A Follow-up Examination of 138 Cases of Subarachnoid Hemorrhage

Acta Nedica Scandinavica. Yol. CXXXVIII, fasc. I, 1950.

From the Medical Clinic of the University of Upsala (Sweden) (Head: Professor Erik

(then Head : Associated Professor Kout Liedholm). Ask-Upmark), and the Medical Clinic of the University of Lund (Sweden)

A Follow-up Examination of 138 Cases of Subnrachnoid Hern0rrhage.l

BY

ERIK ASK-UPMARK, 31. D., Upsala (Sweden), and

DAVID INGVAR, M. K., Lund (Sweden).

(Submitted for publication December 3, 1949.)

Ge n era1 In trod 11 cti on.

The spontaneous subarachnoid hemorrhages represent a topic of considerable anatomical, clinical and forensic interest. Anatomically, the pathogenesis has been conclusively elucidated not least so by recently performed investigations. Clini- cally, the symptomatology has been well known since decades but a most important recent achievement is the knowledge about the neurosurgical accessibility in many instances. Forensic medicine, finally, has taken a vivid interest in the subject since it is becoming realized that subarachnoid hemorrhages may sometimes be responsible for instances of sudden death. Personally, we have witnessed subarachnoid hemorrhage occurring for instance in a man whilst hunting and the death in such a case might easily be erroneously ascribed to accident, or as- sault. From historical point of view the sudden death of the Swedish Crown Prince Charles August in 1810 might be recalled. He died on the horse back whilst inspec- ting the troops on the heath of Quidinge and considerable suspicions arose about poisoning, even so as to induce the exilation of his physician, Rossi. Yet, there can be no doubt but that death was due to a spontaneous subarachnoid hemorrhage, as evidenced already at the post mortem. This necropsy was being performed by Rossi together with three professors from the medical faculty at Lund (Engelhart, Liljevalch and Florman) and was much later (1865) discussed in a paper by Amnzeus of Upsala, who stuck to the post mortem observations and emphatically denied the idea of poisoning, forwarded: originally by Berzelius.

It is perfectly obvious, that a disease of so dramatic character and 90 frequently

1 The present paper w a ~ presented to The International Society of Internal Medicine in Base1 Sept. 1948, to Upsala Medical Society as The Hwasser Lecture Oct. 1948, to the Swedish Society for Internal Medicine Nov. 1948 and to The IV. International Neurological Conge& in Paris h p t . 1949.

16 ERIK ASK-UPMARK AND DAVID INQVAR.

of fatal outcome, has attracted the interest of numerous authors. In Sweden the topic has been covered by Karl PetrBn, Ehrenberg, Sven Ingvar, Ask-Upmark and others and abroad by several distinguished scientists, as exemplified by the monograph of Dandy (1945), where references are to be had. Whilst it may be concluded that our knowledge about the anatomy, the pathophysiology and the symptomatology seems to be fairly complete, recent attempts to approach the disease neurosurgically make i t urgently desirable to improve our knowledge about the prognosis. Valuable contributions to this subject have been published recent1 y by Magee (1943), by Wolff (1948) and by Hamby (1948). The present investigation was outlined by one of us (A.-U.) already in 1944 and represents an attempt to present further evidence.

Our study is based on the collected material of the medical clinics of the Swe- dish universities (Upsala and Lund). Whilst most of the cases in Lund during the years 1934-1945 as well as in Upsala 1947-1948 were seen personally whilst in the hospital by one of us (A.-U.), we are well aware of the fact that this paper is to a considerable extent based upon and derived from the labour of others, who have been in charge of these cases. We wish to extend our respectful acknowledge- ment as well to these colleagues [in Upsala particularly Bergmark, in Lund Karl Petr6n (t) and Sven Ingvar (t)] as to everybody else who has been instrumental in assisting our follow-up examination, not least so the gentlemen of the clergy who have given us valuable help in tracing the individual cases. Appreciated impul- ses have been obtained by one of us (A.-U.) on visits to San Francisco (department of Naffziger, 1947), t o Oxford (department of Sir Hugh Cairns, 1948) and t o Edinburgh (department of Norman Dott, 1948), the last two visits being gener- ously sponsored by the British Council.

Material and Occurrence.

