CEREBROSPINAL SYMPTOM-CONIPLEXES The characteristic signs of these forms of endogenous diseases are nystagnius, strabismus, disturbances of speech (bradylalie) deficiency of the intellect, spasm (spasticity), and palsies of the legs and arms. Other symptoms that occur with varying regularity are: atrophy of the optic nerve, ocular palsies, tremors, wobbling of the head, athe tosis, disturbances of deglutition and other bulbar symptoms, isolated palsies, weakness of the sphincters and finally—indicating a transition to the myopathies,—muscular atrophy and pseudohypertrophy. The grouping of these multifarious morbid symptoms in an individual case or individual families is subject to wide variations; hence the publi cation of numerous interesting cases belonging to this category and the erection of a series of different types of familial palsies, which, as has been shown above, cannot be regarded a.s nosologic entities.
The onset of the disease, or, in other words, the manifestation of the first symptoms may occur at any- age; most commonly children beyond the tenth year of life are attacked. There are also cases, how ever, in which the first symptoms make their appearance very early, so that the dividing line between congenital and endogenous conditions cannot be sharply drawn. Boys as well as girls are attacked, some times in the same family, and the disease may be transmitted through the mother as well as through the father. Consanguinity appears to play an important role. Sporadic cases without any demonstrable hereditary or family elements are rare in this form of endogenous disease, or else they are not recognized as cases of cerebrospinal spastic paralysis.
The initial symptoms are extremely variable. Among the earliest are spasms in the legs, which interfere with walking and lead to contrac tures. At the same time, or a little later, nystagmus and slow speech make their appearance. Still later, awkwardness of the hands, tremors, choreic and ataxic movements, and often wobbling of the entire body are added to the picture. The intelligence suffers later in the course of the disease. Mingled with these cardinal symptoms of family spastic paralysis, the manifestations already mentioned, particularly ocular palsies and bulbar symptoms (disturbances of deglutition, dtib bling of saliva, forced laughter, etc.) are frequently encountered. Trophic changes in the skin and in the osseous system have been very rarely observed.
Special attention should be called to certain symptoms of the cerebrospinal family diseases because they suggest other forms of endogenous diseases, or represent intermediate forms between the two. We refer to cases with disturbances of coordination, as in hereclitary ataxia, with muscular atrophies of the spinal type and, finally, pseudo hypertrophy of individual muscles. Such cases show very clearly the
impossibility of delineating definite clinical pictures in the familial spastic palsies.
Among the numerous cases belonging to this category those reported by Pelizaius, _Freud, Krafft-Ebing, -Wagner, Luce, Bruns, Gee, Pribram, Dreschfeld, Haushalter, Bouchard, Homen, Ganghofner, Jendrassik, Spitler, Holly, and Oppenheim are deserving of special mention.
The clinical course in diseases belonging to this group is slowly progressive, and the disease clues not materially- interfere with the patient's comfort. When, however, he is confined to bed on account of the increasing motor disturbances, when dysphagia and loss of blad der control become pronounced, a fatal termination may at any time be brought about by direct sequelai of the disease such as bedsores, pneu monia or cystitis. This slowly progressive character of the disease, interrupted by periods of temporary arrest of the symptoms, is char acteristic of this group of nervous conditions and, when ft case is under observation for some time, assumes a diagnostic importance. Next to the progressiveness, an hereditary or family tendency is significant in a diagnostic sense. These two are the only reliable diagnostic aids, and in the absence of both. or if they cannot be elicited with certainty, the differential diagnosis from cerebral infantile palsy, brain syphilis or neoplasm is difficult.
Post-mortem examination of these cases reveals some remarkable findings. Degeneration of extensive segments of the spinal tract, par ticularly- the pyramidal tracts, the lateral cerebellar tracts and the po.sterior columns is found. These pathologic findings are described as system-diseases, and we speak of a combined system-disease when, as in this instance, several systems of nerve tracts in the spinal cord are affected (Kahler-Pick, Westphal, Strumpell). Compared with these changes due to a congenital defect others, such as local inflammation of the meninges, cellular degeneration, and the like, are of minor impor tance. The question whether the degenerations are primary, or second ary to other endogenous diseases, is still under discussion, and its deci sion is rendered more difficult by the fact that most of the eases that come to autopsy are of long standing—years or decades—so that abun dant opportunities must have been present for the development of second ary changes in the central nervous system. In the case of the combined system-diseases which occur in this group of family affections—for some occur after intoxication and other diseases—the degeneration is probably regarded by most neurologists as primary in character.