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Table 2 Lesion profiles of the E200G case

From: Genetic CJD with a novel E200G mutation in the prion protein gene and comparison with E200K mutation cases

Pathology\ Brain regions PrPScdepositiona Vacuolationb Reactive astrocytosisc
Frontal Layers 5, 6 ++/+++ (S/PL/FG) Layer 6 +++ Pattern1: layer 1 +
Layers 2–5 ++ Pattern 2: layers 1–4 ++
Layers 1–4 + (FG)   Pattern 3: layers 1–6 +++
Parietal Layers 5, 6 ++ Layers 5–6 ++ Pattern1: layer 6 ++
(S/PL/FG) Layers 2–4 + Pattern 2: layers 2–6 ++
Layers 1–4 + (FG)
Temporal Layers 5, 6 ++/+++ (FG) Lattern 1: Pattern 1: layers 1–5 ++++
Layers 1–4 ++ (FG) Layers 3–6 +++ Pattern 2: layers 1–5 +/++
Layer 2 SS
Pattern 2:
Layers 5–6 +++
Layers 2–4 ++++ (SS)
Occipital Layers 5, 6 + (FG) Layer 6 + Layers 1–6 −/+
Layers 1–4 −/+ (FG)
Cingulate Layers 1–6 +++ (FG) Layers 2–3 ++ Layers 2–6 ++++
Layers 4–5 +++
Layer 6 ++
Insula Layers 5, 6 +++ (FG) Layers 5–6 +++ Layers 1–4 +++
Layers 1–4 ++ (FG) Layers 2–3 ++ Layers 5, 6 −
Caudate +++ (S, FG) +++ +++
Putamen +++ (S, FG) +++ +++
Globus pallidus Ext. +++ (FG) Ext. + Ext.++/+++
Int. ++/+++ (FG) Int. + Int. ++
Thalamusd Rostral: med.+/++ (S), lat. − Rostral: med.++, lat.−/+ Rostral: med.++/+++, lat. +/++
Middle: med. + (S), lat. − Middle: med.++, lat.+ Middle & caudal: med. ++/+++, lat. −/+
Caudal: med. 1+ but focal 2+, lat. - Caudal: med.++/+++, lat. +
Amygdala ++/+++ (FG) +++ ++/+++
Hippocampusd Dentate gyrus, CA4, CA3, and CA2: −/+ CA4, CA3, CA2, and CA1 prox. – CA4, CA3 ++
CA2, CA1 prox. –
CA1: prox. ++, CA1 dist.+++ (FG) CA1 dis. ++ CA1 dis. ++,
Subiculucm: +++ Subiculum: Subiculum ++,
Entorhinal cortex: layers 5, 6 ++/+++ Layer 4 +++ Entorhinal cortex: layers 2–6 ++
Layer 2 +/++
Layers 1–4 −/++ (FG) Entorhinal cortex Transentorhinal cortex: layers 2–6 +++
Transentorhinal cortex: layers 1–6 +++ (FG) : layers 2,4 +++
Transentorhinal cortex
  : layers 2–6 +++
Cerebellum Molecular and granular layers −/+ Molecular and granular Layers – dentate nucleus + Molecular layer ++/+++
Granular layer +/++
Dentate nucleus +
Dentate nucleus +
Midbrain Periaqueductal gray and tegmentum + (FG) Periaqueductal gray ++ Periaqueductal gray +
Tegmentum + Tegmentum ++/+++
Substantia nigra ++/+++ (FG) ++ +/++
Medulla Tegmentum ++ (FG) Tegmentum + Tegmentum +
Inf. olive ++/+++ (FG) Inf. olive - Inf. olive +
  1. a PrPSc deposition scored on a 4-level scale: - absent, + mild, ++ moderate, +++ severe; b Vacuolation scored on a 5-level scale - absent, + mild, ++ moderate, +++ moderately severe, ++++/SS very severe/status spongiosis. c Reactive Astrocytosis scored on a 5 -point scale - absent, + mild, ++ moderate, +++ moderately severe, ++++ very severe. d data shown is from UCSF pathological analysis. NPDPSC analyses were generally in agreement with UCSF analyses except NPDPSC found +++ PrPSc in medial thalamus and hippocampus. Abbreviations: S Synaptic, PL Plaque-like, FG Finely granular, SS Status spongiosus, ext. Externa, int. Interna, med. Medial, lat. Lateral, prox. Proximal, dis. Distal, inf. Inferior.
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Acta Neuropathologica Communications

ISSN: 2051-5960

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