The material was represented by 138 cases. 65 cases belonged to Upsala, 73 cases to Lund.

With regard to the general xcurrence i t may be said that subarachnoidal hemorrhages (SH) are less common than are vascular lesions of the central nervous system parenchyma but more common than subdural and epidural hematomas. It is necessary to distinguish between the occurrence of SH and the occurrence of aneurysms of the subarachnoidal arteries. Although the SH in the very majority of cases depend upon the rupture of an aneurysm, occasionally instances may be observed where the SH is caused by another lesion (vide infra). On the other hand all aneurysms do not rupture, causing a SH. With regard to the aneurysms they have been registered by Sikl et al. in 19 instances out of 8,959 post mortems, by Krayenbuhl in 21 instances out of 7,452 post mortems. There are reasons to believe this incidence to be much too rare, and Luft has it that the occurrence of such aneurysms should be about 1 %.'

1 In a recent report Suter found 27 oases of such aneurysma in a series of 5,960 necropaies performed at the department of pathology in St. Gallen, Switzerland.

138 CASES OF SUBARACHBOID IIEMOBRHAQE. 17

Sex: In the present material 65 were men, 73 women. The same slight dominance of females is to be recognized in several other papers on the subject. Hamby (1948), for instance, had 55 men and 75 women. With regard to the occurrence of aneurysms the same may be said to be true: thus Dandy (1945) had 47 males and 61 females and the statistical report by Mc Donald and Korb (1939) had 519 men and 574 women. An apparent exception seems to be the report of Magee but the explanation in this case is that Magee's study was based on material from the armed forces.

Although the general impression of a certain predilection for females apparently was confirmed by our material, i t should be observed on the one hand that the difference was not overwhelmingly large, on the other hand that a slight excess of females in the population, as present a t least in Sweden, may account €or a t least a part of the difference.

Age: I n the very majority the patients were between 30 and 60, only in a few cases less than 20. This is in conformity with the recent reports, f. ex. those of Magee and Hamby, but somewhat divergent from the text books, where the prevailing age is often given as 20-40. The details of the representation with regard to sex and age are given in the following table.

Table 1.

Age at onset of the disease.

10-19 .................................. 6 1 20-29 .................................. 7 6 30-39 .................................. 11 12 40-49 .................................. 10 19 50-59 .................................. 20 22 60-69 .................................. 9 10 70-79 .................................. 2 3

Age Men Women

All 65 73

Season: No conclusive predilection for any particular season was to be derived from this material. This may be due to the absence of any such predilections or to the limited size of the material. It may be mentioned, however, that during the months of the spring (March, April, May) the predilection of SH for females was particularly outstanding, although the difference from the other seasons was not statistically conclusive.

Eliciting cause: It is almost generally assumed that the SH in the vast majority of cases is spontaneous, and accordingly not precipitated by any undue physical strain. Thus, Richardson and Hyland (1941) considered this factor to be present only in some 18 per cent of all cases. Magee (1943), in reviewing 160 instances of SH, was able to register the occurrence of undue muscular exertion preceding the SH in only 15 cases. In the report of Hamby (1948) the majority of patients were engaged in ordinary activities such as sleeping, sitting, driving or walking a t the time of onset of SH. It was admitted, however, that some few of the patients were

2- 501078. Ac ta med. Scandinau. Vol . CXXXVIII.


engaged in activities that might elevate the blood pressure (for example wrestling, coitus, moving heavy furniture). Pardee, in discussing the paper of Sands, stressed the importance of coitus as an eliciting cause. In the material of Lassen and Vang- gard 10 patients out of 43 gave an eliciting cause (passing the stools, coitus etc.). In our own material it was a striking feature that most instances of SH occurred during daytime, not least so in the morning. It is impossible, however, to pass a judgement about the importance of physical strain on a material which neces- sarily is rather heterogeneous with regard to the completeness of the records. There can be no doubt but that the SH is quite often unpreceded by any particular activity of the patient, hence justifying the term ))spontaneous)). On the other hand, if the records were scrutinized it was quite often possible to trace an eliciting external cause. This cause might be what would most appropriately be called a Valsalva-situation (for instance when lifting a heavy burden, bike-riding against the wind, defaecation, coitus etc.), but there were other instances where a rise of the blood pressure in connection with wrath or with the jumping into cold water preceded the onset and in a t least one case the SH occurred in a lady whilst placed with her head inside the hot helmet of a beauty parlor. The last mentioned observation seems to entail a warning against uncritical use of heat treatment when the head is concerned (for instance diathermy in salpingitis or sinui tis).

