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A 67-year-old man with facial droop, and vertigo

Laila Nasser MD BHSc, Siobhan Deshauer MD BMus, Kyla Caners MD, Amitabha Chakraborty MD n Cite as: CMAJ 2020 June 8;192:E626-9. doi: 10.1503/cmaj.190474

67-year-old white man presented to the emergency department with a 10-day history of left-sided facial numbness, vertigo and nausea, along with new-onset gaitA imbalance. He had been treated for 1 week with prednisone (45 mg/d) and amoxicillin (500 mg 3 times daily) for suspected Bell palsy and concomitant sinus infection. The patient’s medical history included hypercholesterolemia, retracted. colon cancer resection without recurrence (9 years previously) and smoking. His medications included rosuvastatin and acetyl- salicylic acid for primary prevention. He had no history of travel or weight loss, fever or night sweats. On examination, the patient had head deviation to the left in lateral flexion at rest (Figure 1). He had left-sided facial asymmetry sparing the forehead (Figure 2) and decreased been facial sensation over the V2–V3 distribution. He also had ptosis and an inability to close his left eye, with associated Figure 1: Head deviation to the left in lateral flexion at rest in a 67-year- horizontal nystagmus on rightward gaze (Figure 3). His pupils old man with a 10-day history of left-sided facial numbness, vertigo and were equal and reactive to light, with normal visual acuity and nausea, and new onset of gait imbalance. visual field testing. Hearing was decreased on the left. His gait was ataxic with a wide base and he could not achievehas tandem gait. Patellar reflex was brisk and plantar response upgoing on the right. The remaining neurologic examination was normal, including midline tongue, palate rise, cerebellar finger-to-nose testing, tone, sensation and motor testing. Findings on cardiovascular, respiratory, lymphatic and abdominal examinations were normal, with no evidence of dysautonomia. Figure 2: Symmetric forehead with raising of the eyebrows. On initial blood work, thearticle patient’s complete blood count, electrolytes and creatinine were within normal limits. His elec- trocardiogram showed normal sinus rhythm.

Which syndrome is most in keeping with this patient’sThis deficits? a. Weber syndrome b. Partially treated Bell palsy c. Benedikt syndrome d. Lateral medullary syndrome

The patient presented with left-sided facial droop and numb- ness, ptosis, horizontal nystagmus and ataxia, which are Figure 3: Ptosis of the left eye (A) and inability to close left eye with blinking (B).

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ISSUE 23 ISSUE | been for central ner- This patient does not have typical risk factors disease of Multiple sclerosis (c) is a multifocal demyelinating been Posterior circulation ischemic Posterior circulation system lymphoma Primary central nervous multiple sclerosis First presentation of vasculitis Central nervous system Further blood work showed a normal B Further blood work

mon cause of lateral medullary syndrome. Given the patient’s Given the patient’s mon cause of lateral medullary syndrome. be must event thromboembolic a history, smoking and sex age, ruled out first. vous system lymphoma (e.g., immunodeficiency such as from such as from vous system lymphoma (e.g., immunodeficiency yr) and he presented HIV), but is in the right age group (45–65 a new lesion. This with neurologic changes in the context of diagnosis (b) cannot yet be ruled out. presents in adults the central nervous system, which typically than men. A small pro- aged 20–50 years, more often in women older than 60 years. portion of those affected present at age What is the most likely diagnosis? What is the most a. b. c. d. remain ischemic (a) circulation Posterior evaluation of the and posterior fossa. In addition to a In addition fossa. and posterior brainstem of the evaluation in the lesion observed or neoplastic demyelinating visible on cerebellar infarct was a small remote right (Figure 5), matter, in the deep white cerebral atrophy MRI, with moderate changes or ischemic of chronic microangiopathic suggestive (not shown). demyelination Clinically isolated syndromes of multiple sclerosis typically affect Clinically isolated syndromes of multiple sedimentation rate (10 mm/h), and negative HIV and Venereal (10 mm/h), and negative HIV and Venereal sedimentation rate abnormalities No screening. test Laboratory Research Disease telemetry and echocardiogram. were found on cardiac VOLUME 192 VOLUME | ­

