a case of spontaneous anterior clinoid process mucocele presenting with orbital apex syndrome. This was treated successfully with anterior clinoidectomy for decompression.

Recurrent cranial neuropathy in diabetes

Case report A 59-year-old man presented to our department with sudden onset of right-sided ptosis (figure 1) and diplopia, preceded by pain behind the right orbit. This patient had a left sixth nerve palsy a year ago, which had resolved spontaneously. His past history was notable for type 2 diabetes of 15 years duration. On examination, the visual acuity, colour vision and visual fields were normal. Both pupils reacted equally to light. Retinal examination showed features consistent with non-proliferative diabetic retinopathy. There was ptosis of the right eye (figure 1). On lifting the eyelid, abduction was observed in the natural position, with pupillary sparing (figure 2), consistent with a diagnosis of third nerve palsy. The results from examination of other cranial nerves, pyramidal tracts, sensory and cerebellar systems were normal. Glycaemic control was satisfactory on a combination of insulin and metformin with an admission HbA1C of 7.2%. Blood investigations including full blood count, renal and liver functions, lipid profile, serum electrolytes, erythrocyte sedimentation rate, ANA, viral markers and cerebrospinal fluid analysis were within normal limits. A brain MRI did not reveal any structural lesions, especially around the cavernous sinuses or posterior orbits. A clinical diagnosis of recurrent cranial neuropathy secondary to diabetes was considered and the patient made a complete recovery within 12 weeks of this presentation.