Acute onset diplopia. Methods. Introduction. Una O Colmain, Claire Gilmour and Caroline J. MacEwen. Acta Ophthalmologica 2014

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1 Acute onset diplopia Una O Colmain, Claire Gilmour and Caroline J. MacEwen Ninewells Hospital and Medical School, Dundee, UK ABSTRACT. Purpose: To investigate the clinical features of all patients with acute onset diplopia presenting to the ophthalmology department. Methods: The notes of every patient who presented with acute onset (<4-week duration) diplopia to the ophthalmology clinic over a 2-year period were reviewed. Data regarding clinical features, underlying aetiology, past medical history, investigations and outcomes were extracted. Results: One hundred and forty-nine patients presented with 53.7% having an isolated third, fourth or sixth nerve palsy, 10.7% a mechanical cause, 10.1% a dysfunction of higher control, 8.1% decompensation of a pre-existing heterophoria, 6.7% idiopathic, 5.4% causes of monocular diplopia and 5.3% another diagnosis. Neuroradiological investigation identified that <5% of patients had a serious underlying pathology, which required immediate management; 80.5% had a diagnosis and underlying aetiology, which were obvious at presentation based only on clinical information and evaluation. Conclusions: Acute onset diplopia is an uncommon and challenging presentation for the ophthalmologist to manage. These results demonstrate that the aetiology is commonly identifiable at the first presentation based on clinical evaluation, only a small percentage require urgent radiological investigation and a small minority of cases are likely to have serious emergent pathology. Key words: binocular CNS disorders, intracranial cranial nerve, palsy diplopia aetiology microvascular nervous system diseases complications Acta Ophthalmol. 2014: 92: ª 2013 The Authors Acta Ophthalmologica ª 2013 Acta Ophthalmologica Scandinavica Foundation. Published by Blackwell Publishing Ltd. doi: /aos Introduction Acute onset diplopia is an uncommon, but significant and potentially serious, presenting symptom to ophthalmology departments, general practitioners (GPs) and optometrists (Glaser 1990; Morris 1991; Comer et al. 2007). The patient with sudden diplopia is challenging to manage acutely as the possibility of serious systemic associations such as demyelinating disease, vascular disease and neoplasia (Glaser 1990) causes an increased level of concern amongst clinicians. There is a small number of case series of diplopia as the presenting symptom to primary care ophthalmology, which have demonstrated the underlying aetiology for all causes of diplopia (Yapp 1973; Morris 1991; Comer et al. 2007); these articles highlight the diverse range of causes, including chronic conditions. Murchison et al. (2011) published the outcomes of neuroimaging in acute onset isolated cranial neuropathies, and the results reinforce the low yield of neuroimaging in such cases. We present a case series that includes only patients with acute onset <4 weeks of diplopia and investigate the aetiologies and outcomes of these cases; the aim is to explore the clinical features of those who demonstrated serious underlying pathology. Methods A retrospective case note review was performed of every patient who presented to the ophthalmology clinic with acute (<4 weeks) onset diplopia between 1 January 2009 and 31 December Patients included those who were self-referrals as well as those referred from optometrists, GPs and other specialties. All patients were seen by an ophthalmologist on presentation and an orthoptist within 72 hr. We extracted information on age, clinical features, diagnosis, aetiology, investigations and outcomes. Any patients who were under orthoptic or ophthalmology department care with an ongoing condition that could have caused their symptoms were excluded. We used the following definitions when describing conditions: a microvascular cranial nerve palsy is an isolated nerve palsy in a patient over 50 years of age with risk factors such as hypertension and diabetes; the clinical signs improve over 3 6 months and there is no deterioration or additional signs. Multiple sclerosis (MS) is ascribed if the patient has been given this diagnosis from a neurologist or has an MRI scan showing white matter lesions suggestive of demyelination. Myasthenia gravis (MG) is diagnosed based on clinical findings such as fatiguability, improvement with the ice pack test and with an improvement on treatment with pyridostigmine (Glaser 1990) all of our 382

