use of a napkin or handkerchief. The quantity is sometimes enormous and always appears greater than normal. When the masseters and pterygoids are involved, which is usually at a late stage, but may be an initial condition, mastication is feeble or impossible. Finally, their complete paralysis allows the mandible to hang loosely, increases the opening of the drooping mouth and the salivary overflow. The jawjerk is abolished except in those cases where spastic symptoms elsewhere point to the association of the pyramidal tracts in the lesion. It is then increased. The lips usually show marked atrophy and are sensibly thinned. This is sometimes obscured by the fatty deposit, but in the final stages is practically a constant condition. The palate follows the lips in order of involvement in a majority of cases. Its loss of muscular tone is manifested by the nasal voice tones, which also adds to the lack of clearness in the pronunciation of the linguopalatals, and it turns "p" and "b" into the nasal resonant " m." When the palate is fully palsied it hangs loosely in the pharynx without reflex action or voluntary movement. As it can no longer shut off the nasal spaces, fluids often regurgitate through the nose, and even food masses may be forced into the nasal fossæ. When the pharynx is involved, the difficulties of swallowing reach their maximum. Food is now prone to enter the respiratory tract and a violent, fatiguing, and alarming cough is often produced. The danger of pulmonary engorgement, aspiration pneumonia, and heart-failure is intensified by the pneumogastric paralysis that is often present. Alimentation becomes so difficult that the esophageal tube must be used or the patient must be fed by the bowel. To these difficulties is added, sooner or later, a paralysis of the larynx. The adductors are usually most affected and the glottis stands wide open, serving neither the purposes of phonation nor protection to the trachea against the entrance of foreign material. In very rare cases unilateral or bilateral abductor palsy is found, and in the latter case inspiratory stridor indicates the dangerous respiratory difficulty. Both pharyngeal and laryngeal reflexes are abolished. The voice is extinguished. Failing respiratory and cardiac action leads to a fatal termination, which may come suddenly at any period of the disease. It is often induced by aspiration pneumonia or caused by suffocation due to blocking of the respiratory passage by a mass of food. The pulse is likely to become frequent, weak, and irregular. True anginal attacks are not infrequent. Syncope may occur and prove fatal. The pneumogastric involvement further shows itself in a feebleness of respiration, so that coughing and other active expiratory efforts become almost impossible, adding greatly to the danger of choking and to the general discomfort of the patient. It is somewhat remarkable that polyuria and glycosuria are seldom encountered. The controlling centers in the bulb are in close proximity to those invaded by this disease. It will be noticed that the nuclei selected are purely motor and trophic, and that the disease spreads not so much by contiguity as along lines of associated function. This is one of the distinctive habits of the discase, and serves here, as in other progressive maladies of the cerebro spinal axis, to draw earnest attention to this factor in the study of every case. The electrical examination of the atrophic muscles presents considerable difficulty, excepting in the lower face and the masseters. The changes found are practically a quantitative reduction to all currents as fiber after fiber disappears. The reaction of degeneration is wanting. The reflexes are correspondingly diminished and abolished, excepting in those cases in which disease involves the pyramidal tracts. Sensation is not markedly affected, nor is the sense of taste notably disturbed in pure cases. The temperature remains uninfluenced throughout, save by intercurrent accidents. Course. The disease is one of insidious onset and its steadily progressive course is characteristic. In a few exceptional cases the disease presents intermissions, but remissions are practically unknown. The duration of the malady from inception to fatal termination may be roughly stated as from one to five years. Leyden reports one case of seven years' duration, but, on the other hand, intercurrent maladies and the suffocative, cardiac, and pulmonary accidents to which the disease lays the patient liable may cut life short at any moment. The increasing feebleness and malnutrition at the same time add to the gravity of the situation. As above indicated, the disease first manifests itself in the tongue and progressively invades the lips, pharynx, palate, masticators, and larynx. This is a usual sequence, but not a necessary one. Any modification of it may