Reactions of vessel walls and brain parenchyma to the accumulation of Gaucher cells in the Norrbottnian type (type III) of XXXX .

@entity1 @entity3026 @entity19831 @entity1120
Splenectomy in @entity1 with the Norrbottnian type of @entity1120 is followed by increased blood levels of @entity19831 and impaired neurological and mental status. High blood levels are associated with an increased accumulation of @entity19831 in perivascular Gaucher cells in the brain compared to non-splenectomised cases. Surrounding the Gaucher cell infiltrates there is loss of neurons and slight @entity3026 . The brains of four cases with the Norrbottnian type of @entity1120 were examined by immunohistochemical stains in an attempt to further characterize the perivascular Gaucher cells and to examine the reactions of the vessel walls and brain parenchyma to the accumulation of Gaucher cells. The perivascular storage cells showed granular staining with antibodies to muramidase and alpha 1-anti-chymotrypsin confirming that they are blood-derived macrophages belonging to the monocyte-macrophage system. The Gaucher cells contained material positive for antisera to plasma proteins strongly suggesting that large molecules (including @entity19831 ) can escape from the blood and be taken up by the macrophages in @entity1120 . The storage cells were surrounded by a reticulin network stained by antisera to collagen type III, type IV and laminin. The infiltrates were bounded from the brain parenchyma by a membrane strongly positive with antiserum for the basal lamina protein collagen type IV and laminin. The formation of a basal lamina around the Gaucher cell cuffs probably constitutes a protective phenomenon governing the brain parenchyma against the foreign cells. A focal loss of neurons but only minor loss of axons could be demonstrated with the antiserum to neurofilament.(ABSTRACT TRUNCATED AT 250 WORDS)