Clinical Anatomy.

A hemorrhage within the subarachnoid space may generally be brought about

1. A cerebral hemorrhage, perforating into the subarachnoid space. 2. An arteriovenous aneurysm on the convexity of the hemisphere. 3. An arterial aneurysm on the base of the brain.

Whilst i t may be admitted that difficulties may arise in the indivual case to decide the lesion responsible i t may on the other hand be maintained that in the vast majority of cases the clinical history in connection with the bedside observations will be able to furnish sufficient evidence t o settle the diagnosis. Arterio- graphy, surgical intervention and post mortem observations will, however, always represent valuable and frequently necessary additional evidence. The present investigation is concerned only with SH due with certainty or a t least a fair probability to the rupture of arterial aneurysms a t the base of the brain. It has rightly been emphasized, by Dandy, that these aneurysms may be caused by

by one of three underlying conditions.

1. Embolic mycotic lesions in septic endocarditis. 2. Arteriosclerosis. 3. Congenital malformations.

Cases, where the SH reasonably was due to the rupture of a mycotic aneurysm are not included in the present material. The occurrence of a SH in the course of an endocarditis lenta is considered as sufficient evidence of this etiology, not

138 CASES OF SUBARACKNOID HEMORRHAGE. 19

least so during the period when heparin was used in connection with chemotherapy. Whilst we do not deny that arteriosclerosis occasionally may be responsible for the establishment of an aneurysm we feel convinced that the vast majority of all arterial SH are due to the bursting of an aneurysm, established on the basis of malformation. There are, as a matter of fact, three reasons why a teratological etiology seems to be fairly well established.

1. The embryological differentiation of the cerebral arteries from a basal rete- formation, where abundant opportunities to deficient involutions of temporary arteries are to be recognized. In certain mammalia, for instance in camels, rather entangled and serpiginated arteries are still t o be recognized on the base of the brain and one of us (A,-U.) had has the opportunity to observe an aneurysm of an artery on the base of the brain in a lama, where the vascular conditions are pretty similar to those prevailing in camels. For a detailed review of the question of rete mirabile caroticum may be referred to Ask-Upmark (1935, 1945).

2. Dandy has called attention to the fact that certain malformations of the circle of Willis, such as abnormally large communications or asymmetric behaviour of the vessels, are twice as common in individuals with aneurysms of the arteries a t the base of the brain (the circle of Willis and its main branches) as in other individuals.

3. The occurrence in the same individual of other malformations seems to tip the balance in favour of the teratological character of the aneurysms in question. Such malformations are, for instance, coarctation of the aorta and polycystic kidneys. I n this material 47 cases were examined post mortem. There is no record of any malformation of the heart or the aorta among these cases; on the other hand we have seen basal arterial aneurysms (unruptured) in cases of aortic coarctation. In no less than 9 instances, however, kidney malformations were to be registered: in 5 cases polycystic kidneys, in 1 case horseshoe kidney, in 1 case double ureters, in 1 case hydronephrosis on one side in connection with an accessory kidney, in 1 case marked fetal lobulation. The occasional coincidence between SH and polycystic kidney has been observed previously by Ask-Upmark and by Snapper.l Considering the evidence just mentioned, surely it may be said that polycystic kidneys do not appear in some 10 % of the population.

Whilst no attempt will be made to discuss the details of the teratogenesis, attention may be called to the fact that there are as yet, to the best of our knowledge, no comprehensive study of the anatomy of the basal cerebral arteries in new-borns and infants. Such an investigation has now been scheduled in Upsala.

With regard t o the anatomy there are some facts of considerable practical importance to be stressed.

1. I n most cases there is just one aneurysm. Dandy found a multiplicity in no less than 15 yo in his study of the conditions in 108 patients. I n our own material the records were unfortunately not always complete but out of 28 cases where the localization of the aneurysm was to be identified did only 1 case present 2 aneurysms.