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The patient was correctly identified as having a as having a was correctly identified The patient 1 Sensory deficit in the ipsilateral face is related to ischemia Sensory deficit in the This 1 gnomonic for lateral medullary syndrome (or Wallenberg Wallenberg (or syndrome medullary for lateral ­gnomonic Presuming a central cause, a computed tomography (CT) tomography (CT) Presuming a central cause, a computed Both (a) Weber and (c) Benedikt syndromes have contra Both (a) Weber and (c) Benedikt syndromes Bell palsy (b) is a peripheral cause of seventh nerve palsy and cause of seventh nerve palsy and Bell palsy (b) is a peripheral The patient’s upgoing plantar response and brisk reflexes indi- The patient’s upgoing angiogram was ordered in the emergency department to rule out angiogram was ordered in the emergency and the patient was a stroke. The CT scan had no abnormalities for further investiga- admitted to the internal medicine service (MRI) for further tion, including magnetic resonance imaging lateral hemiplegia (rather than sensory loss) and contralateral loss) and contralateral lateral hemiplegia (rather than sensory ataxia). gait disturbance (rather than vertigo and Horner syndrome can occur in Weber and Benedikt syndromes Benedikt syndromes Horner syndrome can occur in Weber and out- sympathetic disrupting circulation posterior from put, and also in Bell palsy. of the trigeminal nucleus, where vertigo and ataxia are a result ofa result ataxia are and nucleus, where vertigo trigeminal of the nuclei (Figure 4). The patient’s resultingan insult to the vestibular for dysfunction of ocular alignment. head tilt is compensation of a central cause by motor involvement is differentiated from change not would Partial treatment face. and lower forehead the and his constel- the distribution of the patient’s , brisk reflexes) is lation of other symptoms (i.e., gait disturbance, not in keeping with peripheral palsy. central cause of seventh nerve palsy (rather than peripheral), peripheral), palsy (rather than of seventh nerve central cause his forehead. The forehead receives as his motor deficit spared corticobul- dorsal multiple of result a as input cortical bilateral the brainstem. bar tract crossings in neuron lesion, also suggesting a central cate an upper motor cause.

Pathoanatomy of lateral medullary syndrome. Credit: Amy Ajay. Ajay. Figure 4: Pathoanatomy of lateral medullary syndrome. Credit: Amy syndrome) (d). Typical features include ipsilateral loss of pain, loss of pain, include ipsilateral (d). Typical features syndrome) Horner syn- the face; ipsilateral and sensation in temperature hemianesthesia, vertigo, body contralateral drome; and patho and nausea. PRACTICE diffusion-weighted imaging(DWI). E628 T (B) coronalviews.Bothdisplaya1-cm well-circumscribedhyperintense Figure 5:Magneticresonanceimagingscan ofthebrainin(A)axialand the opticnerve,cerebellumorspinalcord. acquired wereT plastic lesion,withnoperilesional edemaormasseffect.Sequences disseminated inspace(inthecentralnervoussystem)ortime scler not yetmeet the McDonald criteriafor diagnosis of multiple MRI andnohistoryofotherneurologicdeficits,thepatientdid 2 signalintheleftpons(arrows),suggestive ofademyelinatingorneo-

