The authors have reported four cases where the study of peripheral nerve biopsy revealed the presence of abnormal cells in the endoneurial space. Their bulky nuclei contain in fact numerous deposits more or less tubular reminiscent of the intranuclear structures observed in the multiple sclerosis brains, and other very rare diseases. Exceptionally it seems have these images been described in peripheral nerves. It must be stressed that only one patient had an inflammatory type disease. The meaning of these images is discussed, though no definite conclusion could be reached.

Aged↗

PubMedDate not supplied

Fabry's disease: an ultrastructural study of muscle and peripheral nerve.

A 9-year-old boy, presented with disseminated angiomatous lesions on the right leg. Ultrastructural examination of muscle and peripheral nerve showed numerous lamellar inclusions characteristic of Fabry's disease in the vessel's walls and perineurial cells. In addition, there were typical lamellar inclusions in some muscle fibers. Large myelinated fibers were reduced in number and some myelinated fibers showed segmental demyelination. Some lipid deposits displaying a lamellar pattern were occasionally seen in a few myelinated and unmyelinated axons. These abnormalities of nervous fibers are uncommon in Fabry's disease.

Child↗

PubMedDate not supplied

Lhermitte-Duclos type cerebellum hamartoma and Cowden disease.

A Lhermitte-Duclos type cerebellum hamartoma is reported in a woman with a diffuse hamartomatous condition involving the breast, thyroid, colon, skin, and kidney. The family history demonstrated the autosomal dominant transmission of this hamartomatous syndrome, and indicated the diagnosis of Cowden disease. This observation supports the recent hypothesis of Lhermitte-Duclos disease associated with Cowden disease as being a single phakomatosis.

Axons↗

PubMedDate not supplied

Immunoelectron identification of endoneurial IgM deposits in four patients with Waldenström's macroglobulinemia: a specific ultrastructural pattern related to the presence of cryoglobulin in one case.

In four patients with Waldenström's macroglobulinemia and peripheral neuropathy, direct immunofluorescence on a peripheral nerve biopsy revealed the presence of IgM deposits in the endoneurium. At ultrastructural examination, these endoneurial deposits were granulo-fibrillar in three cases, and tubular in the fourth, in whom a cryoglobulin was evidenced in the serum. In each case the endoneurial deposits were also identified as IgM by post-embedding immunoelectron microscopy with good preservation of the characteristic ultrastructural details.

Aged↗

PubMedDate not supplied

[Anatomical and clinical study of a case of uveitis and multiple sclerosis (author's transl)].

Lesions of the uveal tract can be an ocular manifestation of multiple sclerosis. The authors report on a clinical and anatomical study of a severe case of uveitis in a patient with multiple sclerosis confirmed by an anatomical examination of the nervous system. They review the observations published in the literature which were concerned mainly with clinical findings.

Brain↗

PubMedDate not supplied

Pathologic correlations between ocular and cerebral lesions in 36 AIDS patients.

The eyes and brain have rarely been studied simultaneously in AIDS patients. We have examined both the eyes and brain of 36 patients autopsied these past 3 years. Ten cases had concomitant involvement of eyes and brain by the same pathology: cryptococcus neoformans in 1, aspergillus in another, cytomegalovirus (CMV) infection in 5, and B cell lymphomas in 2 (1 patient had the last 2 disorders associated). One patient presented pericapillary microhemorrhages in the retina of the left ocular globe as well as in the right occipital lobe. Six other patients had isolated CMV retinopathy and 4 others had B cell cerebral lymphomas. Eleven patients with foci of cerebral toxoplasmosis did not have visible cysts of toxoplasma gondii in their retina, in agreement with the other series devoted to the concomitant study of the eyes and brain in AIDS patients. On the other hand, their simultaneous involvement in an AIDS patient must first point to CMV infection.

AIDS Dementia Complex↗