Document nzZR0bJ595axLZKp4y63g9Ew

V*<'M'hfr V VV dWjiiKV <?, N19411 1,1 * * ' ' i,i i I V V, S1" ->. ' , , ' \,vj . ,V 4 1 1 * f * '-X V * ' -' i-'V -A f-'t'Or'.l";-''- '; Y\ '; ^r1*-, rvTL ., iV ** U> i \, ;/, ii '\y>/y % y ^*"j // v,!,iH! f t f 'V ', j ___ .--'i s ' *'. , -: T- CWs' i , / '"Ml' ' '^ , 5 'i f ctaf - ' V;Y . ` "N o r w o o d " BRAND '> Nhh\P$:i<< /, '*' `r'` V TO DUPLICATE THIS FOLDER ORDER "4r, y . , . ,,,,ifi'.'jK<>,j />oj,i'~ f5!o1$e^Verti*cl<c NO. 91-1/3 .S#9 1" " i -e ,H k ** -' M A D S I N U. 0 . A. .. ' Y;.YY';;V Y ' * v > 1 1 " i * * . s * tV /- ' ^ - V * `v A r 1 5 v i> ^ \ ! * . > r *1 '*. 4-/,i^ v /^* fc ' >'(, / v t A, V' - <.1 -f A ? f ' papeihsnger by tz-ade. Since June of 1 ^ 5 Us has suffered from a progressively incapacitating disease.,characterized at the onset by slurring of speech, then by '`jumping, '* weakness and easting .of the muscles of the upper arms and thorax, marked weight loss despite adequate food ingestion, progressive loss of the power of speech, difficulty in swallowing foods, and gradual restrict! of food and fluid intake to puitacecus foods to avoid choking and nasal regurgitation. In the l&3t 18 months a habit of a humorless laugh when under emotional stress, a hanging jav, and a drooping mouth have developed. ' ihe patient always preferred papering to painting, but was boss painter for the Fred Eglehoff Company for 1 1/2 years before World War II. As foreman he mixed the paste into paints and was in the room when spray painting was done by others. luring this period of employment none of the man or himself bad colic or other symptoms attributable to lead exposure. In November 1242 all occu pational exposure to lead ceased when h vent into a war plant as a maintenance mechanic. The first symptoms of the present Illness began seven months later.* the informants for the history were the patient and his wife, and two carbons from the Industrial Commission's file, OD 44675- One of these carbons is a recommendation by Charles P . Darker, who states B... (the patient) first became ill about June, 19**12.... * Ibis is specifically denied by both the patient and the wife, who state the illness began in June, 1 9 4 % months after the* patient left theemplcy of the Eglehoff Company. K E 0016654 o c c u p a tio n a l d is e a s e w e f i l e d , th e c la im 'v a a denied- by th e in d u s t r ia l C e s is s io n in D ecem ber, 1945. V lt h th e h e lp o f C . H . K H is le y and A s s o c ia te s ' th e case m s a p p e a le d . In J u n e , 1946, th e . M e d ica l A ppeal Beard refused- to a llo t? th e c la im . Ga th e a d v ic e o f S ir. K n is le y th e case m s then re fe rre d to u s . On p h y s ic a l e x a m in a tio n th e p a tie n t was found to be a la rg e fram ed nan w ith narked w a s tin g and weakness o f th e m uscles o f s a s t ic s t io n , th e n e c k , th e th o ra x and th e up per m s . He made a few g ru n tin g n o is e s when tr y in g to t a l k , and seemed u n a b le to . a r t ic u la t e '. Most o f th e tim e- th e jaw hung down w ith th e mouth " o p en , th e w asted tongue im m o b ile , T h e re was l i t t l e change in exp res s io n , but th e fo ld s o f th e s k in were n o t sm oothed, th e re v e ro c o n s ta n t ir r e g u la r f ls e ic u la lio n s in v o lv in g p a r t ic u la r ly th e m uscles o f th e th ia x , sh o u ld er g ir d le and upper a rm . th e jaw je r k was p re s e n t and gag r e f le x a c tiv e , th e deep tendon and s u p e r fic ia l s k in r e fle x e s were a l l a c t iv e , w ith no clonus o r p a th o lo g ic a l * efBesses, iiie r w as m arked ago-edem a. Ho senso ry ch an g es, a t a x ia , or- lo s s o f p o s itio n o r v ib r a to r y s m s -w ere fo u n d . He seemed o rie n te d and a w a re , and annoyed by th e hum orless lau g h th a t he u tte re d a t fre q u e n t in te r v a ls d u rin g th e e x am in a tio n . , L ab o rato ry s tu d ie s were lim ite d to th e study o f th e s p in a l !p A - ' i : V -Masotttre-, and blood and u rin e sam ples f o r le a d . The c e re b ro s p in a l f lu id dynam ics w ere n o rm a l, end th e s p in a l f l u i d was c le a r and K 0016-655 llifferentihl, diagnosis ' - ' . ' The differential diagnosis of the patient fs syndrome can be considered as among themany conditions in which progressive m ating and atrophy of muscles occur, and from other