. CEREBRAL SCLEROSIS The term cerebral sclerosis is used to describe a number of patho logic conditions characterized macroscopically by thickening, contrac tion and brownish discoloration of the brain substance, microscopically by proliferation of the connective tissue, particularly in the septa of the brain, and in the perivascular tissue, and by thickening of the vessel walls. The degree to which the nerve tissue is involved in the process varies; ganglion cells and nerve fibres may atrophy or degenerate and disappear completely. Sclerosis may affect only certain portions of the brain, forming hard knotty tumors (tuberous sclerosis), or an entire lobe (lobar sclerosis), or it may cause shrinking and atrophy of an entire hemisphere either of the cerebrum or of the cerebellum (hemispherical form of sclerosis).
These forms of sclerosis merely represent the terminal conditions of some severe cerebral process and may accordingly be due to a variety of causes. They occur as the result of intra-uterine diseases (contbined with porencephaly); they may be the products of brain softening clue to hereditary syphilis, or represent the remains of an inflammatory disease of the brain, etc. Accordingly, the clinical symptoms which mark the onset or subsequent course of these sclerotic conditions are by no means uniform. In the beginning they frequently resemble the symptoms of a congenital paralysis, encephalitis, brain embolism, etc.; later in the course of the disease the variegated picture of cerebral infantile palsy is simulated.
IIence but little is gained by collecting these various forms of sclerosis into a common group, be it never so eomprehensible (Richard iere) nor is the subject of eerebral disease in children thereby rendered more intelligible. A better plan is to analyze the causes of sclerosis and subdivide them into various groups, according to the etiology and clinical symptoms. It should he noted, hon-ever, that the central nervous system in childhood exhibits a. peculiar tendency to react to morbid processes by the formation of fibrous tissue.
Conspicuous in this group of anatomical findings is a condition which has been greatly studied in recent 3-ears and has received the name of diffuse cerebral sclerosis (Striimpell, TIeubner, Busse, Schmaus, Bullard, Ifugo, Weif, Frankl-Hoehwart, and others). The symptoms
of the disease are not present at birth, but develop later "often in the midst of perfeet health," altliough the child may have previously exhibited some defect in its mentally or bodily development. Diffuse sclerosis is especially apt to attack children in the first years of life, older children and adults are rarely affected.
The causes of diffuse cerebral sclerosis are not known. Syphilis, parental alcoholism, and traumatism -are mentioned. In one ease of Pfaundler's (private communication) syphilis, parental consanguinity, and alcoholism and beginning paralysis in the father were present.
The symptoms of the disease arc both psychic and somatic.
As a rule the first thing that is noticed is a diminution of the intelli gence, a sluggishness in the child's movements, a loss of interest. in its surroundings. This psychic change may be considerably increased in the subsequent course of the disease and complete icliocy may ultimately develop. The power of speech is rapidly lost and the child expresses itself only in grunting, inarticulate sounds. There are eases in which the intelligence remains approximately.- normal and is indeed retained until death. Among the bodily symptoms a3vkwardness and sluggish ness in the movements of the body are first. noted. The muscular tone is increased; the muscles are tense and boardlike; rigidity soon develops and ultimately leacls to contractures. The muscular rigidity is not confined to the extremities but attaeks the muscles of the face and the muscles of mastication as well. Sometimes the legs are first involved, in other cases the rigidity appears in all the extremities at the same time. At the height of the (I iSCaSe the child, owing to the spastie contractures, is deprived of all power of motion; the arms are flexed and pressed against the trunk, the legs are extended and (-Tossed; there arc dis tressing convulsions affecting the muscles of the jaw and general muscular twitchings which may be rhythmical, and absolute inability to carry out any intentional movements. Tremor, ataxia and athetosis are not infrequently present. True paralysis and atrophy clo not occur; but the children rapidly emaciate during the subsequent course of the disease.