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DIFFERENTIAL DIAGNOSIS
FOR BILATERAL ABNORMALITIES
OF THE BASAL GANGLIA
AND THALAMUS
Dr Roshan Valentine
PG Resident
St Jo...
INTRODUCTION
• Abnormalities of basal ganglia seen in various conditions.
• MR – IOC
• CT - emergency situations – altered...
ANATOMY
• Deep gray matter include paired BG
and thalamus
• Here we restrict to abnormalities of
lentiform nucleus and cau...
ANATOMY
• LFN is rich in mitochondria , vascular supply , neurotransmitters –
high metabolic activity and oxygen uptake - ...
ANATOMY
BLOOD SUPPLY
• BG : Medial and lateral
lenticulostriate arteries
• Thalamus : PCA and PCOM
• Venous drainage : dee...
TOXIC POISONING
• CAUSE : MC - CO , methanol and CN
• Impairs mitochondrial enzyme in electron transport chain .
• CLINICA...
TOXIC POISONING
IMAGING FINDINGS
• CO – GP(MC) : T1 & T2 hyperintense + DWI RD , Delayed
leukoencephalopathy
• CN , Meth :...
LIVER DISEASE
• CAUSATIVE AGENT : Due to nitrogenous waste crossing BBB
• CLINICAL FEATURES : cirrhosis with portal htn /i...
NON KETOTIC HYPERGLYCEMIA
• CLINICAL FEATURES : poorly controlled diabetes with chorea ,
hemiballismus +/- altered mental ...
HYPOGLYCEMIA
• CLINICAL FEATURES : Diabetic pts, Seizures , focal neurological
deficits and coma.
• Extent of brain damage...
HYPOXIC ISCHEMIC ENCEPHALOPATHY
• CAUSE : Result of cardiac arrest /drowning/asphyxiation
IMAGING FINDINGS:
• Mild HIE : w...
HYPOXIC ISCHEMIC ENCEPHALOPATHY
• Earliest finding(after 2 hrs) : Increased SI of the affected areas on
DW
• T2W : hyperin...
LEIGH DISEASE
• Subacute necrotizing encephalopathy
• CAUSE : Disorder in ATP synthesis at ETC
• CLINICAL FEATURES : Centr...
WILSON DISEASE
• CAUSE : Accumulation of Cu due to ceruloplasmin deficiency.
• CLINICAL FEATURES : dysarthria, dystonia, t...
OSMOTIC MYELINOLYSIS
• CAUSE : electrolyte imbalance, chronically alcoholic pts, chronically
debilitated organ transplant ...
OSMOTIC MYELINOLYSIS
IMAGING FINDINGS:
• MRI : T1 and T2 hyperintensity in affected areas
• Central pontine myelinolysis :...
WERNICKE ENCEPHALOPATHY
• CAUSE: Vit B1 def , Chronic alcoholics , GI or hemat neoplasm ,
chronic dialysis , prolonged TPN...
NEURODEGENERATION WITH BRAIN IRON
ACCUMULATION (NBIA)
• Heterogenous group of disorder with brain degeneration and
excessi...
Creutzfeldt –jakob Disease
• Transmissible fatal neurodegenerative disorder caused by prions
• CLINICAL FEATURES : rapidly...
FAHR DISEASE
• Bilateral striopallidodentate calcinosis
• B/L symmetric deposition of Ca and other minerals in BG ,Thalamu...
DEEP CVT
• CAUSE : hypercoagulable states , OCPS , vasculitis ,
intracranial / systemic infections
IMAGING FINDINGS:
• Sup...
ARTERIAL OCCLUSION
• CLINICAL FEATURES : Agitation , obtundation , coma , memory
dysfunction and various ocular changes
IM...
Figure 19. Basilar artery occlusion in a 61-year-old
man with ocular signs and severe obtundation. (a)
Diffusionweighted M...
NEURO – BEHCET DISEASE
• Behcet syndrome : uveitis , oral ulcers and genital ulcers.
• CNS involvementin 4-49%
• CLINICAL ...
FLAVI VIRUS ENCEPHALITIS
• Eg : Japanese encephalitis, West nile fever , Murray valley fever
IMAGING FINDINGS:
• Symmetric...
CEREBRAL TOXOPLASMOSIS
• CAUSE : Toxoplasma gondii typically in imuncompromised pts
MAGING FINDINGS:
• Multiple focal lesi...
PRIMARY CNS LYMPHOMA
• CAUSE : immunocompetent and immunocompromised
IMAGING FINDINGS:
• T2 hypointensity and high attenua...
