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NEURO IMAGING IN
MOVEMENT DISORDERS




                 DR. A.V. SRINIVASAN
Clinical Syndrome Approach
     2.1. WHAT ARE THE MOVEMENTS

       AKINETIC OR DYSKINETIC
       TREMOR
       JERKS             Myclonus
                         Chorea
                         Tic
       SPASMS            Dystonia
                         Rhythmic / arhythmic
                         Stereo typed / in consistant
                         Continous
                         Action
                         Paroxysms


             Slide - 2                       NEURO IMAGING IN MOVEMENT DISORDERS
Clinical Syndrome Approach
    Akinetic rigid syndrome
    Dystonic syndrome
    Choreic syndrome
    Tic syndrome
    Myoclonic syndrome




                 Slide - 3     NEURO IMAGING IN MOVEMENT DISORDERS
ODD DYSKINESIAS
   ODDTREMOR
   ODD JERKS
      FOCAL MYOCLONUS
      CORTICAL MYOCLONUS
   GIANT SOMATO SENSORY
   HYPEREXPLEXIA
   ODD SPASMS




            Slide - 4      NEURO IMAGING IN MOVEMENT DISORDERS
Brain Iron
    Iron is a key trace metal relating to brain function. The major
     functions in which iron performs a role include:
        Oxidative phosphorylation
        Dopamine synthesis and degradation
        Hydroxyl free-radical formation




                  Slide - 5                  NEURO IMAGING IN MOVEMENT DISORDERS
Brain Iron
 A. Neurodegenerative
 i. Parkinson’s Plus                            Putamen
          a. Striatonigral Degeneration
          b. Shy-Drager
          c. Olivopontocerebellar Atrophy
 ii. Hallervorden-Spatz                         Globus Pallidus
                                                (+ Red Nucleus / Nigra)
 iii.Alzheimer’s                                Cortical


 B. Metabolic
 i. Hypothyroidism                                Generalized
 ii. [Hepatic failure exhibits signal hyperintensity in the globus pallidus on T1 images


                       Slide - 6                           NEURO IMAGING IN MOVEMENT DISORDERS
Brain Iron

    C. Hemorrhage
        Intracerebral Hematoma   Site of Bleed
        Hemorrhagic Infarction   Gyral
        Vascular Malformation    Interstices / Margin




                 Slide - 7               NEURO IMAGING IN MOVEMENT DISORDERS
Brain Iron
 D. Demyelination

 i.      Multiple Sclerosis (severe)         Thalamus /Putamen

 ii.      Microangiopathic
        Leukoencephalopathy            Putamen

 iii.    Radiation injury              White Matter/ Putamen


                    Slide - 8                 NEURO IMAGING IN MOVEMENT DISORDERS
INVESTIGATIONS FOR PRIMARY MOVEMENT
DISORDERS
    Imaging (MRI)
    Exclusion of Wilson <50)
    Genentic testing
    Routine blood wing Biochemistry
    Syphilis




                 Slide - 9             NEURO IMAGING IN MOVEMENT DISORDERS
Investigation in Secondary Movement
Disorders
    GENERAL PLAN
       Extent of nervous system involvement
       Psychometric evaluation
       EEG (epilepti form discharges)
       ENMG (peripheral neruropathy)
       EMG and VEP




                 Slide - 10              NEURO IMAGING IN MOVEMENT DISORDERS
Role of CT
    CONDITIONS THAT MAY PRODUCE WHITE MATTER
     CHANGE ON BRAIN CT OR MRI AND WHICH MAY CAUSE
     MOVEMENT DISORDERS.
       Alexander’s disease
       Krabbe’s disease
       Polycystic lipomembranous osteodyplasia
       Adeno – myelo – Leukodystrophy
       Metachromatic leukodystrophy
       Mitrochondrial diorders.


              Slide - 11         NEURO IMAGING IN MOVEMENT DISORDERS
DEGENERATIVE AND SYSTEMIC DISORDERS
    Polyeystic lipomembraneous                  Hand X-rays, skin biopsy
    Degeneration
    Hallervorden Spatz disease                  Brain MRI
    Pallidal degenerations                      Brain MRI
    Alzheimer’s disease                         Brain MRI
    Multi – infarct state                       Brain MRI
    Binswanger’s subcortical arteriosclerotic   Brain MRI
     Encephalopathy
    Normal pressure hydrocephalus               Brain MRI
    Dementia pugilistica                        Brain MRI
    Calcification of the basal ganglia          Brain CT and MRI
    Cerebral anoxia
     (Co and cyanide poisoning)                  Brain MRI


                      Slide - 12                             NEURO IMAGING IN MOVEMENT DISORDERS
Wilson’s Disease

