Casimiro, C

Herrero Valverde, A

Martins, R

Peres, J

Unidade Local de Saúde Amadora / Sintra

206  LETTERS  TO  THE  EDITORSubacute sclerosingpanencephalitis presenting asrapidly progressive dementia ina  young adultDemencia rápidamente progresiva  en adultojoven como forma  de presentación  depanencefalitis esclerosante subagudaDear  Editor:Subacute  sclerosing  panencephalitis  (SSP)  is  a  neurodegen-erative  disorder  caused  by  persistent  infection  with  measlesvirus.1 It is  characterised  by  progressive  cognitive  and  motorimpairment  and  associated  with  myoclonus.1,2 The  conditionis  rarely  observed  in adult  patients;  when  this  does  occur,SSP  manifests  with  atypical  symptoms.3We  present  the  case  of  a 25-year-old  man with  no  rele-vant  medical  history  who  developed  behavioural  alterationsFigure  1  (1a  and  1b)  Axial  FLAIR  MR  images  (12  months  after  symptom  onset)  showing  diffuse  hyperintensities  in cortical  andsubcortical  regions  of the  parietal  and  temporal  lobes.  (2a/b,  3a/b)  Axial  FLAIR  MR  images  (26  months  and  8  years  after  symptomonset, respectively)  revealing  disease  progression,  with  severe  cerebellar  and  brainstem  atrophy  and a  diffuse,  extensive  lesion  tothe periventricular  white  matter. Please cite this article as: Martins R, Peres J, Casimiro C,Valverde A. Demencia rápidamente progresiva en adulto joven comoforma de presentación de panencefalitis esclerosante subaguda.Neurología. 2018;33:206—207.and  cognitive  deficits  over  the  course  of  3  years  due  toSSP.  The  initial  neurological  examination  revealed  inatten-tion,  impaired  executive  function,  poor  language  fluency,motor  perseveration,  predominantly  right-sided  ideomotorapraxia,  asomatognosia,  agraphaesthesia,  and  right  hemi-paresis.  A  brain  MRI scan  revealed  an extensive  left-sidedcortico-subcortical  temporoparietal  lesion,  as  well  as  othersmaller  lesions  in the  bilateral  subcortical  white  matter  onT2-weighted  and  FLAIR  sequences;  lesions  showed  no  con-trast  uptake  and the  results  of a  cerebral  angiography  studywere  normal  (Fig.  1: 1a  and 1b).  Serial  electroencephalogra-phy  revealed  diffuse  slowing,  with  theta  rhythm  in  posteriorregions  and  no  periodic  activity.We excluded  prion  disease,  autoimmune  encephalopathy,inflammatory  vasculopathy,  and  progressive  multifocalleukoencephalopathy.  The  results  from  serology  tests andautoimmune  studies  were  negative.  A lumbar  punctureyielded  the  following  results:  one red  blood  cell,  51  mg/dLglucose,  54  mg/dL  proteins,  and a  high  IgG index  (1.74)  withpresence  of  oligoclonal  bands.  Results  from  CSF polymerasechain  reaction  for  Tropheryma  whippelii  and herpes  sim-plex,  varicella-zoster,  and  JC  viruses;  syphilis  serology;  andDocument downloaded from http://www.elsevier.es, day 03/07/2018. This copy is for personal use. Any transmission of this document by any media or format is strictly prohibited.LETTERS  TO  THE  EDITOR  20714-3-3  protein  determination  were  all  negative.  Radiologi-cal  studies  and  CSF anti-neuronal  antibody  determinationsrevealed  no tumours.The  clinical,  imaging,  and  laboratory  findings  led tosuspicion  of  SSP;  diagnosis  was  confirmed  by  high  anti-measles  virus  antibody  titres  in the serum  and CSF (1:10and  1:134,  respectively).  The  patient  had  no  history  ofinfection  or  exposure  to  measles  virus  and  had  been  vac-cinated  against  the  virus.  In the following  6  months,  thepatient’s  clinical  symptoms  worsened  progressively:  he dis-played  myoclonus  in the  form of  periodic  supraversion  eyemovements  and  refractory  tonic-clonic  seizures.  A  follow-upMRI  scan  revealed  severe  cerebellar  and  brainstem  atrophy(Fig.  1: 2a,  2b,  3a, and  3b). The  patient  is  currently  in avegetative  state,  displaying  akinetic  mutism.SSP  typically  presents  during  the  first  and  second  decadesof  life;  only  100 cases of  adult-onset  SSP  are described  inthe  literature.4 Incidence  of  the  condition  decreased  by  90%after  the  introduction  of the measles  vaccine  into  the vac-cination  schedule.5 A causal relationship  between  measlesvaccination  and  SSP  has  not been  determined.  