RT Book, Section
A1 Stover, Natividad P.
A1 Watts, Ray L.
A2 Watts, Ray L.
A2 Standaert, David G.
A2 Obeso, Jose A.
SR Print(0)
ID 55796880
T1 Chapter 22. Corticobasal Degeneration
T2 Movement Disorders, 3e
YR 2012
FD 2012
PB The McGraw-Hill Companies
PP New York, NY
SN 978-0-07-161312-5
LK neurology.mhmedical.com/content.aspx?aid=55796880
RD 2024/04/19
AB Corticobasal  degeneration  (CBD)  is  a  neurodegenerative  disorder  that  has  gained  interest  of  neurologists,  neuropathologists,  and  other  neuroscientists  in  the  last  four  to  five  decades.  Over  the  past  years,  there  has  been  a  tendency  to  consider  CBD  as  a  syndrome  more  than  a  nosologic  entity  because  of  the  heterogeneity  of  clinical  presentations  and  overlap  of  the  clinical  and  pathological  features  with  other  neurodegenerative  diseases.  The  patients  with  the  classical  motor  presentation  may  be  still  very  characteristic,  and  currently  most  of  the  pathologically  confirmed  cases  of  CBD  can  be  divided  into  two  distinct  groups:  (1)  patients  that  present  mainly  with  a  movement  disorder  and  (2)  patients  that  develop  cognitive  problems.  As  the  disease  progresses,  most  of  the  patients  may  manifest  both  types  of  symptoms.  The  lack  of  biomarkers  for  the  diagnosis  of  the  different  neurodegenerative  processes  makes  the  premortem  diagnosis  of  these  disorders  even  more  inaccurate  and  difficult  to  differentiate  in  a  clinical  setting.  CBD  was  originally  called  corticodentatonigral  degeneration  with  neuronal  achromasia,  based  in  the  pathological  findings  of  three  original  cases  described  by  Rebeiz  and  colleagues  in  1967.1  The  term  “CBD”  was  initially  used  by  Gibb  and  colleagues  in  1989,2  and  in  2000,  the  term  CBD  Syndrome  was  proposed  by  Kertesz.3  Other  names  used  to  describe  the  cases  of  CBD  included  corticonigral  degeneration  with  neuronal  achromasia,4  corticodentatonigral  degeneration,5  cortical-basal  ganglionic  degeneration,6  and  myoclonic  dystonia.7  In  the  nineteenth  century,  Jean-Martin  Charcot,  Alfred  Vulpian,  and  their  disciples  described  cases  of  atypical  Parkinson's  disease  (PD)  with  jerking  movements  and  abnormal  posture  of  the  limbs,  called  originally  “hemiplegic  parkinsonism.”8,9  Many  of  these  clinical  cases  lack  a  complete  description,  since  the  characterization  of  many  neurologic  signs  and  symptoms  such  as  apraxia,  dystonia,  cortical  sensory  deficits,  and  myoclonus  were  not  a  regular  part  of  the  neurological  examination  performed  at  that  time.  The  description  of  some  of  Charcot's  atypical  parkinsonian  cases  has  some  of  the  distinctive  features  that  we  consider  today  associated  with  CBD.  Jacques  Lhermitte  in  1925  described  a  clinical  case  to  the  French  Neurological  Society  that  is  suggestive  of  CBD.10  “A  67-year-old  carpenter  with  progressive  right  hand  clumsiness,  unable  to  ambulate  independently  at  age  of  72,  with  the  right  arm  flexed  and  an  involuntary  right  arm  movement  ‘like  a  foreign  body,’  unable  to  recognize  objects  in  his  right  hand,  in  spite  of  normal  primary  sensation.”