Multiple Embolic Strokes and Multiple Sclerosis ... · SM Journal of Neurological Disorders and...

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SM Journal of Neurological Disorders and Stroke How to cite this article Nelson F and Jafari M. Multiple Embolic Strokes and Multiple Sclerosis Misdiagnosis, Case Report. SM J Neurol Disord Stroke. 2018; 4(2): 1016s6. OPEN ACCESS Introduction e diagnosis of multiple sclerosis (MS) has relied upon accumulation of clinical presentation, radiological and laboratory investigations that leads to a positive diagnosis and can help to eliminate deferential diagnoses [1]. However, despite well validated diagnostic criteria misdiagnosis remains a problem that has remarkable implications for patients, health care providers and systems [2]. In a study by Solomon et al., [2] two percent of diagnoses and syndromes mistaken for MS were magnetic resonance imaging (MRI) changes caused by vascular disease. is issue could expose patients to immunomodulatory therapy and causes significant morbidity. In this case report we present a patient with multiple embolic ischemic stroke (IS) which was misdiagnosed as MS. Case report A 69 year old right handed female was evaluated as a new patient in the MS clinic at the M Health’s Clinics and Surgery Center at the University of Minnesota, Minneapolis to establish care. Her history included neurological deficits back to 1966 (age 17) described as leſt extremity numbness and tingling sensation that lasted a few weeks. She was symptom free until age 31 in 1980 when she was diagnosed with right optic neuritis with complete return to her previous normal baseline. She was referred to the University of Minnesota where she was diagnosed with MS and had intravenous steroids. She had multiple “relapses” for the next 3 years and then she seemed to go into complete remission without any evidence of relapses of progression since 1983, she was never prescribed a disease modifying therapy once they became available. During her interview she reported she has been doing well ever since. On the day of presentation, she reported no significant neurological symptoms except for occasional tingling sensation in distal extremities throughout which was in the process of being evaluated by the neuromuscular division. She did not have any other symptoms related to MS. Her medical history was significant for diabetes mellitus (DM), hypertension, rheumatoid arthritis (RA), peripheral neuropathy likely due to DM, thyroid disease, hypertriglyceridemia, and gout. She was on prednisone 5 mg every day tapering from 10 mg po every day for the treatment of RA. Physical examination showed a gait which was antalgic and unsteady, with hip tilting to the right, she walked with the help of a front wheel walker. Pinprick sensation diminished markedly beyond the ankle bilaterally. e rest of the examination was normal. Further diagnostic workup revealed atrial fibrillation on electrocardiography. Echocardiography showed mild leſt ventricle hypertrophy probably due chronic hypertension. Her most recent MRI of the brain with and without contrast from 2013 showed multiple T2-weighted (W) hyper intensities mostly in the subcortical white matter, less than 3 mm (most) and some larger lesions which were not typical of MS based on location, size, shape and lack of contrast or corresponding T1-W hypointenstiesor black holes (Figure 1). No previous films were available for comparison. Lumbar MRI revealed evidence of multiple disc herniation at L4-5, L5-S1. e patient was explained that the findings on neurological exam and MRI as well as her risk factors and findings on ancillary testing were not supportive of the diagnosis of MS but were more suggestive of embolic ischemic infarcts which were misdiagnosed as MS based on original symptoms, of note by the time the first MRI was obtained in this patients which the date of is unclear she was already carrying the MS diagnosis which was not questioned. e patient was started on low dose aspirin and referred to the stroke division for complete work up and further Case Report Multiple Embolic Strokes and Multiple Sclerosis Misdiagnosis, Case Report Flavia Nelson # and Mostafa Jafari* # Department of Neurology, University of Minnesota, Minneapolis, MN, USA # authors contributed equally Article Information Received date: Dec 01, 2018 Accepted date: Dec 11, 2018 Published date: Dec 12, 2018 *Corresponding author Mostafa Jafari, Department of Neurology, University of Minnesota, MMC 295, 420 Delaware Street S.E. Minneapolis, MN 55455, USA, Tel: 6124422879; Email: [email protected] Distributed under Creative Commons CC-BY 4.0 Keywords Multiple sclerosis, Ischemic stroke; Misdiagnosis Abstract A 69-year-old female with a diagnosis of multiple sclerosis (MS) was referred to our department to establish care for MS. However, the initial clinical and radiographic examinations were not typical for MS. The atypical physical examination, presence of sub cortical hyperintense T2 weighted FLAIR lesions, lack of evidence for old lesions, and presence of a trial fibrillation led us to replace the diagnosis with multiple ischemic infarcts. The current case report indicates the importance of detailed physical examination and interpretation of the imaging data to prevent confirmation bias.

