optic nerves/eyes ->to Roman

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Hi Roman,

While sorting through some older journal articles, I found these 2

abstract with a reference to eyes and CMT. One is from 1977, the other from

1999.

I am not certain about how to explain Argyll-on-like pupils. The ish

ophthalmologist Argyll on (1837-1909) is best known for his

description of the syphilitic pupil. He also made

important contributions concerning the ocular effects of physostigmine and to

filtration surgery for glaucoma.

The Argyll on pupil, a miotic pupil that fails to react to direct light,

has been described for more than a century. Originally associated with tabes

dorsalis, the sign has now been found in a number of conditions with lesions in

the area of the nucleus of Edinger-Westphal. Magnetic resonance imaging studies

have localized the lesion in patients with sarcoidosis and multiple sclerosis.

With the declining incidence of neurosyphilis, the sign is increasingly likely

to indicate another cause, although an assiduous search for clues should also be

undertaken.

~ Gretchen

Eur Neurol. 1977;16(1-6):172-5.

Argyll-on-like pupils in the neural type of Charcot-Marie-Tooth

disease.

Salisachs P, Lapresle J.

The authors report of Argyll-on-like pupils in three patients

presenting the neural type of Charcot-Marie-Tooth (CMT) disease with

peroneal muscular atrophy. Up to now the light-near dissociation had

been reported in the hypertrophic variety of CMT disease. Stress is

laid upon the fact that the presence of the light-near dissociation in

patients with CMT disease does not help in clinically differentiating

the neural from the hypertrophic type of this disease.

J Neurol Neurosurg Psychiatry. 1999 Jun;66(6):779-82.

Axonal phenotype of Charcot-Marie-Tooth disease associated with a mutation in

the myelin protein zero gene.

Chapon F, Latour P, Diraison P, Schaeffer S, Vandenberghe A.

Laboratoire de Neuropathologie, Centre Hospitalier Universitaire de Caen, 14033

Caen, France.

A French family had Charcot-Marie-Tooth disease type 2 (CMT2) which was

characterised by late onset of peripheral neuropathy involvement, Argyll

on-like pupils, dysphagia, and deafness. Electrophysiological studies and

nerve biopsy defined the neuropathy as axonal type. Genetic analysis of myelin

protein zero (MPZ) found a mutation in codon 124 resulting in substitution of

threonine by methionine. One of the patients, presently 30 years old, showed

only Argyll on-like pupils as an objective sign but no clinical or

electrophysiological signs of peripheral neuropathy.