Isolated Pupil-Involving Third Nerve Palsy as the First Presentation of Sarcoidosis

Abstract

Neurosarcoidosis is seen in 5–15% of patients with systemic sarcoidosis. The most common cranial nerve presentations are optic neuropathy and facial nerve palsy. The authors present a case of sarcoidosis presenting with a pupil-involving third nerve palsy. The patient responded to corticosteroid therapy with resolution of investigations her cranial nerve palsy but progressed to develop cerebellar signs. This is the first documented case of a pupil-involving third nerve palsy occurring as the first presentation of neurosarcoidosis. Although typically a pupil-involving third nerve palsy necessitates urgent neuroimaging to rule out a posterior communicating artery aneurysm, it is important to recognise inflammatory causes in the differential diagnosis.     

Transsphenoidal diplopia

A 46-year-old man developed a pupil-involving incomplete third cranial nerve palsy after undergoing transsphenoidal resection of a pituitary macroadenoma. A CT scan 1 week later revealed postoperative changes, with no new mass lesion, hemorrhage, or orbital fracture. The third cranial nerve palsy spontaneously improved over the ensuing week. Postoperative ocular motor palsy is an uncommon complication of transsphenoidal pituitary surgery. Neuro-imaging should be performed to exclude the presence of a new compressive lesion.     

Massive Oculomotor Nerve Enlargement: A Case of Presumed Schwannomatosis

A 45-year-old man presented with a slowly progressive pupil-involving third nerve palsy. Magnetic resonance imaging (MRI) revealed a tubular lesion extending from the interpeduncular cistern through the cavernous sinus and into the left orbit where it branched into a superior and an inferior division, clearly outlining the anatomy of the third cranial nerve. Multiple other, less pronounced, enlarged cranial nerves were noted. The differential diagnosis included chronic inflammatory demyelinating polyneuropathy (CIDP), hereditary motor and sensory neuropathy (HMSN), neurofibromatosis (NF), and schwannomatosis. The absence of other muscle weakness and of sensory symptoms combined with normal peripheral nerve conduction studies effectively ruled out the hypertrophic polyneuropathies and pointed to a syndromic cause of multiple benign peripheral nerve sheath tumours (PNSTs). The authors are treating this case as presumed schwannomatosis, a syndrome similar to NF2 with much lower frequency of acoustic neuromas.     

Isolated cranial nerve III palsy as the presenting manifestation of HIV-related large B-cell lymphoma: clinical, radiological and postmortem observations: report of a case and review of the literature

Rarely can a neurologically isolated cranial nerve III palsy be the presenting manifestation of central nervous system lymphoma. We detail the clinical, radiological, and pathological features of a previously healthy 45-year-old man presenting with an isolated, pupil-involving, right cranial nerve III palsy due to human immunodefiency virus (HIV) related non-Hodgkin lymphoma. Magnetic resonance imaging demonstrated bilateral peripheral cranial nerve III enhancement with no brain parenchymal or leptomeningeal abnormalities. Cerebrospinal fluid analysis revealed a monocytic pleocytosis with an elevated protein concentration and depressed glucose level. Morphologic and flow cytometric analysis of the cerebrospinal fluid was compatible with a large B-cell lymphoma. Serologic tests for HIV were positive. Postmortem examination of the brain revealed malignant lymphomatous cell infiltration of both cranial nerve III, diffuse leptomeningeal disease and focal superficial subependymal and subpial invasion. Based on our review of the literature, we were able to find only 10 detailed cases of cranial nerve III palsy as the presenting manifestation of central nervous system lymphoma. Furthermore, none of the previously reported cases correlated the magnetic resonance imaging findings with the gross and histopathologic observations.     

Acute Transient Oculomotor Nerve Palsy from Presumed Cavernous Angioma in an Infant

Cavernous angiomas of the cranial nerves are extremely rare, and those of the oculomotor (third) cranial nerve are rarer still, with no previous cases having been reported in the literature. We report herein the first case of a presumed cavernous angioma involving the subarachnoid portion of the left third nerve presenting as an acute left third nerve palsy in an infant. The child was followed without intervention and the palsy completely resolved. Given the poor functional results with attempted surgical excision and the potential for spontaneous improvement in oculomotor nerve function, it is reasonable to follow patients with these lesions without intervention.     

