Triple-A syndrome with prominent ophthalmic features and a novel mutation in the AAAS gene: a case report.

Triple-A syndrome (Allgrove syndrome) is an autosomal recessive disorder characterized by adrenal insufficiency, alacrima, achalasia, and - occasionally - autonomic instability. Mutations have been found in the AAAS gene on 12q13

Connections between the lacrimal gland and sensory trigeminal neurons: a WGA/HRP study in the cynomolgous monkey

The sensory innervation of the lacrimal gland (LG) in the cynomolgous monkey was studied using the retrograde wheat germ agglutinin/horsereadish peroxidase (WGA/HRP) tracer technique. A small solidified piece of WGA/HRP was implanted in the LG. Labelled sensory first-order neurons were found in the ipsilateral trigeminal ganglion (TG) and in the ipsilateral mesencephalic trigeminal nucleus (MTN). The distribution of labelled TG neurons was restricted to ophthalmic and maxillary ganglionic parts. Sensory innervation of LG by primary afferents is not only restricted to TG; an MTN involvement has also been found. This may imply that there is a central sensory role in the production and release of tears

Re-evaluation and quantification of the different sources of nerve fibres supplying the rat eye

The denervation and/or the removal of peripheral nerve ganglia are useful surgical techniques for studying the source and distribution of peripheral nerves in all organs, including the eye. The amount and distribution of the remaining nerve fibres supplying the eye (after sectioning of various types of nervous fibres and/or removal of nerve ganglia) were evaluated in the rat. Male Sprague–Dawley rats were anaesthetized and one or more of the following nervous tissues were removed: superior cervical ganglion, main ciliary ganglion, pterygopalatine ganglion, trigeminal ganglion and the ophthalmic-maxillary nerve. In some animals, chemical sympathectomy was performed by administration of 6-OH dopamine. The eyes were cut in serial sections, but only three regions (cornea, iris and choroid) were harvested and submitted for various nerve fibre staining techniques. The results were quantified and statistically analysed. Superior cervical ganglionectomy and/or chemical sympathectomy induced the destruction of almost all the catecholaminergic nerve fibres in the three examined regions of the rat eye. Removal of the ciliary ganglion (partial parasympathectomy) caused the destruction of about 60% of the cholinergic nerve fibres of the same regions of the rat eye, while subtotal parasympathectomy destroyed about 80% of the cholinergic nerve fibres. Surgical transsection of the ophthalmo-maxillary nerve or the removal of the trigeminal ganglion led to a degeneration of almost all sensitive nerve fibres of the three examined regions of the rat eye. The denervation experiments confirmed the presence of the different types of nerve fibres (sympathetic, parasympathetic and sensitive) in the three studied structures of the rat eye