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Table 2 Cases characteristics

From: A clinicopathological study of selected cognitive impairment cases in Lothian, Scotland: enhanced CJD surveillance in the 65 + population group

Demographic characteristics  N %
Total cases 30 (100%)
Sex
 Male 19 (63%)
 Female 11 (37%)
Age at study referral
 65 – 69 9 (30%)
 70 – 74 8 (27%)
 75 – 79 8 (27%)
 80 +  5 (16%)
Status
 Alive 9 (30%)
 Dead 21 (70%)
Referral characteristics N %
Referral specialty
 Ann Rowling Clinic 15 (50%)
 Neurology 4 (13%)
 Psychiatry of Old Age 8 (27%)
 Medicine of the Elderly 3 (10%)
Interval between onset of symptoms and referral to the study Mean = 7 years (1 – 13 years, std dev 4.1)
By age group
 65 – 69 Mean = 8 years (2 – 13 years, std dev 4.5)
 70 – 74 Mean = 6 years (3 – 11 years, std dev 3.4)
 75 – 79 Mean = 8 years (1 – 13 years, std dev 4.5)
 80 +  Mean = 5 years (2 – 10 years, std dev 3.3)
Clinical characteristics N %
Age at onset of symptoms Mean = 66 years (53 – 82 years, std dev. 7.8)
Suspected diagnosis   
 Atypical Alzheimer's Disease 11 (37%)
 Frontotemporal dementia 4 (13%)
 Mixed a 6 (20%)
 Unclear/unknown 9 (30%)
CJD-consistent features b
 Cerebellar ataxia 14 (47%)
 Involuntary movements 8 (27%)
 Rapid progression 7 (23%)
 Sensory features 2 (7%)
 Visual disturbance 1 (3%)
Duration of illness (from the onset of symptoms to censor date/death) Mean = 9.5 years (1.1 – 17.4 years, std dev 4.7)
Symptoms
a) Forgetfulness/ memory impairment 28 (93%)
b) Visuospatial difficulties (in recognising objects, familiar people, reading difficulties) 22 (73%)
c) Executive dysfunction (impaired problem solving/judgement) 29 (97%)
d) Language disturbance
 Receptive 18 (60%)
 Expressive 26 (87%)
e) Psychiatric symptoms
 Depression 24 (80%)
 Anxiety 19 (63%)
 Delusions 6 (20%)
 Behavioural disturbance 20 (67%)
 Impulsive/socially inappropriate 14 (47%)
 Change in food preferences 12 (40%)
 Aggressive 10 (33%)
 Lacking in empathy 14 (47%)
 Repetitive movements/behaviours 17 (57%)
 Apathy/ withdrawal 21 (70%)
f) Hallucinations
 Visual 8 (27%)
 Audio 3 (10%)
g) No insight 16 (53%)
h) Disturbance of gait 21 (70%)
i) Bedbound 3 (10%)
j) Dysarthria 10 (33%)
k) Visual Impairment 3 (10%)
l) Fluctuations of symptoms 3 (10%)
m) Weakness of limbs 6 (20%)
n) Clumsiness of limbs 9 (30%)
o) Slowness of movement 18 (60%)
p) Apraxia 23 (77%)
q) Involuntary movements
 Tremor 18 (60%)
 Jerking 12 (40%)
 Dystonia 0 0
 Alien limb 1 (3%)
r) Seizures 4 (13%)
 Akinetic mutism 0 0
t) Sensory symptoms
 Numbness/tingly/paraesthesia 5 (17%)
 Pain/burning/discomfort) 3 (10%)
Other relevant histories N %
Surgical history (including transplant)
 No surgery 2 7%
 1 – 2 10 33%
 3 – 4 9 30%
 5 +  9 30%
Blood transfusion history c
 No 29 97%
 Yes 1 3%
Previous injections d   
 No 24 80%
 Yes 6 20%
Family history of dementia
 No 14 47%
 Yes 13 43%
 Unsure/unclear/not known 3 10%
Occupation history   
 High-risk occupations e 7 23%
 Other 23 77%
  1. a mixed: early-onset Alzheimer’s Disease / vascular dementia (3 cases); Corticobasal syndrome/ Progressive supranuclear palsy (1); Frontotemporal dementia /vascular (1)
  2. b some cases presented with more than one feature
  3. c including blood components or plasma products e.g., immunoglobulins or albumins
  4. d as part of treatment e.g., human growth hormone, human gonadotrophin, insulin, fertility treatment
  5. e included: healthcare professions in medical, dentistry, nursing, and paramedical. Laboratory-based professions that dealt with animals, pharmaceutical, or other related research. Professions in veterinary medicine, animal farming, and the meat industry
  6. Note: Std dev is standard deviation