2 patients with clinical findings of myasthenia are referred to a neurologist. Intracranial aneurysms and masses are diagnosed using MR or CT with contrast and or angiography. A decompensated heterophoria is defined as concomitant strabismus with full range of ocular movements; the typical onset is intermittent or gradual and there may be a history of strabismus in childhood. These patients usually have an extended motor fusion range. Age-related restrictions are defined as restrictions, typically of elevation or abduction, of insidious onset that may occur in older age (Davidson & Knox 2002). We had a small number of patients with nerve palsies as a result of aetiologies that are uncommon but well described in the literature: herpes zoster ophthalmicus (HZO) (Edgerton 1942; Marsh et al. 1977), intracranial vasculitis (Glaser 1990) and postcoronary angiography (Muqit et al. 2007). All scans in this study were reported by a radiologist. The indications that we apply for scanning a patient in the acute phase are as follows: (i) nerve palsy with evidence of other neurological features, for example, multiple nerve palsies, peripheral neurological signs or papilloedema, (ii) atypical clinical behaviour for microvascular aetiology or lack of associated risk factors for microvascular aetiology and (iii) significant trauma. Scans were requested if there was progression of signs or symptoms at future follow-up (Glaser 1990). Results One hundred and forty-nine patients presented during the 2-year study period. There were 83 males and 66 females with an age range of 8 96 years and a median age of 57.5 years. The duration of symptoms ranged from a few minutes to 4 weeks. The duration of follow-up was from none to 22 months. The aetiologies are shown by group and age in Figure one. There were 80 (53.7%) isolated nerve palsies, of which 38 (25.5%) were isolated sixth nerve palsies, 24 (16.1%) fourth nerve palsies and 18 (12.1%) third nerve palsies. Mechanical causes accounted for 16 (10.7%) patients including diagnoses of blowout fracture (4), facial injury (5), thyroid eye disease (2) and age-related restrictions (5). Dysfunction of higher control mechanisms of ocular movements accounted for 15 (10.1%) cases. These included internuclear ophthalmoplegia (INO) (7), horizontal gaze palsy (4), skew deviation (2), saccade pursuit dysfunction (1) and convergence spasm (1). Twelve patients (8.1%) had a decompensated heterophoria. Eight patients (5.4%) had monocular diplopia, with causes including cataract and Horner s syndrome (ptosis). Ten patients (6.7%) had a normal examination when they were seen in the clinic six of these patients had reported transient symptoms, the remaining four had a normal examination with no diplopia demonstrated when examined. Multiple muscle involvement was present in seven patients (4.7%). The underlying aetiology was microvascular in 39 (26.2%) cases, decompensatory in 23 (15.4%), trauma in 17 (11.4%) (blunt head trauma causing fourth nerve palsy in eight and orbital trauma in nine), idiopathic in 12 (8.1%), MS in 11 (7.4%), monocular in eight (5.4%), metastatic cancer in eight (5.4%), HZO in six (4%), MG in six (4%), intracranial tumour in four (2.7%), intracranial aneurysm in three (2%), stroke in three (2%), thyroid eye disease (TED) in two (1.3%), agerelated weakness in two (1.3%) and intracranial vasculitis, sinusitis cellulitis, Creutzfeld Jacob disease (CJD), convergence spasm and postangiography each in one case (3.4%) (Figure two). The cause of the diplopia in the patients with horizontal gaze palsies was concurrent esotropia in two cases and intermittent convergence excess as a compensatory mechanism for complete bilateral horizontal palsy in another two. The patient with saccade pursuit dysfunction had a known diagnosis of CJD and essentially had complete lack of control of her eye movements, which was most accurately clinically described as dysfunction of saccades pursuits. This lady was morbidly unwell and died shortly after presentation to the eye department. In 75 (50.3%) patients, the underlying aetiology of their diagnosis was obtained from the history together with the examination at presentation. This number includes 33 (22.1%) patients who had an isolated nerve palsy with associated microvascular risk factors, 17 (11.4%) with a history of trauma, 8 (5.4%) who were known to have MS, 7 (4.7%) who had a known advanced cancer, 6 (4%) with HZO and 1 (0.7%) patient each with an already diagnosed stroke, intracranial vasculitis