BULBAR DISEASES In the discussion of congenital diseases we referred to conditions characterized by the presence of ocular palsies and, more rarely, of other cranial nerves, and in accordance with the autopsy findings of lieubner we ascribed these conditions to a congenital aplasia of the nuclear region. Similar palsies of cranial nerves may also develop in earliest childhood, due most probably to hereditary or familial causes. Palsies of this kind, when affecting the ocular muscles, were described by illobius among the congenital conditions and his description of the clinical picture of infantile nuclear atrophy was made to include these cases also. This classification, however, was given up by later authors (Kunn and especially Peritz). According to these authors the nuclei of the cranial nerves are subject to the same abiotic processes as the motor cells of the spinal eord, and in that ease certain forms of ophthalmo plegia and bulbar symptoms entirely analagous to spinal muscular atrophy may develop, which either become arrested at a certain stage or have a tendency to progress indefinitely. We have autopsy reports (lieuhner and Nag) which confirm the existence of this medullary form of endogenous cranial nerve palsies and which definitely prove the occurrence of atrophy nf the corresponding nuclei. Clinically also we observe intermediate forms between spinal and bulbar diseases; thus, spinal palsies, on the one hand. may lead to bulbar diseases and on the other hand, cases beginning with bulbar symptoms may later develop paralysis of the skeletal muscles.
1. I le j algae proyressire bulbar paralysis is often observed in brothers and sisters. The first symptoms usually appear late (from the Gth to the 10th year of life) and consist in disturbances of speech and degluti tion. sluggishness of the muscles of expression, dribbling of saliva and failure of the lachrymal secretion. Later on atrophy and tremor of the tongue, a mask-like expression of the face, paralysis of the soft palate, hoarseness, weakness of the muscles of mastication, irregularity of the respiration and of the pulse are superadcled, and the child usually dies. Occasionally the disease remains stationary for some time.
2. Progressire ophthalmoplegia affects either all the ocular muscles or only individual ones, especially the levator palpehrte superioris and one abdueens. The internal muscles of the eye always escape. Com binations with palsies of other cranial nerves (facial) are not rare. The ultimate. effect of these disturbances of the ocular mu.scles is per manent interference with the movements of the eye, but life is not endangered.
Although these disturbances, which are due to a nuclear lesion, arc analogous to the spinal diseases, it may possibly be justifiable with Kuno to regard disturbances of the ocular muscles occurring early and associated with changes in the peripheral eye muscles as dystrophic processes. The subject has been very little studied and further investigations would be acceptable.
(a) Pseudoparalytic Myastbenia The investigations of a number of authors in recent years have led to the recognition of a clinical picture, the pathology of which is still unknown, but which is probably: to be included among the endogenous diseases (possibly a primary muscular affection).
The characteristic feature of the disease is a weakness of the bul bar muscles, including the ocular muscles, manifesting itself in gradual loss of function and particularly in greatly diminished resistance to fatigue. Thus, the individual may be able to close the eyes, to speak, or, in severe cases, to swallow once or twice; but he is unable to repeat the acts and several minutes must elapse before he is again able to do so. The muscles of the trunk, extremity and back of the neck exhibit a similar behavior. The muscles are not atrophic and there are no re actions of degeneration; but irritation with the faradic current in a very short time fails to produce a contraction, and a short period of rest is required before the contraction again takes place (myasthenic re action). So far the disease has been observed chiefly in young indi viduals, but not in children. Pathological examination of the nervous system yielils absolutely negative results, but quite recently certain in constant changes in the muscles (cell accumulations) have been found which are difficult to interpret. Curiously, patients with this disease are sometimes the subjects of other grave affections, particularly neoplasms in the mediastinum (with persistent thymus), suggesting the possibility that the injury to the muscles may be due to the action of some morbid metabolic products. Tiut as, on the other hand, congenital disturbances are sometimes associated with myasthenia, opini OTIS incline to the theory of a congenital defect capable, under certain conditions, of causing the disease. The prognosis is very grave although the disease May become arrested and show no tendency to progress for some time and apparent improvement may take place, most of the cases terminate fatally. from dyspncea, the entrance of food into the windpipe, asphyxia or inanition.