Menkes' syndrome with vascular and adrenergic nerve abnormalities.

Abstract:

:Using the formaldehyde-induced fluorescence method, we found a peculiar rosary-type swelling of the adrenergic axons in the peripheral nerves, deficiency of the perivascular adrenergic plexuses in the visceral and cerebral arteries, and reduction of noradrenergic fluorescence in the tegmental and hypothalamic regions of a 3-year-old boy who had typical Menkes' syndrome (kinky hair syndrome). The nigrostriated neurons retained moderate intensity of fluorescence compared with those in postmortem (control) brains. Histologically, marked dilatation of the visceral, meningeal and cerebral arteries were noted. Copper deficiency, the cause of this disease, induces failure of central and peripheral noradrenergic neurons and leads to abnormal vasodilatation.

journal_name

Arch Pathol Lab Med

authors

Uno H,Arya S,Laxova R,Gilbert EF

subject

Has Abstract

pub_date

1983-06-01 00:00:00

pages

286-9

issue

6

eissn

0003-9985

issn

1543-2165

journal_volume

107

pub_type

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