2 . Whereas the size of the aneurysm may vary between that of half a pea or a ~~ -~

1 Since this paper was written Suter has called attention to the same correlation.

20 ERIK -4SK-UPMARK AND DAVID INQVAR.

cranberry and that of a thumbend, most aneurysms are located excentrically to the axis of the artery, not unfrequently also more or less pedunculated.

3. The vast majority of the aneurysms responsible for the SH are to be found in the anterior half of the circle of Willis. This is a most important point since so far only aneurysms so located may be approached neurosurgically. Thus, Dandy had it that out of 133 aneurysms 120 belonged to the carotid segment of the circle

Fig. 1. Diagram on the localisation of the aneurysm in the present material as elicited post mortem or during operation. No conclusive difference is present between the right and the left side and although statements to this effect have sometimes been made in the literature a consideration of big materials,

as collected for instance by Mc Donald and Korb, fails to reveal any such predilection.

of Willis. I n the paper of Hamby 40 aneurysms out of 46 belonged to the carotid segment and in our own material 27 aneurysms out of 29. A diagram of our own cases is reproduced in fig. 1.

4. Aneurysms located to the anterior communicating artery are common and important. With Dandy they did represent 25 out of 133 aneurysms, with Hamby 12 out of 46, with ourselves 7 out of 29. It will be seen that about one aneurysm out of four is 80 located. Whether this predilection for such a short vessel ie determined only by evolutionary or also by hemodynarnic factors cannot for the present be determined. It is of practical importance, however, to remember that these aneurysms are particularly apt to be overlooked at the necropsies, since they quite frequently are situated on the brain side of the vessel, since they are usually small and since they are especially liable to rupture towards the brain. No doubt

138 CASES OF SUBARACHNOID HEMORRHAQE. 21

because of mechanical reasons the hemorrhage hence ensuing is apt to penetrate into the brain, complicating both the clinical and the anatomical picture. Since the maintenance a t least of the left anterior cerebral artery is of vital importance these aneurysms represent quite a particular problem when to be dealt with neurosurgical1 y.

5. I n fatal cases i t is quite common to encounter a severe pulmonary oedema. This observation, which to the best of our knowledge has not been stressed before, lends support to the animal experiments performed by Luisada who was able by means of raising the pressure within a limited segment of a subarachnoidal artery to induce a pulmonary oedema. It is, moreover, a well established fact that pulmonary oedema as met with in cardiac disorders is elicited from the nervous system. It may accordingly not be entirely out of the way to pay some attention to this matter when dealing with cases of SH.

A remarkable feature with regard to the SH is their predilection for the female sex (cfr., however, p. 17). It should be observed that aneurysms of the coronary arteries, although rare, present a most outstanding predilection for males: Scott, a t the Boston City Hospital, has recently described 47 aneurysms of the coronary arteries. 15 were interpreted as congenital, all but one occurring in males. In 20 more cases where another etiology was to be presumed 13 occurred in men, 7 in women. With regard to the SH a t least 2 instances of the present material appeared in women during advanced stages of pregnancy. Since anyhow a certain proportion of the population may be assumed to be pregnant this does not seem unreasonable, all the more since there were several female patients who had performed numerous deliveries (up to 10) before the appearance of SH; in one case a delivery occurred a few years after a SH (with no complication). There is, however, some evidence converging towards the peculiar position of the female arterial system when SH are concerned:

1. We know ever since the fundamental investigations of Gene11 that the follic- ular hormon exerts a relaxing effect on the smooth musculature. This effect ac- counts, for instance, for the dilatations of the ureters to be registered during pregnancy. On the other hand a deficiency of this hormone is t o be blamed for the appearance of the so-called biliary dyskinesias in women, in whom the gall bladder has been removed (Liedberg, Ask-Upmark, unpublished observations).

2. It is a well known phenomenon, touched upon by several authors (for instance Ask-Upmark) that aneurysms of the artery of the spleen may be encountered during pregnancy. These aneurysms may give a quite peculiar clinical syndrome and they may, moreover, burst, resulting into an intraperitoneal hemorrhage (usually within bursa omentalis), which quite often rapidly becomes fatal.

