Table 2 Summary of neuropathological findings in three cases of G51D mutation
|  | Neuronal loss | Neuronal α-synuclein pathology | Oligodendroglial α-synuclein inclusions | ||||
|---|---|---|---|---|---|---|---|
| Â | Â | Annular or crescent | Globular | Diffuse | NFT-like | Threads | Â |
Cortex | Â | Â | Â | Â | Â | Â | Â |
 Frontal | + | ++ | +/++ | + | - /+ | ++ | −/+ |
 Motor | - /+ | ++ | +/++ | + | - /+ | ++/+++ | - /+ |
 Temporal | +/+++ | +++ | +/++ | +/++ | - /+ | +++ | - /+ |
 Parietal | + | +/+++ | ++ | + | - /+ | ++/+++ | - |
 Occipital | - | + | - /++ | - /+ | - /+ | - /+ | - /+ |
 Cingulate | +/+++ | +++ | ++ | +/++ | - /+ | +++ | - |
 Insular | +/+++ | +++ | +/+++ | +/++ | - /+ | +++ | - |
Sub-cortical white matter | Â | Â | Â | Â | Â | Â | Â |
 Frontal | N/A | N/A | N/A | N/A | N/A | + | + |
 Motor | N/A | N/A | N/A | N/A | N/A | + | +/++ |
 Temporal | N/A | N/A | N/A | N/A | N/A | + | + |
 Parietal | N/A | N/A | N/A | N/A | N/A | + | + |
 Occipital | N/A | N/A | N/A | N/A | N/A | + | - /+ |
 Cingulate | N/A | N/A | N/A | N/A | N/A | + | + |
Internal capsule | N/A | N/A | N/A | N/A | N/A | +/++ | + |
External capsule | N/A | N/A | N/A | N/A | N/A | ++ | + |
Amygdala | −/+++ | +/++ | ++/+++ | +/++ | - | +++ | - /+ |
Hippocampus | Â | Â | Â | Â | Â | Â | Â |
 Dentate fascia | - | ++/+++ | + | + | - | +/++ | - |
 CA4 | −/++ | +/++ | +/++ | + | - /+ | ++/+++ | - /+ |
 CA3 | −/+++ | - /+ | +/++ | −/+++ | - | ++/+++ | - |
 CA2 | +/+++ | - | −/++ | −/+++ | - | ++/+++ | - |
 CA1 | −/++ | +/+++ | +/++ | +/++ | + | ++/+++ | - |
 Subiculum | - /+ | +/++ | +/++ | +/++ | - /+ | ++/+++ | - |
 Entorhinal cortex | −/++ | +++ | +/++ | +/++ | - /+ | ++/+++ | - |
 Transentorhinal cortex | +/++ | ++/+++ | +/+++ | +/++ | - /+ | ++/+++ | - |
Caudate | + | - /+ | +/+++ | ++ | - /+ | ++/+++ | - |
Putamen | - /+ | −/++ | +/+++ | ++/+++ | - /+ | ++/+++ | - |
Globus pallidus | - /+ | −/++ | - /+ | - | - | + | - /+ |
Thalamus | - | - | −/+ | −/++ | - | −/++ | - /+ |
Subthalamic nucleus | - | - | - | - /+ | - | −/++ | - /+ |
Red nucleus | - | - | - | - | - | + | + |
IIIrd nerve nucleus | - | - /+ | +/++ | +/+++ | - | +/+++ | - |
Substantia nigra | +++ | - | −/++ | −/+++ | - /+ | ++/+++ | - /+ |
Locus coeruleus | ++/+++ | - | −/++ | −/+++ | - | ++/+++ | - |
Pontine nuclei | - | - | - | + | - /+ | + | - /+ |
Pontine base white matter | N/A | N/A | N/A | N/A | N/A | +/++ | +/++ |
Dorsal motor nucleus of vagus | +/+++ | - | −/+ | −/+++ | −/+ | ++/+++ | - |
Twelfth nerve nucleus | - | - | - | - | - | + | - |
Inferior olive | −/+ | - | - | −/++ | - | + | - |
Cerebellar hemisphere Purkinje cells | +/++ | - | - | - | - | - | N/A |
Cerebellar hemisphere white matter | N/A | N/A | N/A | N/A | N/A | +/++ | +/++ |
Dentate nucleus | - | - | - | - | - | - | - |