TABLE 3.
TDP-43 and p50 Staining in HMN7B
| Region | p50 | TDP-43 Pathology |
|||||
|---|---|---|---|---|---|---|---|
| NCIs | DNs | PVIs | GCIs | Spheroids | NIIs | ||
| Substantia nigra | + | − | − | − | − | − | − |
| Midbrain tegmentum | + | − | − | − | − | − | − |
| Locus coeruleus | + | − | − | − | − | − | − |
| Pontine tegmentum | + | − | − | − | − | − | − |
| Spinal cord anterior horn | + | − | − | − | − | − | − |
p50-positive NCIs were presence (+) or absence (−). TDP-43 pathology was scored on 5-point scale as—(0), absent; + (0.5), rarely observed; ++ (1), sparse; +++ (2), moderate and ++ ++ (3), frequent.
DNs, dystrophic neurites; GCIs, oligodendroglial cytoplasmic inclusions; HMN7B, distal hereditary motor neuropathy 7B; NCIs, neuronal cytoplasmic inclusions; NIIs, neuronal intranuclear inclusions; PVIs, perivascular astrocytic inclusions; TDP-43, TAR DNA-binding protein of 43 kDa.