Sporadic CJD: Clinical features

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Sporadic CJD Clinical features
Dementia Ataxia Myoclonus Multifocal neurological
signs Akinetic mutism Median duration 4.5 months
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Sporadic CJD psychiatric features
Saw psychiatrist initially 16 (36) Admitted to
psychiatry 17 Depression (meds) 25
(22) Delusions 24 Visual hallucinations 41
Auditory hallucinations 2
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CJD
Spongiosis/neuronal loss Prion
protein staining Astrocytosis
Control
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Sporadic CJD Epidemiology

• 1 case/million a year
• Worldwide
• Risk factors

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CJD subtypes
Sporadic 85 Familial 10-15 Iatrogenic
lt5 Variant
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Iatrogenic CJD
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Chemicals and processes ineffective against TSE
agents
Chemical disinfectants Gaseous disinfectants Physi
cal processes Alcohol Ethylene
oxide Boiling Ammonia Formaldehyde Dry heat
(600C) ß-propiolactone Ionising
radiation Formalin UV radiation Hydrochloric
acid Microwave radiation Hydrogen
peroxide Peracetic acid Phenolics SDS (5)
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Whats the infectious agent

• Slow virus ?
• Virino ?
• Prion ?

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PrPC
PrPSc
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Variant CJD Clinical features
Psychiatric presentation Painful sensory
symptoms Dementia Ataxia Myoclonus/chorea/dyston
ia Multifocal neurological signs Upgaze
paresis Median duration 14 months
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Variant CJD differential diagnosis
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Single cases Hong Kong USA Canada Italy Japan The
Netherlands Saudi Arabia Spain Portugal
161
UK
14
France
4
Republic of Ireland
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Variant CJD Epidemiology
Geography Food consumption Operations/vaccinations
Age Other risk factors
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Codon 129 status
MM
MV
VV
Sporadic CJD () n121
Controls ()
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Variant CJD the future?
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Diagnostic tests for CJD

• EEG
• CSF
• Imaging
• Tonsil biopsy

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CSF proteins in CJD

• Increased protein
• Oligoclonal bands?
• Cell counts
• Brain-specific proteins

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CSF brain-specific protein results in SpCJD
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WHO diagnostic criteria for sporadic CJD

• Progressive dementia
• At least 2 out of the following
• myoclonus
• visual or cerebellar
• pyramidal/extrapyramidal
• akinetic mutism
• Typical EEG (any clinical duration) and/or
• Positive 14-3-3 (total duration to death lt2
  years)
• (Routine investigations normal)

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T2-weighted MRI
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Proton density-weighted MRI
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FLAIR MRI
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Diffusion-weighted MRI
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T2-weighted MRI
Sporadic CJD
Sporadic CJD
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T2-weighted MRI
Normal
Variant CJD
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Proton density-weighted MRI
Normal
Variant CJD
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FLAIR MRI
Normal
Variant CJD
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