That such lesions in the cord may be the consequence of chronic hydrocephalus is well argued by Pekelharing in regard to his own recently published case, which in some respects closely resembles that by Müller. The patient was a boy in whom muscular paresis was congenital, and who from birth had exhibited deficient intelligence with an abnormally large head. At the autopsy, made at fourteen, ventricular effusion was found in the brain, and in the cord irregular dilatation of the central canal and great dilatation of blood-vessels and accumulation of leucocytes in its immediate neighborhood. Some ganglion-cells in the inner and anterior groups of the anterior cornua were shrunken and deprived of their prolongations. The author suggests that in this case the cerebral hydrocephalus was the primary disease; that the central canal in the spinal cord was dilated by extension of the effusion from the brain; that a partial reabsorption of such effusion had caused hyperæmia ex vacuo in the tissue immediately surrounding the canal; and that the emigration of leucocytes and partial alteration of the ganglion-cells both resulted from this hyperæmia.
In Müller's case the central canal and adjacent tissue were also the part of the cord most diseased; but the canal was obliterated by proliferation of the ependyma, not dilated. In Barth's case also75 the central canal of the cord was found obliterated. The patient was a man of forty-four, who since the age of forty had suffered from stiffness in the left ankle and difficulty of walking. After a year the stiffness extended to the right ankle; in two years the paresis had mounted to the thighs, and was accompanied by severe pains. Paresis and pain then appeared in the upper extremities, which gradually atrophied. After two years the patient was entirely confined to bed, and two years later was unable even to sit up. Later, the muscles of the neck became hypertrophied. No mention is made of perceptible hypertrophy in other muscles, nor of contractions or tremors other than fibrillary. But at the autopsy was discovered a lateral sclerosis extending the entire length of the cord, associated with partial atrophy of the ganglion-cells in the anterior cornua. In both the gray and white substances the blood-vessels were dilated, and, as already stated, the central canal was obliterated. The brain was healthy. The supinators of the upper extremities, the gastrocnemii at the lower, were richly infiltrated with fat streaked with long bands of connective tissue; the remaining muscles were atrophied.
75 Archiv der Heilkunde, xii. 2, 1871.
The anatomical lesions in this case are identical with those of the special symptom-complex described by Charcot as amyotrophic lateral sclerosis. Certain symptoms of lateral sclerosis are wanting to complete the clinical history, but at least as many are lacking for a typical history of pseudo-hypertrophic paralysis. Only the muscles of the neck hypertrophied: the gastrocnemii and adductors, primarily atrophied, later regained some of their original size. The fatty infiltration of the calf and muscles was unattended by pseudo-enlargement or by retraction: it resembled a fatty substitution due to nerve-paralysis, rather than the hyperplastic process of pseudo-hypertrophy.
Setting aside the three foregoing cases, three remain which, together with an unimpeachable history of pseudo-hypertrophic paralysis, show positive lesions in the spinal cord. The first and the most famous was made upon a patient of Gowers by Lockhart Clarke.76 Changes were found scattered through the entire length of the cord. "In the upper cervical region were patches of incipient disintegration in the gray network of the lateral portion of the cord, the lateral white columns being healthy. Here and there in the gray substance of the anterior and posterior cornua the intercellular matrix was wasted and disintegrated, especially in the neighborhood of the blood-vessels and at the bottom of the anterior median fissure. Here were accumulated globules of myeline and other débris of nerve-tissue. The blood-vessels were distended, their perivascular spaces enlarged. Patches of disintegration of nerve-fibres of the lateral and posterior columns were seen in the lower cervical and in the dorsal regions. Globules of myeline and masses of fatty matter were at some points accumulated at the entrance of the posterior nerve-roots, and even, to a much less extent, adjacent to the anterior roots. The most extensive lesion existed in the lowest part of the dorsal region. In the lateral gray substance on each side was an area of softening containing an actual cavity just outside each posterior vesicular column. The latter remained undamaged.
"The anterior cornua throughout the cord were perfectly normal, though the processes of the cells were perhaps less distinct than elsewhere. Further, notwithstanding the spots of disintegration in the lateral columns there was in them no change comparable to that of lateral sclerosis."77
76 Med.-Chir. Trans., 1874.
77 This autopsy was made on a boy of fifteen, in whom the calves began to hypertrophy at three, and reached their maximum size at five.
The second post-mortem was made by Ross on a patient belonging to Leech: "In the lumbar region of the cord the normal loose and spongy texture of the central column was replaced by a somewhat dense and fibrillated tissue, in which no trace of ganglion-cells could be found. The blood-vessels were enlarged and their walls thickened. In the anterior cornua the ganglion-cells had completely disappeared from the median area, the anterior group, and from the margins of all the other groups. This atrophic process extended into the dorsal and cervical region, and in the latter the central column was changed in the manner already described."78
78 Loc. cit., p. 207. Patient was nine years old at time of death; the disease had begun with paresis at two; was well developed at nine.