PRIMARY BILATERAL THALAMIC GLIOMA
• Thalamus is affected in 1-1.5% of brain tumors / contiguous spread
from pineal germ ce...
NEUROFIBROMATOSIS TYPE I
• MC neurocutaneous syndrome
• CLINICAL FEATURES : café-au-lait spots, axillary freckling, Lisch
...
IMAGING
LAB IXCL.HISTORY
DX
LAB EVALUATION
Immunoassay Toxoplasmosis and Flavivirus
Eeg + CSF analysis CJD
Vit B1 assay Wernickes
Serum glucose Hypogl...
IMAGING
LAB IXCL.HISTORY
DX
• CLINICAL HISTORY
• Suicide attempt
• cardiac arrest
• diabetic hypoglycemia
• hyperglycemia
• Hiv – AIDS
• Vit deficienc...
IMAGING
LAB IXCL.HISTORY
DX
IMAGING FINDINGS
B/L symmetric LN andCN Systemic /metabolic
Asymmetric focal/Discrete Infection/neoplasm
Thalamus+ BG Hypo...
ASSOCIATED ABNORMALITIES
Diffuse or focal cortical involvement Hypoxia , hypoglycemia and CJD
Diffuse and bilateral white ...
ASSOCIATED ABNORMALITIES
T2W HYPERINTENSITY Acute diseases of deep grey
matter nuclei
T1W HYPERINTENSITY Hepatic dis , Mn ...
Bilateral basal ganglia abnormalities - MRI
Bilateral basal ganglia abnormalities - MRI
Bilateral basal ganglia abnormalities - MRI
Bilateral basal ganglia abnormalities - MRI
Bilateral basal ganglia abnormalities - MRI
Bilateral basal ganglia abnormalities - MRI
Bilateral basal ganglia abnormalities - MRI
Bilateral basal ganglia abnormalities - MRI
Bilateral basal ganglia abnormalities - MRI
Bilateral basal ganglia abnormalities - MRI
Bilateral basal ganglia abnormalities - MRI
Bilateral basal ganglia abnormalities - MRI
Bilateral basal ganglia abnormalities - MRI
Bilateral basal ganglia abnormalities - MRI
Bilateral basal ganglia abnormalities - MRI
Bilateral basal ganglia abnormalities - MRI
Bilateral basal ganglia abnormalities - MRI
Bilateral basal ganglia abnormalities - MRI
Bilateral basal ganglia abnormalities - MRI
Bilateral basal ganglia abnormalities - MRI
Bilateral basal ganglia abnormalities - MRI
Bilateral basal ganglia abnormalities - MRI
Bilateral basal ganglia abnormalities - MRI
Bilateral basal ganglia abnormalities - MRI
Bilateral basal ganglia abnormalities - MRI
Bilateral basal ganglia abnormalities - MRI
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Bilateral basal ganglia abnormalities - MRI

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Bilateral basal ganglia abnormalities , mri , co poisoning , leighs disease , liver cirrhosis , HIE

Published in: Health & Medicine
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Bilateral basal ganglia abnormalities - MRI

  1. 1. DIFFERENTIAL DIAGNOSIS FOR BILATERAL ABNORMALITIES OF THE BASAL GANGLIA AND THALAMUS Dr Roshan Valentine PG Resident St Johns Medical College
  2. 2. INTRODUCTION • Abnormalities of basal ganglia seen in various conditions. • MR – IOC • CT - emergency situations – altered sensorium / acute seizures. • In this article , we review the MRI anatomy of basal ganglia and pathology conditions of these brain structures
  3. 3. ANATOMY • Deep gray matter include paired BG and thalamus • Here we restrict to abnormalities of lentiform nucleus and caudate nucleus • Lentiform N : GP(D) + Putamen(I) • Caudate N (I) • Basal ganglia involved in production of movement and in memory , cognition and emotion.
