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Table 5 Autopsy Neuropathology

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

  N %
Study consented 22 (73%)
  of whom deceased 14 (64%)
  of which performed 10 (71%)
Summary of cases (10)
  Codon 129 Suspected clinical diagnosis Atypical features noted Autopsy neuropathology findings
ID 1 MM Senile dementia,
? Alzheimer’s disease
Fluent dysphagia (very early on), extreme behavioural disturbance Alzheimer’s Disease- Neuropathological changes (AD-NC), neocortical Lewy body, severe cerebrovascular disease
ID 2 MV Early-onset Alzheimer’s disease, mixed vascular Seizures AD-NC
ID 3 MM Early-onset Alzheimer’s disease Rapid deterioration, balance impaired AD-NC, Lewy body dementia
ID 4 MV Early-onset Alzheimer’s disease Frontal features, slow progression AD-NC
ID 5 MM Early-onset Alzheimer’s or vascular dementia Unclear AD-NC, severe cerebral amyloid angiopathy, moderate non-amyloid small vessel disease
ID 6 MM Early-onset frontal–temporal dementia Unusual rate of progression Non-specific neurodegenerative disordera
ID 7 MM ? vascular dementia with behavioural and psychological symptoms of dementia (BPSD) Considered previously to have a mild cognitive impairment, but evolved quickly to dementia with BPSD Vascular dementia, microinfarcts, lacunar infarcts
ID 8 Not determined Complex syndrome, parkinsonian features ? Parkinsonism in dementia, hallucinations. Did not meet the criteria for Lewy body dementia, frontal–temporal dementia, vascular dementia with parkinsonism Parkinson’s disease, neocortical Lewy body dementia
ID 9 MV Progressive supranuclear palsy Possible progressive supranuclear palsy, but insufficient evidence to fulfil diagnostic criteria Neocortical Lewy body disease limbic predominant age-related TDP43 encephalopathy (stage 3)
ID 10 Not determined Alzheimer’s disease, mixed vascular dementia Mixed Alzheimer's or vascular dementia, rapid decline, parkinsonian features Lewy body dementia
Age at onset of symptoms Mean = 65.4 years (56 – 79 years, std dev 7.1)
Interval between onset of symptoms and referral to the study Mean = 8 years (2 – 13 years, std dev 3.8)
Duration of illness (from the onset of symptoms to death) Mean = 9.9 years (3 – 16 years, std dev 4.4)
  1. Could not be characterised either clinically or pathologically; a rapidly progressive neurodegenerative disorder clinically thought to be frontotemporal dementia although with late onset cerebellar ataxia