diseases in vhich bulbar signs and symptoms are clearly in evidence. Such illnesses as thyrotoxicosis, arthritic muscular atrophy, syringomyelia, cervi cal rib, myotonia congenita, progressive muscular dystrophy, perl- pher&l neuritis, infectious neuronitis, cervical pachymeningitis, and secondary lesions of the affected nerves by neoplasm, have certain superficial resemblances to the patientfs syndrome, but a clinical knowledge of these syndromes and the studies of the patient rule out such conditions. Myasthenia gravis, tumors of the brain stem, multiple sclerosis, tuberous sclerosis, bilateral vascular lesions of the brain stem as from thrombosis or hemorrhage, central nervous system syphilis, and post-encephalitic end arteriosclerotic Parkln- aonismus may cause bulbar or pseudo-bulbar signs; these conditions have all been ruled out by the studies of the patient. the history, physical findings and laboratory studies are diagnostic of the bulbar type of progressive muscular atrophy. Etiology of the presenting syndrome The immediate probi&a for decision in this case is whether the occupational exposure to lead caused the disease. This problem KE 0016656 in th development of progressive mscular atrophy 5 {6) the " '- ' character of the presenting symptomatologyf and* (f) the necessary criteria for the diagnosis, of lead: poisoning. -- ... - ' " * <*) .gsttaaugs* m m . W i M lafl- ;:' ' . . " M' As a painter* the patient presumably had some exposure to lead-containing pigments. Of importance are the facts he preferred papering to painting and did more of the former I the principal exposure to paints was as foreman for the Fred Eglehoff Company, during which time there was no exposure to dry pigments, no spray painting by the patient* and no note of symptcms commonly attributed to lead poisoning in himself or the ereu; and the patient has never bad such symptoms * other than the parexyms of abdominal pain at intervals throughout the present illness. It say be inferred that the patient has had less exposure to lead than the average pro fessional house-painter* and that he has neither had symptoms he irne attributed to lead exposure ether than the present illness *nor had symptoms that might W so attributed. (2) lime relation between the occupational exposure to lead and the development of the syndrome The patient had no occupational exposure to lead after. v..^.;. leaving th employ of the Fred Eglehoff Company in the fall of 19^2* and had none vhlle vorhing as & maintenance mechanic for a H S 0016657 .. .. (3> The ortent of the present exposure to lead The illness has been eteadily progressive since it began. i ' ; ; .. . . 'V-.- <>< .vsr.'.'- At the present tine the blood end urine lead levels are within the cormsl rang. This is proof that there has bees no recent hazardous exposure to lead It assy be concluded that the patient's illness has progressed in the absence of hazardous lead exposure. (%) The etiology of progressive muscular atrophy It is generally agreed that the etiology of most cases of progressive muscular trophy is unknown. The great majority of . .* > - . V. . . patients with this disease have had no occupational exposure to lead. Also, the vast majority of patients with hazardoua occupa tional exposure to lead, and with symptoms attributable to such exposure, do not develop this disease. Sr. Eehoe has never seen or had brought to his attention a case of progressive muscular atrophy following undoubted lead poisoning. The experience of Jones Collier and V. Bussell Brain is the same* ml have never seen nor have I been able to find-"in -the numerous records of the ' national Hospital (in London) any cases in which undoubted lead poisoning has been followed by typical muscular atrophy (1). However.