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Table 1 Cognition and neuromuscular status – amyotrophy in prion disease

From: A patient with Creutzfeldt-Jakob disease presenting with amyotrophy: a case report

  Hospital day
  Day 1 Day 7 Day 10 Day 16 Day 30
Cognition:      
Mini mental state examination 25 - 23 9 0
Clock drawing test 4 - 3 0 0
Total functional capacity 12 - 2 1 0
Addenbrooke’s cognitive assessment (revised) 83 - 72 38 0
• Attention and orientation 16/18 - 13/18 4/18 0
• Memory 22/26 - 20/26 13/26 0
• Fluency 10/14 - 8/14 8/14 0
• Language 22/26 - 20/26 13/26 0
Visuospatial 13/16 - 11/16 0/16 0
Neuromuscular:      
Neck F 5* 5 4 3 2
  E 5 5 4 3 2
Hip F 4(L), 5(R) 3(L), 3(R) 3(L), 3(R) 3(L), 3(R) 2(L), 2(R)
  E 4, 5 3, 3 3, 3 3, 3 2, 2
Hip ABD 4, 5 3, 4 3, 3 3, 3 2, 2
  ADD 4, 5 3, 4 3, 3 3, 3 2, 2
Knee F 4, 5 3, 3 3, 3 3, 3 2, 2
  E 4, 5 3, 3 3, 3 3, 3 2, 2
Ankle DF 5, 5 3, 3 3, 3 3, 3 2, 2
  PF 5, 5 4, 4 3, 3 3, 3 2, 2
  INV 5, 5 3, 3 2, 2 2, 2 2, 2
  EV 5, 5 3, 3 2, 2 2, 2 2, 2
Shoulder ABD 5, 5 5, 5 4, 4 3, 3 2, 2
  F 5, 5 5, 5 4, 4 3, 3 2, 2
  E 5, 5 5, 5 4, 4 3, 3 2, 2
Elbow F 5, 5 5, 5 3, 3 3, 3 2, 2
  E 5, 5 5, 5 3, 3 3, 3 2, 2
Wrist F 5, 5 5, 5 3, 3 3, 3 2, 2
  E 5, 5 5, 5 3, 3 3, 3 2, 2
Finger F 5, 5 5, 5 3, 3 3, 3 2, 2
  E 5, 5 5, 5 3, 3 3, 3 2, 2
  1. *Medical Research Council Scale 0 to 5. F Flexion, E Extension, ABD Abduction, ADD Adduction, PF Plantar flexion, INV Inversion, EV Eversion, DF Dorsiflexion, L Left, R Right.
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