Transcript of Multiple Embolic Strokes and Multiple Sclerosis ... · SM Journal of Neurological Disorders and...

Page 1: Multiple Embolic Strokes and Multiple Sclerosis ... · SM Journal of Neurological Disorders and Stroke How to cite this article Nelson F and Jafari M. Multiple Embolic Strokes and

SM Journal of Neurological Disorders and Stroke

How to cite this article Nelson F and Jafari M. Multiple Embolic Strokes and Multiple Sclerosis Misdiagnosis, Case Report. SM J Neurol Disord Stroke. 2018; 4(2): 1016s6.OPEN ACCESS

IntroductionThe diagnosis of multiple sclerosis (MS) has relied upon accumulation of clinical presentation,

radiological and laboratory investigations that leads to a positive diagnosis and can help to eliminate deferential diagnoses [1]. However, despite well validated diagnostic criteria misdiagnosis remains a problem that has remarkable implications for patients, health care providers and systems [2]. In a study by Solomon et al., [2] two percent of diagnoses and syndromes mistaken for MS were magnetic resonance imaging (MRI) changes caused by vascular disease. This issue could expose patients to immunomodulatory therapy and causes significant morbidity. In this case report we present a patient with multiple embolic ischemic stroke (IS) which was misdiagnosed as MS.

Case reportA 69 year old right handed female was evaluated as a new patient in the MS clinic at the M

Health’s Clinics and Surgery Center at the University of Minnesota, Minneapolis to establish care. Her history included neurological deficits back to 1966 (age 17) described as left extremity numbness and tingling sensation that lasted a few weeks. She was symptom free until age 31 in 1980 when she was diagnosed with right optic neuritis with complete return to her previous normal baseline. She was referred to the University of Minnesota where she was diagnosed with MS and had intravenous steroids. She had multiple “relapses” for the next 3 years and then she seemed to go into complete remission without any evidence of relapses of progression since 1983, she was never prescribed a disease modifying therapy once they became available. During her interview she reported she has been doing well ever since. On the day of presentation, she reported no significant neurological symptoms except for occasional tingling sensation in distal extremities throughout which was in the process of being evaluated by the neuromuscular division. She did not have any other symptoms related to MS. Her medical history was significant for diabetes mellitus (DM), hypertension, rheumatoid arthritis (RA), peripheral neuropathy likely due to DM, thyroid disease, hypertriglyceridemia, and gout. She was on prednisone 5 mg every day tapering from 10 mg po every day for the treatment of RA. Physical examination showed a gait which was antalgic and unsteady, with hip tilting to the right, she walked with the help of a front wheel walker. Pinprick sensation diminished markedly beyond the ankle bilaterally. The rest of the examination was normal. Further diagnostic workup revealed atrial fibrillation on electrocardiography. Echocardiography showed mild left ventricle hypertrophy probably due chronic hypertension. Her most recent MRI of the brain with and without contrast from 2013 showed multiple T2-weighted (W) hyper intensities mostly in the subcortical white matter, less than 3 mm (most) and some larger lesions which were not typical of MS based on location, size, shape and lack of contrast or corresponding T1-W hypointenstiesor black holes (Figure 1).

