THlE

4i95TM GI,FADUA~TEMEDICAw JOu1?NALVOL. IV. DECEMBER, I928. No. 39

CONTENTSPAGE

rHE SECONDARY FORNIS OF MENTAL DEFICIENCY ... ... ... ... ... 37By R. M. STEWART, M.D., Ch.B., M.R.C.P.Edin., ).P.M.

POST-GRADUATE LEC TURE ON FRACTURES, WITH EXP[.ANATORV X-RAYPHOTOGRAPHS, ILLUSTRATIONS AND APPARATUS ... ... ... 51

By WILLIAM TURNER, M.S., F.R.C.S.

EDITORIAL NOTES ... ... .... .. ... 53

POST-GRADUATE NEWS ... ... ... ... ... ... ... ... ... 54

FELLOWSHIP OF MEDICINE AND POST-GRADUATE MEDICAL ASSOCIATION.-SPECIAL COURSES ... ... ... ... ... v

THE SECONDARY FORMSOF'

MENTAL DEFICIENCY.!y R. M. STEWART,

M.D., CH.T., M.R.C.P.EDIN., D.P.M.

MENTAL defect may properly be regardedas a disorder of the mind, and in approach-ing its study we find the making of a satis-factory classificationi just as difficult as inthe wider domain of psychiatry. In theearly part of last century menital defectiveswere classified largely on a physical basis,their ancatomical defects or- stigmata beingused for the invention of racial types-theKalmuck, Aztec, Papuani, and so on. At asomewhat later date a niew classification wasattemptecl on the basis of pathological

anatomy, anid this in its tuLrni has beeni suLper-seded by a scheme evolved from the statnd-poilnt of causation, which divides ameintiainito twNo classes: the primary forms, deter-mined by nieulopathic inheritanice; and theseconidary formis, determinied by acquireclfactors operating before, at, or after birth.Thie for-mer at-e said to accounlt for go percenit. and the latter io per cent. of all cases,but in my experienice too much emphasishas beeni placed oni a history of mentaldefect in the parents or niear anicestors, andI believe that future resear-ch will show thatmiiore than- a quarter of all cases of mentaldefect are due to acqulired factors.

In actuLal practice it is niot always easy, oreveni possible, to determin-e whether mientaldefect is innate or- acquired, and doubtlessin the causation of some cases botli giolups

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THE SECONDARY FORMS OF MENTAL DEFICIENCY

of factors are concerned. None the less,the division of amentia into primary andsecondary forms meets clinical demandsfairly well, and is probably the most usefulfor descriptive purposes.

At the outset it should, perhaps, beemphasized that the life history of the in-dividual commences, not with birth, butconception, so that for the first nine monthsthe child, though not leading an independentexistence, is none the less exposed tonumerous extraneous influences. This ante-natal period is capable of subdivision intothe period of embryonic life, marked by a

forms of mental deficiency sustained at thisperiod.

Thirdly, there is the post-natal period, inthe course of which the brain of the growingchild may be damaged by the toxins ofacute infections, by the rupture of veinsduring convulsions, by direct trauma, andso 011.

Returning to the pre-natal period, whenthe nourishment of the foetus is effectedthrough the placental circulation, it is logicalto assume that nletabolic disturbances ordisease in the mother may also injuriouslyaffect the brain of the offspring. Certain

FIG. 1.

phase of rapid development, and the periodof fcetal life, which is principally concernedwith growth. Doubtless, at any period ofintra-uterine life the brain is liable to acci-dents which may impede or arrest its forma-tive processes, but it is especially duringthe second period, when the foetus becomesnourished by maternal blood, that the com-mon types of gross lesion occur. At biiththere is further possibility of damage to thebrain. Difficult and prolonged labour, in-strumental delivery, and especially asphyxianeonatorum, are considered to be importantagents in the catusation of certain paralytic

fevers, and notably small-pox, are known tooccur in utero, and may possibly be thecause of arrest of brain development, buton this point we have as yet little exactknowledge, and for an undisputed andclassical example of intra-uterine infection itis necessary to fall back on syphilis. Mostobservers are agreed that this disease haslittle influence as an ante-conceptional causeof mental deficiency, but an infection inearly uterine life may unquestionably beresponsible for arrested development of thebrain and the production of feeble-minded-ness, if it does not kill the foetus outright.

