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TRIPLE PTOSIS ABSTRACT This is a case in which aberrant regeneration after a remote facial palsy confounds the diagnosis of an early ipsilateral third nerve palsy. CASE HISTORY A 54 year old Caucasian male presented to the emergency department for an acute increase in left ptosis x 1 day. The patient had a previously existing left ptosis residual from a left lower motor neuron facial palsy in 2004. Initial assessment by the ER physician revealed left eye ptosis with accompanying left retrobulbar pain. Pupils and extraocular motilities were recorded as normal. Other cranial nerve testing was remarkable for left facial weakness involving the upper and lower face. No other focal neurological deficits observed. CBC, electrolytes plus and head CT were obtained. (See Laboratory/Radiology Studies). CT of the head without contrast revealed no acute intracranial hemorrhage, infarct or mass. The patient was admitted to neurology service for workup of recurrent left facial lower motor neuron palsy vs acute facial nerve palsy of infectious etiology vs. ptosis of unclear etiology (unlikely myasthenia gravis). The patient was consulted to optometry for an evaluation of the ptosis. OPTOMETRY CASE HISTORY The patient presented to the optometry clinic complaining of an exacerbation of longstanding left ptosis upon wakening x 1 day with accompanying significant left retrobulbar pain which started 3 days prior. He denied double vision, blurry vision, photophobia, eyelid swelling, variability of ptosis size. He noted no additional facial weakness from baseline and reported the ability to fully close the left eye. His wife who accompanied him to the exam claimed that the change in his facial appearance was limited to the left lid. He additionally denied recent trauma or MVA, new rash or history of shingles, changes in hearing, tick bite, difficulty swallowing, dysarthria, numbness or tingling. Dana Bastarache 1 CASE HISTORY CONTINUED Ocular History h/o left lower motor neuron facial palsy 2004 with longstanding left ptosis Mild nonproliferative diabetic retinopathy OU H/o classic migraines diagnosed at 18 years old. Frequency: 2- 3x/year Choroidal Nevus OD Mild Myopia with Presbyopia OU Medical History Diabetes Mellitus Type II x 20 years Hypertension Hyperlipidemia Coronary Artery Disease Obseity Fibromyalgia PTSD Medications Insulin Metformin Clopidogrel Metoprolol Lovastatin Valsartan Amitriptyline Cetirizine Hydrochlorothiazi de Gabapentin Nabumenton EXAMINATION BCVA OD 20/20, OS 20/20 Confrontation Fields Full OD, OS Pupils Equal, round reactive to light OD, OS (-)APD EOMs OD: full *SEE PHOTOS* OS: partial limitation supraduction, adduction Dana Bastarache 2 EXAMINATION continued Alternating Cover test: Cranial Nerve Testing CN VII: 1+ action left frontalis 3+ action orbicularis oculi 1+ orbicularis oris CN II, IV, V, VI, VIII, IX, X, XI, XII unremarkable. CNI not tested Eyelid Position Palpebral Fissure OD 9mm OS 3mm Marginal Reflex Distance OD 3mm OS -2mm Levator Function OD 14mm OS 12mm Worsening of left ptosis on mouth opening and puffing cheeks *SEE PHOTOS* Hertel Exophthal. OD 15mm OS 13mm Resistance to Retropulsion Negative bilaterally , left globe tender to touch Photo1: Resting eyelid position Base 96mm Photo 2: Complete closure of eye on opening of mouth Photo 3:Complete closure of eye on puffing checks Dana Bastarache 3 EXAMINATION continued Anterior Segment Unremarkable OU (-)Cogan lid twich Handheld Goldman Applanation Tonometry OD: 10 mmHg OS: 11mmHg @ 5:01p Dilated Fundus Examination Optic nerves with distinct borders, pink healthy rim 360 OU. No evidence of papilledema. Choroidal nevus 1/3DD temporal to macula OD flat with distinct borders. No evidence of hemorrhages, cotton wools spots, IRMA, venous beading, neovascularization OU Humphrey Visual Field 24-2 Unreliable OU, non-neurological field LABORATORY/RADIOLOGY STUDIES CBC Test Name WBC RBC HGB HCT MCV MCH MCHC RDW-CV PLT MPV NEUT % LYMPH % MONO % EOS % BASO % NEUT, ABS MONO, ABS EOS, ABS BASO, ABS Result 5.65 K/cmm 4.28 L M/cmm 13.1 L g/dL 38.4 L % 89.7 fL 30.6 pg 34.1 gm/dL 