Perivascular cavitation of the basal ganglia in gargoylism.

  title={Perivascular cavitation of the basal ganglia in gargoylism.},
  author={R. M. Norman and Henry Urich and N. E. France},
  journal={The Journal of mental science},
A case of gargoylism has been described in which the most striking pathological feature was the presence of extensive cavitation of the basal ganglia of the brain. These spaces were perivascular and differed from the small cysts commonly found in the cerebral white matter in gargoylism only in their size and localization. The patient was deaf and had no corneal opacities and there was a similarly affected male sibling. The case may therefore belong to that variant of gargoylism in which there… 

Nodular Lesions of Choroid Plexus in Hurler Disease

Neuropathologic examination of six brains from children with Hurler disease revealed nodular lesions in the glomus of choroid plexus caused by proliferation of vacuolated pericytes, fibroblasts, and

The mucopolysaccharidoses: characterization by cranial MR imaging.

It is postulate that in the natural course of MPS, cribriform changes occurred first, followed by white-matter changes and, last, atrophy, which may suggest a poorer prognosis.

MR imaging of cavitary lesions in the brain with Hurler/Scheie.

Her MR images showed large cavities within the white matter, as well as diffuse white matter changes and ventricular enlargement, which are typical of mucopolysaccharidoses.

Hurler's Syndrome and the Hearing Organ

  • G. Kelemen
  • Medicine
    The Journal of Laryngology & Otology
  • 1966
The case histories as presented below enumerate the details of the symptomatology and otological relations here only publications including histopathological reports on the temporal bones will be considered.

Computed Tomography and Magnetic Resonance Imaging of the Brain in Hurler's Disease

MRI and CT scans of the brain in five patients with Hurler's disease revealed low attenuation areas in the centrum semiovale and peritrigonal white matter, and it is suggested that the cystic areas seen on MRI correspond to perivascular lacunae seen in histopathologic material.

Diseases of Carbohydrate Metabolism

The concept of lysosomal disability caused by deficiencies of specific lysOSomal enzymes has proven extremely useful for the understanding of many other storage diseases.

Macrophages migration through the brain parenchyma to the perivascular space following particle ingestion.

Two macrophage responses to colloidal carbon in the central nervous system are indicated: an indolent population remains in the injection site for at least 1 month and a second population of macrophages emigrates through the neuropil.