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THE SECONDARY FORMS OF MENTAL DEFICIENCY

To what extent must syphilis be held re-sponsible for mental deficiency is still amatter of considerable controversy. Re-garded by some as merely a concomitant ofno greater significance than, say, an infectionwith the tubercle bacillus, by others theSpirochceta pallida is assigned a most im-portant part in the genesis of amentia.

Sir Frederick Mott, in his evidence beforethe Royal Commission on the care andcontrol of the feeble-minded, expressed theopinion that, " Syphilis is an active agent in

become negative when the child reachesadolescence, and consequently, in compar-ing statistics from different sources, mis-leading conclusions may be arrived at unlessthe ages of the patients investigated areknown. The results may also be expectedto vary according to the social status of thegroup examined, for in an institution filledwith the children of slum dwellers, theincidence of congenital syphilis is likely tobe higher than in a private home for defectivechildren of the upper classes. Probably

FIG. 2.

the production of congenital weakness andthe degeneracy that accompanies it." Otherworkers have found no reliable evidence tosupport such a view, and Goddard goes sofar as to say, "One cannot help having thefeeling that if syphilis is a cause of feeble-mindedness, it does so only under mostfavourable conditions." The evidence de-rived from the application of the Wasser-mann reaction is equally conflicting, andcan, I think, only be explained by a differ-ence in the quality of the material investi-gated. In my experience age is a factor ofsome importance, as a positive reaction may

also the type of case investigated is of someimportance, the incidence of syphilis beinglower in a series of unselected cases than ina similar number of selected, on account ofspecial physical disabilities-infantile palsies,and so on. A few years ago I investigated800 patients in the Leavesden Mental Hos-pital, and obtained a positive Wassermannreaction in I9'75 per cent. The majority ofthe patients in this hospital are in the lowergrades of mental deficiency, many of thembeing afflicted by physical infirmity, parti-cularly nervous disease. It cannot, there-fore, be claimed that the cases in this study

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THE SECONDARY FORMS OF MENTAL DEFICIENCY

were unselected, but the results approximatevery closely to those of my colleague, Dr.Gordon, who in a recent investigation of575 mentally defective found a positiveWassermann in i5'65 per cent.There is, I think, no doubt that congenital

syphilis has undergone considerable modi-fication in type since the beginning of thiscentury, possibly because the infection is ofa milder type. The more usual clinicalsigns such as rhagades, Hutchinsonianteeth, and eye changes, form a clinicalpicture which is only seldom met with, andstatistics based on the presence or absenceof the classical signs will lead to a veryerroneous idea of the incidence of thesyphilis taint. In a series of 173 casesshowing a positive Wassermann, I was onlysuccessful in finding two who presented theclassical triad of bilateral eighth-nervedeafness, keratitis and notched incisors.Stigmata of congenital syphilis may indeedbe entirely absent. On the other hand,in late congenital syphilis the Wassermannreaction may be negative, although thepatient presents obtrusive signs of thedisease. In looking for evidence of syphi-litic infection, much information of dia-gnostic value can often be obtained froman examination of the larger joints.Thickening and broadening of the bonystructures in their neighbourhood is verysuggestive of congenital syphilis, particularlyin the case of the elbow-joint, where there isoften a limited range of movement, or anabnormality of the carrying angle to whichStoll has given the term "knock-kneeelbow."

THE PATHOLOGICAL ANATOMY OFSYPHILITIC AMENTIA.

The cerebral lesions of syphilitic amentiaare not well known, and the following briefexposition may therefore be of some interest.A certain number of developmental ano-malies, such as anencephaly, porencephalyand microcephaly, have been recorded, andin a few cases uncomplicated by paralysis

I have been able to find imperfect develop-ment and numerical deficiency of thecortical nerve cells. Usually, however,when syphilis affects the nervous systemof the foetus, it does so in an unmistakablemanner, the pathological changes being ofa gross character easily recognizable by thenaked eye.The lesion most often found is of the

meningo-vascular type, and though thebrain may show marked damage in oneparticular area, multiple diffuse affectionsare the rule. Pathologically, these resemblefairly closely the changes found in adult

FIG. 3.

syphilis, but the alterations in the meningesand vessels have seldom the cellularcharacter which distinguishes a progressivesyphilitic process, and vascular occlusionleads to a sclerosis rather than a softeningof: cerebral convolutions. Depending onthe stage at which it develops, the syphiliticlesion may be accompanied by secondaryatrophy in those portions of the nervoussystem with which the affected areas arefunctionally related. The characteristicdiffuseness of the syphilitic process is wellillustrated by the following two cases.

