Case Study: Diagnosis and Progression of Creutzfeldt-Jakob Disease in a Geriatric Patient, Slides of Geriatrics

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Geriatrics Rounds

• 71 yr RHD male with past Hx of MI, HTN,

dyslipidemia, gout, OA, referred to Neurology for

incoordination

• Normal exam in ER except Lt hand dysmetria
• EKG( q waves, prior MI), blood work Normal
• CT Head  Normal
• Discharged with Cerebellar Stroke as provisional

diagnosis & stroke workup initiated

• Echocardiogram, Carotid USG, MRI brain

 22 Feb 2011: back to ER

 Progressive symptoms:

 Lt hand incordination

 Gait unsteadiness

 Lt facial weakness

 Slurred speech

 Minor dysphagia of solids

 Weight loss 15 lbs over 6 weeks

 Chronic cough

 Hematuria (occasionally)

 Blood on toilet paper since ASA ( x 3 weeks)

DTR Lt Rt

Biceps 3+ 3+

Triceps 3+ 3+

Brachioradialis 3+ 3+

Knee 3+ 3+

Ankle 3+ 3+

Plantar’s Equivocal

Cerebellar Exam

Marked dysfunction Lt UE & LE

Marked dysdiadochokinesia Lt UE

Marked dysrhythmogenesis Lt LE

Intention tremor Lt UE

Noted Lt UE apraxia

(ideomotor/ideational)

Gait:

Markedly unsteady , leaning to left

Localization?

Differential?

Investigations?

Course in Hospital:

23 Feb 2011 : MOCA 30/

24 Feb 2011:Worsening of cerebellar findings bilaterally

EEG done

Over next week: Increasing dysphagia to solids Increased Lt hand spasticity, tremor, slurred speech Lt hand/leg myoclonic jerks, Startle reflex -ve Worsening Lt UE/LE strenght 4-/5, worsening of gait/ motility Pronounced Lt facial droop Mild behavioral changes/ paranoia: noted by family

07 March 2011 MOCA: 18/30( visuospatial/executive, attention , language)

08 March 2011: EEG: Intermittent 1 Hz periodic discharges, max Rt , background slowing

• EEG 24 Feb
• EEG 08 March

PET

PET

Creutzfeldt-Jakob Disease

Prion Disease

 Prion: small infectious pathogen containing protein but lacking

nucleic acid

 Non-pathological membrane bound protein in neuronal and non-

neuronal cells

 Conversion to active, accumulating isomer

 Intracellular accumulation of prions = apoptosis, cell death,

degenerative neurologic disease

 CJD (and variants), kuru, Gerstmann-Straüssler-Scheinker

syndrome, familial fatal insomnia