TABLE 4.
Regional Distribution and Severity of Lewy Pathology in a Case With Fabry Disease
| Case | 1 | 2 | |
|---|---|---|---|
| aon/ob | Anterior olfactory nucleus and olfactory tract/olfactory bulb | +++/++ | − |
| dm | Dorsal motor nucleus of the vagal nerve (X) | ++ | − |
| ap | Area postrema | + | − |
| irz | Intermediate reticular zone | ++ | − |
| ro | Obscure raphe nucleus | + | − |
| rm | Great raphe nucleus | ++ | NA |
| gig | Gigantocellular nucleus of the reticular formation | ++ | NA |
| ph | Prepositus hypoglossal nucleus | ++ | NA |
| fa | Motor nucleus of the facial nerve (VII) | + | NA |
| tr | Spinal tract nucleus of the trigeminal nerve | + | NA |
| lc | Locus coeruleus | +++ | − |
| pc | Central reticular nucleus of the pons | + | − |
| dr | Dorsal raphe nucleus | +++ | − |
| cr | Central raphe nucleus | + | − |
| sn | Substantia nigra, pars compacta | +++ | − |
| pa | Paranigral nucleus | + | NA |
| pg | Pigmented parabrachial nucleus | ++ | NA |
| ppt | Pedunculopontine tegmental nucleus | +++ | − |
| oc | Oculomotor nucleus (III) | + | NA |
| ce | Central nucleus of the amygdala | + | − |
| ba | Basal nucleus of the amygdala | + | − |
| la | Lateral nucleus of the amygdala | + | − |
| ct | Cortical nucleus of the amygdala | + | NA |
| ac | Accessory cortical nucleus of the amygdala | ++ | − |
| me | Medial nucleus of the amygdala | + | − |
| db | Interstitial nucleus of the diagonal band | ++ | − |
| bn | Basal nucleus of Meynert | ++ | NA |
| so | Hypothalamic supraoptic nucleus | + | − |
| pv | Hypothalamic paraventricular nucleus | + | NA |
| tm | Hypothalamic tuberomamillary nucleus | ++ | NA |
| tl | Hypothalamic lateral tuberal nucleus | + | NA |
| in | Thalamic intralaminar nuclei | + | NA |
| rt | Reticular nucleus of the thalamus | + | NA |
| pu | Putamen | +++ | − |
| nc | Caudate nucleus | ++ | NA |
| CA2 | Ammon’s horn, second sector | + | − |
| tre | Transentorhinal cortex | + | − |
| ent | Entorhinal cortex | + | − |
| pi | Prepiriform cortex (Brodmann Area 51) | + | NA |
| ai | Agranular insular cortex, layer Vb | + | NA |
| ci | Anterior cingulate gyrus, layer Vb | + | NA |
| af | Anterolateral funiculus of the spinal cord | + | NA |
| lam 1 | Layer 1 of the spinal cord | + | NA |
| IML | Intermediolateral nucleus (layer 7) of the spinal cord | + | NA |
The pathology was assessed semiquantitatively: − absent or not detectable; + slight; ++ moderate; +++ severe. Only regions that displayed Lewy pathology are shown. For accompanying heat maps, see Supplementary Data Tables.
NA, not available.