Fig. I shows the appearance of thecerebral hemispheres of a syphilitic idiot,

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THE SECONDARY FORMS OF MENTAL DEFICIENCY

hemiplegic from birth, who died in hertwenty-first year. The left cerebral hemi-sphere is smaller than the right, and thepia-arachnoid covering both much thickened.Certain of the gyri above the left Sylvianfissure are shrunken and sclerotic(fig. 2), andon coronal section the left lateral ventricleis found to be dilated (fig. 3). Both theinternal capsule and lenticular nucleus havebeen largely destroyed by an old softeningin their neighbourhood. At the base thereis a marked degree of leptomeningitis, thearteries are thickened and the cerebellumshows a crossed atrophy of its righthemisphere.

FIG. 4.

The second case (fig. 4) is that of a syphi-litic idiot with right hemiplegia. The brainis small and shows a chronic leptomeningitisaffecting its entire surface. All the con-volutions behind the right precentral gyrusshow the appearance known as microgyria(fig. 5), and on the left side the lower two-thirds of the motor cortex are atrophied andmuch firmer than other portions of thecortex (fig. 6). For the latter condition itis convenient to employ the term secondarymicrogyria. In both these patients theblood Wassermann was strongly positive,but the spinal fluids showed practically nodeparture from the normal. This seems to

FIG. 5.

be quite the usual serological finding insyphilitic aments who survive to adolescenceor adult life, and may, I think, be interpretedas indicating a non-progressive form oflesion. In contrast to the parenchymatousforms, the damage to the brain is sustainedat an early period, the spirochaete dies orbecomes latent, and the patient is left withpermanent damage to the nervous system.Certain writers have expressed the view thatthe amentia of syphilis terminates in thedevelopment of juvenile general paralysis,but this is certainly not my experience, andI have seen only one patient in whom thispossibility is at all likely. The patient is anepileptic idiot, aged 25, who, in addition toa positive Wassermann reaction in his bloodand spinal fluid, gives a typical paretic curvewith the colloidal gold test. It is now nearly

FIG. 6.-Pseudo-microgyria.

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five years since the tests were performed,and as yet there are no other signs suggestiveof general paralysis.The mental enfeeblement which accom-

panies congenital syphilis may be of anydegree, though as a rule those who exhibitparalysis are low-grade imbeciles or idiots.Owing to the multiplicity and diffuseness ofthe lesions the paralysis, when present, mayassume many different forms. An investi-gation of 173 syphilitic aments at Leavesdenrevealed 2 with tetraplegia, 12 with diplegia,17 with paraplegia, i8 with hemiplegia, and4 with hydrocephalus. Syphilitic amentsare frequently epileptic, and in the seriesquoted above 48 per cent. were subjectto fits.

Treatiment.-I have no experience in thetreatment of very young syphilitic aments,but in the adolescent stage the results aredisappointing, and I have never seen anymental improvement follow treatment by thevarious arsenical preparations commonly inuse. Nevertheless, the recognition of thistype of amentia is not without value, for itdoes occasionally permit one to arrest at anearly stage lesions which, if untreated, leadto gross disfigurement.. Unless the possi-bility of syphilis is borne in mind, syphiliticbone disease may be mistaken for that oftuberculosis, and indolent ulcers on theextremities may be wrongly ascribed to theinfluence of circulatory defects. Syphiliticaments, and especially those with paralysis,benefit very little from the exhibition ofbromides or luminal, but in some, treatmentwith the arsenicals diminishes the numberand severity of the fits in a gratifyingmanner.

JUVENILE GENERAL PARALYSIS.Although juvenile general paralysis may

not develop until puberty, or even later, theinvasion of the nervous system by theorganism of syphilis probably takes place atan early age, and the disease may thereforebe regarded as a form of secondary amentia.It is by no means so rare as was once thought,

and in the last few years I have seen elevencases. From the adult type it differs in thatthe duration is usually longer and remissionsare practically unknown.The usual history is of a gradual mental

and physical deterioration, occurring atschool age and in some cases initiatedby convulsions. The child becomes in-creasingly stupid, indifferent to its sur-roundings, dirty in its habits, and eventuallybed-ridden with contractured limbs, markedemaciation, and in fact all the classicalfeatures which distinguish the third stageof the adult type. Neurological signscharacteristic of general paralysis are in-