No previous films were available for comparison. Lumbar MRI revealed evidence of multiple disc herniation at L4-5, L5-S1. The patient was explained that the findings on neurological exam and MRI as well as her risk factors and findings on ancillary testing were not supportive of the diagnosis of MS but were more suggestive of embolic ischemic infarcts which were misdiagnosed as MS based on original symptoms, of note by the time the first MRI was obtained in this patients which the date of is unclear she was already carrying the MS diagnosis which was not questioned. The patient was started on low dose aspirin and referred to the stroke division for complete work up and further

Case Report

Multiple Embolic Strokes and Multiple Sclerosis Misdiagnosis, Case ReportFlavia Nelson# and Mostafa Jafari*#

Department of Neurology, University of Minnesota, Minneapolis, MN, USA#authors contributed equally

Article Information

Received date: Dec 01, 2018 Accepted date: Dec 11, 2018 Published date: Dec 12, 2018

*Corresponding author

Mostafa Jafari, Department of Neurology, University of Minnesota, MMC 295, 420 Delaware Street S.E. Minneapolis, MN 55455, USA, Tel: 6124422879; Email: [email protected]

Distributed under Creative Commons CC-BY 4.0

Keywords Multiple sclerosis, Ischemic stroke; Misdiagnosis

Abstract

A 69-year-old female with a diagnosis of multiple sclerosis (MS) was referred to our department to establish care for MS. However, the initial clinical and radiographic examinations were not typical for MS. The atypical physical examination, presence of sub cortical hyperintense T2 weighted FLAIR lesions, lack of evidence for old lesions, and presence of a trial fibrillation led us to replace the diagnosis with multiple ischemic infarcts. The current case report indicates the importance of detailed physical examination and interpretation of the imaging data to prevent confirmation bias.

Page 2: Multiple Embolic Strokes and Multiple Sclerosis ... · SM Journal of Neurological Disorders and Stroke How to cite this article Nelson F and Jafari M. Multiple Embolic Strokes and

Citation: Nelson F and Jafari M. Multiple Embolic Strokes and Multiple Sclerosis Misdiagnosis, Case Report. M J Neurol Disord Stroke. 2018; 4(2): 1016s6.

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recommendations on need for anticoagulation and scheduled for follow up visit with us after MRI/MRA of head and neck and while waiting for appointment with stroke. The patient was not completely surprised as she found it somewhat atypical to have been so stable for 35 years.

DiscussionA combination of diagnostic criteria that establish dissemination

in space andtime by MRI include the McDonald criteria [3] and the 2016 Magnetic Resonance Imaging in Multiple Sclerosis (MAGNIMS) criteria [4]. However diagnosis of MS could be a challenge since there is no specific biomarker or blood test [5]. A big challenge is that, like MS, many alternate diagnoses lack specific biomarkers and rely on clinical judgment and expertise. We presented a case of embolic ischemic stroke in the context of atrial fibrillation which was misdiagnosed with MS due to neurologic deficits and multiple subcortical white matter lesions in the brain MRI detected after the clinical diagnosis and before MS lesions were more clearly characterized. This is not an uncommon mistake especially when the diagnosis is made by neurologist with minimal exposure to the disease and or updates in diagnosis such as the case of clinicians practicing in rural areas. Acute, pseudo stroke manifestations of MS are rare, only a few cases of sudden aphasia, sudden deafness, or hemiplegia have been described [6]. These manifestations can be confused with the acute phase of stroke or vice versa. Differentiating acute and subacute IS lesions from acute demyelinating lesions of MS is not possible with conventional MRI, however, diffusion-weighted MR imaging (DWI) should facilitate the differential diagnosis.7Both acute demyelinating and acute IS lesions are enhanced on post-contrast T1-W and both are hyperintense on DWI. Neuroimaging studies of patients with acute IS and MS have revealed apparent diffusion coefficient (ADC) differences between demyelinating and ischemic lesions [7]. ADC values decline rapidly after ischemia [8]. Conversely, MS lesions are associated with high ADC values which reflect the increase of the extracellular space either by extracellular edema or by demyelination [9]. However, ADC values may decline in acute MS lesions due to the release of inflammatory cytokines, which would induce mitochondrial dysfunction responsible for cytotoxic

edema [10]. Moreover, periventricular lesions are more specific for MS diagnosis [11]. Our patient’s brain MRI imaging revealed multiple T2-W hyper intensities mostly in the subcortical white matter which was not consistent with MS based on location, size and shape.