be presented. The encroachments of related nuclear diseases at lower and higher levels give rise to different trains of symptoms, which, however, in their full development furnish very similar pictures. The course pursued in any given case can be understood by reference to the anatomical and especially to the functional relationship of the bulbar nuclei. The occurrence of a bronchitis, bronchopneumonia, angina pectoris, or of suffocative attacks is often of fatal import. Diagnosis. The diagnosis of a well-developed and unmixed case presents little difficulty. The course of the disease is of the first importance. The facies can hardly be mistaken. Palsy of both facial nerves gives rise to feebleness of the lips, but the upper part of the face does not escape and glossopharyngeal symptoms are lacking. Diphtheric palatal palsy may raise a doubt unless the clinical history of the infection is available. In this condition the lips and tongue escape, the onset is somewhat abrupt, and the usual course is toward recovery. Great difficulty may be presented in cases of multiple neuritis, but in them we have sensory disturbances, the reaction of degeneration, and marked symptoms in the extremities. When secondary to tabes, insular sclerosis, syringomyelia, and amyotrophic lateral sclerosis, the highly characteristic symptoms of these various diseases are prominent. As an extension process from the cord upward, or from the ocular nuclei downward, its development is preceded by the well-marked evidence of these prior states, which persist and increase during the evolution of the bulbar paralysis. The greatest diagnostic difficulty is presented by cases of the pseudobulbar paralyses. Pseudobulbar Paralyses.-These are (1) organic and (2) asthenic. The organic variety is due to more or less symmetrical lesions involving the posterior group of cranial nerves at any point from their cortical centers to their peripheral trunks. We distinguish a cerebral form due to bilateral cortical or subcortical vascular lesions. The onset is abrupt, and usually developed in two stages. A hemiplegic or apoplectic case presents a second stroke, this time from a lesion in the sound hemisphere, and the bulbar palsy is at once established or completed. It is only very rarely limited to the parts definitely elected by true bulbar palsy, does not present the atrophy, and the reflexes are retained or exaggerated. A radicular form follows acute bulbar myelitis and hemorrhage into or softening of the bulb. These are rare affections of sudden onset. The lesion does not spare the motor tracts for the limbs, and other bulbar functions do not escape. Tumor may similarly produce a pseudobulbar palsy, but the distinctive symptoms of an intracranial growth-headaches, vomiting, vertigo, and papillitis-are added. A basilar form is occasioned by tumors of the base and basilar meningitis, especially of the syphilitic variety. A pseudobulbar paralysis of this form is among the greatest rarities, for obvious anatomical reasons. Finally we have a neuritic form. This is usually only a part of a more widely distributed or multiple neuritis. Asthenic Bulbar Paralysis. This disorder, as described by Strümpell, may mimic polio-encephalitis inferior very closely. Previous to his communication a similar case was reported by Jolly,2 under the title of "Myasthenia Gravis Pseudoparalytica." Since that time cases have been reported by Murri,3 Pineles, Bruns, 5 Collins," Hallervorden, Kojewnikoff, and others. The condition in several instances has terminated fatally, but no changes in the bulbar nuclei were discovered. Apparently the functional deficit had not reached a degree sufficiently intense to produce cell-changes that were observable under ordinary methods of examination. In a case reported by Widal and Marenesco, disintegration of the chromaphilic elements was demonstrated by the Nissl and Marchi methods. The paralysis, which comes on more or less insidiously, especially involves the tongue, lips, and pharynx, but the eyes and extremities are also affected to a certain degree. Fibrillary twitching is wanting, the reflexes are not disturbed, and electrical responses are only modified by the fatigue induced by their repetition. It is found, as in bulbar paralysis, that rest seems to improve the paralytic features, but that the muscles involved show an extraordinary susceptibility to fatigue. Jolly's case showed ready muscular exhaustion under electrical stimulus, but this has not been found in some other cases. In some instances there is a tendency to improve and to relapse, as in the case of Collins, in which "Deut. Zeit. f. Nervenheilk.," Bd. 8. "Berlin. klin. Wochens.," Jan. 7, 1895. 4" Wien. Jahrbuch f. Psychiat., " vol. xiii. "Internat. Med. Mag.," April, 1896. "Deut. Zeit. f. Nervenheilk.," Nov., 1896. 3 “Policlinico,” vol. ii, 1895. 