FIG. 7.

variably present, and persistent grinding ofthe teeth is in my experience a symptom ofsome diagnostic value. As in other formsof syphilitic amentia, stigmata of congenitalsyphilis may be entirely absent, and not afew of the cases I have seen have hadquite pleasant features (fig. 7). In the elevenpatients I have had under my care, fourwere mentally deficient from birth, and sevenshowed no failure of intellect until puberty,or even later. In one case optic atrophywas the first symptom, and in two transientparalysis occurred at the age of 6 years. Withregard to the microscopic appearances, spiro-chaetes are usually abundant in the cere-

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THE ECONDARY FORMS OF MENTAL DEFICIENCY

bral cortex; otherwise the morbid histologyresembles that of the adult form.The prognosis in juvenile general paralysis

is bad. Treatment by induced malaria haslittle or no effect on the mental state of thepatient, though it does appear to prolong life.One patient whom I treated five years agois still alive, bereft of all intelligence andhelpless in every way.

CRETINISM.Cretinism or congenital myxcedema is

caused by the loss of the secretion of thethyroid gland, and the mental deficiencywhich constitutes one of its chief symptomsis the direct consequence of that loss. Inspite of the absence of the thyroid, typicalsymptoms are not usually present at birth,probably because the autocoids from themother's thyroid are conveyed through theplacenta, and after birth through her milk.Being improvable, cretins often come firstunder notice in general hospitals, and thenumber admitted to institutions for thementally defective is very small. Thephysical signs present a certain uniformityof type and are not easily confused withthose of any other disease. The body isstunted, podgy and heavy-looking. Thehead is large in proportion to the size of thebody and is dolicocephalic. The sub-cutaneous tissue is infiltrated with solidcedema which imparts to the face its heavyexpression. The cheeks, are fat and baggy,the complexion earthy, the nose pug-shaped,the eyes set far apart, the tongue large andprotruding, and the teeth carious. Elasticswellings may be present in the supra-clavicular regions and axilla. The largependant abdomen frequently shows anumbilical hernia. The hair is abundant,but coarse and dry. The limbs are short,broad, and often rickety. Many cretins aredeaf and dumb, and in some the lower limbsare paralysed. These appearances are wellseen in the accompanying illustration (fig. 8)of a male sporadic cretin, aged 25. The

patient, of Polish origin, was foundabandoned in London, and on admissionmeasured 3 ft. 2 in. in height. He had adental age of 12 years and weighed 4 st. 3 lb.He was a profound idiot, unable to articulateor to walk. He had a positive Wassermannin the blood and a number of dischargingsinuses were present in the neck and rightleg. After a year's treatment with thyroidextract his weight fell to 3 st. and hebegan to take some interest in his surround-ings. His clinical history was brought to anabrupt termination by the sudden develop-

FIG. 8.

ment of thymic symptoms. At the post-mortem examination we found extensivesyphilitic lesions in the heart, aorta, andliver. The thyroid gland was representedby a small mass not larger than a pea which,on microscopic examination, was seen toconsist of a few small vesicles with anepithelial covering of cubical cells and acertain amount of colloid material (fig. 9).The brain of the cretin is large, heavy, andsimply convoluted; the cortical neuronesare imperfectly developed and binucleatedcells not uncommon. Masses of iron-freepigment lying in relation to the corticalvessels seem to be a characteristic feature inthe disease.The degree of mental defect in the spor-

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44 THE SECONDARY FORMS OF MENTAL DEFICIENCY

adic cretin varies from profound idiocy toslight mental enfeeblement. As a rule heis apathetic, placid and good-tempered,though if provoked he may show outburstsof violence. The amount of mental im-provement depends very largely on the ageat which treatment is commenced. Infantcretins may attain a normal mental level,but in those of a maturer age the mentaldevelopment is never commensurate withthe physical, some degree of feeble-minded-ness being always the end result. Occa-sionally the alteration in the mental state isnot altogether in the direction one would

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FIG. 9.-Thyroid of Cretin.

wish, for I have seen a sluggish, inoffensivecretin transformed by thyroid treatment intoa vicious lout, who used his new-foundstrength for tyrannizing weaker patients anddemolishing the ward furniture.

It is, of course, essential to keep up thetreatment throughout the patient's life, andit is interesting to note that treated cretinsseem to age very slowly, retaining thephysiognomy of youth well into adult life.The patient whose photograph I show you(fig. IO) looks like a child of io, though heis actually in his twenty-fifth year.

