In one of Bannister's cases the first upper molar of the left side sometimes ached, and the gum and bone were so wasted as to expose its roots for a considerable distance. Falling out of the teeth has been observed in a few other cases.
Atrophy of the tongue is associated with the facial wasting in a certain percentage of cases, and the uvula and soft palate are also sometimes wasted. On the other hand, atrophy of the tongue has been observed as an isolated phenomena.
Atrophy of the bones of the face is frequently present, and has been determined by careful measurements. It will show to a greater or lesser extent according to the age of the patient. When the disease arises after the bony development has been fully acquired, there will be little external evidence of osseous involvement; when it begins during the time of active growth of the bone, as it does not infrequently, the arrest in the skeleton will be very apparent. Thus in a number of cases beginning in early childhood bone atrophy has been a very marked feature.
Eulenburg says that the functions of taste, smell, hearing, and seeing are not interfered with in any of the reported cases; but Bannister reports impairment of the sense of taste in both of his cases. In one the whole left half of the tongue was involved, in the other only the left posterior third. In the first of these, in which the whole left half of the tongue was involved, both the glosso-pharyngeal and the chorda tympani nerves were affected. According to Bannister, the significance of this observation is that either the glosso-pharyngeal and fifth nerves are both involved, or that the taste-fibres for the base as well as tip of the tongue are derived from the last-named nerve. In this case, however, which was due to a severe injury, the headache, mental confusion, loss of hearing, etc. more probably indicated a widespread intracranial lesion, affecting to some degree the nucleus of the glosso-pharyngeal. In the other case, which was apparently a typical one of unilateral facial atrophy in its early stage, no history of traumatism was present, and the sense of taste, which was carefully tested several times, was seriously impaired over the left posterior third of the tongue.
Bannister discusses the probable cause of impairment of taste in each case, as follows: “The atrophy seemed to involve the region supplied by the second division of the fifth nerve as much as, or more than, the other divisions; and on this is situated the spheno-palatine ganglion which gives off the Vidian nerve, the upper division of which, the superior superficial petrosal nerve, is supposed by Schiff to contain the taste-fibres, which return from the lingual nerve through the chorda tympani. The usual theory of this disease is that it depends upon an affection of the trophic fibres contained in the fifth nerve or one or more of its divisions. Accepting this theory, Baerwinkel,16 noticing a case of unilateral atrophy of the face affecting only the second division of the fifth, and, as he held, indicating trouble with the spheno-palatine ganglion, its trophic centre, mentions the fact that there was no impairment of taste in the anterior portions of the tongue as rather against the hypothesis of Schiff as to the course of the taste-fibres. At the same time he gives two other observations of lesions of the trifacial and the seventh nerve that favor it. In our patient I should have expected a priori to have found the taste affected in the anterior portion of the tongue, if anywhere, but the reverse was the fact. It is difficult to suppose an accidental lesion of the glosso-pharyngeal coexisting with the one of the trigeminus that produces the atrophy when there are no more signs of nervous disorder than there were in this case. On the other hand, the taste-fibres of the glosso-pharyngeal are not generally supposed to have such connections with the fifth nerve as to be involved in its disease, while those of the chorda tympani, of the connections of which with the trigeminus there is much more physiological evidence, escape. The observation is a contradictory one, and I cannot at present explain it.”
16 Deutsch. Archiv f. klin. Med., xvii. 1, 1875.
So far as I know, ophthalmoscopic changes have never been noted. In a few cases enophthalmos, or sinking in of the eye, doubtless due to the disappearance of orbital fat as the disease advances, has been recorded.
Defects of hearing, and even partial deafness, have been reported in a very limited number of cases, making it questionable whether this symptom is a coincidence or a complication. In the case reported by me careful examination of the membrane of the tympanum by W. Ziegler of the ear department of the Hospital of the University of Pennsylvania showed atrophy of this membrane.
Eulenburg speaks of one case in which the external atrophy had reached as far as the larynx, and in which the pronunciation of the letter r was somewhat impeded. Beverly Robinson,17 reports a case accompanied with slight paralysis of the adductors of the vocal cords. The laryngeal paralysis in this case improved under treatment, which led Robinson to believe that the relation between the two affections might have been only one of coincidence.
17 Am. Journ. Med. Sci., October, 1878, p. 437.