Table 5.
Distribution of FUS-positive lesions in NIFID and atypical FTLD-U
| Anatomical region | NIFID cases |
Atypical FTLD-U cases |
||||||||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| 1* | 2 | 3 | 4 | 5 | 6 | 7 | 1 | 2 | 3 | 4 | 5 | 6 | 7 | |
| Cerebral cortex | ||||||||||||||
| Frontal cortex | abpv | abpt | abp | abp | avt | at | NA | abt | abt | ab | abt | ab | ab | ab |
| Temporal cortex | NA | abp | abp | abp | avt | at | ab | abt | abt | NA | abt | ab | 0 | ab |
| Limbic system | ||||||||||||||
| Hippocampus GCL | NA | abpvc | abvpc | ab | a | 0 | NA | bv | bvc | bvc | bvc | bv | bv | bvc |
| Subiculum | NA | abp | abp | ab | avt | a | NA | 0 | bt | bvt | bv | b | bv | bv |
| Entorhinal | NA | abp | abp | ab | 0 | a | NA | abv | bt | bvt | bv | NA | bvt | bv |
| Fusiform | NA | abp | abp | ab | at | at | NA | abv | bt | abvt | abvt | NA | bvt | bv |
| Subcortical nuclei | ||||||||||||||
| Striatum | NA | ba | NA | abvt | abvt | abt | NA | abt | abvt | abt | abt | abt | NA | NA |
| Globus pallidus | NA | bt | NA | abv | abv | ab | NA | NA | 0 | ab | ab | ab | NA | 0 |
| Thalamus | NA | ag | NA | abv | abv | ab | NA | NA | NA | NA | NA | NA | 0 | NA |
| Brainstem | ||||||||||||||
| Substantia nigra | NA | gl bv | gl | gl | gl t | gl | gl | NA | NA | gl v | gl vt | gl | gl | gl |
| Red nucleus | NA | b | NA | a | bvt | a | NA | b | NA | 0 | 0 | NA | b | 0 |
| Locus coeruleus | NA | b | gl | gl | gl | 0 | NA | NA | 0 | NA | gl vt | gl | gl | 0 |
| Pontine base | NA | b | gl b | gl b | gl bv | gl b | gl b | gl | gl vt | v | gl | gl | gl | gl v |
| 12th nerve | NA | gl s | gl s | gl s | gl svt | gl s | NA | NA | NA | NA | NA | gl s | gl s | NA |
| Spinal cord | ||||||||||||||
| Cervical | NA | NA | NA | gl sg | NA | NA | NA | NA | gl sg | NA | NA | gl sg | NA | NA |
| Thoracic | NA | NA | NA | gl s | gl st | gl gs | NA | NA | gl sg | NA | NA | NA | NA | NA |
| Lumbar | NA | NA | gl sg | NA | NA | gl sg | NA | NA | gl sg | NA | NA | NA | NA | gl sg |
| Sacral | NA | NA | NA | gl s | NA | na | NA | NA | gl sg | NA | NA | NA | NA | NA |
* Patient NIFID1 was diagnosed with a frontal cortical biopsy.
Neuronal cytoplasmic inclusion morphologies: a = annular/crescent; b = bean shaped; g = granular; gl = globular; p = Pick body like; s = skein-like filamentous. Neuronal intranuclear inclusion morphologies: c = circular; v = vermiform.; t = FUS-positive neuropil threads; t = FUS-positive neuropil threads; NA = not available; GCL = granule cell layer.