TABLE 2.
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Neuropathological Features in SCA2

Patient 1
Patient 2
Patient 3
Neuronal Loss*1C2-IR†pTDP-43-IR‡Neuronal Loss*1C2-IR†pTDP-43-IR‡Neuronal Loss*1C2-IR†pTDP-43-IR‡
Cerebral cortex (frontal)−++++−++++++++++
Cerebral cortex (occipital)−+++−++++++++
Hippocampus−+−−+++−−++−
Dentate gyrus−−−−−−−−−
Amygdala−−−−−−−−−
Cerebral white matter−+++−−+−++++
Caudate nucleus−++−−++++++
Putamen−++−−++++++
Pallidum++++−−+++++
Thalamus+++++++++++++++++++
Oculomotor nucleus−+++−+++−++++−
Red nucleus++++++++++++++++++++++
Substantia nigra++++++++++++++++++++++++
Pontine nucleus+++++++++++++++++++++++−
Locus coeruleus−++++−++−++−
Hypoglossal nucleus−+−+++−+++−
Vestibular nucleus−+−++−++−
Inferior olivary nucleus++++++++++++++++++++++++
Anterior horn++++++++++++++++++++++++++
Cerebellar cortex+++++++++++++++++++++++++++
Dentate nucleus−++−+++−++++
Patient 1
Patient 2
Patient 3
Neuronal Loss*1C2-IR†pTDP-43-IR‡Neuronal Loss*1C2-IR†pTDP-43-IR‡Neuronal Loss*1C2-IR†pTDP-43-IR‡
Cerebral cortex (frontal)−++++−++++++++++
Cerebral cortex (occipital)−+++−++++++++
Hippocampus−+−−+++−−++−
Dentate gyrus−−−−−−−−−
Amygdala−−−−−−−−−
Cerebral white matter−+++−−+−++++
Caudate nucleus−++−−++++++
Putamen−++−−++++++
Pallidum++++−−+++++
Thalamus+++++++++++++++++++
Oculomotor nucleus−+++−+++−++++−
Red nucleus++++++++++++++++++++++
Substantia nigra++++++++++++++++++++++++
Pontine nucleus+++++++++++++++++++++++−
Locus coeruleus−++++−++−++−
Hypoglossal nucleus−+−+++−+++−
Vestibular nucleus−+−++−++−
Inferior olivary nucleus++++++++++++++++++++++++
Anterior horn++++++++++++++++++++++++++
Cerebellar cortex+++++++++++++++++++++++++++
Dentate nucleus−++−+++−++++
*

Neuronal loss; −: <10% (none), +: 10%–30% (mild), ++: 31%–60% (moderate), +++:>60% (severe).

**

1C2, frequency of 1C2 immunoreactivity; ‘−‘:*<1% (none), +:1%–20% (low), ++: 21%–40% (intermediate), +++: 41%–60% (intermediate to high), ++++: 61%–80% (high), +++++: >80%(extremely high).

***

Phosphorylated transactivation-responsive DNA-binding protein 43 immunoreactive frequency; −: 1% (none), +: 1%–5% (low), ++: 6%–10% (intermediate), +++: >10% (high).

TABLE 2.
Open in new tab

Neuropathological Features in SCA2

Patient 1
Patient 2
Patient 3
Neuronal Loss*1C2-IR†pTDP-43-IR‡Neuronal Loss*1C2-IR†pTDP-43-IR‡Neuronal Loss*1C2-IR†pTDP-43-IR‡
Cerebral cortex (frontal)−++++−++++++++++
Cerebral cortex (occipital)−+++−++++++++
Hippocampus−+−−+++−−++−
Dentate gyrus−−−−−−−−−
Amygdala−−−−−−−−−
Cerebral white matter−+++−−+−++++
Caudate nucleus−++−−++++++
Putamen−++−−++++++
Pallidum++++−−+++++
Thalamus+++++++++++++++++++
Oculomotor nucleus−+++−+++−++++−
Red nucleus++++++++++++++++++++++
Substantia nigra++++++++++++++++++++++++
Pontine nucleus+++++++++++++++++++++++−
Locus coeruleus−++++−++−++−
Hypoglossal nucleus−+−+++−+++−
Vestibular nucleus−+−++−++−
Inferior olivary nucleus++++++++++++++++++++++++
Anterior horn++++++++++++++++++++++++++
Cerebellar cortex+++++++++++++++++++++++++++
Dentate nucleus−++−+++−++++
Patient 1
Patient 2
Patient 3
Neuronal Loss*1C2-IR†pTDP-43-IR‡Neuronal Loss*1C2-IR†pTDP-43-IR‡Neuronal Loss*1C2-IR†pTDP-43-IR‡
Cerebral cortex (frontal)−++++−++++++++++
Cerebral cortex (occipital)−+++−++++++++
Hippocampus−+−−+++−−++−
Dentate gyrus−−−−−−−−−
Amygdala−−−−−−−−−
Cerebral white matter−+++−−+−++++
Caudate nucleus−++−−++++++
Putamen−++−−++++++
Pallidum++++−−+++++
Thalamus+++++++++++++++++++
Oculomotor nucleus−+++−+++−++++−
Red nucleus++++++++++++++++++++++
Substantia nigra++++++++++++++++++++++++
Pontine nucleus+++++++++++++++++++++++−
Locus coeruleus−++++−++−++−
Hypoglossal nucleus−+−+++−+++−
Vestibular nucleus−+−++−++−
Inferior olivary nucleus++++++++++++++++++++++++
Anterior horn++++++++++++++++++++++++++
Cerebellar cortex+++++++++++++++++++++++++++
Dentate nucleus−++−+++−++++
*

Neuronal loss; −: <10% (none), +: 10%–30% (mild), ++: 31%–60% (moderate), +++:>60% (severe).

**

1C2, frequency of 1C2 immunoreactivity; ‘−‘:*<1% (none), +:1%–20% (low), ++: 21%–40% (intermediate), +++: 41%–60% (intermediate to high), ++++: 61%–80% (high), +++++: >80%(extremely high).

***

Phosphorylated transactivation-responsive DNA-binding protein 43 immunoreactive frequency; −: 1% (none), +: 1%–5% (low), ++: 6%–10% (intermediate), +++: >10% (high).

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