3. A certain predilection of the SH for the periods of menstruation has been registered by several authors, for instance Froin, Ehrenberg and Sven Ingvar. The present material does neither confirm nor deny those observations, which, however, seem to be entirely reasonable. 4. It is, ceteris paribus, more dangerous for a female to have a SH than for a

maIe. This quite outstanding prognostic difference will be dealt with in detail later on. Here i t may be sufficient to remember the fact that in most other dis-

22 ERIK ASK-UPMARK AND DAVID INBVAR.

orders a man is more liable to succumb than a woman, other conditions being similar.

5. With regard to the vascular system and its pathology there is a quite considerable difference between males and females. Buergers disease affects practically only men, Raynauds disease practically only women. Coronary infarctions are much more common in males. On the other hand there is a quite peculiar syndrome, which may be called brachiocephalic obliterating arteritis which is confined to women, mostly in their thirties. This syndrome is unusual but one of us (A.-U.) has had the opportunity to observe 3 cases during the last few years (1946- 49). Frovig (1945) has given a comprehensive review of the matter. The real cause is unknown. It may not be out of the way to remember that obliteration of arteries in the neck is a normal phenomenon during evolution, for instance when the so called carotid duct disappears in connection with the development of a neck or when one of the carotid arteries is becoming obliterated in snakes.

6. Meadows (1949) has called attention to the fact that spontaneous carotid cavernous aneurysms with pulsating exophthalmus occur particularly in middle aged or elderly women.

The clinical anatomy of the SH may accordingly be summarized thus: SH is caused by the rupture of an aneurysm, usually located a t the carotid segment of the circle of Willis. This aneurysm is as a rule of malformational character. A transitory increase of the blood pressure may in some instances be of contributory importance for the rupture. Malformations of the kidney were frequently observed a t the post mortem and the same goes with pulmonary oedema.

Clinical Symptomatology.

The nosology of SH is well known and should not be repeated here. The two outstanding characters are on the one hand the extremely sudden onset of meningitic symptoms, to be compared with the perforation of a peptic ulcer into the abdom- inal cavity, on the other hand the appearance of the cerebrospinal fluid, which is hemorrhagic and, on centrifugation, presents xantochromia. With regard to the neurological bedside observations the deciding factor is the topographical position of the aneurysm, underlying the SH. The symptoms may accordingly be confined to those of a severe meningitis but involvement of structures in the neighbourhood such as cranial nerves or brain tissue may entangle the picture considerably. There is, for instance, one case on record in the literature who for years had suffered from diabetes insipidus, a syndrome which disappeared after the rupture of the aneurysm exerting pressure on the para-pituitary structures. Palsies of the extraocular nerves, interference with the visual field, primary optic atrophy, trigeminal neuralgia, exophthalmus, retinal hemorrhages, choked discs, unconsciousness, hemiplegia and monoplegia are some of several symptoms to be encountered. Wellknown observations from the laboratory are the transitorily increased blood sugar level, the temporary increase of non-protein nitrogen, the occurrence of glucosuria, albuminuria and acidosis. We have found a quite considerable increase of the sedimentation rate in these cases, usually reaching its

138 CASES OF SUBARACHNOID HEMORRHAGE. 23

maximum during the second or third week and returning to normal levels after 6-8 weeks.

The question whether premonitory symptoms are to be elicited or not may be answered as follows.

In several instances no such symptoms are to be encountered, the sudden onset of a dramatic clinical syndrome being similar to a lightning from a blue heaven.