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B....

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;r.l·,il.:fa.r,:iir-r.*.···

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FIG. 10.

This case may also be used to illustratewhat may happen at the beginning ofthyroid treatment if steps are not taken tosafeguard those bones which have to bearthe weight of the body. When the treat-ment was commenced the boy had perfectlystraight limbs, but in a few months theybecame bent into the position illustrated(fig. 11), and all subsequent efforts to correctthe deformity have proved unavailing.

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FIG. 11.

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MACROCEPHALY.The first variety to be considered in the

group of large-headed or macrocephaliccases is the condition known as hydro-cephalus.The essential feature of internal hydro-

cephalus is an excess of fluid in the cerebralventricles which become distended, some-times to an enormous degree. The causevaries in different cases, but the greatmajority are a sequel of meningitis in theneighbourhood of the fourth ventricle,which blocks the foramina of Magendieand Luscka so that cerebro-spinal fluidcannot escape to the points of absorption,the microscopic villi in relation to the largevenous sinuses. Fig. 12 illustrates the

FIG. 12.

appearance of the floor of the fourth ven-tricle in a case of obstructive hydrocephalus.Owing to the marked thickening of theependyma, no details of structure can bemade out on the floor which is traversedby two abnormally-placed vessels. Thepatient was an epileptic imbecile, who diedof fits in his thirty-third year. His headmeasured 70 cm. in circumference, and inaddition to somatic signs of congenitalsyphilis he had a positive Wassermannreaction in both blood and cerebro-spinalfluid.More rarely the ventricular foramina are

freely open, but the absorbing power ofthe arachnoid space is diminished, or the

cerebro-spinal fluid is prevented from reach-ing the absorbing surface by adhesions atthe base of the brain.

In a typical case the ventricles are widelydilated, the corpus callosum is reduced to athin membranous sheet, and the cerebralhemispheres are converted into two largevesicles which collapse when the ventriclesare punctured. The sulci are diminishedin depth, the convolutions are broad andflattened, and the atrophy is sometimes sopronounced that the cortex and underlyingwhite matter have a thickness of only a fewmillimetres. Clinically the appearance ofthe hydrocephalic is very striking. Thecranium is much enlarged, bradycephalicand globular in form with the greatestcircumference at the temples. The heightof the forehead is exaggerated, the eyeswidely set with sclerotics showing abovethe iris. Percussion of the skull is accom-panied by an increased feeling of resistance,and the scalp is thin and tightly stretched.When the degree of hydrocephalus ismarked, there is often interference withsight and hearing, and spastic paraplegiais not uncommon.

Hydrocephalic patients are usually placid,affectionate, and the least troublesome ofall aments. In spite of the fact that thecerebral hemispheres may be extremelyatrophied, the mental defect rarely exceedsthat of imbecility, and many hydro-cephalics respond well to training and canbe taught to occupy themselves with simplehandicrafts.One patient under my care, whose head

measures 271 in. in circumference, has anexceptionally good memory for the ordinaryevents of hospital life and an extraordinarycapacity for calculating dates.

If, for example, he is given any date, aftera few moments' pause, in which he rocks hisbody to and fro, he is able to state the dayof the week correctly. I have tested thiscapacity for calculating dates as far back asDecember 25, 1812, and have very rarely

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found him make a mistake. His pheno-menal aptitude in this direction is all themore remarkable, as he is totally unable toperform simple problems in arithmetic.He cannot, for example, multiply three byeight nor add together the number of daysin two months.

DIFFUSE SCLEROSIS.A second type of macrocephaly is that in

which there is enlargement of the entirebrain without distension of the ventricles.Diffuse sclerosis, as the condition is termed,is decidedly rare and not very easy to dis-tinguish from hydrocephalus. The dia-gnosis rests on the observation that inhypertrophy the enlargement of the skull ismost pronounced above the superciliaryridges, and the head is square rather thanround. The pathological basis appears tobe a diffuse neuroglial sclerosis whichrenders the consistence of the brain muchfirmer and heavier than normal. Patientswith cerebral hypertrophy are usually of lowmentality and are subject to epileptic fits,muscular weakness, and tremor.

TUBEROUS SCLEROSIS.

Although this disease is not associatedwith enlargement of the head, it belongs tothe forms of sclerotic amentia and mayconveniently be considered here. Clinically,tuberous sclerosis is characterized by theoccurrence of epilepsy in conjunction withmental defect.According to Freedman, the disease begins

between the fourth and seventh months offoetal life and is neither familial norhereditary.