12.5 % 159 K/cmm 10.0 fL 66.0 % 25.1 % 5.5 % 2.7 % 0.5 % 3.73 K/cmm 0.31 K/cmm 0.15 K/cmm 0.03 K/cmm Ref Range 4.5 - 10.0 4.7 - 5.7 14 - 17 40 – 51 80 - 101 27 - 34 31.0 - 36.0 11.5 - 15.0 130 - 450 9.0 - 12.0 40.0 - 75.0 10 - 55 2 - 12 0.0 - 7.0 0-2 1.8 - 6.5 0.11 - 0.59 0.0 - 0.7 0 - 0.2 Sed Rate 38 CRP 1.28 mg/L HB A1C 8.9 CT head without contrast MRI of brain with and without gadolinium There is no evidence of intra-axial or extra-axial mass, midline shift, acute hemorrhage or infarct. The sulci, ventricles and cisterns are normal. The orbits are unremarkable. Skull and extracranial soft tissues are unremarkable. Conclusion: No acute intracranial abnormality. Impression: No retrobulbar mass identified. Unrevealing Dana Bastarache 4 DIFFERENTIAL DIAGNOSIS of chief complaint: acute unilateral ptosis CN III palsy Brow ptosis from facial palsy (pseudoptosis) Myasthenia Gravis Acute Horner’s Syndrome Mechanical ptosis (ie acute hordeolum) DIAGNOSIS : TRIPLE PTOSIS 1. History of left lower motor neuron facial palsy 2004 with known longstanding left ptosis secondary to aberrant regeneration 2. Synkinesis manifesting as increasing left ptosis when patient opens mouth widely or puff cheeks following facial palsy 3. Painful, partial, pupil-sparing left CNIII palsy, primarily involving supraduction and adduction We were unable to clinically observe an infraduction deficit of the left eye however cover test inferiorly revealed a small left hyperphoria suggesting sub-clinical limitation. This is a case in which aberrant regeneration after a remote facial palsy confounded the diagnosis of an early ipsilateral third nerve palsy. To complicate the diagnosis further, the palsy was partial and manifested as subtle EOM limitations with a spared the pupil. Another confusing variable was that the patient initially denied diplopia which is usually one of the presenting complaints in an acute CN III palsy. The most common reason that diplopia is not elicited upon an acute CN III palsy is because the ptosis covers the pupil. The other less common reasons are that the extraocular muscles are so minimally affected that the patient has some fusional vergence to hold the eye into partial alignment or that the images are so far apart that the patient is unaware of the second image. The patient denied diplopia in primary gaze even with the lid held but could be elicited in all other directions of gaze. All of these variables led to this diagnosis being overlooked by multiple physicians prior to optometry evaluation. Therefore the “triple ptosis” diagnosis was formulated from the following patient characteristics: Ptosis One: from aberrant regeneration following facial palsy Ptosis Two: increased ptosis with facial animation via synkinesis Ptosis Three: from acute CN III palsy DISCUSSION In a discussion revolving around ptosis it is important to review eyelid anatomy. Normal eyelid movements are supplied by 3 entities: 1. Facial Nerve (CN VII) innervates the orbicularis oculi to close both the upper and lower lid 2. Oculomotor nerve (CN III) innervates the levator palpebrae superioris to elevate the lid 3. Sympatheics innervate the superior tarsal muscle (Mueller) to augment opening of the lid and also the inferior tarsal muscle Dana Bastarache 5 It is commonly known that during an acute facial palsy a “brow ptosis” can occur secondary to weakness of the frontalis muscle forcing the brow to sit at or below the superior orbital rim. Aberrant regeneration, on the other hand, is an under recognized cause of ptosis following a facial palsy. During regeneration and repair of neurons after a palsy, some fibers may take an unusual course and connect to other muscles innervated by CN VII. Aberrant regeneration following a facial palsy may give rise to an ipsilateral ptosis because of increased orbicularis tone. Signs of aberrant regeneration include reduced palpebral aperture, with reduced upper and lower marginal reflex distance. 