Table 2 Cases characteristics
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% |