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Letter to the Editor
Cerebellar alterations: an infrequent presentation of neurosyphilis
Cerebelopatía por sífilis: una presentación infrecuente de neurolúes
J. Millaa, A. Aceitunob, J. Francoa,
Corresponding author
Jonathan.franco@quironsalud.es

Corresponding author.
, A. Chartea
a Departamento de Medicina Interna, Hospital Universitario Quirón Dexeus, Barcelona, Spain
b Departamento de Neurología, Hospital Universitario Quirón Dexeus, Barcelona, Spain
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    "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">With the era of antibiotics&#44; the prevalence of syphilis decreased&#59; however&#44; it has increased in the past 10 years&#44; with an estimated 18 million cases among people aged between 15 and 49 years&#46;<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">1</span></a> Global incidence is 1&#46;5 cases per 1000 population&#44; with higher risk in homosexual men&#46;<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">2</span></a> Neurosyphilis is the term used to refer to central nervous system involvement&#44; which may develop at any stage&#44; even in the early phase&#46; Its manifestations include asymptomatic neurosyphilis&#44; meningitis&#44; meningovascular syphilis&#44; general paresis&#44; and tabes dorsalis&#44; although there are also &#8220;atypical&#8221; forms&#44; which do not meet clinical criteria for classic forms&#46;<a class="elsevierStyleCrossRef" href="#bib0055"><span class="elsevierStyleSup">3</span></a></p><p id="par0010" class="elsevierStylePara elsevierViewall">We present the case of a 52-year-old homosexual man with a history of secondary syphilis 7 years before&#46; Diagnosis was clinical and results from the non-treponemal &#40;rapid plasma reagin &#91;RPR&#93; of 1&#58;138&#41; and treponemal tests were positive&#46; We administered 2&#46;4 million units of benzathine penicillin&#44; and improvements were observed&#44; with RPR titres decreasing to 1&#58;2&#59; however&#44; at 3 years of follow-up&#44; the same titre persisted and he voluntarily stopped attending his periodic follow-up consultations&#46; The patient attended the emergency department due to a 3-month history of instability and gait disorder&#44; which had worsened in the previous week&#44; when he also developed language impairment&#46; Physical examination revealed mild bulbar dysarthria and dysmetria in all 4 limbs &#40;predominantly in the right side&#41;&#44; as well as gait ataxia with tendency to drift&#44; increased base of support&#44; and Romberg sign&#44; and even falls&#46; Ocular motility showed no alterations&#44; muscle balance was preserved in all 4 limbs&#44; superficial and proprioceptive sensitivity were normal&#44; and deep tendon reflexes were present and symmetrical in all 4 limbs&#46; In the targeted medical history interview&#44; the patient reported that he had not presented associated sphincter alterations or neuropathic pain&#46; A cranial and spinal cord magnetic resonance imaging study revealed chronic small vessel disease in both brain hemispheres&#44; without involvement of the cerebellar parenchyma or spinal cord or pathological contrast uptake&#46; A subsequent spinal cord study with somatosensory evoked potentials yielded normal results&#46; A basic blood analysis &#40;blood count&#44; biochemistry&#44; vitamin B<span class="elsevierStyleInf">12</span> and copper levels&#41; revealed no alterations&#59; in the syphilis serological test&#44; RPR titre was 1&#58;128 and VDRL titre was 1&#58;64&#59; the HIV serology test was negative&#46; A lumbar puncture revealed predominantly lymphocytic pleocytosis &#40;157<span class="elsevierStyleHsp" style=""></span>cells&#47;mm<span class="elsevierStyleSup">3</span>&#41;&#44; high protein levels &#40;57<span class="elsevierStyleHsp" style=""></span>mg&#47;dL&#41;&#44; positive VDRL of 1&#58;4&#44; and positive PCR findings for <span class="elsevierStyleItalic">Treponema pallidum</span>&#46; Differential diagnosis of rapidly progressive cerebellar syndrome should include structural cerebellar lesions &#40;ischaemic&#44; inflammatory&#44; or tumour&#41;&#44; infectious causes &#40;HIV&#44; Epstein-Barr virus&#44; or cytomegalovirus&#41;&#44; autoimmune disease&#44; and paraneoplastic symptoms&#44; which were ruled out in our patient&#46; With the diagnostic suspicion of neurosyphilis of atypical presentation &#40;due to clinical signs suggestive of cerebellar involvement with no parenchymal lesions in the cerebellum and normal spinal cord findings&#41;&#44; we started treatment with intravenous crystalline penicillin for 14 days&#46; One week after treatment onset&#44; the patient&#39;s clinical symptoms improved