Updated: 11/6/2016

Spongiform Encephalopathy / Creutzfeldt-Jakob Disease

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Questions
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Evidence
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Overview
 
 

 
Snapshot
  • A 58-year-old male presents with difficulties in concentration and worsening insomnia. These symptoms have been progressively worsening over the past month. Startle myoclonus is appreciated on physical examination. Electroencephalography shows biphasic sharp wave patterns.
Introduction
  • Prions
    • misfolded intracellular proteins that lead to neurodegeneration
      • PrPc (normal; α-helix isoform) undergoes conformational change into PrPsc (abnormal; β-pleated sheet isoform)
        • PrPsc is resistant to intracellular proteases
          • can further induce PrPc into PrPsc → cytoplasmic vacuoles and neuronal death
  • Spongiform encephalopathy
    • Creutzfeld-Jakob disease (CJD)
      • most common
      • can be sporadic, familial or acquired
        • acquired via iatrogenic or dietary methods
Presentation
  • Symptoms
    • rapidly progressive dementia
      • occurs in weeks to months
    • myoclonus
      • startling the patient can induce myoclonus
    • ataxia
    • distrubances in vision
      • e.g., visual field defects, abnormalities in perception
Evaluation
  • Histology
    • spongiform cortex
      • large intracellular vacuoles 
  • Electroencephalography
    • spike (sharp) wave pattern
  • Cerebral spinal fluid (CSF)
    • 14-3-3 protein
  • Magnetic resonance imaging
    • hyperintensity in the head of the caudate and putamen on T2-weighted and FLAIR images
Differential
  • Alzheimer disease
  • Frontotemporal dementia
  • Vascular dementia
  • HIV-associated dementia
Treatment
  • Supportive
  • No effective treatment
Prognosis, Prevention, and Complications
  • Prognosis
    • death typically within 1 year of symptom onset
  • Prevention
    • iatrogenic
      • screen dura mater and cornea donors
      • incinerate medical tools used in patients with/suspective of CJD
      • recombinant human growth hormone use instead of human derived
  • Complications
    • seizures
    • aspiration pneumonia
PrPsc
 

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Questions (2)

(M1.NE.15.4671) A 65-year-old man with no significant medical history begins to have memory loss and personality changes. Rapidly, over the next few months his symptoms increase in severity. He experiences a rapid mental deterioration associated with sudden, jerking movements, particularly in response to being startled. He has gait disturbances as well. Eventually, he lapses into a coma and dies approximately ten months after the onset of symptoms. Which of the following would most likely be seen on autopsy of the brain in this patient? Tested Concept

QID: 107137
FIGURES:
1

A

10%

(7/72)

2

B

10%

(7/72)

3

C

54%

(39/72)

4

D

17%

(12/72)

5

E

8%

(6/72)

M 2 D

Select Answer to see Preferred Response

(M1.NE.14.24) A 70-year-old woman with no significant medical history begins to experience memory loss and personality changes. Over the next few months, her symptoms become more severe, as she experiences rapid mental deterioration. She also starts to have sudden, jerking movements in response to being startled and gait disturbances. Eventually, she lapses into a coma and dies eight months after the onset of symptoms. What process likely caused this woman's illness? Tested Concept

QID: 101688
1

Loss of dopaminergic neurons in the substantia nigra pars compacta.

8%

(5/66)

2

Autoimmune inflammation and demyelination of the central nervous system.

2%

(1/66)

3

Autoimmune inflammation and demyelination of the peripheral nervous system.

3%

(2/66)

4

Conversion of a protein from an a-helix to a ß-pleated form, which resists degradation.

68%

(45/66)

5

Frontotemporal atrophy and the accumulation of intracellular, aggregated tau protein.

20%

(13/66)

M 2 E

Select Answer to see Preferred Response

Evidence (4)
Topic COMMENTS (6)
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