Acquired, isolated third nerve palsies in infants with cerebrovascular malformations

PURPOSE: To report two infants with acquired, isolated third nerve palsies attributable to intracranial cerebrovascular malformations. DESIGN: Observational case report. METHODS: Two patients are described. Each was examined in a university-based pediatric ophthalmology and neuro-ophthalmology practice. RESULTS: An 8-month-old child presented with a pupil-involving partial left third nerve palsy because of a partially thrombosed fusiform aneurysm of the left internal carotid artery. A 3-month-old infant developed a right third nerve palsy from a giant arteriovenous fistula arising from an M2 branch of the right middle cerebral artery. CONCLUSION: Patients younger than 8 months and 3 months with acquired, isolated third nerve palsies resulting from intracranial cerebrovascular malformations could not be found in a MEDLINE search. In conclusion, at even this young age, acquired, isolated third nerve palsies may be the initial manifestation of an intracranial aneurysm or fistula. Magnetic resonance imaging (MRI) and MRI-angiography were adequate for detecting these processes.     

Third Nerve Palsy as the Presenting Sign of Metastatic Endometrial Carcinoma with Lymphovascular Space Invasion

ABSTRACT

We present a case of endometrial carcinoma (EC) presenting with a third cranial nerve palsy due to metastasis to the cavernous sinus. Although the tumour was only stage 1B, histopathologic features including higher grade and lymphovascular space invasion (LVSI) increase the risk of recurrent and metastatic disease. To our knowledge, this is the first case of EC presenting as a third cranial nerve palsy in the English-language ophthalmic literature. Clinicians should be aware that the risk of metastatic EC including neuro-ophthalmic presentations depends upon not only stage and grade but also the presence of unique histopathological findings such as LVSI.     

Oculomotor nerve paralysis and bilateral facial nerve paralysis as presenting signs of Lyme disease

Lyme disease is a tick-borne illness caused by the spirochete Borrelia burgdorferi . On occasions, patients with Lyme disease present with isolated cranial nerve palsies; multiple palsies have rarely been reported in the literature.We discuss the case of a patient, who presented with a third nerve palsy and bilateral seventh nerve palsies and was subsequently diagnosed as suffering from Lyme disease.To our knowledge, this is the first reported case of Lyme disease presenting with this combination of neurological signs. A 53-year-old male from the Scottish Western Isles presented with a two-day history of double vision. On examination, he was found to have a left third cranial nerve palsy and a left seventh cranial nerve palsy. Five days later, the patient developed a right seventh cranial nerve palsy. Serum and CSF enzymelinked immunosorbent assay (ELISA) and Western blot analysis were both positive for Lyme disease as was CSF polymerase chain reaction (PCR) analysis. Subsequently, the patient gave a history of multiple tick bites over the previous few years. He was commenced on appropriate antibiotic therapy and oral prednisolone. A rapid improvement in his condition was noted. This case demonstrates the importance of considering the diagnosis of Lyme disease in patients with multiple cranial neuropathies, especially those residing in, or with a history of travel to, endemic areas. In addition, it illustrates how early diagnosis, with the institution of appropriate therapy, leads to a prompt improvement in neurological symptoms and signs.     

Neurological Imaging in Acquired Cranial Nerve Palsy: Ophthalmologists vs. Neurologists

ABSTRACT

Purpose: Cranial nerve palsies often require neurological imaging by MRI. Guidelines on whether or not to utilize MRI have been absent or lack clarity. In daily practice, both neurologists and ophthalmologists treat patients with cranial nerve palsy and determine whether neuro-imaging is required. There appear to be differences in policy with respect to neuro-imaging. The question, which will be answered in this study, is the following: to what extent do differences in policy exist between ophthalmologists and neurologists regarding imaging by MRI of patients with acquired ocular cranial nerve palsy?

Method: PubMed database was searched for literature on acquired cranial nerve palsy and MRI scanning performed by ophthalmologists and neurologists. Case series published between 2000 and 2015 were included. The first author screened the literature on eligibility, profession of the authors, and conducted data abstraction.