In a typical case the brain is studded withsclerotic nodules, which on the cortex areslightly raised, umbilicated and yellowish-white in colour. Sub-ependymal tumours,rather like candle gutterings, are found inthe ventricles, and multiple tumours arecommon in the heart, kidneys, skin andother organs.The disease usually begins in early child-

hood with the development of epilepsy, andwhen once initiated the fits become graduallymore and more severe and are often thedirect cause of death.

Mental enfeeblement may be present fromthe first, or the patient may be normal forseveral years and capable of attending schoolfor a few years, but sooner or later signs ofdeterioration are noticed.The child ceases to take interest in its

toys, loses the power of attention, andbecomes increasingly dull and eventuallyfaulty in habits. To this picture there maybe added focal signs such as spaslic paralysis,muscular spasms and speech defect. The

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FIG. .3.FIG. 13.

clue to the recognition of this comparativelyrare disease is often afforded in earlyadolescence by the appearance of adenomasebaceum on the face. The eruption takesthe form of small, firm, whitish or reddishnodules distributed over the cheeks andnose in a butterfly pattern. Between thenodules it is usual to find small teleangiectaseswhich give the complexion a mottled red.Fig. 13 is of a feeble-minded girl in whomthe facial eruption is particularlywell-marked.In other cases there is no eruption on theface, but rather larger nodules may be foundon the trunk. When the skin is entirelyunaffected diagnosis must be largely amatter of conjecture.

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THE SECONDARY FORMS OF lMENTAL DEFICIENCY

No treatment has any effect on the disease,which usually proves fatal in childhood orearly, adult life.

PARALYTIC FORMS.Some degree of paralysis is one of the

commonest symptoms of the more severegrades of amentia, and may be seen inpractically any part of the body. While itis customary to classify infantile cerebralpalsies into those having their onset inintra-uterine life, those due to accidents atbirth, and those acquired subsequent tobirth, in many cases the history is toomeagre to decide to which special group acase shall be assigned, and it is perhapsmore useful to divide paralytic amentia intotwo broad classes: the progressive and thenon-progressive. To the former groupbelong juvenile general paralysis, amauroticfamily idiocy, and certain rarer forms ofcerebral degeneration. In the latter groupone recognizes all forms of cerebral andspinal paralysis which do not of themselveslead to the death of the patient.Among the progressive forms juvenile

general paralysis is by far the commonest,and has already been referred to.Of the other, amaurotic family idiocy is

the best known, and the propriety of includ-ing it in the secondary forms of amentiamay perhaps be questioned, for the diseaseis associated with a definite hereditary taintand occurs almost exclusively in membersof the Hebrew race. But, on the other hand,the distinctive pathological changes and theclinical course of the disease afford goodgrounds for believing that it is of toxicorigin and not due solely to inheritedcauses. As regards its clinical features,mental impairment beginning during theearlier months of life and ending in com-plete idiocy is one of the most strikingsymptoms, and this is accompanied by aprogressive paralysis which leads to a fataltermination at about the age of 2 years.Diminution of vision terminating in absoluteblindness is a constant feature, and is asso-

ciated with changes in the macula lutea andsubsequent optic nerve atrophy. The morbidchanges in the nervous system consist of awidespread degeneration of the ganglioncells of the retina and central nervous system.Amaurotic idiocy is not often encounteredin institutions for the mentally defective,but occasionally one meets with examplesof the rarer juvenile form. In this type,which was first described by Spielmeyerand Vogt, there is no predilection for theJewish race, and as the age of onset is later,and the fundus changes not pathognomonic,the disease is liable to be confused with

FIG. 14.

other conditions, and particularly the juvenileform of general paralysis, which not infre-quently begins at about the same age. Toillustrate the clinical course, the followingcase may be cited.The patient, the second child in a family

of seven, seemed normal in every way until5 years of age, when shle contracted scarletfever. Thereafter she was noticed to havebecome dull and, though able to go toschool, made no progress and was still inthe first standard when 7 years old. Alittle later her eyesight began to fail, and by

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THE SECONDARY FORMS OF MENTAL DEFICIENCY

her thirteenth year she was almost blind.Soon afterwards she commenced to havefits, and when' admitted to hospital in I920she showed nystagmus, inactive pupils,marked speech defect, generalized musculartremor, and loss of sphincter control. Hermental development was equal to that of achild of 8 years. In I924, when she cameunder my care, she was bed-ridden, contrac-tured, and completely devoid of intelligence.The Wassermann reaction was negative.She died three years later from pulmonarytuberculosis. At the autopsy the brain wasfound to be small with atrophy of thecerebral convolutions and folia of the cere-bellum. Microscopic examination showedwidespread degeneration in the ganglioncells (fig. 14), atrophy of all cerebellar layers,and rarefaction of medullary nerve fibres.