    3 movement disorders
        Akinetic-rigid syndrome resembling Parkinsonism
        Dystonic syndrome
        Postural and intentional tremor
    Clinical
        Tremor, rigidity, gait difficulty
        Bulbar signs: indistinct speech, dysphagia
        Hepatic encephalopathy
        Anemia (10%)


                 Slide - 13               NEURO IMAGING IN MOVEMENT DISORDERS
Wilson’s Disease
    Dx
           Kayser-Fleischer  ring
           Elevated urinary copper
           Decreased serum ceruloplasmin
           Elevated bepatic copper

    CT DDX
           Straiatonigraldegeneration
           Metabolic acidosis
           Leigh disease
           2o parkinsonism
           acquired hepatocerebral degeneration
           myeliolysis
           HIE


                    Slide - 14                     NEURO IMAGING IN MOVEMENT DISORDERS
Wilson’s Disease

    MR
       Altered GM and WM signal intensities
       Atrophy of caudate head, brain stem
       Cerebral and cerebellar atrophy
    MR: 3 patterns
           T1               PD/T2
        Variable            increased
        increased           variable
        variable            decreased


                 Slide - 15              NEURO IMAGING IN MOVEMENT DISORDERS
Wilson’s Disease
    Type I
          Putamen, GP, thalamus
          Caudate, claustrum, subthalamus
          Red nucleus, substantia nigra
          Dentate

    Type II
          Globus  pallidus
          Rarely putamen, caudate

    Type III
          Basalganglia
          Thalamus
          Dentate nucleus
          Superior colliculus


                    Slide - 16               NEURO IMAGING IN MOVEMENT DISORDERS
Metabolic
    Metabolic disorders: Gray Matter
       Huntington’s disease
       Hepatocerebral disorders
       Mitochondrial cytompathies
       CO, CO3 poisoning
       Striatonigral degeneration
       Pyruvate dehydrogenase deficiency
       Hallervorden-Spatz disease


                 Slide - 17                NEURO IMAGING IN MOVEMENT DISORDERS
Wernicke-Korsakoff Syndrome
    MR
       Abnormal signal in mamillary bodies
       Periventricular thalamus
       Periaqueductal gray
       + enhancement




                 Slide - 18                  NEURO IMAGING IN MOVEMENT DISORDERS
PRES: Posterior Reversible Encephalopathy
Syndrome
    Imaging
       Subcortical involvement
       Basal ganglia, brainstem unusual
       Cerebellar involvement occasional
       Enhancement/hemorrhage unusual




                 Slide - 19                NEURO IMAGING IN MOVEMENT DISORDERS
Hemochromatosis
   MR
      hypointensity of basal ganglia, thalami
      central white matter
      adenohypophysis
      choroids plexus




                 Slide - 20                NEURO IMAGING IN MOVEMENT DISORDERS
Degenerative Disorders
    Diseases of the Substantia Nigra
        Parkinson’s disease
        Progressive supranuclear palsy
        Straitonigral degeneration
        Hallervorden – Spatz disease




                 Slide - 21              NEURO IMAGING IN MOVEMENT DISORDERS
Parkinson’s Disease
    MR
       Diminished width of pars compacta
       Normal putamina
    MR-guided Pharmacotherapy
       Intracerebroventricular drug delivery
       Parenchymal drug delivery
       Huge population of new patients with neurodegenerative
       disorders potentially amenable to targeted
       pharmacotherapy


                 Slide - 22               NEURO IMAGING IN MOVEMENT DISORDERS
Parkinson’s Disease
    Progressive Supranuclear Palsy
    Pathology
        Atrophy of mesencephalon
        Periaqueductal gliosis and neurofibrillary tangles
    MR
        Subtle enlargement of aqueduct
        Subtle atrophy of periaqueductal gray




                  Slide - 23                 NEURO IMAGING IN MOVEMENT DISORDERS
Parkinson’s Disease
    MR
       Diminished width of pars compacta
       Hypointense dorsolateral putamina
    Hallervorden – Spatz Disease
       Gradual limb stiffness
       Dysarthria, dementia
       50% exhibit choreiform or athetosis
       marked symmetric T2-shortening
          globipallidi
          + pars reticularis

       eye of the tiger appearance

                   Slide - 24                NEURO IMAGING IN MOVEMENT DISORDERS
Differentiation
        Signal Hypointensity T2 <Putamen>
                 Parkinson’s Plus (Multiple System Atrophy)
                 Hypothyroidism
        Signal Hyperintensity T2 <Putamen>
                 Wilson’s
                 Familial Striatal Degeneration
                 Creutzfeldt jakob
                 Anoxia
                 Leigh’s
                 Ophthalmoplegia Plus
                 Pallidoluysian Degeneration
        Signal Hyperintensity T1 <Globus Pallidus>
                 Hepatic insufficiency
                 Anoxia (petechial hemorrhage)



