prominent features of the malady. Sometimes the initial feature consists of attacks of pain of the stabbing, lancinating variety. The advance of the disease may be chronically progressive, or it may show exacerbations or remissions, and continue finally to advance. Sometimes the amelioration is permanent and recovery is possible. In progressive cases death may result from intercurrent maladies, from an apoplectiform attack, from bulbar accidents, or from the exhaustion of terminal dementia.

The duration of the disease, like its anatomical basis and its symptoms, is very variable. It may reach full development within a few months or a year, rarely almost at a bound. It may terminate in a year, last twenty, or recede completely at any time.

In some instances only one or a few of the most notable symptoms of the disease ever develop. These cases constitute the formes frustes of Charcot. Thus the trembling, or the speech defect, or the gait with or without nystagmus, or bulbar symptoms and amyotrophy may predominate, while the other symptoms are only very slightly developed or entirely absent.

Diagnosis. In a well-developed case the diagnosis is easily made. The intention tremor, cerebellospasmodic gait, spastic weakness, nystagmus, and syllabic speech make a distinctive picture that should be recognized at a glance. The differentiation from family ataria may, however, present great difficulty when several members of the same family are affected with multiple sclerosis. Friedreich's type is marked by flaccidity and abolished knee-jerks, the motor difficulty is purely ataxic and never spasmodic, and optic-nerve symptoms are practically always absent. In the heredocerebellar type of family ataxia there are increased reflexes and often optic atrophy, but a long family succession of cases rarely fails us, and the motor difficulty here again is devoid of spasticity and is purely ataxic. In neither type do we find the well-developed syllabic speech nor the intention tremor, though the speech is modified and ataxic and there are choreic movements which might at first mislead.

Hysteria is capable not only of mimicking every objective symptom of insular sclerosis, but sometimes is associated with it in the same patient. It should never be out of mind in making the diagnosis, and the stigmata of the neurosis should be faithfully sought in every instance. Cases showing hysterical signs and the symptoms of multiple sclerosis should be looked upon as probably organic if the tendon reflexes are exaggerated and the plantar reflex is abolished. 1

In the partial cases, mainly marked by one of the prominent characteristics of multiple sclerosis, the diagnosis is much more difficult. In every such instance all the features of insular sclerosis should be sought, and some of them, if only partially developed, will be found to fix the diagnosis, or the presence of signs of other diseases will guide. When trembling predominates, we must exclude paralysis agitans, metallic intoxications, chorea, and hysteria. When the speech defect is most prominent, we must think of paretic dementia and the family ataxias.

1 Buzzard, "Simulation of Hysteria by Organic Disease of the Nervous System," London, 1891.

In cases showing a predominance of cerebellar ataxia intracranial tumors and the family ataxias must be excluded. When the gait is purely spastic, we must exclude transverse myelitis and the combined scleroses of the cord.

Prognosis. While the outlook in a case of multiple sclerosis is always grave, the tendency to remissions and the occasional complete recession of the disease make it distinctly more hopeful than in the destructive sclerotic lesions of tabes and cross-myelitis. It is impossible in a given case to forecast its probable course so long as it is progressing. Apoplectiform seizures followed by paralytic features and aggravation of all the symptoms may occur at any moment; but when the disease has shown remission and improvement we are justified in hoping, with due reservation, for better things.

Treatment. In every case the nature of the origin of the malady will guide therapeutics. If the disease is to be considered as mainly due to infections and to the continuous activity of microbic life, which from time to time leads to new plaques or increases old foci, bactericidal preparations are indicated-quinin in malaria; mercury, iron, arsenic, and salicyl preparations in other septic conditions. Generally speaking, an antisepticemic régime is in order. This may only be valuable when the general resistive powers of the economy are increased by the best hygienic and general health measures. The future may furnish some organic antitoxin remedy more powerful than our present medicaments.

CHAPTER II.

SYPHILIS OF THE NERVOUS SYSTEM.

THE syphilitic lesions of the nervous system, especially of the central apparatus, are of extreme frequency and of almost infinite variety. In this section little more can be attempted than an enumeration of them, with special reference to clinical peculiarities and the most efficacious mode of treatment.

Syphilitic nervous disease may be divided into (1) those due to the active ravages of the virus and (2) those which follow the infection and are not marked by neoplastic products. The one is specific, the other parasyphilitic. The first is microbic, the second toxic or chemical. Such a view of luetic lesions and consequences gives us the best working basis at the present time. Syphilis is capable of transmission to offspring in either form, and we have some hereditary syphilitic diseases of the general nervous system marked by the ordinary gummatous processes, and others due solely to faults of nutrition and toxic action. Again, both the syphilitic and parasyphilitic diseases may be present in

the same individual at the same time or at different periods. The following tabulation may serve to systematize the subject :

Specific lesions of the general nervous system, like specific lesions elsewhere, always originate in the connective tissue or blood-vessels and secondarily affect the parenchyma, disturbing function first and finally destroying the essential cells and fibers of the part. Rarely they arise in bony structures and thence invade the adjoining nervous apparatus or act upon it by pressure. Their peculiarity is in the gummatous plastic exudate and its formative tendency. It is essentially a neoplastic process, which may vary from an appearance of simple inflammation to the accumulation of sizeable tumors. In early stages there is often a marked tendency to regressions and recidives. Old lesions, if of considerable size, may caseate within and cicatrize upon the surface by fibrous organization and fibroid changes may alone remain to indicate the preexisting gummy condition.

