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Washington University Experience | METABOLIC | Menkes disease | 1C1 Menke's Dz (Case 1) 1

1C1-5 Gross appearance of the brain (weight 500 gm, nl ~900 gm). ---- This ventral view of the brain shows particular loss or misshapen appearance of the frontal and temporal lobes. The optic nerves and tracts appear unusually lengthy. Notice the tortuosity and size of the vasculature, particularly that associated with the brainstem. The most impressive gross finding is not visible, and is only demonstrated by palpation which demonstrates marked firmness of the white matter. An unusual result can be seen in later images, in which the soft cortical gray matter has separated from the strikingly firm underlying white matter during handing forming a prominent cleft.

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