*-;there are 'scattered references in the literature in which this question has been elaborated. The first significant reference KE" 001665 it was absent on single determinations. "he {flee} eases of progress!? muscular atrophy showed no lead (in the ezine}*" ihe first author!tat!? specific discussion of the problem was by Gowers* who stated* General muscular atrophy say result from lead poisoning* but this form 1 not* as a rule* progressive in character when the cause has ceased to act. It resemble the ordinary form of progressive muscular atrophy* however* in-seat and features and thus differs from the common atrophic palsy of the extensors that is produced by l e a d (2) He later described two forms of lead palsy, the first, local muscular paralysis followed by wasting* "the second form, characterised by primary atrophy* occurs especially in the intrinsic muscle of the head, but is sometimes extensive end irregular in its distribution* affecting many muscles in all four limbs. The westing is slow* and acecm* panics * instead of succeeding loss of power" {h) He described no cases with bulbar symptoms. H e went on to say *%he cases of general muscular atrophy of saturnine origin scarcely ever present any difficulty (in diagnosis; because they usually supervene on (5).severe lead poisoning that has caused characteristic symptoms " He cautioned that *the recognition of lead-poisoning depends, first , on the character of the symptoms of nerve disturbance* secondly* , on the existence of other indications of the presence of lead in K f 0016659 lost in aa article by 2. A . X . Wilson* published la ISO? (?}* Because tills la the only paper 1 which the diagnosis of "amyotro phic lateral sclerosis of toxic origin has been specifically entertained * the cases which were used as examples are suasaariced below. .- . - ,. ,; . Case X . A compositor* aged ^2* began his trade at the age of twelve and continued until Incapacitated by wrist drop at thirty-five. He had had colic about three months before. *& ceased work as & compositor* but still had occasionally to do with, lead..... Three years ago the weakness and wasting se-emed to have commenced afresh* as ho began to notice a certain difficulty in keeping his head erect* end a certain feebleness about his shoulders On examination the findings included profound acculer atrophy involving principally the upper arm muscles * wi th some weakness * atrophy and spasticity of the anterior tibial muscles * a steppage gait, s'typical extensor plantar response on the.left* and a ,;, distinct tendency to extension on the right. There were no bulbar signs, "although his articultIon has become a little indistinct Case 2 . A tinsmith of 22 vith 16 years experience had never suffered from lead colic* although there was '% good deal of lead in the solder used by him. He developed cramping and weakness in extension in the fingers of the left hand, which progressed to HE 0016860 involvement of other muscle. groups.of the extremities Constant painful ramps:la the calves continued. She deep reflexes were brisk'sad the plantar responses remained-extensor. Case 3 . ihe patient % s a j^-yesr-old engine fitter vho *hs& not, apparently, come into direct contact with lead. twelve years before he (had) contracted syphilis8' Sixteen months before he " vas seen he began to notice weakness and numbness of the extensors of the right middle finger, this progressed to involve the other fingers, and the wrist. At the end of the year there was m s ting of the forearm end weakness of the right elbow and shoulder, three months before he m s seen the seme changes began in the left hand and arm* He had to hold up hie heed with his hand to keep it from falling forward as he walked, and he had increasing difficulty in passing water. In addition to the findings of atrophy and slight spasm of the affected muscles he had Argy 11-Bobertsoa pupils, a tremulous tongue that appeared slightly atrophic on fee right side, and a double extensor plantar response. , : --- ' ' Cess % Was a 6>year old painter and paper-hanger, who had had an attack of lead colic at 60. Six months before he m s seta he developed "sciaticaa i n 'the right buttock and thigh, fhre months later had had reaping of the fingers of the hands, and developed a bilateral wrist drop. On physical exemiixaticaa he had complete double K'f 0 0 1 6 8 6 1 1 In each there is the same unmistakable (except perhaps, in Case 2) source of poisoning, vis., lesi, the action of which had con-* tir^ued o v e r s considerable period. ,,;s% . . ,, - ; ; 2 In each the onset Is characteristic of an ordinar? lead pals?, vis., double drop-wrist. The degree and duration of this lead pals? before the appearance of other symptoms war? greatly in the different cases. / .. : ':- 2* She lead palsy......is follow! by progressive amyotrophy of Irregular distribution, affecting flexors as well as extensors..... (and) is associated with cramps is the limbs, fibrillations in the diseased muscles , sometimes with involuntary sporadic movements, and with weakness proportional to the m a t i n g . * We say conclude from study of these cases that Wilson does not follow Gower1s criteria in making the etiologlc diagnosis, but that he likewise restricts his cases to those "in which the onset is characteristic of lead palsy.