7 “Archiv f. Psychiatrie,” vol. xxviii. case the special senses of sight and hearing also showed rapid exhaustion. The condition is marked essentially by neurasthenia, affecting particularly the motor apparatus. The prognosis is grave, as a fair proportion of the cases terminate fatally through asphyxia. Treatment. In pure polio-encephalitis inferior chronica the prognosis is fatal. Curative treatment is, therefore, out of the question, but much can be done to alleviate the distressing condition of the patient and to obviate the laryngeal, pulmonary, and nutritive dangers that threaten him with suffocation, asphyxiation, pneumonia, and inanition. The hypersecretion of saliva may be checked by atropin, which also furnishes a reliable heart-stimulant. The stomach-tube and rectal alimentation are our means to avoid strangulation and to secure nutrition. Recourse to tracheotomy may be had in abductor laryngeal paralysis. Electricity is of use in exercising the muscles of the face, tongue, and gullet. The faradic current is sufficient. The large, indifferent electrode may be placed on the back of the neck, and a smaller, active electrode is then brought into contact with the lips, masseters, and tongue. By placing it over the pomum adami swallowing efforts are induced. Care must be exercised not to fatigue muscles already wasted. Energetic courses of silver, ergot, phosphorus, zinc, picrotoxin, and mercury are mentioned only to condemn them, and anything else that pulls down the waning strength of the patient must be avoided. Tonics and strengthening measures are of value. Acute bulbar palsy, or acute bulbar myelitis, is due to the same infections that set up acute myelitis. It may furnish the terminal stage of the chronic form, or it may result from an upward extension of a cord-lesion. The symptoms with which we are familiar in the chronic variety are rapidly evolved and often associated with febrile disturbances, headache, and somnolence. As the disease gains the pneumogastric nuclei, death becomes imminent and results through respiratory failure. Combined Forms of Polio-encephalitis.-The various combinations of chronic superior and inferior polio-encephalitis by extension have been alluded to in describing them separately. In some very rare cases the nuclear invasion falls upon upper and lower cranial nuclei practically at once. The resulting picture is an aggregate of the simpler ones. From their vital nature, the presence of pneumogastric symptoms dominates the outlook. PART III. DISEASES OF THE BRAIN PROPER. CHAPTER I. THE CEREBRAL CORTEX-LOCALIZATION. General Considerations.-The subject of localization of function in the cerebral cortex has attained great importance and its literature vast proportions. The practical considerations will be briefly and somewhat dogmatically set forth. Many points are still under debate, awaiting further experience and experiment, and some of these problems it is likely will never reach solution. It is well determined that there is a definite area of the cortex that is closely associated with motor functions. That this zone is purely motor is open to serious question. The general trend of belief is that it is also sensory, at least in part, and that motion does not even originate in it. As a working scheme we may consider that motion is represented in three levels: First, in the gray matter of the spinal cord; second, in the Rolandic area of the cortex; third, in the highest level of conscious thought, probably in the frontal region of the brain. The spinal level may be considered that of reflex, vegetative automatism, the Rolandic level that of motor memories, and the frontal area that of conscious, selective, and intelligent action. Thus, destruction of the highest level leaves automatic and memory action practically unimpaired, as in the experiments of Goltz, who removed the entire cerebrum of dogs without depriving them of muscular motion or bodily function. In the automatism of dementia the motor memories are likewise preserved. The mid-level, the Rolandic region, may be destroyed, leaving consciousness of volitional motions and the will to execute them, but the memory of their muscular production is gone, and they default, as, for instance, in motor aphasia. If the lowest or spinal level be destroyed, the mind and the memory organ have lost their tool and peripheral paralysis obtains. There is no difficulty in conceiving certain cortical areas to be memory organs, as in the case of the higher visual centers in the parietal lobe. We may, however, go further. All thought contains the two ideas of motion and sensation. They can not be separated, and without them consciousness is impossible. Indeed, they are in a certain sense identical. Motion is to the mind but the sensation of a change of position, and sensation is only the recognition of 155 |