SCHILDER'S ENCEPHALITIS.

Brief allusion may also be made to theeven rarer form of progressive paralysis towhich Schilder has given the name en-cephalitis periaxialis diffusa. This disease,which may attack adults as well as children,is characterized by mental reduction, pro-gressive blindness and bilateral paralysis,and a fatal termination in a few years. Theprincipal pathological changes consist ofdemyelination of the white matter of thebrain, spreading symmetrically from theoccipital poles, and sparing the subcorticalband of white matter. The patient in theaccompanying illustration (fig. I5) is animbecile girl in whom the'disease has pro-gressed very slowly during the past five years.Two other members of the family have beenunder the care of a neurologist, and in onethe diagnosis has been confirmed by post-mortem examination. The general appear-ance of the patient corresponds very closelyto the "attitude of adoration" describedby Freud in his study of the diplegias ofchildhood. The head is retracted with theeyes staring upwards, the elbows are pressedinto the sides, and the hands are heldpronated below the face. Mentally, the

patient is childish, timid and resistive. Shecan recognize different individuals by thesound of their voices, but has little know-ledge of time, and a mental age of 8 years.PARALYTIC AMENTIA- NON-PROGRESSIVE.This group is a large one, and includes

all cases in which there is old-standingdestruction of the motor neurones. We mayconsequently meet with monoplegia, hemi-plegia, diplegia, paraplegia, and certain formsassociated with involuntary movements.The paralyses of intra-uterine origin may

be the expression of gross cerebral defects,polio-encephalitis,and agenesis of the corticalneurones. Birth palsies may have as their

FIG. 15.

pathological basis meningeal haemorrhage,lobar sclerosis, cysts, and partial atrophies,while the post-natal forms may be caused byvascular lesions, meningitis and encephalitis.

It is often stated that while diplegias arefor the most part congenitally determined,the hemiplegias and monoplegias are ofpost-natal origin, but to this rule there aremany exceptions. The pathological lesionsdisplay much variety in their mode of origin,and to illustrate how difficult it is to arriveat any decision as to the pathological natureor extent of a lesion causing an infantilepalsy, I may refer briefly to the followingcases:-

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THE SECONDARY FORMS OF MENTAL DEFICIENCY

The first was a helpless idiot, aged 24,whose condition was stated to be causedby a head injury at birth. The patient hada divergent strabismus of both eyes and wasparalysed on the right side, the right armand leg being markedly wasted, but noshorter than those on the opposite side.Slight flattening of the cranium was presentover the left parietal bone. At the autopsythe brain was found to be unusually small,and the left cerebral hemisphere sclerosedand shrunken to almost half the size of theother hemisphere.The second was that of an epileptic im-

becile, aged i8, with partial paralysis of theleft arm and leg, dating from birth. Hewas able to wash, dress, and feed himself,and had a fair knowledge of time and place.When occupied with ward work he employedprincipally the right arm and hand, althoughhe could hold objects with his left hand,and was able to bring it into use when sweep-ing or in cleaning utensils. The left footwas inclined to drag a little, but it did nothinder his progress to any extent, for hecould climb stairs without much difficulty.At the post-mortem examination the craniumwas noted to be somewhat flattened in theneighbourhood of the right parietal bone,and on exposing the brain a large cavitywas found in the substance of the rightcerebral hemisphere and covered by a thinsheet of arachnoid membrane. When thiswas removed it was seen that the cavitymerged into the body and posterior horn ofthe lateral ventricle. The convolutions sur-rounding showed a somewhat radial distri-bution, and those absorbed in the cavityincluded the greater part of the pre- andpost-central gyri and portions of the middletemporal and superior and middle frontalconvolutions. The defect was therefore atypical example of true porencephaly, andwas probably determined by an obliterationof certain branches of the right middlecerebral artery.To describe the numerous types of para-