Result: Ten case series were found eligible for analysis. A total of 889 cranial nerve palsies were described, 770 by ophthalmologists and 119 by neurologists. The age range of patients in all case series was 2 to 96 years of age. The oculomotor nerve was investigated in 162 patients, the trochlear nerve in 131 patients, and the abducens nerve in 486 patients. All neurologists (n=3) and 2 out of 7 investigated ophthalmologists recommended performing MRI scanning in every patient who presented with an ocular cranial nerve palsy, while 5 ophthalmologists (5/7) opted to triage patients for risk factors associated with cranial nerve palsies prior to ordering MRI imaging. When different groups of patients were viewed separately, it became apparent that almost all specialists agreed that every patient with a third nerve palsy and patients under 50 years of age should undergo MRI scanning. In patients with fourth nerve palsy, MRI scanning was not indicated.

Conclusion: The neurologists in this study were more likely to perform MRI scanning in every patient presenting with ocular cranial nerve palsy. Ophthalmologists were more likely to determine risk factors associated with cranial nerve palsy, which they took into account when deciding whether or not to perform neurological imaging in patients aged more than 50 years or presenting with abducens nerve palsy.     

BB embolus causing monocular blindness in a 9-year-old boy

PURPOSE: To report a case of monocular blindness resulting from intravascular bullet migration. DESIGN: Observational case report. METHODS: A 9-year-old boy presented to the emergency room with a penetrating BB gun injury to the chest. RESULTS: Carotid angiography localized the bullet to the right internal carotid artery. He subsequently suffered blindness in the right eye, a right pupil-involving third nerve palsy, left hemiparesis, and a probable left homonymous hemianopia. CONCLUSION: BB gun injuries may result in devastating ophthalmologic as well as systemic consequences due to bullet embolization.     

Pediatric ocular motor cranial nerve palsy: Demographics and etiological profile

Purpose:The aim of this study was to describe epidemiological and clinical characteristics of pediatric ocular motor cranial nerve palsy.Methods:This was a retrospective record-based study, carried out at a tertiary eye care hospital in India, between January 2011 and January 2015 and included patients up to 16 years of age at the time of presentation, diagnosed with third, fourth, sixth nerve palsy or a combination of these with other cranial nerve palsy. Data analyzed included demographic details, etiologies, presence or absence of amblyopia, relevant investigations, and management.Results:A total of 90 cases were included in the study. Eighty patients (88.88%) presented with isolated nerve palsy. Forty-three patients (47.77%) had congenital nerve palsy. The most common nerve involved was third (n = 35, 38.88%) followed by sixth (n = 23, 25.55%) and fourth nerve (n = 22, 24.44%). Most common cause of third and fourth cranial nerve palsy was congenital (n = 18, 51.42% and n = 17, 77.30%, respectively), while it was trauma for the sixth nerve (n = 7, 30.40%). Amblyopia was most frequently associated with third cranial nerve palsy (n = 27, 77.14%). The radio-imaging yield was maximum (n = 7, 70%) for combined cranial nerve palsy. Overall 44 (48.88%) patients were managed conservatively, while 46 (51.11%) patients needed squint with or without ptosis surgery.Conclusion:The most common ocular motor cranial nerve involved in the pediatric population was the third cranial nerve, and it was found to be the most amblyogenic in this age group. The neuroimaging yield was maximum for combined cranial nerve palsy. The most common conservatively managed nerve palsy in this study group was the fourth nerve palsy.     

False and non-localizing signs in neuro-ophthalmology

The absolute reliance on abnormal neuro-ophthalmologic findings as signposts for particular lesions of the neuraxis must be tempered by a working knowledge of false and nonlocalizing signs and symptoms. Transient monocular visual loss or complex visual hallucinations may lead to neuroanatomic ambiguity, and elevated intracranial pressure is not the most common cause of swollen optic disks in late life. Sixth nerve palsy associated with increased intracranial pressure is possibly the best-known false localizing sign, but other ocular motility disturbances (divergence paresis, convergence insufficiency, and skew deviation) also elude localization. Lastly, the localizing pitfalls of anisocoria--Horner and Raeder syndromes, physiologic anisocoria, pupil-involving third nerve palsy, and benign pupillary dilation--are discussed.     

Third cranial nerve palsy in children.