and CJD. This does not include those with monocular diplopia or transient symptoms that accounted for another 15 (10.1%) patients. As well as the 75 above, a further 30 (20.1%) had findings on clinical examination alone at initial presentation that explained their diplopia these were long-standing decompensated squint superior oblique palsy (18), myasthenia gravis (4), age-related restrictions (2), TED (2) or a normal examination (4). Using our criteria for scanning a patient as outlined above, neuroradiological investigations were performed on 47 (31.5%) patients in our series. Twenty-one (14.1%) patients underwent urgent scanning due to the presence of additional or suspicious features, seven were scanned urgently due to trauma and 19 were scanned nonurgently. Of the 21 patients who were scanned urgently, six (4%) patients required emergency management the diagnoses and management of these patients are outlined in Table 1. Excluding the six patients who required emergency management, the remaining 15 patients who were scanned urgently did not require urgent treatment or urgent referral to another specialty as a result of the outcome of their scan. The indications for these urgent scans were as follows: young patient with sixth nerve palsy (3), sixth nerve palsy plus stroke (1), child young adult with fourth nerve palsy (2), multiple extraocular muscle involvement plus nystagmus (1), multiple extraocular muscle involvement plus ptosis (1), multiple and bilateral muscle involvement (1), young patient with dysfunction of higher control of eye movements (2), history of cancer plus dysfunction of higher control of eye movements (1) and dysfunction of higher control of eye movements in older patient (not compliant with our protocol) (3). The diagnoses of these patients were demyelination (4), myasthenia (1), ischaemia (3), sinusits orbital cellulitis (1) and stroke (2), and four were normal studies. Seven (4.7%) patients were scanned urgently due to a history of acute trauma. Nineteen patients were 383

3 Table 1. The clinical features of those requiring emergency management Diagnosis Aetiology Management III palsy pupil involved P Comm Aneurysm Neuroradiologically coiled III palsy pupil involved P Comm Aneurysm Neuroradiologically coiled III palsy pupil involved + Proptosis Orbital Mass Referred to oncology III palsy pupil spared + Fall Pituitary tumour Neurosurgery VI palsy + Papilloedema Abducens schwannoma Referred to neurosurgery Monocular diplopia Horner s syndrome Carotid dissection Referred to vascular surgery III palsy: Third nerve palsy; P Comm Aneurysm: Posterior communicating artery aneurysm; VI palsy: Sixth nerve palsy. Table 2. Indications for and results of nonurgent neuroradiological investigations Diagnosis Indication for scan Result VI Palsy Metastatic cancer Intracranial mass Metastatic cancer Intracranial mass MS Clinical stroke Pontine stroke Progression over months Cavernous sinus aneurysm III Palsy Lymphoma MRI features of cerebral lymphoma IV Palsy Acquired palsy Ischaemic changes Acquired palsy Acquired palsy, young adult Blunt head trauma Blunt head trauma Traumatic head injury Mechanical Suspected TED EOM enlargement TED Changing atypical clinical features MG diagnosed later Supranuclear Idiopathic gaze palsy Idiopathic gaze palsy Idiopathic INO Suspected MS Persistent symptoms VI palsy, Sixth nerve palsy; III palsy, Third nerve palsy; IV palsy, Fourth nerve palsy; MS, Multiple sclerosis; INO, Internuclear ophthalmoplegia;, Nil abnormal detected; EOM, Extraocular muscle; TED, Thyroid eye disease. Table 3. Aetiology at presentation and progression to neuroradiological investigations. Diagnosis Aetiology evident at presentation Urgent scans scanned nonurgently one of these patients presented with a sixth nerve palsy that worsened on follow-up and transpired to have an intracavernous carotid artery aneurysm that was managed conservatively but the Routine scans Required urgent management VI Palsy IV Palsy III Palsy Supranuclear Complex Mechanical Monocular 8 8 1* Decompensation Required urgent scan for diagnosis VI, sixth nerve palsy; IV, fourth nerve palsy; III, third nerve palsy;, normal examination at presentation. *Acute onset Horner s syndrome. remaining 18 did not have any findings on scanning, which would have changed the patient s management if discovered earlier. The indications for and the results of these scans are detailed in Table 2. Table 3 shows the number of cases in each diagnosis for whom there was an obvious underlying aetiology and