NEURO IMAGING IN NEURO IMAGING IN MOVEMENT DISORDERSMOVEMENT DISORDERS

DR. A.V. SRINIVASANDR. A.V. SRINIVASAN

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Clinical Syndrome ApproachClinical Syndrome Approach 2.1. WHAT ARE THE MOVEMENTS

AKINETIC OR DYSKINETICTREMOR

JERKS MyclonusChoreaTic

SPASMS DystoniaRhythmic / arhythmicStereo typed / in consistantContinousActionParoxysms

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Clinical Syndrome ApproachClinical Syndrome Approach Akinetic rigid syndrome Dystonic syndrome Choreic syndrome Tic syndrome Myoclonic syndrome

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ODD DYSKINESIASODD DYSKINESIAS ODDTREMOR ODD JERKS

FOCAL MYOCLONUS CORTICAL MYOCLONUS

GIANT SOMATO SENSORY HYPEREXPLEXIA ODD SPASMS

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Brain IronBrain 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

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Brain IronBrain Iron

A. Neurodegenerativei. 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. Metabolici.   Hypothyroidism Generalizedii.  [Hepatic failure exhibits signal hyperintensity in the globus pallidus on T1 images

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Brain IronBrain Iron

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

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Brain IronBrain Iron

D. Demyelination i.    Multiple Sclerosis (severe) Thalamus /Putamen

ii.     Microangiopathic Leukoencephalopathy Putamen

iii.    Radiation injury White Matter/ Putamen

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INVESTIGATIONS FOR PRIMARY MOVEMENT INVESTIGATIONS FOR PRIMARY MOVEMENT DISORDERSDISORDERS

Imaging (MRI) Exclusion of Wilson <50) Genentic testing Routine blood wing Biochemistry Syphilis

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Investigation in Secondary Movement Investigation in Secondary Movement DisordersDisorders

GENERAL PLAN Extent of nervous system involvement Psychometric evaluation EEG (epilepti form discharges) ENMG (peripheral neruropathy) EMG and VEP

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Role of CTRole 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.

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DEGENERATIVE AND SYSTEMIC DISORDERSDEGENERATIVE 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

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Wilson’s DiseaseWilson’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%)

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Wilson’s DiseaseWilson’s Disease Dx

Kayser-Fleischer ring Elevated urinary copper Decreased serum ceruloplasmin Elevated bepatic copper

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

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Wilson’s DiseaseWilson’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 increasedincreased variablevariable decreased

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Type I Putamen, GP, thalamus Caudate, claustrum, subthalamus Red nucleus, substantia nigra Dentate

Type II Globus pallidus Rarely putamen, caudate

Type III Basal ganglia Thalamus Dentate nucleus Superior colliculus

Wilson’s DiseaseWilson’s Disease

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MetabolicMetabolic Metabolic disorders: Gray Matter

Huntington’s disease Hepatocerebral disorders Mitochondrial cytompathies CO, CO3 poisoning

Striatonigral degeneration Pyruvate dehydrogenase deficiency Hallervorden-Spatz disease

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Wernicke-Korsakoff SyndromeWernicke-Korsakoff Syndrome MR

Abnormal signal in mamillary bodies Periventricular thalamus Periaqueductal gray + enhancement

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PRES: Posterior Reversible Encephalopathy PRES: Posterior Reversible Encephalopathy SyndromeSyndrome

Imaging Subcortical involvement Basal ganglia, brainstem unusual Cerebellar involvement occasional Enhancement/hemorrhage unusual

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HemochromatosisHemochromatosis

MR hypointensity of basal ganglia, thalami central white matter adenohypophysis choroids plexus

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Degenerative DisordersDegenerative Disorders Diseases of the Substantia Nigra

Parkinson’s disease Progressive supranuclear palsy Straitonigral degeneration Hallervorden – Spatz disease

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Parkinson’s DiseaseParkinson’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

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Parkinson’s DiseaseParkinson’s Disease Progressive Supranuclear Palsy Pathology

Atrophy of mesencephalon Periaqueductal gliosis and neurofibrillary tangles

MR Subtle enlargement of aqueduct Subtle atrophy of periaqueductal gray

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Parkinson’s DiseaseParkinson’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

globi pallidi + pars reticularis

eye of the tiger appearance

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DifferentiationDifferentiation 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)