The  cases  ofSSP  described  in patients  vaccinated  against  measles  virusare  thought  to  be  due  to subclinical  measles  infection  withinthe  first  year  of  life.1As  in  our  case,  most  cases of  adult-onset  SSP  are not  asso-ciated  with  a  history  of  infection  or  exposure  to  measlesvirus.3 Atypical  symptoms  are  also  more  frequent  in  adult-onset  SSP.2,3 Our  patient  experienced  progressive  motorand  cognitive  impairment  without  generalised  myoclonus  orperiodic  EEG  activity;  these  findings  are  typical  in  advancedstages  of  the  disease.3 High anti-measles  virus  antibodytitres  in the  serum  and  CSF  confirm  the diagnosis  of  SSPdue  to  their  high  sensitivity,  specificity,  and  positive  predic-tive  value.1,2 Death  usually  occurs  1-3  years  after  symptomonset;  there  is  currently  no  effective  treatment  for  thecondition.2 A combination  of  intrathecal  interferon  alfa andoral  isoprinosine  has  shown  an efficacy  of  30%  to  35%  in clin-ical  trials.6,7 Our  patient  did  not  start treatment  due  to  theadvanced  stage  of the  condition.In conclusion,  a  diagnosis  of SSP  must  be  consideredin  young  patients  with  progressive  cognitive  impairment.Atypical  symptoms  are frequent  in adult-onset  SSP,  espe-cially  in cases with  compatible  serology  and imagingfindings.References1. Garg R.  Subacute sclerosing panencephalitis. J Neurol.2008;255:1861—71.2. Gutierrez J, Issacson PS, Koppel BS. Subacute sclerosing panen-cephalitis: an update. Develop Med Child Neurol. 2010;52:901—7.3. Singer C, Lang AE, Suchowersky O. Adult-onset subacute scleros-ing panencephalitis: case reports and review of literature. MovDisord. 1997;12:342—53.4. Prashanth LK, Taly AB, Ravi V, Sinha S, Arunodaya GR. Adultonset subacute sclerosing panencephalitis: clinical profile of 39patients from a tertiary care center. J  Neurol Neurossurg Pshy-chiatry. 2006;77:630—3.5. Campbell H, Andrews N, Brown KE, Miller E. Review of the effectof measles vaccination on  the epidemiology of  SSPE. Int J  Epi-demiol. 2007;36:1334—48.6. Anlar B, Saactci I, Köse G, Yalaz K.  MRI findings in subacute scle-rosing panencephalitis. Neurology. 1996;47:1278—83.7. Eroglu E, Gokcil Z, Bek S, Ulas UH, Ozdag MF, Odabasi Z.  Long-term follow-up of  patients with adult-onset subacute sclerosingpanencephalitis. J  Neurol Sci. 2008;275:113—5.R.  Martins a,∗,  J.  Peres a, C.  Casimirob,  A.  Valverde a,ca Servicio  de  Neurología,  Hospital  Prof.  Doutor  FernandoFonseca,  Amadora,  Lisboa,  Portugalb Servicio  de Neurorradiología,  Hospital  Prof.  DoutorFernando  Fonseca,  Amadora,  Lisboa,  Portugalc CEDOC,  Nova  Medical  School,  Universidade  Nova  deLisboa,  Lisboa,  Portugal∗Corresponding  author.E-mail  address:  ritadossantosmartins@gmail.com(R.  Martins).2173-5808/© 2017 Sociedad Espan˜ola  de Neurolog´ıa. Published by ElsevierEspan˜a, S.L.U. This is  an open access article under the CCBY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).Locating the site ofcerebrospinal fluid leak in apatient  with low cerebrospinalfluid  pressureLocalización del punto  de  fuga de líquidocefalorraquídeo en  un  caso de hipotensión delíquido cefalorraquídeo Please cite this article as: García Martín G, Rodríguez Belli AO,Padilla Parrado F, Aguilar Cuevas R.  Localización del punto de fugade líquido cefalorraquídeo en un caso de hipotensión de líquidocefalorraquídeo. Neurología. 2018;33:207—209.Dear  Editor:Low  cerebrospinal  fluid  (CSF) pressure  due  to  meningeal  tearis  an infrequent  but  widely  reported  condition,  often  associ-ated  with  typical  neuroimaging  findings  that  are  helpful  fordiagnosis.  However,  it is  relatively  infrequent  for  the  site ofthe  CSF  leak to  be located,  especially  in cases  secondary  tomild  trauma;  the upper  thoracic  spine  and cervico-thoracicjunction  are the  most  frequent  locations.1 Locating  the siteof the CSF leak  is  relevant  due  to  the possible  prognosticimplications.We  present  a case  with  striking  neuroimaging  findings,which  enabled  us to clearly  locate the site  of  the  CSF  leakat  the  lumbar  level  (a  rare  location);  this was  essential  toestablish  a  definitive  treatment.Our  patient  was  a 30-year  old  woman  with  no  rele-vant  personal  history,  who  visited  our  department  due  toDocument downloaded from http://www.elsevier.es, day 03/07/2018. This copy is for personal use. Any transmission of this document by any media or format is strictly prohibited.

Demencia rápidamente progresiva en adulto joven como forma de presentación de panencefalitis esclerosante subaguda

R. Martins

J. Peres

C. Casimiro

A. Valverde

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Neurología

http://repositorio.hff.min-saude.pt/bitstream/10400.10/2019/1/Neurologia.%202018%20Apr.pdf

Demencia rápidamente progresiva en adulto joven como forma de presentación de panencefalitis esclerosante subaguda

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