Table 3.
Analysis of CNS Histopathology of BMT-treated MPS I Cats
| Animal # | 82766 | 4874 | 4296 | 4297 | 4347 | 4366 | 4372 | 4583 | BMT | BMT | |
|---|---|---|---|---|---|---|---|---|---|---|---|
| Treatment Status | Untreated | Untreated | BMT | BMT | BMT | BMT | BMT | BMT | Average | St Dev | |
| Age at euthanasia (months) | 33.6 | 52.8 | 35.8 | 2.2 | 36.8 | 36.3 | 17.7 | 17.2 | |||
| Neuronal granulation1 |
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| Cerebral Cortex | Frequency | 5 | 5 | 4.0 | 3.0 | 3.0 | 2.0 | 3.0 | 3.0 | 3.0 | 0.6 |
| Intensity | 5 | 5 | 3.0 | 3.0 | 4.0 | 3.5 | 4.5 | 3.5 | 3.6 | 0.6 | |
| Caudate Nucleus | Frequency | 5 | 5 | 3.0 | NA | 3.0 | 3.0 | 3.0 | 3.0 | 3.0 | 0.0 |
| Intensity | 5 | 5 | 4.0 | NA | 4.0 | 4.0 | 3.0 | 4.0 | 3.8 | 0.4 | |
| Hippocampus | Frequency | 5 | 5 | 4.0 | 4.0 | 4.0 | 4.0 | 3.0 | 3.0 | 3.7 | 0.5 |
| Intensity | 5 | 5 | 3.0 | 3.0 | 3.0 | 4.0 | 3.0 | 3.0 | 3.2 | 0.4 | |
| Thalamus | Frequency | 5 | 5 | 4.0 | 4.0 | 4.0 | 4.0 | 3.0 | 3.0 | 3.7 | 0.5 |
| Intensity | 5 | 5 | 3.0 | 4.0 | 3.0 | 4.0 | 3.0 | 3.0 | 3.3 | 0.5 | |
| Brain Stem | Frequency | 5 | 5 | 4.0 | NA | 4.0 | 4.0 | 3.0 | 3.0 | 3.6 | 0.5 |
| Intensity | 5 | 5 | 4.0 | NA | 4.0 | 4.0 | 4.0 | 4.0 | 4.0 | 0.0 | |
| Cerebellum | Frequency | 5 | 5 | 4.0 | 4.0 | 4.0 | 4.0 | 3.0 | 4.0 | 3.8 | 0.4 |
| Intensity | 5 | 5 | 4.0 | 4.0 | 4.0 | 4.0 | 4.0 | 4.0 | 4.0 | 0.0 | |
| Spinal Cord | Frequency | 5 | 5 | 4.0 | NA | 3.0 | 4.0 | 3.0 | NA | 3.5 | 0.6 |
| Intensity | 5 | 5 | 4.0 | NA | 4.0 | 5.0 | 3.0 | NA | 4.0 | 0.8 | |
| Fibroblast Vacuolation1 and Mononuclear Cells Infiltration2 |
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| Perivascular Space | |||||||||||
| Vacuolation | Frequency | 5 | 5 | 4.0 | 4.0 | 4.0 | 4.0 | 2.0 | 4.0 | 3.7 | 0.8 |
| Intensity |
5 | 5 | 3.0 | 2.0 | 2.0 | 2.0 | 2.0 | 3.0 | 2.3 | 0.5 | |
| Infiltration | Frequency | 5 | 5 | 3.0 | 1.0 | 1.0 | 1.5 | 0.5 | 2.0 | 1.5 | 0.9 |
| Intensity | 5 | 5 | 2.5 | 1.0 | 1.0 | 1.5 | 0.5 | 2.0 | 1.4 | 0.7 | |
| Meninges | |||||||||||
| Vacuolation | Frequency | 5 | 5 | 2.5 | 2.0 | 2.0 | 1.0 | 2.0 | 2.0 | 1.9 | 0.5 |
| Intensity |
5 | 5 | 2.5 | 2.0 | 2.0 | 1.0 | 2.0 | 2.0 | 1.9 | 0.5 | |
| Infiltration | Frequency | 5 | 5 | 2.5 | 2.0 | 1.0 | 1.0 | 2.0 | 2.0 | 1.8 | 0.6 |
| Intensity | 5 | 5 | 2.5 | 2.0 | 2.0 | 1.0 | 2.0 | 2.0 | 1.9 | 0.5 | |
| Choroid Plexus | |||||||||||
| Vacuolation | Frequency | 5 | 5 | 3.0 | 2.0 | 2.0 | 2.0 | 3.0 | 1.0 | 2.2 | 0.8 |
| Intensity |
5 | 5 | 3.0 | 3.0 | 2.0 | 2.0 | 3.0 | 1.0 | 2.3 | 0.8 | |
| Infiltration | Frequency | 5 | 5 | 1.0 | 1.0 | 1.0 | 1.0 | 1.0 | 0.5 | 0.9 | 0.2 |
| Intensity | 5 | 5 | 1.0 | 1.0 | 1.0 | 1.0 | 1.0 | 0.5 | 0.9 | 0.2 | |
1
absence = 0; presence in up to 10% of the cells = 1; up to 30% = 2; up to 50% = 3; up to 80% = 4; more than 80% = 5
2
absence = 0; presence in up to 10% of the vessels = 1; up to 30% = 2; up to 50% = 3; up to 80% = 4; more than 80% = 5; NA = not assessed