  4. 4. ANATOMY • LFN is rich in mitochondria , vascular supply , neurotransmitters – high metabolic activity and oxygen uptake - vulnerable to metabolic abnormalities • Thalamus is paired structure on either side of third ventricles. • Responsible for relaying sensory and motor signals to and from the cerebral cortex • Disorders of thalamus affects consciousness and abnormalities of sensation
  5. 5. ANATOMY BLOOD SUPPLY • BG : Medial and lateral lenticulostriate arteries • Thalamus : PCA and PCOM • Venous drainage : deep venous system ; internal cerebral veins - basal vein of Rosenthal - great vein of galen
  6. 6. TOXIC POISONING • CAUSE : MC - CO , methanol and CN • Impairs mitochondrial enzyme in electron transport chain . • CLINICAL FEATURES : Acute cognitive impairment /coma , optic neuritis (methanol) DIAGNOSIS • Toxicology and Lab tests , imaging ( assess brain damage)
  7. 7. TOXIC POISONING IMAGING FINDINGS • CO – GP(MC) : T1 & T2 hyperintense + DWI RD , Delayed leukoencephalopathy • CN , Meth : Hemorrhagic necrosis of putamen • Meth : White matter edema
  8. 8. LIVER DISEASE • CAUSATIVE AGENT : Due to nitrogenous waste crossing BBB • CLINICAL FEATURES : cirrhosis with portal htn /iatrogenic (TIPSS) • IMAGING FINDINGS : - GP and SN : hyper on T1( due to Mn deposn) - Reversible post transplantation. - Acute hyperammonemia : bilaterally symmetric swelling, T2 prolongation restricted diffusion in the basal ganglia, insular cortex, and cingulate gyrus - MRS : Detection of glutamate-glutamine
  9. 9. NON KETOTIC HYPERGLYCEMIA • CLINICAL FEATURES : poorly controlled diabetes with chorea , hemiballismus +/- altered mental status. • Treatable condition which shows resolution of findings when performed 2-12 months later IMAGING FINDINGS ⁻ CT: B/L or rarely U/L hyperattenuation of GP or CN ⁻ MR : hyperintensity on T1 and variable intensity in T2
  10. 10. HYPOGLYCEMIA • CLINICAL FEATURES : Diabetic pts, Seizures , focal neurological deficits and coma. • Extent of brain damage depends on severity and duration of hypoglycemia. IMAGING FINDINGS: • T2 hyperintensity in cerebral cortex , hippocampi and BG • Mild reversible hypoglycemia – transient and isolated WMI with true diff restriction involving splenium , internal capsule and corona radiata. • BG INVOLVEMENT : POOR PX
  11. 11. HYPOXIC ISCHEMIC ENCEPHALOPATHY • CAUSE : Result of cardiac arrest /drowning/asphyxiation IMAGING FINDINGS: • Mild HIE : water shed zones • Severe HIE : grey matter structures like cerebral cortex , BG and hippocampi. • Brainstem and WM are typically spared. • CT : Diffuse edema , decreased attenuation of the cortical gray matter with loss of normal gray matter–white matter differentiation, BG and Thalamus • WHITE CEREBELLUM SIGN : diffuse cerebral damage results in lower attn. of cerebral parenchyma , compared to cerebellum and BG which are spared – POOR PX
  12. 12. HYPOXIC ISCHEMIC ENCEPHALOPATHY • Earliest finding(after 2 hrs) : Increased SI of the affected areas on DW • T2W : hyperintensity and swelling of affected areas ( after 24 hrs) • Delayed : T2 hyperintensity in subcortical WM
  13. 13. LEIGH DISEASE • Subacute necrotizing encephalopathy • CAUSE : Disorder in ATP synthesis at ETC • CLINICAL FEATURES : Central hypotonia , developmental regression/arrest , ophthalmoplegia , resp and bulbar dysfunction and ataxia. IMAGING FINDINGS: • T2 hyperintensity in BG , periaquedeuctal region , cerebral peduncles and putamen. • MRS : High lactate levels in BG DIAGNOSIS : Imaging + Elevated serum and CSF lactate levels.
  14. 14. WILSON DISEASE • CAUSE : Accumulation of Cu due to ceruloplasmin deficiency. • CLINICAL FEATURES : dysarthria, dystonia, tremors, ataxia, Parkinsonian symptoms, and psychiatric problems. IMAGING FINDINGS: • MR : T2 hyperintensity in Putamen(MC) • GP , caudate nucleus , thalamus • Less common : cortical and subcortical region , mesencephalon , pons , vermis and dentate nuclei • DWI restriction in early stages
  15. 15. OSMOTIC MYELINOLYSIS • CAUSE : electrolyte imbalance, chronically alcoholic pts, chronically debilitated organ transplant pts , rapid overcorrection of hyponatremia. • Oligodendroglial cells are more susceptible to osmotic stresses.