­ This osis, whichstipulatesthatdemyelinatinglesionsmustbe 1 , T 2 , fluid-attenuatedinversionrecovery (FLAIR)and article CMAJ 2 With only 1 lesion on Withonly1 lesion | JUNE8, 2020 has | VOLUME 192 presentation ofmultiplesclerosis. most correctansweroftheoptionspresentedaboveis(c),first ating process,andhavingruledoutaposteriorstroke(a),the is 50years. condition thatoccursmoreofteninmen;meanageatdiagnosis osis canbeacuteandmimicstroke,cannotyetruledout. weeks surrounding illness (more ple sclerosislesionsinthebrainaretransientlyenhanced next besttestisanMRIbrainscanwithgadolinium(d),asmulti- nosis includedaneoplasticlesionordemyelinatingdisease.The Given theresultsofourpatient’sbrainMRI,differentialdiag- d. c. b. a. What isthenextbestdiagnosticstep? (multiple attacks). tion tomakethediagnosisofmultiplesclerosis. detect demyelinationandopticneuritisbutarenotusedinisola- MRI ofthespine.Visualevokedpotentialsareclassicallyusedto demyelinating lesionssuggestiveofmultiplesclerosiswithan out centralnervoussystemlymphoma.Wecheckedforfurther the presenceofneoplasticcellsandforflowcytometrytorule ence ofoligoclonalbandingconsistentwithmultiplesclerosis, workup. Thecerebrospinalfluidwasusedtotestforthepres- and orderedanMRIofthespine(b)tocompletethispatient’s undertook alumbarpuncture(a)toobtaincerebrospinalfluid this testwasnotreadilyavailableinouracutesetting,sowe were co metry didnotshowevidenceofclonalT-orB-cellpopulations. cerebrospinal fluid. Oligoclonal banding was negative. Flow cyto for aslightlyelevatedproteinlevelof0.68(normal<0.45)g/L balance, andhis facialdroopandeyefindingswere stillpresent. toms wereimprovingbuthestill hadongoingdisturbancestohis with disseminationinspaceand time. the patientnowmetMcDonald criteriaformultiplesclerosis, suggestive ofactivedemyelination. Thesefindingsindicatedthat pontine lesionaswellintherightperiventricularwhitematter, incomplete ringenhancementinthepreviouslyvisualizedleft MRI enhancedwithgadoliniumwasperformed.Itdisplayed Fabry diseasewasexcludedwithnormalgenetictesting. level, normalcalciumlevelandunremarkablechestradiography. ocular examination, negative angiotensin-converting enzyme metry and echocardiogram. With an MRI the rarityofcondition,itisnotmostlikelydiagnosis. tion rate.Giventheabruptonsetofthispatient’ssymptomsand cific neurologicsymptomsandnormalerythrocytesedimenta-

Primary centralnervoussystemvasculitis(d)isanuncommon Magnetic resonanceimagingofthebrainwithgadolinium Visual evokedpotentials Magnetic resonanceimagingofthespine Lumbar puncture The inpatientneurologyandneuro-ophthalmologyservices The resultsofthelumbarpuncturewereunremarkableexcept At the time of the repeat MRI of the head, the patient’s symp- A monthaftertheinitialMRIofpatient’shead,arepeat been A cardioemboliceventwasunlikely,giventhenormaltele nsulted and ruled out neurosarcoidosis with a normal 1 | Itclassicallypresentswithalongprodrome,nonspe- ISSUE 23 5,6 However,thepresentationofmultiplescler retracted. so than in the spine). suggesting a demyelin- 8 7 However, in the in the ­ ­ ­ PRACTICE ​ E629 It is crucial to recognize the It is crucial

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. 7th ed. Philadelphia: Lippincott Williams & Williams & . 7th ed. Philadelphia: Lippincott retracted. The authors thank Amy Ajay BHSc (McMaster

neuroanatomy Divisions of Emergency Medicine (Nasser, Caners) andDivisions of Emergency Medicine (Nasser, Caners)