I n other instances, where circumstances allow a detailed penetration of the previous history, symptoms of the aneurysm may precede those of the SH for hours, days, months or years. Rather suddenly appearing severe pains in the front head, the temporal region or the eye for hours or days previous to the SH may indicate ail expansion of the aneurysm. The well known syndrome of ))migraine ophthal- moplegiqueo is rather suggestive of an intracran a1 aneurysm, bordering to the extraocular nerves. It may further be questioned whether not migraine in the usual sense of the word is more common in the history of SH than in the average population. Wolff, whose contributions to our knowledge of headaches are wellknown, is of the opinion that a history of migraine may be traced in some 40 yo of these cases as compa.red with 9 yo in the population a t large. We have for years had our attention on this question but the records do not allow us to pass a definite judgment although i t may be mentioned that in G out of 11 cases, resulting in a fatality, a history of migraine for years was to be elicited. It is, moreover, well known that the intracranial structures sensible to pain are the basal arteries (Wolff 1948). That a distension of an artery may represent the mechanism eliciting the pain is, finally, evidenced as well by the omigraine ophtalmoplegiqueo as, for the external carotid system, by the investigations of Graham and Wolff (1939). On the other hand we do not attempt to suggest that migraine is always present in the earlier history of SH, neither that all instances of migraine have as their substratum an intracranial aneurysm. It should, for instance, be recollected that the predilection of migraine for females is by far more considerable than the corresponding predilection of arterial aneurysms a t the base of the brain. There is, however, one phenomenon in migraine which is extremely suggestive of activities of the brain structures encircled by the circle of Willis and that is the urina spastica. Inciden- tally, the same symptom may be observed in connection with attacks of paroxysmal tachycardia and one of us (A.-U.) has had the opportunity to observe a case where the attacks of tachycardia were being called forth by a leakage of cerebrospinal fluid occurring once in a while through the nose (a fracture of lamina cribrosa). It may be recalled that draining of the cerebrospinal fluid (by means of lumbar puncture) was one of the measures applied by Pickering and Hess in their fundamental study on headache (reduction of the pressure a t the outside of the arteries).

Prognosis.

When dealing with the subsequent course of a disorder as the SH it is perfectly

On the one hand several records of cases dying on their first admission are apt evident that several sources of errors are to be considered.


to lack important informations pertaining to their previous history: if a person is brought into the hospital unconscious and subsequently dies the anamnestic record is quite often liable to be incomplete. On the other hand it should be emphasized that no follow up examination is ever complete until the cases are traced to their bitter end: the survivors, who are perhaps today up and about, doing perfectly well, may or may not succumb to-morrow to a recurrent attack of SH, of which w0 know nothing today. Moreover, instances of arterial intracranial aneurysms, where no rupture has occurred will not be able t o be considered by the investigations; with regard to these cases references may be had with Dandy. Finally, several patients who survived their first attack and left the department died later a t home where no necropsy was to be obtained and the final diagnosis accordingly had to be based upon bed-side evidence viz. death certificates only. As Dandy puts the matter: osubsequent ruptures after apparent healing are exceedingly common and may occur ten years or more after the initial break.))

With these reservations the following may be said about the prognosis. Richardson and Hyland had a material of 118 cases: 61 were fatal, 57 survived,

37 of whom were still alive 1-10 years later. Magee judged the fate of 150 cases from his board of pension. 52 cases died in

the initial attack, 98 survived this first attack. In 50 out of the 98 survivors recurrences occurrred, resulting in a fatality in 32 instances. Altogether there were accordingly 66 survivors, but it should be remembered on the one hand that the material was representative only for males, on the other hand that the vast majority of cases were traced only for a short time, hence only in 14 cases for 1 year or more, only in 4 cases for 2 years, only in one case for 4 years. Most recurrences did appear during the first month.

Dandy’s excellent monograph deals mainly with the arterial aneurysms but he mentions that out of 108 patients the aneurysm ruptured in 64 patients: ))Over half of the patients with ruptured aneurysm died within 48 hours, and half of these within 24 hours.)) If the mycotic and arteriosclerotic aneurysms are excluded there remain 59 instances of ruptured aneurysms with SH. 30 died during the first attack and 6 more whilst being operated upon during this attack. Of the 23 survivors recurrences occurred in 15 instances, 12 of which were fatal. Altogether there remain just 11 survivors. One case was (successfully) operated upon 22 years after the first rupture, otherwise the recurrences occurred within 1 month in 5 instances, after more than 1 year in 6 instances (1 9/12, 2, 3 9/12, 5 5/12, 11, 12 years), and after more than 1 month but less than 1 year in the rest of them.

Wolf, Goodell and Wolff studied 46 cases of SH at the New York Hospital. Only 5 died during the initial attack, recurrences were noted in 24 instances, 10 of them were fatal and the total amount of survivors were 31. The recurrences were in most cases (20) to be registered within 6 weeks (9 fatalities), only in 4 cases after more than 8 weeks (one fatality, after 5 years). I n 17 instances the patients were traced for more than one year, in 5 instances for more than 5 years.