Acquired syphilis affects the central nervous system by specific lesions in a very considerable proportion of all luetic cases. Hjelmann, leaving out of the question both tabes and paretic dementia, states that from 1 to 2 per cent. of all cases of syphilis develop cerebrospinal lesions, and that of those presenting the tertiary features of the infection, twelve per cent. show invasion of the cerebrospinal axis. Fournier puts this latter percentage at twenty-one. In the great majority of cases the syphilitic process is wide-spread and somewhat haphazard in its location. Very rarely do cases of spinal syphilis lack evidences of cerebral involvement at some period, but cerebral syphilis, on the contrary, is sometimes devoid of spinal complications. We may, for descriptive purposes, divide these cases of acquired active syphilitic lesions into the cerebral and the spinal. Their common association should never be forgotten.

CEREBRAL SYPHILIS.

Syphilitic cerebral meningitis is perhaps the most frequent form of cerebral syphilis, and usually, to some extent, complicates all other varieties of encephalic lues. The favorite location is the basilar area, especially the interpeduncular space, from which it frequently extends upward on to the convexity of the parietal, and particularly of the frontal, lobes. So commonly is the base affected that, speaking clinically and according to Charcot, it may never be considered exempt, even when the symptoms point to the convexity alone. The distribution recalls that of tuberculosis. A gelatinous transparent substance infiltrates the soft meninges about the circle of Willis and the optic chiasm. It is composed of small, round, embryonic cells, and is very vascular and viscoid. Cascation and sclerotic degeneration mark the lesion later. The adjoining or enveloped nerves and vessels may be injured. Neuritic atrophy on the one hand and obliterating arteritis on the other are thus set up. Extension of the process to the nerve-sheaths and interstitial structure accounts for the very frequent symptoms on the part of the cranial nerves in cerebral syphilis.

C

In the field of syphilitic meningitis gummy masses, or gummata, are usually encountered. They vary in size from miliary bodies to tumors as large as filberts, which may be solitary, few, or many in number. Their favorite location is at the base or on the convexity, especially on the frontal lobes, but no portion of the brain is exempt. Sometimes they form on the surface or in the thickness of the dura mater, or deeply within the brain, on some septal infolding or penetrating blood-vessel. They comport themselves as do brain-tumors generally, and give rise to similar localizing signs and symptoms. Sclerous or fibrous degeneration marks the later stages of all these specific neoplastic lesions, and gives rise to what is sometimes called sclerous specific meningitis.

Fig. 184.-Ocular palsy in a case of brain syphilis in which at one time there was a crossed hemiplegia, right face and left extremities. The right eye overacts, turning upward and outward in looking to the right.

Syphilitic cerebritis may exist as a direct extension from a specific meningitis or gumma, or may occur more or less independently. It may present itself as a diffuse, gummatous encephalitis, or as localized encephalic gummata which tend to caseate, or it may appear in circumscribed patches or plaques, especially in the pontine and peduncular surfaces, which may end in sclerous patches. Every syphilitic process in the brain entails a surrounding zone of cerebral softening or cerebritis.

Syphilitic Cerebral Arteritis.-Syphilitic endarteritis in the brain is a comparatively common accident. This may result in the obliteration of the large basilar vessels secondarily to a gummatous menin

gitis, or it may develop as a primary locus of syphilitic activity. Its legitimate result is a localized ischemia, which, if complete, results in thrombotic brain-softening. Heubner insists upon its frequency in the basal ganglia. The middle cerebral arteries appear also to be frequently affected either in the numerous small branches of the Sylvian or by the complete obliteration of this motor-zone vessel.

It is also now established that cerebral hemorrhages may follow a specific arteritis. This rarely occurs within the brain-substance, but more often takes place in the large and comparatively unsupported basilar vessels, resulting in a foudroyant apoplectic stroke almost immediately fatal.

Syphilitic Lesions of the Cranial Nerves.-Owing to their exposed position at the base, where specific cerebral invasion is most common, the cranial nerves are very commonly implicated. The relative frequency with which they are affected diminishes from the chiasm and optic nerves backward toward the bulb. The order is as follows: Optic nerves, motor oculi, abducens, trifacial, facial, auditory, and only rarely the glossopharyngeal, vagus, and hypoglossus.

The motor oculi, or third nerve, is affected in about forty per cent. of the cases, and shows a particular vulnerability on the part of the fibers supplying the levator of the upper lid. Hence the frequency and significance of ptosis. Inequalities of the pupil and pupillary stasis are of equal importance and frequency. Referable to the affection of the chiasm and optic nerves and tracts we find all forms of irregular and unequal contractions of the visual fields. Optic neuritis is very frequently present and atrophy may result. The trifacial may be involved in one or all its branches, causing disturbed sensibility sometimes, but more frequently neuralgic pains of corresponding distribution. Infrequently we have facial palsy, and in rare cases this is associated with deafness on the same side.

The amount of injury to a nerve varies within wide limits, but often presents the suggestive peculiarity of advance and recession, giving rise to temporary, fugaceous palsies and temporary sensory features, such as amblyopia, lasting a day or two. After several such attacks the disability is likely to persist permanently and indicates a destructive change in the nerve, which commences as an interstitial infiltration and neuritis.

The general symptoms of cerebral syphilis are modified by the particular lesions that are present. These, as a rule, are multiform, but with special prominence of certain features. One case attracts most attention by its eye symptoms, another by its neuralgic pains, another by its convulsive manifestations, another by its stupor or mania or paralysis.

There is always, or nearly always, an initial period—a premonitory period that it is of the greatest importance to recognize. Then only does medication offer a fair chance of effecting a cure. Once passed, irreparable damage is done, and the best that can be hoped for is to check further mischief and leave the brain and nerves scarred with cicatricial tissue. Brain-syphilis appears, ordinarily, during the secondary period, but may occur at any date after the first few months. Thus the first