                     Slide - 25                            NEURO IMAGING IN MOVEMENT DISORDERS

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Neuro imaging in movement disorders

  • 1. NEURO IMAGING IN MOVEMENT DISORDERS DR. A.V. SRINIVASAN
  • 2. Clinical Syndrome Approach 2.1. WHAT ARE THE MOVEMENTS AKINETIC OR DYSKINETIC TREMOR JERKS Myclonus Chorea Tic SPASMS Dystonia Rhythmic / arhythmic Stereo typed / in consistant Continous Action Paroxysms Slide - 2 NEURO IMAGING IN MOVEMENT DISORDERS
  • 3. Clinical Syndrome Approach  Akinetic rigid syndrome  Dystonic syndrome  Choreic syndrome  Tic syndrome  Myoclonic syndrome Slide - 3 NEURO IMAGING IN MOVEMENT DISORDERS
  • 4. ODD DYSKINESIAS  ODDTREMOR  ODD JERKS FOCAL MYOCLONUS CORTICAL MYOCLONUS  GIANT SOMATO SENSORY  HYPEREXPLEXIA  ODD SPASMS Slide - 4 NEURO IMAGING IN MOVEMENT DISORDERS
  • 5. Brain Iron  Iron is a key trace metal relating to brain function. The major functions in which iron performs a role include: Oxidative phosphorylation Dopamine synthesis and degradation Hydroxyl free-radical formation Slide - 5 NEURO IMAGING IN MOVEMENT DISORDERS
  • 6. Brain Iron A. Neurodegenerative i. Parkinson’s Plus Putamen a. Striatonigral Degeneration b. Shy-Drager c. Olivopontocerebellar Atrophy ii. Hallervorden-Spatz Globus Pallidus (+ Red Nucleus / Nigra) iii.Alzheimer’s Cortical B. Metabolic i. Hypothyroidism Generalized ii. [Hepatic failure exhibits signal hyperintensity in the globus pallidus on T1 images Slide - 6 NEURO IMAGING IN MOVEMENT DISORDERS
  • 7. Brain Iron  C. Hemorrhage Intracerebral Hematoma Site of Bleed Hemorrhagic Infarction Gyral Vascular Malformation Interstices / Margin Slide - 7 NEURO IMAGING IN MOVEMENT DISORDERS
  • 8. Brain Iron D. Demyelination i. Multiple Sclerosis (severe) Thalamus /Putamen ii. Microangiopathic Leukoencephalopathy Putamen iii. Radiation injury White Matter/ Putamen Slide - 8 NEURO IMAGING IN MOVEMENT DISORDERS
  • 9. INVESTIGATIONS FOR PRIMARY MOVEMENT DISORDERS  Imaging (MRI)  Exclusion of Wilson <50)  Genentic testing  Routine blood wing Biochemistry  Syphilis Slide - 9 NEURO IMAGING IN MOVEMENT DISORDERS
  • 10. Investigation in Secondary Movement Disorders  GENERAL PLAN Extent of nervous system involvement Psychometric evaluation EEG (epilepti form discharges) ENMG (peripheral neruropathy) EMG and VEP Slide - 10 NEURO IMAGING IN MOVEMENT DISORDERS
  • 11. Role of CT  CONDITIONS THAT MAY PRODUCE WHITE MATTER CHANGE ON BRAIN CT OR MRI AND WHICH MAY CAUSE MOVEMENT DISORDERS. Alexander’s disease Krabbe’s disease Polycystic lipomembranous osteodyplasia Adeno – myelo – Leukodystrophy Metachromatic leukodystrophy Mitrochondrial diorders. Slide - 11 NEURO IMAGING IN MOVEMENT DISORDERS
  • 12. DEGENERATIVE AND SYSTEMIC DISORDERS  Polyeystic lipomembraneous Hand X-rays, skin biopsy  Degeneration  Hallervorden Spatz disease Brain MRI  Pallidal degenerations Brain MRI  Alzheimer’s disease Brain MRI  Multi – infarct state Brain MRI  Binswanger’s subcortical arteriosclerotic Brain MRI Encephalopathy  Normal pressure hydrocephalus Brain MRI  Dementia pugilistica Brain MRI  Calcification of the basal ganglia Brain CT and MRI  Cerebral anoxia (Co and cyanide poisoning) Brain MRI Slide - 12 NEURO IMAGING IN MOVEMENT DISORDERS
  • 13. Wilson’s Disease  3 movement disorders Akinetic-rigid syndrome resembling Parkinsonism Dystonic syndrome Postural and intentional tremor  Clinical Tremor, rigidity, gait difficulty Bulbar signs: indistinct speech, dysphagia Hepatic encephalopathy Anemia (10%) Slide - 13 NEURO IMAGING IN MOVEMENT DISORDERS
