Abnormalities of the basal ganglia and thalami

1
Abnormalities of the basal ganglia and thalami

• S.Alj, M.Ouali Idrissi, N. Cherif El Idrissi El
  Ganouni, O.Essadki, A.OusehalRadiology
  department, Ibn Tofail Hospital , Cadi Ayyad
  University, Marrakech.

NR4
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Introduction

• Several diseases may cause MR signal
  abnormalities of the basal ganglia and thalami.
• Signal abnormalities are usually non specific.
• Analysis of the clinical manifestations , type of
  signal, location of the lesions and associated
  abnormalities can help to achieve the correct
  diagnosis.

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Methods and patients

• The study included patients with signal
  abormalities of basal ganglia and thalami in MRI.
  
• MRI technique
• -MRI 1,5 Tesla.
• -with T1, T2 , T2 ,Flair and diffusion sequences
  .

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Results

• 1-Epidemiological features
• Thirteen patients were included in the study .
• There was seven women and 6 men.
• Patients were aged between 8 and 70 years (mean
  age 27,15).

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Results

• 2-Clinical manifestations

symptoms Number
seizure 5
decreasing of consciousness 4
stroke 3
Visual impairment 1
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Results

• 3-Imaging features
• 3.1-Location of the signal abnormalities
• 3.2-Associated abnormalities
• White matter abnormalities were found in 4
  patients

Abnormalities location Case number
Basal ganglia 10
Nucleus dentatus 5
Thalami 2
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Results

• 3.3-etiology

Etiology Number of cases
Fahrs disease 4
Neurolupus 2
Wilson disease 1
Multiple sclerosis 1
Behcet disease 1
Creusfeldt Jacob disease 1
Hypoglycemia 1
No etiology found 2
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Figure 1 Fahrs disease Calcifications of the
basal ganglia and thalami on CT Bilateral and
symetric hyperintensity of the basal ganglia and
thalami on T1 WI
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Fahrs disease

• Comment
• Rare clinical entity
• characterized by bilateral calcifications of the
  basal ganglia, thalami, dentate nuclei of the
  cerebellum, and the white matter of the cerebral
  hemisphere
• Clinical manifestations characterized by
  movement disorders, dementia and behavioral
  disorders
• Computed tomography calcifications are visible
  as high-density areas.
• Magnetic resonance image the calcifications have
  different signal intensities. Its probably
  related to the stage of the disease, and the
  volume of the calcium deposit.

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Figure 2 Wilson disease. T2-weighted MR image
depicts bilaterally symmetric areas of abnormal
T2 hypersignal in the thalamus, putamina and
caudate nuclei.
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Wilson disease

• Comment
• caused by the accumulation of copper resulting
  from a deficiency of ceruloplasmin.
• Clinical manifestations dysarthria, tremors,
  ataxia, Parkinsonian symptoms, and psychiatric
  problems. The Kayser-Fleisher rings in the cornea
  is characteristic when found.
• MRI
• - areas of T2 hyperintensity in the putamen (a
  common finding),
• -Other locations of the signal abnormalities
  globus pallidus, caudate nuclei, and thalamus
  (ventrolateral aspect ).
• -The cortical and subcortical regions,
  mesencephalon, pons, vermis, and dentate nuclei
  may also be involved.

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Figure 3 Creutzfeldt Jacob disease 30 years aged
patient with rapidely progressive demantia.
Bilateral hyperintensities on FLAIR WI of
cerebral cortex and basal ganglia .
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Creutzfeldt jacob disease(CJD)

• Comment
• fatal neurodegenerative disorder caused by prions
• Four main subtypes sporadic, familial,
  iatrogenic, and variant CJD
• Clinical manifestations rapidly progressive
  dementia, myoclonus.
• Electroencephalography Characteristic periodic
  sharp-wave complexes.
• MRI of sporadic CJD
• diffusion-weighted MR imaging increasingly
  important for the diagnosis
• Bilateral restricted diffusion of cerebral cortex
  and basal ganglia .

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Figure 4Hypoglycemia 70 years aged patient type
2 diabetic . Bilateral FLAIR hypersignal in the
temporal and occipital cerebral cortex, and basal
ganglia.
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Hypoglycemia

• Comment
• Brain damage is dependent on the severity and
  duration of hypoglycaemia
• Clinical manifestation spatients with severe
  hypoglycemia have coma and are typically diabetic
  receiving treatment with oral hypoglycemic agents
• MRI
• -bilateral T2 prolongation in the cerebral
  cortex, hippocampi, and basal ganglia.
• -transient white matter abnormalities DW MR
  findings, involving the splenium of the corpus
  callosum, internal capsules and corona radiata
  have been reported in milder hypoglycemia

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Figure 4Neuro-Behçet Disease 40 years aged
patient with Behcet disease . Hyperintense
lesions of the left midbrain, cerebellar
hemispheres and the basal ganglia.
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Neuro-Behçet Disease

• multisystemic, recurrent inflammatory disorder of
  unknown cause
• Clinical manifestations triad of uveitis, oral
  ulcers, and genital with neurological ulcers
  manifestations (headache, dysarthria, cerebellar
  signs, sensory signs)
• MRI
• -lesions hyperintense on T2-WI , hypointense on
  T1-WI, enhance after contrast material
  administration
• -Involving the brainstem, basal ganglia
  (bilateral involvement in one-third of cases),
  and thalamus .
• -Less commonly, the white matter of the cerebral
  hemispheres and cervicothoracic spinal cord are
  involved.

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Other

• Comment
• Other inflammatory and demyelinating disease may
  present with basal ganglia and thalami lesions
  (Multiple sclerosis , systemic lupus with
  neurological manifestations..)
• Clinical manifestations variety of neurological
  deficits
• MRI (MS)
• -Gray matter T2-hypointensity,
• -suggestive of excessive iron deposition
• -associated with worsening disability in patients
  with MS

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Conclusion

• Signal abnormalities of basal ganglia are usually
  non specific.
• Clinical manifestations and the location of this
  signal abnormalities led to diagnosis.
• The etiologies vary widely corresponding
  generally to uncommon diseases (such Fahrs
  disease), or to a rare manifestation of some
  commun pathologies (such multiple sclerosis).

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References

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• 4-M Neema and al. Deep gray matter involvement on
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