Hamby has recently published a most important investigation from the Buffalo General Hospital. 130 cases of SH were examined. I n 44 the patient died during


the first attack. I n 46 cases recurrencies occurred with 37 fatalities. Altogether there were 49 survivors.1 Neither age nor sex was found to be a determining factor. Unconsciousness was an ominous sign and patients with recurrences )>had only about half the chance of recovery of those with a single episode)).

Our own material is represented by 138 cases (65 males and 73 females). 38 died during the initial attack (18 males, 20 females), 100 survived the initial attack (47 males, 53 females). These survivors have all been traced by questionairies to the clergy, those still alive by personal questionairies. If the subsequent course after the initial attack had not been entirely smooth or if any doubt still was present the patients were seen personally by one of us, closely interrogated and examined. This tracing part of the work was being performed for the Upsala patients by Ask-Upmark, for the Lund patients by Ingvar. If the patients had entered any hospital after their first admission the records of this hospital (mostly the clinics concerned) were of course scrutinized as well. It turned out that recurrent attacks had occurred with certainty or a fair probability in 10 males (4 fatalities) and 29 females (19 fatalities). 10 men and 3 women had succumbed to other dis- eases so that a t the time of the follow up examination there were still alive 33 males (6 of whom had had reccurrencies) and 31 females (10 recurrencies).

With regard to the period of time for which the individual cases were traced the following table attempts to furnish some evidence.

Table 2. For 1 year or less 14 cases were traced 11 recurrencies with 8 fatalities. For 1 up to 5 years 27 o )> o : 15 P B 8 0

For 5 up to 10 years 27 )) D o : 6 o >> 4 D

For 10 up t o 33 years 32 )) o : 7 o 0 3 0

Altogether 100 )) D u : 39 B o 23 >> Males 47 D 0 > > ' : l o b 0 4 b

Females 53 >) )> u : 29 n o 23 D

Whereas hence most recurrencies occurred during the first 5 years they might appear as late as 20 years after the initial attack (in one nurse whose initial attack was elicited by lifting a patient). As will be seen 59 cases have been traced for 5 years or more.

An important question seems to be represented by the survival time for the cases succumbing to their initial attack, This time we have been able to ascertain in our own material as well as in that of Dandy. It turned out that the fatalities might occur after a few hours, hence the very first day. It might on the other hand be postponed even so as not t o occur until the 25th day (in such cases it is of course impossible to exclude that a new SH has occurred but as far as the clinical and pathological records allowed a judgment the SH was solitary). On an average these cases died on the 9th day. This is, in our opinion, a most important fact which should be considered when dealing with such patients diagnostically and thera- peutically (vide infra).

Whereas the immediate mortality (i. e . during the initial attack) was very much -

These figures have been obtained by considering the 14 cases, dying a t home as well.


the same with males and females it will be seen that the reccurrencies are more frequent with females (approximately 3 times as frequent) and more deadly as well (19 out of 29 as against 4 out of 10). To the best of our knowledge, this observation has hitherto not been stressed. On the other hand there were more men (10) than women (3) who a t the time of the follow up examination had died from other causes (cancer etc.). From the point of view of SH they have been included in the ))survivors)).

A brief summary may be given of the materials referred to in this study.' The 6 cases of Dandy given parentetically died in connection with surgical intervention. The ))survivors)) include those who have managed to pull through their recurrencies and also those who have later died from other causes, unrelated to SH.

Table 3.

Total numbers Died during Recurrencies Recurrencies Survivors of cases. first attack. altogether fatal in all

Magee .................. 150 52 50 32 GG Dandy .................. 59 30 ( + G I 15 12 11

Hamby ................. 130 44 146 '39 ,448 Upsala-Lund ........... 138 38 39 23 77

Wolff e t al. ............. 46 5 24 10 31

-~ ~. _ _ ._

Altogether 523 1G9 (+6) 174 114 233

' 14 cases dying since discharge from vascular accidents have here been considered as recurrencies. 2 Including one case not traced.