lysis which may be encountered would

occupy more time than is at my disposal,and I may therefore pass on to a considera-tion of the mental features.To a considerable extent the situation of

the cerebral lesion is a factor in determiningthe degree of intellectual defect. Grossbrain disease limited to one hemisphere iscompatible with the milder forms of amentia,but when both hemispheres are involved theeffect on the mind is always serious, forobviously disease of both halves of the brainmust have a more disturbing influence uponthe mental development of the child than aunilateral lesion. Moreover, gross cerebrallesions are sometimes merely complicatingor reinforcing factors in an amentia origin-ally determined by neuropathic inheritance,and in such cases, where both innate andacquired factors have been in operation, themental defect is always marked, and oftenaccompanied by gross stigmata of degenera-tion which give to the primary ament hisugly features. Diplegic patients are excep-tional in that, though both halves of thebrain are involved, their intelligence maybe only slightly impaired, and they do notas a rule.suffer from epilepsy. On the otherhand, patients with paralysis limited to onehalf of the body often develop epilepsy inthe early years of life and continue to havefits throughout their existence. Minorepilepsy is relatively uncommon, and inmy experience has no influence on thedefective's behaviour, but severe and uncon-trollable epilepsy leads to a progressivedeterioration in bodily and mental health,and many of its victims display the explosiveepileptic temperament so often seen in theinsane.

Another complicating feature in paralysedaments may be the presence of involuntarymovements, particularly athetosis. Whenbilateral, the condition usually affects theface as well as the limbs, and with grimacingthere may be pronounced articulatorydefects.

Marie has stated that in double athetosisthere is no disturbance of intelligence, but

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in the few cases I have seen it has beenpossible to detect some degree of feeble-mindedness. Patients of this type are,however, usually cheerful,-and are interestedin all that goes on.GENERAL MANAGEMENT AND TREATMENT.

In conclusion, I would like to say a fewwords about the management of secondaryaments.

Mental defect is a permanent and in-eradicable condition, and consequently itsvictims require life-long care and control.Much may be done in some cases by carefulhome training, but unless the parents, andespecially the mother, are able to devote aconsiderable part of their time and energyto this task, the patient will be better off ina special institution where he is able toassociate on equal terms with companions,and thus be stimulated by the spirit ofemulation.The class of ament with whom I have

dealt does not make very promising materialfor education in the "three R's," and forthe majority our efforts must aim at makingthe best use of such capacity as they possessby means of industrial or technical training.It would take too long to detail the differentways in which this can be accomplished, but,briefly stated, the preliminary training con-sists in exercising the sense organs, inarousing spontaneity and attention, and indeveloping the reasoning powers as far as ispossible. In idiots, even the power of instinc-tive attention is very feeble, and the most onecan expect of them is the establishment of vhabits of cleanliness. With low-grade im-beciles rather more can be done, and in theirtraining it is essential to make free use of playand organized games, care being taken thatthe periods of exertion are short. For thehigher-grade secondary aments the provisionof suitable indoor occupation such assewing, knitting, mat-making, raffia work,chair-caning, and so on, is essential, and itis here that the services of a trained instructorare so useful.

In paralysed aments loss of movementin the affected limbs is seldom absolute,and much improvement can sometimes beeffected. At the outset movement and co-ordination of the larger muscle groups areencouraged by teaching the child to graspand move to their appropriate place largewooden insets, bricks and rings. Rhythmicexercises which involve synchronous move-ments are also useful, and at the end of sixmonths, if these measures are carried outdaily, the patient may have gained sufficientmastery of his limbs to be able to walk andto feed himself.

Dribbling at the moutli is a common andunpleasant symptom in paralytic amentia,and since it has seldom an organic basis, thehabit may be eradicated by exercises directedtowards strengthening the muscles of themouth and pharynx. For this purpose oneemploys whistles, trumpets and pipes forblowing bubbles.As regards general hygienic measures,

mental defectives require to be warmlyclad, and their paralysed limbs must bemassaged and kept warm if chilblains areto be avoided. In the winter the numberof articles soiled at night is always greaterthan at other seasons, for nothing so con-duces to the habit of bed-wetting as cold,and the night nurse must be instructed touse extra blankets and to rouse his faultypatients at regular intervals.The scope of medical treatment is re-

stricted and, apart from the treatment ofepilepsy, cretinism and syphilitic mani-festations, medicinal measures are largelyconfined to combating sleeplessness andregulating the excretory organs.

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