and he was able to walk&#59; Romberg test was negative&#46;</p><p id="par0015" class="elsevierStylePara elsevierViewall">The decrease in the mortality associated with HIV&#44; due to the effectiveness of antiretroviral treatment&#44; has caused an increase in the incidence of sexually transmitted infections&#44; including syphilis&#46; This has led to the reappearance of neurological manifestations &#40;neurosyphilis&#41;&#46;<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">2</span></a> Untreated syphilis may result in neurological impairment in 4&#37;-10&#37; of patients&#44; but cases have also been described in immunocompetent patients receiving appropriate antibiotic treatment of the primary infection&#46;<a class="elsevierStyleCrossRef" href="#bib0060"><span class="elsevierStyleSup">4</span></a></p><p id="par0020" class="elsevierStylePara elsevierViewall">To our knowledge&#44; there are no previous reports of neurosyphilis with cerebellar alterations&#44; although the wide spectrum of clinical manifestations of syphilis is widely known&#59; syphilis was historically known as &#8220;the great simulator&#46;&#8221; Classic forms of presentation include syphilitic meningitis&#44; meningovascular variant&#44; general paresis&#44; and tabes dorsalis&#46; However&#44; there is an &#8220;atypical&#8221; form that may present with unusual manifestations&#44; such as seizures&#44; psychiatric symptoms&#44; altered level of consciousness&#44; and behavioural alterations similar to those of viral or autoimmune encephalitis&#46;<a class="elsevierStyleCrossRefs" href="#bib0055"><span class="elsevierStyleSup">3&#44;5</span></a> In our case&#44; we should mention the early clinical improvement with antibiotic treatment&#44; which may suggest that progression time was shorter&#59; in more chronic stages&#44; such as tabes dorsalis or general paresis&#44; the damage is irreversible&#46;</p><p id="par0025" class="elsevierStylePara elsevierViewall">Another controversial issue is the indication of lumbar puncture during diagnosis of syphilis&#59; it is currently indicated in patients with neurological&#44; ophthalmic&#44; or ear signs and symptoms&#59; tertiary syphilis in another part of the body&#59; or treatment failure &#40;including failure to decrease titres in non-treponemal blood tests&#41;&#46;<a class="elsevierStyleCrossRef" href="#bib0070"><span class="elsevierStyleSup">6</span></a> However&#44; a more controversial recommendation is that lumbar puncture should be performed in those patients with syphilis in any stage who present high titres in non-treponemal tests during the initial diagnosis &#40;RPR<span class="elsevierStyleHsp" style=""></span>&#8805;<span class="elsevierStyleHsp" style=""></span>1&#58;32&#41;&#44; regardless of whether the patient presents neurological symptoms or is HIV-positive&#44; since in these patients&#44; the risk of presenting asymptomatic neurosyphilis is 11 times higher than among those with lower titres&#46;<a class="elsevierStyleCrossRef" href="#bib0075"><span class="elsevierStyleSup">7</span></a> Our patient presented high titres during the first diagnosis of secondary syphilis &#40;RPR 1&#58;134&#41;&#46; Twenty percent of patients with asymptomatic neurosyphilis develop the symptomatic form during the first 10 years&#44; especially when they present high pleocytosis or high cerebrospinal fluid protein levels&#46; In this scenario&#44; there is controversy regarding the actions to be taken in daily practice&#44; as more studies are needed to assess whether it is necessary to actively screen for and treat asymptomatic neurosyphilis&#46;<a class="elsevierStyleCrossRef" href="#bib0080"><span class="elsevierStyleSup">8</span></a></p><p id="par0030" class="elsevierStylePara elsevierViewall">In conclusion&#44; the incidence of syphilis is increasing both in immunosuppressed and in immunocompetent patients&#46; This leads to a higher incidence of central nervous system infection due to syphilis &#40;neurosyphilis&#41;&#46; Neurological symptoms may vary greatly&#44; from the classic form to atypical manifestations&#46; Healthcare professionals should be aware of this increasing incidence and the possibility of unusual neurological symptoms in neurosyphilis&#44; as early treatment may prevent sequelae and improve survival&#44; as in our patient&#46;</p></span>"
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        "nota" => "<p class="elsevierStyleNotepara" id="npar0005">Please cite this article as&#58; Milla J&#44; Aceituno A&#44; Franco J&#44; Charte A&#46; Cerebelopat&#237;a por s&#237;filis&#58; una presentaci&#243;n infrecuente de neurol&#250;es&#46; Neurolog&#237;a&#46; 2020&#59;35&#58;443&#8211;444&#46;</p>"
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