30% of patients with Bell’s palsy experience sequelae of the paralysis which can include incomplete motor or sensory regeneration and aberrant regeneration.1 Ptosis following a facial palsy can be exacerbated by synkinesis. Synkinesis refers to the abnormal involuntary facial movement that occurs with voluntary movement of a different facial muscle group. Marin-Amat syndrome describes an acquired synkinesis of the orbicularis oculi and jaw movements. It manifests as an involuntary eyelid closure when the patient is asked to open the mouth widely or to puff the cheeks. The reported incidence of synkinesis in facial nerve palsy varies greatly from 9 to 55%.2 Yamamoto et al found that synkinesis occurs most frequently 24-39 weeks following the onset of facial palsy.3 It is not known when our patient developed ptosis since he transferred care to the clinic several years his palsy. We had on record an identification photo taken from 2009 which was 5 years after the initial incident. CN III is also responsible for eyelid position and paralysis can lead to a partial or complete ptosis. In addition to innervating the levator palpebrae superioris, CN III innervates 4 of the 6 extraocular muscles (superior rectus, inferior rectus, medial rectus and inferior oblique) and also supplies parasympathetic innervation to the sphincter muscle of the iris and to the ciliary body. CN III palsies are either classified as complete or incomplete and either pupil sparing or pupil involving. Complete paralysis refers to total palsy of all four extraocular muscles and levator. Pupil sparing is defined as less than 1 mm of anisocoria and normal pupillary light reflex.4 For the sake of this discussion, we will refer to acquired, non-traumatic and isolated (without associated neurological findings) third nerve palsies only. This subset of CN III palsies can be further classified. Third nerve palsy with: 1. Normal pupil with completely palsied EOM 2. Normal pupil with incomplete palsied EOM 3. Abnormal pupil with partial or complete EOM palsy Our patient’s profile fits category 2. He had a subtle partial paralysis of at least 3 of the 4 muscles innervated by CN III. It has been documented that most patients with extraocular muscle and levator involvement in pupil sparing, incomplete third nerve palsies of vasculopathic origin have a diffuse pattern of paresis.5 This partial and diffuse limitation was also the reason the diagnosis of an acute CN III palsy was that much more difficult to spot, especially to the non-optometric trained eye. This patient also presented with retrobulbar pain. Headache and periocular pain, excruciating enough to overshaow the ptosis and diplopia, occur in at least 50% of patients with microvascular ischemic CN III palsy.6 Ischemic damage to the trigeminal fibers in CN III may be the source of pain in ischemic diabetic CN III palsy.7 The most common location of the pain has been documented around the ipsilateral brow and eye. Literature indicates that 11-33% of patients have pain preceding the onset of visual symptoms. 8 This finding is also consistent with our patient’s presentation. Ischemic lesions of CN III often spare the pupil in theory because the lesion is confined to the core of the nerve and does not affect the peripherally situated pupillary fibers. By contrast, a compressive lesion (ie tumor or aneurysm) would compress the superficial pupillary fibers and impair Dana Bastarache 6 their function. Pupil involvement occurs in as many as 20% of patients with ischemic CN III palsy but anisocoria of more than 1.5mm is exceedingly rare. 9 Incomplete third nerve palsies with pupillary sparing require an MRI to rule out a mass. According to Jacobson and Trobe, for this subset of CN III palsies even those greater than 40 years old, who have vasculopathic factors present, it is recommended to obtain MRI followed by MRA if the MRI doesn’t disclose a non-aneurysmal cause. 10 MRA was recommeded for our patient but was not ordered by neurology. TREATMENT AND MANAGEMENT Plan: 1. Obtain MRI followed by MRA if the MRI is unremarkable. Rule out giant cell arteritis as a cause of ischemic CN III palsy. 