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­surg Psychiatry 2005;76(suppl 2):ii16-22. Wilkins; 2010. been 1980;37:669-70. caused by multiple Qiu W, Wu J-S, Carroll WM, et al. Wallenberg syndrome sclerosis mimicking stroke. J Clin Neurosci 2009;16:1700-2. Rosen’s emergency medicine: concepts M. Rosen’s emergency medicine: Walls R, Hockberger R, Gausche-Hill 2017. and clinical practice. 9th ed. Toronto: Elsevier Canada; Snell RS. Clinical cerebrovascular syndromes. UpToDate; 2017 Caplan L. Posterior circulation [updated 2018 Apr. 30]. of the lateral medullary syndrome. Arch Neurol Ho KL. Uncommon causes JA, et al. Wallenberg’s lateral medullary syndrome: Sacco RL, Freddo L, Bello imaging correlations. Arch Neurol 1993;50:609-14. clinical-magnetic resonance Barkhof F, et al. Diagnosis of multiple sclerosis: Thompson AJ, Banwell BL, 2018;17:162-173. 2017 revisions of the McDonald criteria. Lancet Neurol Filippi M, Preziosa P, Banwell of lesions on magnetic res- BL, et al. Assessment guidelines. Brain 2019; onance imaging in multiple sclerosis: practical 142:1858-75. potentials. J Neurol Walsh P, Kane N, Butler S. The clinical role of evoked Neuro for multiple sclerosis in Swanton JK, Rovira A, Tintore M, et al. MRI criteria a multicentre retro- patients presenting with clinically isolated syndromes: spective study. Lancet Neurol 2007;6:677-86. The lesson from this case is that demyelinating disease can can disease is that demyelinating this case from The lesson invites submissions to “What is your call?” Clinical details CMAJ invites submissions to “What is your call?” Clinical details (including images) are presented with a multiple-choice question about the diagnosis. The answer and a brief discussion of the condi- follow. We specifically invite submissions illustrating commontion or important radiographic and electrocardiographic diagnoses of appeal to a general audience. We require authors to obtain consent from the patient for publication of his or her story. Submit manu- scripts online at http://mc.manuscriptcentral.com/cmaj Competing interests: None declared. This article has been peer reviewed. The authors have obtained patient consent. Affiliations: University; Internal Medicine (Deshauer, Chakraborty), McMaster Chakraborty).Hamilton Health Sciences (Nasser, Deshauer, Caners, toContributors: Kyla Caners and Amitabha Chakraborty contributed Nasser and Siobhan the conception and design of the work. Laila revised the man- Deshauer drafted the manuscript. All of the authors content, gave final uscript critically for important intellectual - account be to agreed and published be to version the of approval able for all aspects of the work. Acknowledgements: Lateral of 4 (Pathoanatomy of Figure the creation for University) Medullary Syndrome). Disclaimer: Siobhan Deshauer is the daughter of Dorian Deshauer, who is a deputy editor with CMAJ. Dorian Deshauer was not involved in the editorial decision-making for this article. Correspondence to: Laila Nasser, [email protected]

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1. 2. 3. 4. 5. 6. 7. 8. 9. present at an atypical age, and that central vertigo or a facial or a facial that central vertigo an atypical age, and present at the first presentation. palsy can be References 10 features of a possible lateral medullary syndrome and order the syndrome and a possible lateral medullary features of central other and stroke for investigate to imaging appropriate when warranted. as multiple sclerosis, causes, such

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The first step is to recognizeis to The first step 5 lesion at the C1–C2 level (arrow). article 2

It found that the presence of lesions that lesions of presence the that found It 7 This A multicentre retrospective study examined MRI criteria This 6 The final diagnosis in this case was multiple sclerosis, multiple sclerosis, this case was The final diagnosis in Three months later, an MRI of the spine showed a small, non- Three months later, an MRI of the spine showed the patient’s initial At a follow-up appointment 5 months after the constellation of symptoms including facial nerve palsy and the constellation of symptoms including facial nerve palsy and central vertigo, then pursue a broad differential of intracranial - lym system nervous central ischemia, includes that pathology phoma, vasculitis and multiple sclerosis (as an atypical cause). fulfilling McDonald criteria with the patient having 1 “attack” and objective clinical evidence of an indicative lesion on MRI in 2 areasleft and (periventricular system nervous the central of pons). enhancing demyelinating lesion at the C1–C2 level (Figure 6). enhancing demyelinating lesion at the C1–C2 presentation of multi- presentation, the diagnosis of an atypical The patient continues ple sclerosis was confirmed by neurology. sclerosis clinic. to be monitored regularly by the multiple

This case highlights an atypical onset of multiple sclerosis. The multiple sclerosis. The This case highlights an atypical onset of medullary syndrome;patient presented with a new onset of lateral that multiple sclerosis however, it was only with further testing medul- lateral with association common most The diagnosed. was artery stroke, but lary syndrome is posterior inferior cerebellar other causes. a number of there are Discussion Magnetic resonance imaging scan of the cervical spine 3 monthsFigure 6: Magnetic resonance imaging scan of the cervical after presentation, showing a T are disseminated in both time and space from 2 MRI scans has a higher specificity and risk for clinically definite multiple sclerosis or time in dissemination either meeting lesions than dissemination in space criteria alone. The study found that multiple sclerosis criteria can be met in patients presenting with clinically isolated syndromes, as in our patient. for patients with clinically isolated syndromes and thought to for patients with clinically isolated syndromes and thought to sclerosis. multiple have