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Neuropathology

External hydrocephalus, whatever the causes are, exhibits dilation of the subarachnoid space, with no increase in collagenous fibers or cellular elements, but an increase in CSF. The causative encephaloclastic disease may be diagnosed on the basis of other phenomena, such as liver cirrhosis in alcoholics, or loss of neurons in the presence of plaques and tangles in senile dementia or Alzheimer's disease.

Typical macroscopic features of internal hydro-cephalus include an enlarged ventricular system (Fig. 4.12a, b) (Weller and Shulman 1972), interstitial edema, disruption of the ependymal cells lining the ventricle, and axonal and myelin destruction in the periventricular white matter (Del Bigio 2004). Secondary changes in neurons reflect compensation to the stress or ultimately the disconnection. Proliferating astrocytes and/or gliosis (Fig. 4.12c) replace in part the interrupted ependymal cell line. These glial nodules appear granular or like small tumors (Fig. 4.12c) upon macroscopic inspection of the inner surface of the ventricles (Fig. 4.12b). There is also a partial reestablishment of flattened ependymal

Fig. 4.12a-c. Internal hydrocephalus. a Expanding ventricular system associated with an atrophy of white matter; b the ventricular surface is commonly marked by a granular surface structure, which (c) microscopically is characterized by multiple glial nodules which replace lost segments of the ependymal layer (H&E, magnification X200)

Fig. 4.12a-c. Internal hydrocephalus. a Expanding ventricular system associated with an atrophy of white matter; b the ventricular surface is commonly marked by a granular surface structure, which (c) microscopically is characterized by multiple glial nodules which replace lost segments of the ependymal layer (H&E, magnification X200)

cells, and a decline in the number of axons with parallel proliferation of glial fibers in the periventricular white matter. In chronic hydrocephalus with high pressure hydrocephalus, a flattening of the gyral crests is seen (Fig. 4.13a); in addition a small reactive glial zone around the ventricular system (Fig. 4.13b) may develop which can be separated from the intact white matter at autopsy (Fig. 4.13c).

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