  16. 16. OSMOTIC MYELINOLYSIS IMAGING FINDINGS: • MRI : T1 and T2 hyperintensity in affected areas • Central pontine myelinolysis : Symmetric trident shaped / bat wing shaped T2 /FLAIR hyperintensity in central pons • Ventrolateral pons and pontine portion of CST are spared • Extrapontine myelinolysis : T2 hyperintensity in GP , putamen , thalamus and cerebellum. • DWI R in early stages – not typical though DIAGNOSIS : Imaging + serial Na measurement
  17. 17. WERNICKE ENCEPHALOPATHY • CAUSE: Vit B1 def , Chronic alcoholics , GI or hemat neoplasm , chronic dialysis , prolonged TPN without vit supplementation. • CLINICAL FEATURES : altered consciousness, ocular dysfunction, and ataxia IMAGING FINDINGS: • MRI : Symmetric T2 hyperintensity in medial thalamus , periaqueductal area , mammillary body and tectal plate. • Petechial hemorrhage , diffusion restriction and contrast enhancement of affected areas
  18. 18. NEURODEGENERATION WITH BRAIN IRON ACCUMULATION (NBIA) • Heterogenous group of disorder with brain degeneration and excessive iron deposition in basal ganglia(PAN K 2 gene mutation.) • 2 types • Classic early onset , rapidly progressive(halloverden spatz) • Atypical late onset and slowly progressive • CLINICAL FEATURES : pyramidal or extrapyramidal signs, dystonia, and dysarthria IMAGING FINDINGS: • MR : B/L T2 hypointensity in GP with high signal intensity center - EYE OF THE TIGER appearance
  19. 19. Creutzfeldt –jakob Disease • Transmissible fatal neurodegenerative disorder caused by prions • CLINICAL FEATURES : rapidly progressive dementia, myoclonus, and multifocal neurologic dysfunction. • DIAGNOSIS: Brain Biopsy /autopsy, periodic charp wave complexes at EEG IMAGING FINDINGS: • MRI : DWI R of cerebral cortex and basal ganglia • Variant CJD : Bovine spongiform encephalopathy • B/L lesions in pulvinar nuclei of thalamus(pulvinar sign/hockey stick sign)
  20. 20. FAHR DISEASE • Bilateral striopallidodentate calcinosis • B/L symmetric deposition of Ca and other minerals in BG ,Thalamus , Dentate nuclei and centrum semiovale in absence of HYPOPARATHYROIDISM. • CLINICAL FEATURES : Headache , vertigo , movt disorders , syncope , seizures , coma, dementia , parkinsonism , chorea, dystonia etc. IMAGING FINDINGS: • MRI/CT : B/l symmetric dense calcifications in BG , dentate nuclei , thalamus , subcortical white matter .
  21. 21. DEEP CVT • CAUSE : hypercoagulable states , OCPS , vasculitis , intracranial / systemic infections IMAGING FINDINGS: • Superficial CVT: cerebral edema and venous infraction of cerebral cortex near vertex. • Deep CVT: Venous HTN , b/L involvement of thalamus and basal ganglia. • MRI/CT: Venous HTN and cerebral edema results in T2 hyperintensity in thalamus , BG , internal capsule and DWM • Hemorhhagic transformation common • MR venogram : evaluation of thrombus
  22. 22. ARTERIAL OCCLUSION • CLINICAL FEATURES : Agitation , obtundation , coma , memory dysfunction and various ocular changes IMAGING FINDINGS: • Acute infarcts : T2 hyperintensity and DWI R with occlusion seen on MRA based on the artery occluded. • Artery of Percheron infarct : B/l symmetric paramedian parts of thalamus and midbrain on both sides.
  23. 23. Figure 19. Basilar artery occlusion in a 61-year-old man with ocular signs and severe obtundation. (a) Diffusionweighted MR image shows bilateral hyperintense areas in the paramedian thalamus (arrows). (b) Timeof- flight MR angiogram clearly depicts occlusion of the rostral portion of the basilar artery (arrow). (c, d) Noncontrast CT scans obtained 3 days later show bilateral subacute infarcts of the thalamus (arrows in c) and an infarct in the right cerebellar hemisphere (arrow in d).
