Basal Ganglia - Amazon S3€¦ · Immune Cross-Reactivity of the Basal Ganglia – PANDAS...

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Transcript of Basal Ganglia - Amazon S3€¦ · Immune Cross-Reactivity of the Basal Ganglia – PANDAS...

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Basal Ganglia

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Basal Ganglia Anatomical Review

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Basal Ganglia Anatomical Grouping

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Basal Ganglia Anatomical Grouping

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Basal Ganglia

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Basal Ganglia Direct and Indirect Pathways

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Basal Ganglia Loops

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Figure 1

The Lancet 2014 384, 523-531DOI: (10.1016/S0140-6736(13)62418-6) Copyright © 2014 Elsevier Ltd Terms and Conditions

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Basal Ganglia Loops

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Basal Ganglia Somatotopic Organization

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7  Concepts  for  The  Rate  Model  

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Basal Ganglia Indirect Pathway

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Motor Tic Disorder

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Sydenham’s Chorea

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Hemichorea--Hemiballism after Diabetic Ketoacidosis

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Basal Ganglia Direct Pathway

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Figure 3

The Lancet 2014 384, 523-531DOI: (10.1016/S0140-6736(13)62418-6) Copyright © 2014 Elsevier Ltd Terms and Conditions

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Young Onset Parkinson’s Disease

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Parkinson’s Feature of Re-Emergent Tremor

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Video Examples

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Unified  Parkinson’s  Disease    Ra%ng  Scale  

•  Hand  Movements  •  O  =  Normal  •  1  =  mild  slowing,  and/or  reduc,on  in  amplitude  •  2  =  moderately  impaired,  definite  and  early  fa,guing,  may  have  occasional  arrests    

•  3  =  severely  impaired,  frequent  hesita,ons  and  arrest  •  4  =  can  barely  perform    

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Basal Ganglia Hyperdirect Pathway

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Neurodegenerative Diseases of the Basal Ganglia

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Lewy body disease in Parkinson’s

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Lewy body disease in Parkinson’s

Parkinson  disease,  Lewy  body:  The  Lewy  body  is  the  pathological  hallmark  of  Parkinson  disease.  The  Lewy  body  inclusion  shows  an  eosinophilic  core  surrounded  by  a  pale  halo  (arrow).  The  protein  alpha-­‐synuclein  is  a  component  of  the  Lewy  body.  

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Degenerative Substantia Nigra in Parkinson’s Disease

The  lea  shows  Parkinson's  disease  with  a  pale  substan,a  nigra  compared  to  a  normal  ,ssue  on  the  right    

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Genetic Neurodegenerative Disorder – Hunington’s Disease

Microscopically,  the  caudate  nucleus  in  Hun,ngton's  disease  demonstrates  loss  of  neurons  along  with  gliosis.  

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Hun,ngton's  disease  is  shown  grossly  in  this  coronal  sec,on  of  the  brain.  It  demonstrates  atrophy  of  the  caudate  with  resultant  increase  in  size  of  lateral  ventricles.  

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Lacunar Infarction of the Basal Ganglia

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Lacunar  infarct  (<1cm):  A  rela,vely  small  cys,c  lesion  in  the  putamen  secondary  to  small  vessel  disease.  Common  loca,ons  for  lacunes  include  basal  ganglia,  pons,  internal  capsule,  thalamus  and  cerebral  white  maSer.  

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Lacunar Infarcts

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Mechanisms that Injure the Basal Ganglia

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Poewe  W,  Djamshidian-­‐Tehrani  A.  Movement  disorders  in  systemic  diseases.  Neurol  Clin.  2015  Feb;33(1):269-­‐97.  

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Poewe  W,  Djamshidian-­‐Tehrani  A.  Movement  disorders  in  systemic  diseases.  Neurol  Clin.  2015  Feb;33(1):269-­‐97.  

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Poewe  W,  Djamshidian-­‐Tehrani  A.  Movement  disorders  in  systemic  diseases.  Neurol  Clin.  2015  Feb;33(1):269-­‐97.  

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Carbon  monoxide  poisoning:  During  the  first  few  hours  aaer  carbon  monoxide  poisoning,  the  brain  is  swollen,  congested  and  cherry-­‐red.  Aaer  24-­‐48  hours  of  survival,  scaSered  petechial  hemorrhages  may  be  seen  in  white  maSer  with  larger  hemorrhages  in  the  pallidum  (arrows).  

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Pesticide Induced Destruction of Basal Ganglia

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96  96  

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Infectious Destruction of Basal Ganglia

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Immune Cross-Reactivity of the Basal Ganglia – PANDAS

(Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcus)

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Iron Accumulation Induced Destruction of the Basal Ganglia

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Globus Pallidus Iron Accumulation

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Copper Toxicity (Wilson’s Disease) Destruction of the Basal Ganglia

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Wilson  disease  (hepatolen%cular  degenera%on):  Wilson  disease  is  an  autosomal  recessive  disorder  caused  by  muta,ons  in  a  copper  transport  gene  (ATP7B).  The  putamen  appears  brown  and  shrunken.  The  caudate  is  oaen  severely  affected,  but  pallidum,  thalamus  and  brain  stem  may  be  less  severely  involved.  

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Autoimmune Destruction of Basal Ganglia

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Basal Ganglia and Nutritional Considerations

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