  • 14. Wilson’s Disease  Dx  Kayser-Fleischer ring  Elevated urinary copper  Decreased serum ceruloplasmin  Elevated bepatic copper  CT DDX  Straiatonigraldegeneration  Metabolic acidosis  Leigh disease  2o parkinsonism  acquired hepatocerebral degeneration  myeliolysis  HIE Slide - 14 NEURO IMAGING IN MOVEMENT DISORDERS
  • 15. Wilson’s Disease  MR Altered GM and WM signal intensities Atrophy of caudate head, brain stem Cerebral and cerebellar atrophy  MR: 3 patterns T1 PD/T2 Variable increased increased variable variable decreased Slide - 15 NEURO IMAGING IN MOVEMENT DISORDERS
  • 16. Wilson’s Disease  Type I  Putamen, GP, thalamus  Caudate, claustrum, subthalamus  Red nucleus, substantia nigra  Dentate  Type II  Globus pallidus  Rarely putamen, caudate  Type III  Basalganglia  Thalamus  Dentate nucleus  Superior colliculus Slide - 16 NEURO IMAGING IN MOVEMENT DISORDERS
  • 17. Metabolic  Metabolic disorders: Gray Matter Huntington’s disease Hepatocerebral disorders Mitochondrial cytompathies CO, CO3 poisoning Striatonigral degeneration Pyruvate dehydrogenase deficiency Hallervorden-Spatz disease Slide - 17 NEURO IMAGING IN MOVEMENT DISORDERS
  • 18. Wernicke-Korsakoff Syndrome  MR Abnormal signal in mamillary bodies Periventricular thalamus Periaqueductal gray + enhancement Slide - 18 NEURO IMAGING IN MOVEMENT DISORDERS
  • 19. PRES: Posterior Reversible Encephalopathy Syndrome  Imaging Subcortical involvement Basal ganglia, brainstem unusual Cerebellar involvement occasional Enhancement/hemorrhage unusual Slide - 19 NEURO IMAGING IN MOVEMENT DISORDERS
  • 20. Hemochromatosis  MR hypointensity of basal ganglia, thalami central white matter adenohypophysis choroids plexus Slide - 20 NEURO IMAGING IN MOVEMENT DISORDERS
  • 21. Degenerative Disorders  Diseases of the Substantia Nigra Parkinson’s disease Progressive supranuclear palsy Straitonigral degeneration Hallervorden – Spatz disease Slide - 21 NEURO IMAGING IN MOVEMENT DISORDERS
  • 22. Parkinson’s Disease  MR Diminished width of pars compacta Normal putamina  MR-guided Pharmacotherapy Intracerebroventricular drug delivery Parenchymal drug delivery Huge population of new patients with neurodegenerative disorders potentially amenable to targeted pharmacotherapy Slide - 22 NEURO IMAGING IN MOVEMENT DISORDERS
  • 23. Parkinson’s Disease  Progressive Supranuclear Palsy  Pathology Atrophy of mesencephalon Periaqueductal gliosis and neurofibrillary tangles  MR Subtle enlargement of aqueduct Subtle atrophy of periaqueductal gray Slide - 23 NEURO IMAGING IN MOVEMENT DISORDERS
  • 24. Parkinson’s Disease  MR Diminished width of pars compacta Hypointense dorsolateral putamina  Hallervorden – Spatz Disease Gradual limb stiffness Dysarthria, dementia 50% exhibit choreiform or athetosis marked symmetric T2-shortening  globipallidi  + pars reticularis eye of the tiger appearance Slide - 24 NEURO IMAGING IN MOVEMENT DISORDERS
  • 25. Differentiation  Signal Hypointensity T2 <Putamen>  Parkinson’s Plus (Multiple System Atrophy)  Hypothyroidism  Signal Hyperintensity T2 <Putamen>  Wilson’s  Familial Striatal Degeneration  Creutzfeldt jakob  Anoxia  Leigh’s  Ophthalmoplegia Plus  Pallidoluysian Degeneration  Signal Hyperintensity T1 <Globus Pallidus>  Hepatic insufficiency  Anoxia (petechial hemorrhage) Slide - 25 NEURO IMAGING IN MOVEMENT DISORDERS