the proportion that underwent urgent or nonurgent neuroradiological investigations. The median time to resolution of the microvascular sixth nerve palsies was 3.5 months, microvascular third nerve palsies took slightly longer to resolve, with a median time of 5 months to resolution, with only 72.7% achieving full recovery. There were only three patients with acute onset diplopia who were diagnosed with a microvascular fourth nerve palsy. Each of these patients recovered within 3 months. Of the six (40%) patients who had a microvascular dysfunction of higher control causing diplopia, five completely recovered and one patient with a bilateral INO partially recovered. All were improved by 3 months. Discussion This case series has identified a range of diagnoses causing acute diplopia similar to that already recognized in the literature (Yapp 1973; Morris 1991; Comer et al. 2007). The majority (53.7%) of cases were caused by isolated cranial nerve palsies. It is accepted that isolated cranial nerve palsies do not routinely require neuroradiological investigation (Murchison et al. 2011), but clinicians tend to have a high index of suspicion regarding the possibility of associated pathology with acute onset diplopia (Glaser 1990; O Boyle et al. 1992; Kumudhan et al. 2005; Bodia et al. 2007). This study is reassuring in that it has found that only a small minority of cases of acute onset diplopia (4.7%) have a serious underlying pathology that requires urgent management. A clear history and thorough clinical examination to identify the underlying cause of the diplopia reveals the diagnosis for the majority of cases (70.5%). The absence of other signs or symptoms is encouraging in the context of cranial nerve palsies. Where pathology other than microvascular disease was identified, in the majority of cases (86.6%), the presentation was accompanied by a relevant past medical history or clear clinical findings. 384

4 Number of pa ents Urgent scanning is performed in the context of pupil-involving third nerve palsies and presentations with additional features (e.g. papilloedema). Scanning is also performed as indicated (nonurgently) in situations such as orbital disease, thyroid orbitopathy, in situations of atypical clinical progression (e.g. worsening or changing clinical signs), or to confirm or guide the management of a pre-existing diagnosis (e.g. metastases MS) where the result may affect the treatment of the patient. We found that using these criteria for requesting a scan, there was a high rate of abnormal scan results in these specific groups of patients; each of our patients with pupil-involving third nerve palsies had an emergent and significant abnormality two posterior communicating artery aneurysms and one orbital tumour. One patient who had a partial third nerve palsy and a history of unsteadiness had a pituitary tumour. One patient presented with papilloedema a 21-yearold with a sixth nerve palsy and VI IV III Mech Higher Decomp Monoc Complex Diagnosis Fig. 1. Aetiologies by group and broken down by age - over or under 50 years. % Pa ents Fig. 2. Aetiologies of all diagnoses Ae ology >50 <50 was found to have a schwannoma of the abducens nerve. One elderly patient with an isolated sixth nerve palsy developed progressive signs, and an MRI scan revealed an aneurysm of the intracavernous internal carotid artery. For the junior ophthalmologist in the emergency clinic, our results should be reassuring as we demonstrate that a high proportion of patients with acute diplopia will have a clear diagnosis with an obvious underlying aetiology at presentation. Additionally, all of our patients with serious pathology had clinical signs or evidence of progression that would not be easily missed and that would prompt most ophthalmologists to the appropriate investigation. As this was not a study but a review of practice, a number of our patients were scanned despite not fulfilling our criteria for investigation; all of these scans were normal or showed generalized ischaemic changes, which were in keeping with an otherwise presumed diagnosis of a microvascular aetiology. We checked for the possibility of missed diagnoses by reviewing the files of the patients who failed to attend follow-up appointments, and they did not undergo admission or investigation under other medical specialties (the notes in our hospital are regional in a Health Board that serves a discrete and captive population). A number, 16 (10.7%), of our patients had a previous diagnosis that was a recognizable cause for