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Letter to the Editor
Atypic onset of multiple sclerosis mimicking Susac's Syndrome
Presentación atípica de esclerosis múltiple que imita el síndrome de Susac
Asuman Orhan Varoglua,
Corresponding author
asumanorhan69@gmail.com

Corresponding author.
, Mehmet Akif Balcia, Basak Atalayb
a Department of Neurology, Medical School, Istanbul Medeniyet University, Istanbul, Turkey
b Department of Radiology, Medical School, Istanbul Medeniyet University, Istanbul, Turkey
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and round-shaped lesions in the CC and CS&#46; We describe a female MS patient with polymicrogyria and an unusual initial presentation that mimics SS&#46; A 21-year-old female has been admitted due to fever&#44; encephalopathy&#44; convulsion&#44; cold&#44; and flu symptoms&#44; for 4 days&#46; Cooperation and orientation disorder&#44; apathy&#44; and tetraparesis &#40;3&#8211;4&#47;5&#41; were determined&#46; Serum biochemical parameters and Cerebrospinal fluid analysis &#40;CSF&#41; for the viral panel&#44; culture&#44; and biochemistry were normal&#46; MRI revealed hyperintense lesions in the periventricular region&#44; left cerebellar hemisphere&#44; and CC on Axial T2-weighted and Sagittal T2-weighted images&#46; Furthermore&#44; bilateral polymicrogyria was demonstrated involving both cerebral hemispheres &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>a&#41;&#46; No contrast enhancement was seen&#46; Anti-myelin oligodendrocyte glycoprotein &#40;anti-MOG&#41;&#44; oligoclonal band&#44; and anti-aquaporin-4 &#40;neuromyelitis optica spectrum disease&#41; were negative&#46; The IgG index was 0&#46;55&#46; CSF showed glucose&#58; 74<span class="elsevierStyleHsp" style=""></span>mg&#47;dL &#40;40&#8211;70<span class="elsevierStyleHsp" style=""></span>mg&#47;dl&#41;&#44; sodium &#40;Na&#41;&#58; 147<span class="elsevierStyleHsp" style=""></span>mmol&#47;L &#40;135&#8211;150<span class="elsevierStyleHsp" style=""></span>mmol&#47;L&#41;&#44; chloride &#40;CL&#41;&#58; 122<span class="elsevierStyleHsp" style=""></span>mmol&#47;L &#40;135&#8211;150<span class="elsevierStyleHsp" style=""></span>mmol&#47;L&#41;&#44; and protein&#58; 170<span class="elsevierStyleHsp" style=""></span>mg&#47;dL &#40;150&#8211;450<span class="elsevierStyleHsp" style=""></span>mg&#47;L&#41;&#44; and no cells were observed&#46; <span class="elsevierStyleSup">18</span>F-FDG PET&#47;CT revealed hypometabolic lesions&#46; MR spectroscopy showed both NAA&#47;choline ratio and choline&#47;creatine ratio dropped suggesting demyelination lesions&#46; Therefore&#44; brain tumors were excluded&#46; No hearing loss and BRAO were found&#46; Methylprednisolone &#40;IVP&#44; 1000<span class="elsevierStyleHsp" style=""></span>mg&#47;day&#41; therapy was initiated following the preliminary diagnosis of SS&#46; Levetiracetam 1000<span class="elsevierStyleHsp" style=""></span>mg&#47;day was given&#46; On MRI&#44; substantial regressions were seen&#44; two weeks later&#46; A month later&#44; she was admitted to hospital once more same complaints&#46; Hyperintense lesions were seen in the superior frontal subcortical WM and the right middle cerebellar peduncle &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>b&#41;&#46; New oligoclonal analysis band was positive&#46; She was diagnosed as having atypical-onset MS&#46; A new hyperintense lesion was discovered at C2 level in the second hospitalization &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>c&#41;&#46; After treatment&#44; her symptoms were regressed&#46; She began fingolimod therapy&#44; and no further attacks or lesions were seen for almost two years&#46; Acute disseminated encephalomyelitis &#40;ADEM&#41; has a mildly increased CSF protein level and lymphocytic pleocytosis&#46; ADEM lesions are often bigger&#44; bilateral&#44; asymmetric&#44; and more numerous than MS lesions&#46; Indistinct margins are a common imaging feature of ADEM lesions&#46; This may make it easier to distinguish these lesions from MS lesions&#44; which often have clear-cut edges&#46;<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">2</span></a> In our case&#44; a CSF revealed no cells&#44; and protein level was normal&#46; Additionally&#44; all lesions showed no contrast enhancement&#46; So&#44; ADEM was eliminated&#46; Brain tumors including primary brain lymphoma were excluded by <span class="elsevierStyleSup">18</span>F-FDG PET&#47;CT and MR spectroscopy&#46;<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">2</span></a> BRAO and hearing loss were not found even though she was present encephalopathy and had CS lesions and &#8220;snowball lesions&#8221; in CC seen in SS&#46; Initially&#44; we did not think of MS because MRI revealed hyperintense lesions mimicking SS C&#44; moreover&#44; all lesion&#39;s ages were the same&#46; New lesions with contrast enhancement were seen during the second hospitalization&#44; particularly on the right side of middle cerebellar peduncle and C2 level&#46; Oligoclonal band was positive finally&#44; so she was diagnosed with MS&#46; We also demonstrated that she had bilateral polymicrogyria&#46; There is no report of MS case with polymicrogyria&#46; Independent of isolated lesions&#44; although appearing to be normal&#44; widespread alterations in Gray Matter &#40;GM&#41; and WM might be pathogenic&#46;<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">3</span></a> Although this pathophysiological process&#44; which is ubiquitous in both GM and WM&#44; may have an impact on one another&#44; this impact does not have to be parallel&#46;<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">4</span></a> We speculated that polymicrogyria in both hemispheres changed the way that GM and WM interacted&#44; leading to the development of atypical demyelination lesions in the WM&#46; Therefore&#44; the patient&#39;s initial clinical presentation and MRI findings were unusual for MS&#46; Even at the first presentation&#44; MS should be considered when there are cortical clinical symptoms&#44; &#8220;snowball lesions&#8221; in the CC&#44; and lesions in the CS&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0005">Authors&#8217; contribution</span><p id="par0010" class="elsevierStylePara elsevierViewall">Asuman Orhan Varoglu&#58; Conception&#44; design&#44; supervision&#44; data collection and&#47;or processing&#44; analysis and&#47;or interpretation&#44; literature review&#44; writing&#46; Mehmet Akif Balci&#58; Data collection and&#47;or processing&#46; Basak Atalay&#58; Analysis and&#47;or interpretation&#44; literature review&#46;</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0010">All data are available</span><p id="par0015" class="elsevierStylePara elsevierViewall">Written informed consent was obtained from the patient&#46;</p></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0015">Funding</span><p id="par0020" class="elsevierStylePara elsevierViewall">None&#46;</p></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0020">Conflicts of interest</span><p id="par0025" class="elsevierStylePara elsevierViewall">None&#46;</p></span></span>"
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Original language: English
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