| I-1 | II-1 | II-5 | II-3 | II-10 | III-1 | III-2 | III-4 | III-6 | III-7 | IV-1 | IV-2 | IV-3 | ||
---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
Demographics | Â | ||||||||||||||
Age at onset | Â | Â | Â | Â | Â | 42 | 40 | 39 | Â | 29 | Â | Â | Â | ||
Age at diagnosis | Â | Â | Â | Â | Â | 46 | 41 | 39 | 41 | 35 | 26 | 19 | 5 | ||
Age at death | 42 | 45 | 43 | 41 | 44 | 47 | Â | 40 | Â | Â | Â | Â | Â | ||
Gender | M | F | F | M | M | F | M | F | F | M | F | F | F | ||
Symptoms and signs | Â | Â | Â | Â | Â | Â | Â | Â | Â | Â | Â | Â | Â | ||
Retinopathy |  +  |  +  |  +  |  +  |  +  |  +  |  −  |  +  |  −  |  +  |  −  |  −  |  −  | ||
Seizures |  −  |  −  |  −  |  −  |  −  |  −  |  −  |  +  |  −  |  +  |  −  |  −  |  −  | ||
ICP |  |  |  |  |  |  −  |  −  |  +  |  −  |  −  |  −  |  −  |  −  | ||
Cognitive decline |  +  |  |  |  |  |  +  |  −  |  −  |  −  |  +  |  −  |  −  |  −  | ||
Stroke-like episode |  −  |  −  |  −  |  −  |  −  |  +  |  −  |  +  |  −  |  +  |  −  |  −  |  −  | ||
Nephropathy |  +  |  +  |  +  |  +  |  +  |  +  |  +  |  +  |  −  |  +  |  −  |  −  |  −  | ||
Mesenteric ischemia |  |  |  |  |  |  −  |  −  |  −  |  −  |  +  |  −  |  −  |  −  | ||
Ascites |  +  |  |  |  |  |  −  |  +  |  −  |  −  |  +  |  −  |  −  |  −  | ||
Hypertension |  +  |  +  |  +  |  +  |  +  |  +  |  +  |  +  |  −  |  +  |  −  |  −  |  −  | ||
Lab workups | Â | Â | Â | Â | Â | Â | Â | Â | Â | Â | Â | Â | Â | ||
Proteinuria |  |  |  |  |  |  +  |  +  |  +  |  −  |  +  |  −  |  −  |  −  | ||
GFR (mL/min) | Â | Â | Â | Â | Â | 15.03 | 9.6 | 41.49 | Normal | 38.5 | Normal | Normal | Normal | ||
Hyperlipidemia |  |  |  |  |  |  +  |  +  |  −  |  −  |  +  |  −  |  −  |  −  | ||
Hypoalbuminemia |  |  |  |  |  |  +  |  +  |  +  |  −  |  −  |  −  |  −  |  −  | ||
Normocytic anemia |  |  |  |  |  |  +  |  +  |  +  |  −  |  −  |  −  |  −  |  −  | ||
Hyperfibrinogenemia |  |  |  |  |  |  −  |  −  |  −  |  −  |  +  |  −  |  −  |  −  | ||
Hypothyroidism |  |  |  |  |  |  +  |  −  |  −  |  −  |  −  |  −  |  −  |  −  | ||
Hyperparathyroidism |  |  |  |  |  |  −  |  +  |  −  |  −  |  −  |  −  |  −  |  −  | ||
Inflammatory markers |  |  |  |  |  | ESR, FDP, D-D↑ | IL-6, ESR↑ | FDP, D-D↑ | Normal | ESR, CRP, FDP, D-D↑ | Normal | Normal | Normal | ||
Neuroimagings | Â | Â | Â | Â | Â | Â | Â | Â | Â | Â | Â | Â | Â | ||
Leukoaraosis | Â | Â | Â | Â | Â | Fazekas 3 | Â | Fazekas 1 | Fazekas 1 | Fazekas 3 | None | None | None | ||
Number of rim-enhancing lesion | Â | Â | Â | Â | Â | Multiple | Â | Single | None | Multiple | Â | Â | Â | ||
Mass effect and edema |  |  |  |  |  |  +  |  |  +  | None |  +  | None | None | None | ||
Neuropathology | Â | Â | Â | Â | Â | Â | Â | vasculopathy mimicking CNS vasculitis | Â | Â | Â | Â | Â |