Part 2

The case histories with which its pages are so amply stocked are carefully analyzed in accordance with a broadly chosen plan, and the generalizations that precede and follow them are obviously based on a wide and varied personal experience such as alone could render a familiarity with the literature of the subjects treated adequate to its best usefulness. Both writers were indeed well adapted for this task. Dr. Southard, as everyone is aware, has long been a highly conscientious, ardent and productive worker in the department of pathological anatomy, and of late years a careful student of clinical diagnosis and methods, both at the Danvers State Hospital and still more, at the Psychopathic Hospital which he worked so hard to found; while Dr. Solomon’s researches, in the special field of neurosyphilis, have been of the highest order.

Undoubted as are the merits of the case-system of instruction that has been so much in vogue in recent years, and excellent as is the modern supplementation of this method by the use of published records, the danger is still real that the student will have presented to him a picture of nature in disease that is too diagrammatic, too concise, with the result that while the task of memory is lightened through simplified formulation, the training of the doubting and inquiring instincts is often given too little stimulus and scope. In this book this danger is deliberately met through the casting of emphasis rather on the pluralistic aspects of the processes at stake than (primarily) on their unitary aspects.

The student who utilizes this volume cannot but emerge from his study a more thoughtful person than he was at the period of his entry. He will have seen that clinical rules of thumb cannot be followed to advantage, and that, on the contrary, surprises are to be expected and prepared for. Let the recognition of this fact, if it seems to increase the difficulties in the way of diagnosis, not lead to pessimism in that respect, or to hopelessness in therapeutics. On the contrary the writers’ bias is towards the worth-whileness of clinical efforts and an increased respect for accuracy and thoroughness in the utilization of modern methods of research. The chance is indeed held open that even the gaunt spectre of “General Paresis” may prove to be less terrible than it seems, and for this hope good grounds are given.

It is in this way made clear, on the strength of anatomical evidence of much interest, that even if in the treatment of a given patient, the time arrives when a fatal or unfavorable result seems manifestly foreshadowed, it may be still worth while to renew the treatment with fresh zeal, for the sake of combatting some symptom or exacerbation, for which a locally fresh process furnishes the cause.

Another noteworthy principle here emphasized and illustrated is that the relationship between “functional” (hysterical, neurasthenic, migrainoid) symptoms and the signs (or symptoms) of organic processes is clinically important and worthy of much further study. This is a matter which, in a general sense, has interested me for many years. Above and over the “organic” hovers always the “functional,” as representing the first indication of the marvelous tendency to repair, or substitution, for which the resources of nature are so vast. Yet this functional tendency also has its laws, of which, in their turn, the organic processes display the action in quasi diagrammatic form. Hysteria, neurasthenia, migraine, etc., do not arise _de novo_ in each case, but conform to typical, though not rigid, formulas, susceptible of description. I have recently had the opportunity to study in detail an analogous series of transitions between the movements (and emotions) indicative of apparently purposeless myoclonic movements (on an epileptoid basis) and the movements of surprise, engrossment, purposeful effort, the excitement and joy by which the former were excited and into which they shaded over.

Taken altogether, this book represents work and thought in which, for amount and kind, the neurologists of Boston may take just pride.

JAMES J. PUTNAM.

ST. HUBERT’S, KEENE VALLEY, NEW YORK. _August, 1917._

Me miserable! which way shall I fly Infinite wrath and infinite despair? Which way I fly is Hell; myself am Hell; And, in the lowest deep, a lower deep Still threatening to devour me opens wide, To which the Hell I suffer seems a Heaven.

Paradise Lost, Book IV, lines 73–78.

I. THE NATURE AND FORMS OF SYPHILIS OF THE NERVOUS SYSTEM (NEUROSYPHILIS)

PARADIGM to show possible abundance and variety of symptoms and lesions in DIFFUSE NEUROSYPHILIS (“cerebrospinal syphilis”). Autopsy.

=Case 1.= Mrs. Alice Morton[1] was in the hands of at least five well-known specialists in different branches of medicine and surgery during the nineteen years of her disease. It appears that she acquired syphilis upon marriage at the age of 23 to a man who later became tabetic and acknowledged syphilitic infection previous to marriage. Mrs. Morton remained without children and there were no miscarriages.

At the age of 27, she developed iritis, paresis of the left eye muscles, and ulceration of the throat, with destruction of the uvula. The syphilitic nature of her disease was at once recognized and the classical treatment was given, although, through numerous shifts in consultants, this treatment was never pushed to the limit. At 28 Mrs. M. began to suffer from severe headaches resembling migraine and accompanied by attacks of paræsthesia; at 35, came severe pains in the back and difficulty in walking.