2. Address underlying vasculopathic risk factors and patient education 3. Dispense a patch which can be useful for alleviating diplopia short term. Patient educated regarding depth perception 4. Patient educated not to drive until diplopia is resolved Initial management of an acute, incomplete pupil sparing CN III palsy is to order appropriate imaging as described above to rule out a posterior communicating artery aneurysm and to monitor progression of ophthalmoplegia and/or pupil involvement. The patient was re-examined 1 day and 1 week after initial presentation to the eye clinic. During this time the patient was advised to self monitor his pupils. No changes were detected on subsequent visits. For maximal ophthalmoplegia, the average time from onset has been documented as 34 to 10 days11. Our patient has not yet recovered at 2.5 weeks after onset. Therapy for CN III palsy is directed at the underlying etiology. For this patient, underlying microvascular disease needed to be addressed and vascular risk factors treated. The majority of ischemic third nerve palsies improve within 3 months- 6 months. In a study of 28 patients with ischemic CN III palsy, 68% resolved in four weeks, 96% in eight weeks, and 100% in 12 weeks.12 The most common therapeutic modalities for the treatment of facial synkinesis and aberrant regeneration include botulinum toxin and surgical correction. CLINICAL PEARLS: TRIPLE PTOSIS 1. Aberrant regeneration is an under recognized cause of ptosis following a facial palsy 2. Observation of synkinesis requires facial animation in office 3. Careful attention must be given to extraocular muscles in the setting of an acute unilateral ptosis with or without diplopia. Incomplete CN III palsies can be subtle and missed. 4. If an incomplete CN III palsy presents with a normal pupil, pupil involvement may still evolve and needs close monitoring. Maximal ophthalmoplegia should be present in 10 days. Dana Bastarache 7 References 1. Marsk E, Bylund N, Jonsson L, Hammarstedt L, Engström M, Hadziosmanovic N, et al. Prediction of nonrecovery in Bell's palsy using sunnybrook grading. Laryngoscope. Apr 2012;122(4):901-6. 2. Monteserra L,Benito M. Facial synkinesis and aberrant regernation of facial nerve.In:Jankovic J and Tolosa E (eds). Advances in Neurology. Raven Press: New York, 1998 p211-224 3. Yamamoto E, Nishimura H, Hirono Y. Occurrence of sequelae in Bell's palsy. Acta Otolaryngol Suppl. 1988;446:93-6. 1988;446:93-6. 4. Lee AG, Hayman LA, Brazis PW. The evaluation of isolated third nerve palsy revisited: an update on the evolving role of magnetic resonance, computed tomography, and catheter angiography. Surv Ophthalmo 2002l, pp. 47(2); 137-57 5. Sanders S, Kawasaki A, Purvin VA. Pattern of extraocular muscle weakness in vasculopathic pupilsparing, incomplete third nerve palsy. J Clin Neuroophthalmol, 2001, Vols. 21:256-259. 6. Smith CH. Nuclear and infranuclear ocular motility disorders. In: Miller NR, Newman NJ, eds. Walsh & Hoyt’s Clinical Neuro-ophthalmology. 5th edu Baltimore. 1998;1:1205 7. Bortolami R, D’AlessandroR, Manni E. The origin of pain in ischemic –diabetic third nerve palsy. Arch Neurol 1993; 50:785 8. Wilker SC, Rucker JC, Newman NJ, Blousse V, Tomask RL. Pain in ischemic ocular motor cranial nerve palsies. BrJ Ophthalmol2009.Dec;93:1657-1669 9. Jacobson DM. Pupil involvement in patietns with diabetes-associated oculomotor nerve palsy. Arch ophthalmol 1998;116:723-7. 10. Jacobson DM, Trobe JD. The emerging role of magnetic resonance angiography in the management of patients with third cranial nerve palsy. Am J Ophthalmol 1999, pp. 128;94-96. 11. Renowden SA, Harris KM, Hourihan MD. Isolated atraumatic third nerve palsy: Clinical features and imaging technqiues. Br J Radiol 1993;66:1111-1117 12. Capo H, Kupersmith WF. Evolution of oculomotor nerve palsies. J Clin Neuro-ophthalmol 1992;12:21-25 13. Trobe J. Searching for brain aneurysm in third cranial nerve palsy.ED J Neuro-Ophthal Vol 29. No3, 2009; 171-173 14. Chen C, Malhotra R, Muecke J, Davis G, Selva D. Aberrant facial nerve regeneration: an under recognized cause of ptosis. Eye 2004.18,159-162. 15. Wood, S. Normal pupil findings can give a false sense of security in a presumed cranial nerve III palsy patient: A unique case report. Optometry (2010) 81;505-509 Dana Bastarache 8