  24. 24. NEURO – BEHCET DISEASE • Behcet syndrome : uveitis , oral ulcers and genital ulcers. • CNS involvementin 4-49% • CLINICAL FEATURES : Headache , dysarthria , cerebellar signs , sensory signs and personality change. IMAGING FINDINGS: • T2 hyperintense and T1 hypointense and CE with vasogenic edema at brainstem, basal ganglia (bilateral involvement in one-third of cases), and thalamus , WM(LC)
  25. 25. FLAVI VIRUS ENCEPHALITIS • Eg : Japanese encephalitis, West nile fever , Murray valley fever IMAGING FINDINGS: • Symmetric involvement of deep grey matter JE : T2 hyperintensity in B/L posteromedial thalamus • Intralesional hemorrhages + DWI R • Less common sites : basal ganglia, substantia nigra, red nucleus, pons, hippocampi, cerebral cortex, and cerebellum. • JE and Murray valley fever involve THALAMUS • West nile fever : B/L involvement of thalamus , caudate and lentiform nucleus
  26. 26. CEREBRAL TOXOPLASMOSIS • CAUSE : Toxoplasma gondii typically in imuncompromised pts MAGING FINDINGS: • Multiple focal lesions in the basal ganglia and lobar gray matter– white matter junctions • T2 W : hypo to isointense lesions with prominent mass effect and vasogenic edema +/- hemorrhagic lesions • Post Contrast : nodular/ ring enhancement • MRS : increased lipid levels without elevated choline levels
  27. 27. PRIMARY CNS LYMPHOMA • CAUSE : immunocompetent and immunocompromised IMAGING FINDINGS: • T2 hypointensity and high attenuation in CT involves deep hemispheric periventricular white matter, corpus callosum, and basal ganglia • MRS : Elevated choline levels • Immunocompetent : Solid, homogeneously enhancing lesions • Immunocompromised : Ring enhancement and central necrosis
  28. 28. PRIMARY BILATERAL THALAMIC GLIOMA • Thalamus is affected in 1-1.5% of brain tumors / contiguous spread from pineal germ cell tumors. • CLINICAL FEATURES : behavioral impairment ranging from personality changes to dementia. • IMAGING : T2 hyperintense and T1 isointense B/L symmetric mass with no CE • Low grade tumors characterized by absence of tumor progression on serial MR images,
  29. 29. NEUROFIBROMATOSIS TYPE I • MC neurocutaneous syndrome • CLINICAL FEATURES : café-au-lait spots, axillary freckling, Lisch nodules, neurofibromas, plexiform neurofibromas, optic glioma, bone dysplasias, or pseudoarthrosis. IMAGING FINDINGS: Focal T2 hyperintense and TI hypointense in GP > brainstem and cerebellum • NO mass effect • No surrounding edema • No CE • MRS : high NAA-Ch , Naa-Cr and Cr- Cho ratios
  30. 30. IMAGING LAB IXCL.HISTORY DX
  31. 31. LAB EVALUATION Immunoassay Toxoplasmosis and Flavivirus Eeg + CSF analysis CJD Vit B1 assay Wernickes Serum glucose Hypoglycemia/Hyperglycemia Serum Ceruloplasmin Wilsons disease Serum and CSF Lactate Leigh Disease Serum Ca , P and PTH Fahrs DIsease
  32. 32. IMAGING LAB IXCL.HISTORY DX
  33. 33. • CLINICAL HISTORY • Suicide attempt • cardiac arrest • diabetic hypoglycemia • hyperglycemia • Hiv – AIDS • Vit deficiency • Electrolyte imbalance
  34. 34. IMAGING LAB IXCL.HISTORY DX
  35. 35. IMAGING FINDINGS B/L symmetric LN andCN Systemic /metabolic Asymmetric focal/Discrete Infection/neoplasm Thalamus+ BG Hypoxia , osmotic myelinolysis , wilsons disease , Leighs disease , Fahrs disease , CJD , deep CVT, Infection , Primary CNS lymphoma BG w/o Th Systemic disease ( toxic poisoning , hypoglycemia , hyperglycemia , liver disease , huntingtons disease , NF) B/L Th w/o BG Focal ( arterial occlusion ,Flavivirus , infection )
  36. 36. ASSOCIATED ABNORMALITIES Diffuse or focal cortical involvement Hypoxia , hypoglycemia and CJD Diffuse and bilateral white matter abnormality poisoning , hypoglycemia Brainstem Leigh disease , Myelinolysis , Neuro behcets disease , Basilar artery occlusion Perilesional edema + infiltration outside BG + Th CNS infection + tumors
  37. 37. ASSOCIATED ABNORMALITIES T2W HYPERINTENSITY Acute diseases of deep grey matter nuclei T1W HYPERINTENSITY Hepatic dis , Mn deposition , Hyperglycemia , NF 1 CT Ca – Fahrs disease, Hypoparathyroidism H’age – Poisoning, CNs toxoplasmosis, Venous infarction , JE DWI Acute cytotoxic brain damage in acute infarction, hypoxia, hypoglycemia, CJD, and Wernicke encephalopathy MRS lactate in hypoxia or mitochondrial disease

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