At 36, the migraine attacks began to be accompanied by blurring of vision and dizziness. The difficulty in walking became extreme, affecting particularly the right foot. The legs became spastic, there were pains and hyperæsthesia of the chest, and severe cramps of the legs. Antisyphilitic treatment at this time yielded marked improvement.

During her thirty-sixth year, Mrs. M. sustained curious transient losses of vision and of hearing. She was also irritable, and at this time developed her first pronounced mental symptoms, namely, delusions concerning her relatives. There were also a few seizures of an epileptiform nature.

At 38 there was a spell of total deafness, followed by improvement. The eye muscles were also subject to a variable involvement with intervening spells of improvement. The _knee-jerks were lost, but after a time returned_ in less pronounced form. Shortly, an absolute paralysis and extensive decubitus developed, and death occurred at 39.

The autopsy is briefly summarized below, but it is important in the understanding of Mrs. M.’s case (particularly some of the sensory symptoms and the transiency of certain symptoms) to consider the pre-infective history. Although there seems to be no doubt that the patient acquired syphilis at about 23 years of age from a syphilitic husband, who himself later became tabetic, yet it is of note that the patient was the only child of parents, both of whom also suffered from mental disease. Mrs. M.’s father died of what was called softening of the brain (one should avoid terming _all_ old cases of _so-called_ “softening of the brain” syphilitic, since the older diagnosticians did not always distinguish between non-syphilitic arteriosclerotic effects and syphilitic disease). Mrs. M.’s mother also died insane (confusion and emotional depression). It is clear, then, that we do not need to suppose that every symptom shown by Mrs. M. is directly due to destructive or irritative lesions immediately due to the spirocheta pallida. The case is, in fact, an excellent lesson as to the association of structural and functional effects in neuropathological cases.

Mrs. M. as a child had shown talent, but was somewhat nervous and eccentric. At one time, she had an attack of hysterical dysphasia; at another time, an attack of hysterical dyspnea; during another period, an apparent obsession (kicking the mopboard at regular intervals). Moreover, she had for years suffered from migraines of a severe and unusual type. Both the hysterical tendency and the migrainous tendency became mingled with the results of the neurosyphilis in later stages of the disease in such wise that it was hard to tell exactly where the structural phenomena left off and the functional phenomena began.

For example, at the age of 32, nine years after infection and four years after the earliest nerve symptoms traceable to syphilis, and at about the time of the onset of spinal cord symptoms, an attack was described as follows:

The patient had a very severe attack of migraine (?) yesterday, preceded and accompanied by paraphasia, so severe that for three hours she was unable to make herself understood, and indeed felt “as if her ideas were getting away from her.” This attack was ushered in by a numbness of the forefinger and thumb of the right hand, which lasted for about three hours, though the earlier attacks had lasted for only about ten minutes. During this period the hand felt as if it had been frozen and the loss of muscular power was so great that she was unable to hold objects in the hand. In some of the attacks this paræsthesia has affected the entire left half of the body, and occasionally the right half. Sometimes the seizures come on with great suddenness, so that once, when she was attacked while in the middle of the street, she had considerable difficulty in reaching the sidewalk. After the worst part of the attack is over a certain amount of paraphasia may persist for some days, together with awkwardness in the use of the right hand and numbness. She has had a great deal of nausea and vomiting, without reference to the taking of food.[2]

Bearing in mind the mingling of structural with functional symptoms in this case, let us consider the autopsy findings.

=ANATOMICAL=

=FORMS OF NEUROSYPHILIS=

AUTONOMIC (SYMPATHETIC) NEUROSYPHILIS?

PERIPHERAL NEUROSYPHILIS

CENTRAL NEUROSYPHILIS MENINGEAL VASCULAR PARENCHYMATOUS MENINGOVASCULAR VASCULOPARENCHYMATOUS DIFFUSE ( = MENINGOVASCULOPARENCHYMATOUS)

GUMMA

CHART 1

=CLINICAL FORMS OF NEUROSYPHILIS=

=HEAD AND FEARNSIDES, 1914=

SYPHILIS MENINGOVASCULARIS CEREBRAL FORMS HEMIPLEGIA AFFECTION OF THE CRANIAL NERVES MUSCULAR ATROPHY LATERAL AND COMBINED DEGENERATIONS EPILEPSY

SYPHILIS CENTRALIS DEMENTIA PARALYTICA TABES DORSALIS MUSCULAR ATROPHY OPTIC ATROPHY GASTRIC CRISES EPILEPTIC MANIFESTATIONS