their diplopia at presentation and would have been expected to have an abnormal scan, for example MS, metastatic cancer. Eight (7.6%) of these patients were scanned (nonurgently) when they presented with diplopia, and for this reason, our high rate of abnormal scan results is best interpreted in this context. Complex causes of motility dysfunction, where multiple muscles or nerves are involved, and dysfunctions of higher control of eye movements are two groups of presentations that many clinicians are less confident in assessing and managing; they are uncommon cases and in all but the emergency clinic are managed by subspecialists. Over half (8 15) of our patients with a dysfunction of higher control of eye movements had an obvious cause at presentation. Of the remaining seven, three had demyelinating disease and three had ischaemic changes. Five of the seven patients in our series with multiple muscle involvement had an obvious cause at presentation, and the remaining two had demyelinating disease and ischaemic changes. The obvious causes were all detectable in the history for example, history of metastatic cancer or were evident on thorough clinical examination, for example, fatiguability or positive ice pack test in myasthenia. These results reinforce our findings that with thorough history and examination, even unusual and rare causes of diplopia can be assessed and managed appropriately at their first presentation. Twelve of our patients presented with a decompensation of a heterophoria these cases require the ophthalmologist to distinguish clinically between concomitant and incomitant strabismus; the clinician should take a good history regarding the gradual or intermittent onset of symptoms and a previous ophthalmic history. 385

5 Monocular diplopia is usually obvious from detailed history taking and careful examination. We also had a number of patients with transient symptoms or an entirely normal examination with no demonstrable diplopia none of these cases demonstrated or subsequently developed any significant pathology associated with their episode of diplopia. Acute diplopia is a challenging clinical scenario often managed by junior ophthalmologists in the emergency and out of hours setting, without orthoptic support; our results support the belief that taking a good history, performing a full ophthalmic examination with particular emphasis on eye movements and a neurological examination is sufficient to reach a diagnosis and that urgent investigation in the form of scanning is rarely indicated. Acknowledgements We would like to acknowledge the work of the staff of the orthoptic department at Ninewells hospital who helped with this project. The authors declare that they have no conflict of interest. Some of the results in this article were presented at the Scottish Ophthalmological Club meeting, February References Bodia AA, Ablett M & Inglis A (2007): Bilateral intracavernous carotid artery aneurysms presenting as progressive cranial nerve palsies. Clin Exp Optom 90: Comer RM, Dawson E, Plant G, Acheson JF & Lee JP (2007): Causes and outcomes for patients presenting with diplopia to an eye casualty department. Eye 21: Davidson JH & Knox PC (2002): The effect of ageing on eye movement: a literature review. Br Orthopt J 59: Edgerton AE (1942): Herpes zoster ophthalmicus: report of cases and a review of the literature. Trans Am Ophthalmol Soc 40: Glaser JS (1990): Neuroophthalmology, 2nd edn. Lippincott: Philadelphia. Kumudhan D, Jamieson A & Sanders R (2005): Metastatic carcinoma presenting as cranial nerve palsy. Scott Med J 50: Marsh RJ, Dulley B & Kelly V (1977): External ocular motor palsies in ophthalmic zoster: a review. Br J Ophthalmol 61: Morris RJ (1991): Double vision as a presenting symptom in an ophthalmic casualty department. Eye 5: Muqit MMK, Weir CR, Ballantyne J, Dunn FG & Goodfield NER (2007): Neuro-ophthalmic complications after coronary angiography. Scott Med J 52: 54. Murchison AP, Gilbert ME & Savino PJ (2011): Neuroimaging and acute ocular motor mononeuropathies: a prospective study. Arch Ophthalmol 129: O Boyle JE, Gardiner TA, Oliva A & Enzenauer RW (1992): Sixth nerve palsy as the presenting sign of metastatic prostate cancer: a case report and review of the literature. J Clin Neuroophthalmol 12: Yapp JMS (1973): Diplopia as a presenting symptom. Br Orthopt J 30: Received on Apirl 28th, Accepted on November 11th, Correspondence: Una O Colmain Department of Ophthalmology Ninewells Hospital Dundee DD1 9SY UK Tel: Fax: uocolmain@nhs.net 386