CLINICAL

New-onset initially diagnosed as

Neil Sharma, Ju-Lee Ooi, Rebecca Davie, Palvi Bhardwaj

Case Question 2 divides into superior and inferior branches, Joe, 66 years of age, was referred with What are the causes of an isolated which enter the through the superior a 2-week history of a right upper palsy? orbital fissure. abnormality. He complained of associated The superior division innervates initially, but this subjectively Question 3 the levator palpebrae superioris and improved after a few days as the eye What clinical features raise the index of superior rectus, while the inferior division became more difficult to open. He had a suspicion of a compressive lesion? mild, intermittent headache for 4 weeks, relieved with oral paracetamol. There Question 4 were no other neurological symptoms. What investigation does this patient He had no other symptoms of giant cell urgently require? arteritis. His past medical history included hypercholesterolaemia for which he was Case continued taking regular statin therapy. He was an Joe was referred for an urgent CT ex-smoker with a 40 pack-year history. angiogram. This showed a large Initially, Joe’s general practitioner (GP) unruptured posterior communicating diagnosed conjunctivitis and prescribed artery (Figure 1). He was chloramphenicol drops four times daily. admitted under the neurosurgical team. One week later the symptoms had not improved and Joe was referred to the eye Question 5 Figure 1. 3-dimensional reconstruction image clinic complaining of increasing right What are the surgical options for dealing of the CT-angiogram showing an unruptured periocular pain. On examination, his aneurysm of the posterior communicating artery with an unruptured intracranial aneurysm? (white arrow) visual acuity was 6/5 in each eye. There was near complete ptosis of the right eye. Question 6 The right was fixed and dilated, and What are the important learning points there was no direct or consensual light from this case? Table 1. Common causes of reflex present. Cover testing revealed oculomoter nerve palsy right and hypotropia, while Answer 1 Intracerebral aneurysm extraocular movements showed limited The oculomotor nerve begins as several right adduction and elevation. These nuclei in the dorsal midbrain at the level Infarction findings were consistent with a right, of the superior colliculus. The fascicle /microvascular damage partial, pupil-involving oculomotor nerve emerges from the midbrain and passes Cavernous sinus lesion palsy. into the interpeduncular space. The basilar Trauma Question 1 portion runs parallel to the posterior communicating artery in the subarachnoid Meningitis What is the course of the oculomotor space. The nerve then enters the Giant cell arteritis nerve? cavernous sinus by piercing the dura, and

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innervates the medial rectus, inferior pupillomotor fibres supplied by pial blood easier accessibility and rapid acquisition rectus and inferior oblique muscles. The vessels. By contrast, the microangiopathy time, although this varies depending on branch to inferior oblique also contains associated with microvascular disease the institution.7 preganglionic parasympathetic fibres that affects the vasa nervorum blood supply, innervate the ciliary muscle and spincter causing ischaemia of the main trunk of Answer 5 pupillae. the nerve, while generally sparing the The two main neurosurgical options are superficial pupillomotor fibres. an endovascular coiling procedure or Answer 2 Pain is another important feature of microsurgical clipping of the aneurysm. A There are numerous causes of isolated many third nerve palsies due to posterior study retrospectively analysed outcomes oculomotor nerve palsy. Some of the communicating artery aneurysm. for oculomotor palsy recovery from seven more common causes are listed in Presentation is typically with an ipsilateral patients who underwent endovascular Table 1.1 Microvascular ischaemic disease frontal headache. coiling, compared with 10 patients who due to cardiovascular risk factors is one It is important to remember that these underwent clipping.8 There was no of the most common causes. However, features are not definitive. There have reported difference between the two it is important to consider and exclude been several case reports of patients with groups. potentially life-threatening causes such as a third nerve palsy due to aneurysm that Often the decision is based on a variety giant cell arteritis and intracerebral artery presented with a normally reactive pupil.4 of surgical factors. In this case craniotomy aneurysm. If a pupil-sparing third nerve palsy is not with clipping was deemed the preferred Up to 30% of cases of oculomotor imaged initially, it is necessary to monitor option. Joe underwent this operation the nerve palsy are due to intracerebral closely to ensure the pupil does not following morning. The same afternoon he aneurysm.2 The most common location become involved at a later stage. Similarly, was recovering well (Figure 2). Note the is at the junction of the posterior pain is not universal in aneurysmal near complete right ptosis persists. communicating artery and the internal compression. carotid artery. It is imperative to promptly Answer 6 diagnose these patients, as missing an Answer 4 There are several important lessons to aneurysm that subsequently ruptures Formal catheter angiography has been be learned from this case. These are carries a significant risk for morbidity and considered the gold standard for the summarised in Table 2. It is important to mortality. The average time from the onset diagnosis of cerebral . However, be able to differentiate eyelid ptosis from of a third nerve palsy due to an unruptured it may be associated with a 1–2% risk of eyelid swelling, which may be the result of posterior communicating artery aneurysm systemic or neurological complications.5 an infective process such as conjunctivitis. to major subarachnoid haemorrhage has With an experienced neuro-radiologist Viral conjunctivitis is typically associated been reported as 29 days.3 interpreting the images, modern computed with conjunctival hyperaemia, watery tomography angiography (CTA) and discharge, conjunctival follicles and Answer 3 magnetic resonance angiography scans pre-auricular lymphadenopathy, and is The presence of pupillary involvement should detect nearly all aneurysms often bilateral. Eyelid ptosis is defined raises the index of suspicion for a responsible for an isolated oculomotor as less than 2 mm between the upper compressive aneurysmal lesion, due to nerve palsy.6 Many clinicians prefer CT/CTA eyelid margin and pupil light reflex, or an the characteristic superficial location of the for the initial study because of its relatively asymmetry of more than 2 mm between