CHART 2

=Peripheral neurosyphilis=: The lesions of the cranial nerves were characteristically asymmetrical. Whereas the left third nerve looked entirely normal, the =right third nerve= had its diameter reduced two-thirds. On the other hand, the fourth nerves were equal and apparently normal. The sensory portion of the left fifth nerve was normal; the right fifth nerve was normal. The =right sixth nerve= agreed with the right third nerve in being atrophic, and was in fact reduced to a mere thread without contained nerve fibres at a point 2 mm. from its superficial origin. Although the right third nerve was atrophic, it was the =left seventh and eighth nerves= which had become atrophic; the process had spared the right seventh and eighth nerves. The remainder of the cranial nerves were grossly normal, except that the =optic nerves= had an outer zone of a translucent nature. So far, no spirochetes have been demonstrated in any portion of the nervous system of this case, but such asymmetrical and focal cranial nerve lesions are perhaps due to local spirochetal infection, punctuating (as it were) the diffuse process.

How much of the transient blindness, deafness, and ocular paralysis can be explained on the anatomical findings in these nerves? Possibly a portion of the phenomena can be so explained. Thus, the mechanical conditions of pressure inside and outside these nerves, both in their peripheral course and in their passage through the membranes, can be readily understood to differ during the acute and subacute inflammation, during the process of repair in the pial tissues, and during the process of overgrowth of neuroglia tissue about the superficial origins of the nerves. Of course, the majority of lesions of these nerves were entirely extinct at the time of the autopsy, and their history could be surmised only from the appearances in the _left eighth nerve_. Here occurred a sharply marked focal area of gliosis with apparently total destruction of nerve fibres and related with a _lymphocytosis_ of the investing membrane (one of the few areas of lymphocytosis found anywhere in this case).

If it were not for the pre-infective history, the hysterical dysphasia and dypsnea, the youthful obsessions, the migrainous tendency, and the psychopathic inheritance, we might be tempted to try to explain the transient blindness, the deafness, and ocular palsies on the basis of mechanical and toxic variations in the conditions of the peripheral cranial nerves. The existence of a trace of lymphocytosis in the left eighth nerve leads to the hypothesis that treatment might still be effective in this particular region (see below in discussion of spinal symptoms).

=Spinal neurosyphilis:= Not only the spinal cord but also the posterior and anterior nerve roots exhibited severe lesions. These lesions were both meningeal and parenchymatous. The meningeal process differed in its intensity in different parts of the spinal cord, being severest in the thoracic region. At one point in this region, the dura mater was so firmly attached to the pia mater that the line of demarcation between the two membranes was hard to make out. In fact, it seems clear that there could have been no free intercommunication between the spinal fluid above these adhesions of dura to pia mater and the spinal fluid below the adhesions. Accordingly, it seems that _lumbar puncture_, had it been practised in this case, _would have failed to show features representative of the whole cerebrospinal fluid system_. Moreover, since at no point in this region of adhesions or in the pia mater of the spinal cord below this point, were found any lymphocytes, it seems clear that the ordinary lumbar puncture would have failed to reveal a pleocytosis. Whether this fluid would have yielded a positive globulin and excess albumin test, it is now impossible to say; but it appears that the process in the lower part of the spinal cord was to all intents and purposes extinct.

However, there was one region of more severe inflammatory involvement. The _spinal cord in the cervical region showed a lymphocyte infiltration_ of its vessels amounting to a mild myelitis (meaning, thereby, an inflammatory process of the spinal cord remote from the pia mater). Moreover, in this region, there was, besides the perivascular infiltration of the substance, also an infiltration of the overlying membranes themselves, especially in and near the posterior root zones.

The lessons of this finding are several: The inflammatory process in this case does not appear to have been entirely extinct! Can we not suppose that treatment might still have benefited this local inflammation (perivascular infiltration of the cervical spinal cord substance and overlying lymphocytic meningitis)? Can we not also picture the gradual ascent of the inflammatory lesions from lower segments to higher segments and possibly conceive of the gradual elevation of the zone of hyperæsthesia manifested in this case as following the gradual displacement upward of the lymphocytic process? Are there spirochetes in this tissue? So far none have been discovered, possibly through inaccuracies of available technique. To the neuropathologist, however, the lesion looks like a local reaction to organisms.

In addition to the spinal meningitis, chronic and acute, as above described, there were extensive parenchymatous spinal lesions.

In the first place, the meningitis had affected practically all the posterior roots so that the explanation of the posterior column sclerosis of this case is clear. The meningitis had apparently been so marked, also, that all the fibres anywhere near the periphery of the spinal cord had been likewise destroyed. The posterior columns and the posterior root zones were markedly sclerotic; or as we say (having reference to the overgrowth of neuroglia tissue) gliotic. But there was as much sclerosis (gliosis) of the lateral columns (particularly in the posterior two-thirds) as there was in the posterior columns and root zones. In fact, the entire posterior half or two-thirds of the spinal cord markedly outstripped the anterior portions of the cord in the severity of the gliosis (sclerosis) shown.