PURPOSE: To report the causes and the sensory, motor, and cosmetic results after treatment for oculomotor (third cranial nerve) palsy in children. METHODS: Review of the clinical records of children with a diagnosis of third cranial nerve palsy followed up in a university-based pediatric ophthalmology practice between 1981 and 1996. RESULTS: Forty-nine children with 53 affected eyes were followed up for a mean of 5.5 years. Third cranial nerve palsy was partial in 31 children (32 eyes) and complete in 18 children (21 eyes). The palsy was congenital in 20 eyes and caused by postnatal trauma in 17 eyes. Seventeen eyes had aberrant regeneration and four eyes with partial third cranial nerve palsy had spontaneous resolution. Thirty-six children (38 eyes) were affected before visual maturation (age 8 years), and 25 (27 eyes) had amblyopia. Of the five amblyopic eyes with quantifiable visual acuity, none had measurable improvement of Snellen visual acuity during the follow-up period. Overall, visual acuity was between 6/5 and 6/12 at the last follow-up visit in 31 eyes (58%). Ocular alignment was greatly improved after strabismus procedures, with a mean of 1.5 procedures for patients with partial third cranial nerve palsy and 2.3 procedures for those with complete palsy. Binocular function was difficult to preserve or restore but was achieved for some patients with partial third cranial nerve palsy. CONCLUSIONS: Surgical treatment of third cranial nerve palsy is frequently necessary, especially in cases of complete palsy. Multiple strabismus procedures are often needed to maintain good ocular alignment. Surgery can result in cosmetically acceptable alignment of the eyes, but it rarely results in restoration or achievement of measurable binocular function. Treatment of amblyopia is effective in maintaining the level of visual acuity present at the onset of the third cranial nerve palsy, but improvement in visual acuity is difficult to achieve.     

Pediatric third, fourth, and sixth nerve palsies: a population-based study

PURPOSE: To determine the population-based incidence and cause of cranial nerve palsies affecting ocular motility in children in the circumscribed population of Olmsted County, Minnesota. METHODS: The Rochester Epidemiology Project medical records linkage system captures virtually all medical care provided to Olmsted County residents. By means of this database, all cases of third, fourth, and sixth cranial nerve palsy were identified among county residents less than 18 years of age from 1978 through 1992. Medical records were reviewed to confirm the diagnosis, determine the cause, and document county residency. Incidence rates were adjusted to the age and sex distribution of the 1990 white population in the United States. RESULTS: Over this 15-year period, 36 incidence cases of cranial nerve palsy were identified in 35 children in this defined population. The age-adjusted and sex-adjusted annual incidence of third, fourth, and sixth nerve palsies combined was 7.6 per 100,000 (95% confidence interval, 5.1 to 10.1). The most commonly affected nerve was the fourth (36%), followed by the sixth (33%), the third (22%), and multiple nerve palsies (9%). The most common cause was congenital for third and fourth nerve palsy, undetermined for sixth, and trauma for multiple nerve palsies. Although three cases were associated with neoplasia, a cranial nerve palsy was not present at the time of diagnosis in any case. CONCLUSIONS: Unlike many institutionally based referral series, our population-based study provides data on the incidence and cause of third, fourth, and sixth nerve palsies in a geographically defined population. In contrast to previous institutionally based series, nearly half the cases were congenital in origin, and in no case did intracranial neoplasia present as an isolated nerve palsy.     

Bilateral sixth nerve and left third nerve palsy after head trauma

CASE REPORT: To describe a case of combined bilateral cranial nerve palsy of traumatic origin. To determine the lesions that produce the symptoms is useful to define the final prognosis and the best treatment. DISCUSSION: We report the case of a patient who developed a bilateral sixth nerve and left third nerve palsy after head trauma. The underlying lesion was a diffuse axonal injury. After an observation period during which no spontaneous improvement occurred, we administered botulinum toxin with a successful clinical result. Bilateral combined traumatic cranial nerve palsies are rare. When a diffuse axonal injury is present, the chance of spontaneous resolution is poor.     