ConclusionThis case report raises the issue of the differential diagnosis of

the sudden onset of a neurologic deficit in a young subject, which would likely be either stroke or MS but also raises the issue of patients who were diagnosed with MS prior to or soon after the availability of MRI and the lack of characterization of typical MS lesions in the early stages. We should not only be cautious in interpreting an MRI in this day and age but also question the diagnosis in someone who may have been diagnosed before MRI was available and has an atypical disease course. The current Mc Donald criteria may expedite the diagnosis but also increase the number of false positive MS cases. It is useful to remember that when in doubt, one must repeat MRI in 3-6 months before assigning the MS diagnosis.

References

1. McDonald WI, Compston A, Edan G, Goodkin D, Hartung HP, Lublin FD, et al. Recommended diagnostic criteria for multiple sclerosis: guidelines from the International Panel on the diagnosis of multiple sclerosis. Annals of neurology. 2001; 50: 121-127.

2. Solomon AJ, Bourdette DN, Cross AH, Applebee A, Skidd PM, Howard DB, et al. The contemporary spectrum of multiple sclerosis misdiagnosis: A multicenter study. Neurology. 2016; 87: 1393-1399.

3. Thompson AJ, Banwell BL, Barkhof F, Carroll WM, Coetzee T, Comi G, et al. Diagnosis of multiple sclerosis: 2017 revisions of the McDonald criteria. Lancet Neurol. 2018; 17: 162-173.

4. Filippi M, Rocca MA, Ciccarelli O, De Stefano N, Evangelou N, Kappos L, et al. MRI criteria for the diagnosis of multiple sclerosis: MAGNIMS consensus guidelines. Lancet Neurol. 2016; 15: 292-303.

5. Bove RM, Hauser SL. Diagnosing multiple sclerosis: art and science. Lancet Neurol. 2018; 17: 109-111.

6. Rosso C, Remy P, Creange A, Brugieres P, Cesaro P, Hosseini H. Diffusion-weighted MR imaging characteristics of an acute strokelike form of multiple sclerosis. AJNR Am J Neuroradiol. 2006; 27: 1006-1008.

7. Zivadinov R, Bergsland N, Stosic M, Sharma J, Nussenbaum F, Durfee J, et al. Use of perfusion- and diffusion-weighted imaging in differential diagnosis of acute and chronic ischemic stroke and multiple sclerosis. Neurol Res. 2008; 30: 816-826.

8. Stadnik TW, Demaerel P, Luypaert RR, Chaskis C, Van Rompaey KL, Michotte A, et al. Imaging tutorial: differential diagnosis of bright lesions on diffusion-weighted MR images. Radiographics: a review publication of the Radiological Society of North America, Inc. 2003; 23: e7.

9. Filippi M, Rocca MA, Comi G. The use of quantitative magnetic-resonance-based techniques to monitor the evolution of multiple sclerosis. Lancet Neurol. 2003; 2: 337-346.

10. Gass A, Niendorf T, Hirsch JG. Acute and chronic changes of the apparent diffusion coefficient in neurological disorders--biophysical mechanisms and possible underlying histopathology. J Neurol Sci. 2001; 1: S15-S23.

11. Ruet A, Deloire MS, Ouallet JC, Molinier S, Brochet B. Predictive factors for multiple sclerosis in patients with clinically isolated spinal cord syndrome. Multiple sclerosis (Houndmills, Basingstoke, England). 2011; 17: 312-318.

Figure 1: A) Axial T-2 weighted FLAIR image of the patient demonstrating hyper- intense subcortical white matter lesions. B) Axial T-1 weighted image of the patient showing no black hole lesions.