But although we can explain the posterior column sclerosis, the sclerosis of the posterior root zones and the marginal sclerosis (_Randsklerose_) round the entire periphery of the cord, on the basis of long-standing effects of old meningitis, we cannot thus explain another finding, namely, the destruction of the fibres in the lateral columns. This, in fact, is explained through lesions (mentioned below) that affected the encephalon. The net result of all these lesions of the spinal cord was to leave only the gray matter and a small amount of surrounding fibres (belonging to short tracts uniting nearby segments) intact. Briefly stated, =every long tract in the spinal cord appeared upon examination to be extensively degenerated=. The genesis of this parenchymatous loss was, however, double, being in part due to a local meningeal process (sometimes known as “perimeningitis”) and in part due to a cutting off of the pyramidal tract fibres on both sides by lesions higher up in the nervous system.

=ANATOMICAL FORMULAE=

=MENINGOVASCULOPARENCHYMATOUS INVOLVEMENT=

M, V, P, or Combinations Applied to the Classification of Head and Fearnsides

I. SYPHILIS MENINGOVASCULARIS CEREBRAL FORMS M or V or MV[3] HEMIPLEGIA V AFFECTION OF THE CRANIAL NERVES M MUSCULAR ATROPHY M LATERAL AND COMBINED DEGENERATIONS M EPILEPSY M or V

II. SYPHILIS CENTRALIS DEMENTIA PARALYTICA MVP or VP TABES DORSALIS MP MUSCULAR ATROPHY P OPTIC ATROPHY P GASTRIC CRISES (M? or) P?

EPILEPTIC MANIFESTATIONS P?

CHART 3

Can we offer any explanation of the =partial return of knee-jerks= after their temporary total loss at a certain period of the disease? We may assume that the knee-jerks were functionally lost about a year before the death of the patient through the partial or even almost complete destruction of the entering posterior root fibres at that level of the spinal cord which is directly related with the knee-jerk. The later partial return of the knee-jerks apparently requires us to suppose the maintenance of some fibres and collaterals by which a functional connection can be effected between the fibres of the posterior roots and the anterior horn cells which innervate the quadriceps femoris. Let us now suppose that _pari passu_ with the actual return of the knee-jerks, the destructive processes that are affecting both pyramidal tracts high up in the nervous system are now advancing. It is clear that, whatever inhibitory influence these pyramidal tracts have been exerting up to this time upon the knee-jerk reflex arc, that influence is now to be decidedly reduced in amount and possibly absolutely lost. Upon the loss of such inhibitory influences exerted from above, the few persisting connections of the posterior roots and anterior horn cells are now permitted to resume their functions.

=Encephalic neurosyphilis=: The lesions mentioned above as causing destruction of the pyramidal tracts of the spinal cord were symmetrically destructive and atrophic lesions of the gray matter of both corpora striata with atrophy of the anterior segments of the internal capsules. There was a degenerative process of the corpus callosum especially affecting the forceps minor of the tapetum. The ventricles were largely dilated, indicating a considerable destruction and atrophy of the white matter in general.

After the above discussion of the possible effects of pyramidal tract lesion in this case, it is unnecessary further to discuss the paraplegia produced by the cystic lesions of the corpora striata. The theorist might inquire how these cystic lesions are produced: whether by vascular blocking or by toxic effects of the accumulations of spirochetes. Evidence is lacking which would completely sustain either hypothesis. Still, we do know that lesions almost identical in appearance may be produced by the necrosis consequent to the plugging of nutritive vessels in an organ like the brain supplied with end arteries. Therefore, it is probable that most pathologists would believe these lesions of the corpora striata to be produced by vascular plugging of the nature of thrombosis.

It is worth while to note that there was a suggestion of foci of encephalitis made out upon the gross examination. The cortex in general showed strikingly few lesions. However, the convolutions did show in places numerous ill-defined areas of hyperemia and slight swelling. These areas were of irregular distribution and only a few mm. or cm. in diameter. No gross vascular lesions were demonstrable in connection with these focal areas. Microscopically, however, venous plugs of polymorphonuclear leucocytes were found, and the local hyperemias were found to be largely due to venous congestion. However, very few polymorphonuclear leucocytes were found outside the blood vessels.

The white matter of numerous convolutions showed microscopically certain pale spots suggestive of an early atrophic process. Very possibly these represent a general tendency in the cerebrum to the same process of parenchymatous loss which had proceeded to such a marked degree in the spinal cord.

There was a single large so-called cyst of softening in the cerebellum (1.5 mm. across by 0.5–7.5 cm. in depth).