Table 2. Important learning points from this case

Neurogenic ptosis should not be confused with eyelid swelling from conjunctivitis or periorbital

A third nerve palsy will often present with ptosis, and the may be in an abducted and depressed position: ‘down and out’

When assessing a patient with either ptosis or diplopia, it is imperative to check that the are equal in size and react equally to light

Urgent referral is crucial if there are signs of a third nerve palsy, and neuro-imaging may be Figure 2. Post-operative picture following microvascular clipping of the intracranial aneurysm required to investigate for an intracranial aneurysm Note the near-complete right ptosis It is important to ask about symptoms of giant cell arteritis if the patient is 50 years or older

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the eyes.9 Important causes include Hospitals NHS Trust, Cambridge Cambridgeshire, UK; 3. Lee AG, Hayman LA, Brazis PW. The evaluation congenital ptosis, levator aponeurosis Ophthalmologist, Eye and Specialists, Green of isolated third nerve palsy revisited: An update Square, NSW; Clinical Lecturer, University of Sydney, on the evolving role of magnetic resonance, dehiscence, Horner’s syndrome, Westmead Hospital, Westmead, NSW; Conjoint computed tomography and catheter angiography. and oculomotor nerve Lecturer, University of NSW, Prince of Wales Hospital, Surv Ophthalmol 2002;47:137–57. Sydney, NSW palsy. In any patient with possible eyelid 4. Kissel JT, Burde RM, Klingele TG, Zeiger HE. Rebecca Davie BSc, MBBS, Pupil-sparing oculomotor palsies with internal ptosis, it is mandatory to lift the ptotic Registrar, Cambridge University Hospitals NHS Trust, carotid-posterior communicating aneurysms. Ann lid and assess pupillary light reflexes and Cambridge, Cambridgeshire, UK Neurol 1983:13:149–54. extraocular movements. Any patient with Palvi Bhardwaj MBBS, BMedSci (Orthoptics), 5. Heiserman JE, Dean BL, Hodak JA, et al. Ophthalmology Registrar, Luton and Dunstable Neurologic complications of cerebral angiography. or restricted eye movements University Hospital, Addenbrooke’s Hospital, Am J Neuroradiol 1994;15:1401–07. warrants urgent referral. Cambridge University Hospitals NHS Foundation Trust, 6. Elmalem VI, Hudgins PA, Bruce BB, et al. Cambridge Biomedical Campus, Cambridge, UK Underdiagnosis of posterior communicating Authors Competing interests: None. artery aneurysm in non-invasive brain vascular studies. J Neuroophthalmol 2011;31:103–09. Neil Sharma BSc(Med), MBBS (Hons), MPH, Provenance and peer review: Not commissioned, 7. Trobe JD. Searching for brain aneurysm in MMed (Refract Surg), FRANZCO, Senior Clinical externally peer reviewed. Fellow, Cambridge University Hospitals NHS Trust, third cranial nerve palsy. J Neuroophthalmol Cambridge Cambridgeshire, UK; Ophthalmologist, 2009;29:171–73. Eye and Retina Specialists, Green Square, Sydney, References 8. Ahn JY, Han IB, Yoon PH, et al. Clipping vs. coiling NSW; Consultant Ophthalmologist, Westmead 1. Sadagopan KA, Wasserman BN. Managing the of posterior communicating artery aneurysms Hospital, Westmead, NSW; Clinical Lecturer, patient with oculomotor nerve palsy. Curr Opin with third nerve palsy. 2006;66:121– University of Sydney, Central Clinical School, Sydney, Ophthalmol 2013;24:438–47. 23. NSW. [email protected] 2. Green WR, Hackett ER, Schlezinger NS: Neuro- 9. Small RG, Sabates NR, Burrows D. The Ju-Lee Ooi BSc (Med), MBBS, MPH, GradDipPaed, ophthalmologic evaluation of ocular motor palsies. measurement and definition of ptosis. Ophthal FRANZCO, Clinical Fellow, Cambridge University Arch Ophthalmol 1964;72:154–67. Plast Reconstr Surg 1989;5:171–75.

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