Parasellar syndrome caused by plasma cell leukemia

A 71-year-old man had incomplete third cranial nerve palsy caused by plasma cell leukemia and a parasellar plasmacytoma. This is the first reported case of plasma cell leukemia in which a central nervous system sign was the initial manifestation. Only one previous case of intracranial plasmacytoma associated with plasma cell leukemia has been reported. The characteristics of intracranial plasmacytomas and of plasma cell leukemia are discussed.     

Bilateral ophthalmoplegia during percutaneous transluminal coronary angioplasty

A 61-year-old woman underwent percutaneous transluminal coronary angioplasty (PTCA) to relieve stenosis of the left anterior descending coronary artery. During the procedure ophthalmoplegia with diplopia developed. The diagnosis was partial right third cranial nerve palsy and left fourth cranial nerve palsy. The cause is presumed to have been a microembolic infarct of the right peripheral third nerve coupled with either decompensation of pre-existing left superior oblique palsy or a separate microinfarct of the left trochlear nerve. Over 7 months of follow-up there was partial resolution of the ophthalmoplegia. Ophthalmoplegia during PTCA is rare, and to our knowledge this complication has not been reported in the ophthalmic literature.     

Central Retinal Artery Occlusion and Third Cranial Nerve Palsy Following Nasal Septoplasty

Background

Postoperative vision loss following routine nasal surgery is an extremely rare and devastating complication. We report a case of unilateral blindness due to central retinal artery occlusion associated with third cranial nerve following septoplasty.

Case Report

We report a patient who developed an unusual central retinal artery occlusion with unilateral blindness following nasal surgery under general anesthesia. A 45-year-old man underwent a nasal septal surgery for severe epistaxis. Soon after recovery, the patient noticed loss of vision in his right eye and was unable to lift his upper eyelid. Upon ophthalmic examinations, we determined that he had right-sided third cranial nerve palsy with central retinal artery obstruction and ptosis of right upper eyelid, restriction of ocular movements, and no perception of light in the right eye. Postoperative computerized tomography scan revealed multiple fractures of the left medial orbital wall, including one near the optic canal. Ptosis and ocular defects were recovered partially, but visual loss persisted until the last follow-up.

Conclusion

This paper highlights one case of complete unilateral blindness from direct central retinal artery occlusion associated with third cranial nerve palsy following an apparently uneventful septorhinoplasty. Ophthalmologists and otolaryngologists should therefore be aware of the possible occurrence of such complications.Key Words: Central retinal artery occlusion, Third nerve palsy, Septoplasty, Nasal polypectomy, Visual loss     

The emerging role of magnetic resonance angiography in the management of patients with third cranial nerve palsy.

PURPOSE: To discuss the sensitivity of three-dimensional time-of-flight (3D TOF) magnetic resonance angiography (MRA) for detecting aneurysms causing third cranial nerve palsy and to propose guidelines for the use of MRA in the evaluation of patients with different clinical presentations of oculomotor nerve palsy. METHODS: From a review of the scientific literature, we determined the proportion of aneurysms of various size that cause third nerve palsy, the sensitivity of MRA in detecting aneurysms of these size classes, and the relationship of aneurysmal size to rupture. RESULTS: The data disclose that properly performed and interpreted 3D TOF MRA will overlook only 1.5% of aneurysms producing third cranial nerve palsy that will, if untreated, rupture during the subsequent 8 years. CONCLUSIONS: Magnetic resonance angiography plays an important diagnostic role in the evaluation of patients with isolated third cranial nerve palsy. However, because of the potentially drastic consequences of overlooking an aneurysm, MRA should be the definitive screening test only among patients with a relatively low likelihood of harboring an aneurysm or a relatively high likelihood of suffering a complication from catheter angiography.     

Isolated Unilateral Trochlear Nerve Palsy Due to Ipsilateral Dorsal Midbrain Infarction

A 67-year-old Japanese man with essential hypertension and diabetes mellitus abruptly developed isolated right-sided trochlear nerve palsy without pain. Four days later, cranial magnetic resonance imaging demonstrated a tiny ischaemic lesion in the caudal and dorsal midbrain on the right side, which might involve the trochlear fascicles after its decussation. The trochlear nerve palsy resolved within 4 days while treated with intravenous anti-platelet agent. This is a first reported case of isolated unilateral trochlear nerve palsy due to ipsilateral midbrain infarction.