Synonyms.—Glosso-labio-laryngeal Paralysis; Duchenne's Disease.
Definition.—An acute or chronic disease of middle age, due to involvement of the motor nuclei of the medulla oblongata, and characterized by paralysis of the lips, tongue, larynx, and pharynx, resulting in impairment of speech, phonation, mastication, and deglutition. Two forms of bulbar paralysis are recognized, the acute and the chronic.
Etiology.—Bulbar paralysis is a disease of middle and advanced life, between the ages of forty-five and seventy, and is more frequent in men than in women. It may be due to hemorrhage, embolism or softening, exposure to cold, trauma, or diphtheria. Lead-poisoning and syphilis have also been named as possible causes.
Pathology.—The most pronounced changes are found in the nerve-roots proceeding from the medulla; those of the hypoglossal, glossopharyngeal, vagus, facial, the motor nucleus of the fifth, and spinal accessory, showing distinct atrophy. They are of a gray or grayish-red color, and much shrunken. The microscope reveals degeneration of the nuclei or complete disappearance of the nerve-cells, some increase of the neuroglia, and thickening of the vessel-walls.
Muscular atrophy is limited to the lips, tongue, palate, and muscles of the larynx, and if spinal atrophy be associated, the muscles of the neck, shoulders, or arms are involved in the process.
Symptoms.—The onset is sudden, and may be attended by nausea and vomiting. Articulation is indistinct, especially in the use of the linguals, labials, and dentals: l, m, p, b, t, d, etc.
The lower lip being affected, it drops and there is dribbling of saliva. Deglutition is difficult, with frequent attacks of choking. The mind is impaired, the patient becomes quite emotional and neurasthenic. There may be hemiplegia or crossed facial paralysis.
Chronic Form.—The symptoms of this form are very gradual in their development, the first noticeable feature being a difficulty in controlling the tongue and articulating sounds that depend upon it,—thus words containing e, r, s, 1, k, g, d, t, n, and sh. As the disease progresses, the paralysis of the tongue becomes more pronounced; it can not be protruded, and lies on the floor of the mouth. Next in order, the lips become involved, and the labials p, b, f, v, m, and o are pronounced with difficulty, and blowing- and whistling are impossible. The lips atrophy and drop, exposing the teeth, and the saliva dribbles away.
Following the lips, the palate becomes paralyzed, and liquids are regurgitated through the nose, and the voice becomes nasal. Increased difficulty in swallowing is now experienced, for in addition to the inability of the tongue to carry the bolus backward, there is paralysis of the pharynx. In time the laryngeal muscles become involved, resulting in hoarseness and, finally complete aphonia. In swallowing, food is apt to enter the larynx, producing attacks of choking, and not infrequently particles find their way into the bronchi or lung, giving rise to bronchitis and deglutition pneumonia. At the last the patient's condition is most deplorable and disgusting, for, with mind impaired, he resembles a "driveling idiot." He can not talk, swallow, or close his mouth, and the saliva and particles of food dribble away.
There are no sensory symptoms, and the taste remains normal.
Diagnosis.—This is generally quite easy, the above symptoms being so striking and characteristic that no one need make a mistake in the diagnosis.
Prognosis.—This is unfavorable, though the disease may last for several years. Death usually results from exhaustion, inanition, choking, inspiration pneumonia, or circulatory disturbances.
Treatment.—Since the disease is incurable the treatment will be symptomatic. The general health is to be maintained and the patient instructed in the introduction of the esophageal tube, for feeding will have to be conducted in this way in the last stage of the disease. Electricity is of doubtful utility.