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QUESTION1. PRESENTATION: 60yr old alcoholic man with change in mental status, muscle twitching, fibrillation.2. INX: CTB normal, EEG normal.

Neurology

QUESTION1. PRESENTATION: 60yr old alcoholic man with change in mental status, muscle twitching, fibrillation.2. INX: CTB normal, EEG normal.

1. ANSWER: CJD

2. EXPLANATION (variant CJD vs sporadic CJD)
- Age at presentation: median 29yrs for VCJD | vs 60yrs in SCJD.
- Survival and late dementia: median 14 months for VCJD | vs 4 for SCJD.
- Psychiatric features are an early sign (anxiety, withdrawal, apathy, agitation, a permanent look of fear in the eyes, depression, personality change, insomnia). Hallucinations and delusions may occur—before akinetic mutism.
- Painful sensory symptoms are commoner (foot pain, hyperaesthesia) in VCJD.
- Normal EEG in VCJD | SCJD has a characteristic spike and wave pattern.
- Mean CSF tau-pT181/tau protein ratio is 10-fold higher in VCJD than in SCJD.


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REFERENCES

1. Wilkinson, I. (2017). Oxford handbook of clinical medicine. Oxford: Oxford University Press.
2. Hannaman, R. A., Bullock, L., Hatchell, C. A., & Yoffe, M. (2016). Internal medicine review core curriculum, 2017-2018. CO Springs, CO: MedStudy.
3. Image: no reference available.

Ⓒ A. Manickam 2018

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