* la M s textbook (?) Hilsen adds "two or three more exespl.es have cose to my knowledge# since* the latest (1522) concerns a painter who some eight years ago suffered from an attack of drop-wrist (left more than right) and made a partial recovery! on examination I found atrophic palsy of small hand muscles also of forearms, fibrillary twitches, exaggerated deep reflexes (probable), extensor weakness with little or no spasticity, and unsiistkmble signs of incipient bulbar paralysis (wasting and K E 0016662 encephalopathy, -d on ifch spastic.. parap^ogia^. Sh case / ... of syEasetrleal motor neuritis of the upper limbe resemble oases of ^ progressive muscular atrophy. Eovever * B m a v e l i .makes a diugnoe- tic point of the observation that tbe cases did act progress# after ; tbe cause (?) {lead contaminated drinking va tor) vas removed. These observations may be sumarised by the statuent that Vv tbe evidence used la tbe past to attribute to lead a tiologie role in tbe development of progressive muscular atrophy is tenuous and presumptive, but that no cases bave been so attributed., that have not had peripheral motor palsy tb presenting gyaptos. (6) ghe character of the presenting sympt^&tglogy '^ the illness in tbe present case began with difficulty in articulation. Weakness of tbe forearm and hands is not prominent, ' - > . . . even later in tbe course of the illness* 2be abdominal cramping has -. tf y - apparently been considered as evidence of lead poisoning. Hovever, this bas continued. up to tbe present# in the proven absence (by . :. / \ blood end urine sampling) of hasardons lead exposure. Cramping ...... - --..... .'Tit ... - of effected muscles may be present in progressive muscular atrophy. In suamary* tbe presenting symptomatology as ,not peripheral nerve ; palsy, and vaa not characteristic of lead poisoning. . - CT) .fhe necessary criteria or %h diagnosis of lead poisoning In our opinio the necessary criteria for the diagnosis of lead poisoning are three* '(l) there must have been sufficient H 0016663 ..V; . ... meat of the present illness is completely conjeeturai. lo adequate ' basis for such a conjecture can be found from a study of the patient and the pertinent literature* - S2SSS2EE .. ' 1. The patient is suffering from the bulbar type of pro ' gressive muscular atrophy. 2. The patient has had less exposure to lead than the average professional housepainter* and prior to the present illness had had no symptoms attributable to lead exposure! the illness did not begin until months after the occupational exposure to lead ceased ; the Illness has progressed in the absence of hazardous lead exposure? the evidence used in the past to consider lead an etiolo gical factor in the development of progressive muscular atrophy Is tenuous and presumptive, but even so no cases have been so attri buted that have not had peripheral motor palsy the presenting symp tom* the presenting symptom in the present case vas not peripheral nerve palsy, and m s not even characteristic of lead poisoning* and non of the criteria ve consider necessary for the diagnosis of lead poisoning are satisfied in the present case. . >* The assumption that lead has a causative or contributory relation to the development of the preemit illness is completely conjectural. So adequate basis for such a conjecture can be found" from a study of the patient and the pertinent literature. 8-13-46 -  7 > T 6 , 37-99, 188?. - "' 3* Covers * V . B ., A manual of diseases of the S S L M g l > ?* Blakiston vFfefla.,nd edition*'i8* '1*474* ; 4. ib., id. .*>'51. 5. ib,* Id. .2*951. - . . - .- /$ 6. ib.* id. J *$59* 7. Wilson* S,A .K.* The amyotrophy of chronic lead poisoning* asyothrophle lateral sclerosis of toxic origin, Ber, of Retimi, and Fsychiat., 5*441-455* 19o7* . ~ -- --- -- 8 lb, 3 1940 ; , edited,by A. 5. Bines, Wilkins and Wilkins, 9. Brancoli, Edvin, leraarks on s o m clinical pictures attributable to lead poisoning,.vlth special reference to the neurological saaifestations of oltgahigs, Brit. Med. J ., July 18* 1931. ?v ''/-:> -. ' -y- WsSgt k r 0 0 1 6 I P ..........