It will be seen that the various materials are slightly different from one another, the lowest immediate mortality being found with Wolff, the highest with Dandy. On an average, however, it may be said that approximately 32 yo succumb to the initial attack, that recurrences occur a t least with the same frequency and that the mortality of the recurrencies is about twice as high as during the initial attack. Approximately 44 yo are registered as ))survivors)) but it should be remembered, firstly, that several of these cases have later died from other causes, secondly, that about 1 survivor out of 4 has had a recurrent attack of SH and that new attacks in the future is liable to reduce this group. Finally, several of these survivors are to be considered as seriously crippled, with various neurological handi- caps interfering with their working ability. Of our survivors approximately 50 yo considered themselves able to carry on their occupation as usual, whereas the rest were more or less severely crippled. Somewhat similar figures have been presented by Hamby. I t may, hence, be safely stated that if a perso% has a S H the chances

' The paper of Lassen and Vanggard, although instructive, does not allow a comparison with t h e materials here quoted, since most of the relapses occurred within one month (in one case, however, after 6 years). The material was represented by 43 cases, 12 of whom died a t the first at tack (4 males out of 18 - 8 females out of 25). The death in these instances occurred at 1-15 days after onset (on an average at the 7th day). Out of t he 31 survivors 27 were traced: 5 during 5-10 months, 8 during 11/,-2'/? years, 8 during 3-4'1, years, 6 during 51/2-71/2 years. 20 out of the 27 were considered f i t for work, whereas 7 were considered more or less unable t o cope with their job. It should be observed, however, t ha t only 3 out of the 20 were entirely free from any symptoms, whilst the remaining 17 were more or less hampered by impaired ability of mem- ory, headaches, nervosity etc.


have hitherto only been 1 out of 5 that he will made a good recovery, whilst his chances to become crippled may be judged approximately just as high and he has 3 chances out of 5 to die from his disease sooner or later.

This, indeed, is a very sad future and the question may be raised what measures could be taken to improve the outlook. The following points suggest themselves.

Fig. 2. Arteriography in one of our cases, a young woman. Tho aneurysm is readily seen in the neigh- bourbood of art. communicans ant. In order t o save space only one projection is reproduced: i t goes without saying that a frontal view is necessary as well. This angiography as well as tha t reproduced in the next picture was carried out in the depart.ment of neurosurgery of the Sodersjukhuset, Stockholm,

under the experienced supervision of Dr. Olof Sjoqvist.

1. Since the SH ultimately is due a malformation i t is obviously desirable for the pregnant mother to avoid exposure to German measles during the first 3 months of the pregnancy. Marriage with people belonging to families with domi- nant malformations, such as polycystic kidneys, cannot of course be forbidden, but the possibility has to be taken into consideration that the offspring may present aneurysms of the circle of Willis. We realize that life and love do not, as a rule, take such considerations.

2. If a SH should occur and no possibility of surgical intervention be available the nursing technique becomes of paramount 'importance (in appropriated cases reduction of intracranial pressure by means of suboccipital or even lumbar puncture or by Mg SO4, adequate supply of fluid, if necessary by means of a nasal tube, prevention of complications, such as pneumonia by means of penicillin etc.). Should the patient recover and surgery for one reason or another be out of question it will be important t o prevent situations entailing increase of the blood pres-


sure (anger, exposure to cold, Valsalva-situations /constipation, heavy work etc./) and his life will have to observe the general precautions necessitated by any lesion to the central nervous system (sufficient sleep, fresh air, avoiding of heat and of hard liquors etc.).

3. The most important advance in the treatment of SH is no doubt the neurosurgery, as introduced for these lesions by the pioneer work of Dandy. It may be

Fig. 3. The same case after operation.

readily admitted that age and general condition sometimes make a surgical intervention impossible, that some aneurysms are situated so as to be unavailable to neurosurgical approach and that the mortality of these operations may be high. On the other side the hand may be skilled by experience and it should be remembered

1) that most aneurysms are situated in the carotid segment of the arterial circle and accordingly available

2) that the outlook without operation is so dark that an attempt to improve the prognosis seems entirely justified, not least so in female individuals, where the liability of recurrences is considerable and particularly of course in young female individuals, where the general condition of the patient allows an intervention.

No details regarding the operations can be given by us, references being amply found in the monograph of Dandy. We only want to stress the necessity in such instances to perform an arteriography in order to locate the lesion. This arteriography, and the subsequent operation, should be performed as early as ever possible. Originally, we were inclined to wait for one or two weeks at least, in order


to allow the symptoms to settle down. This attitude is, as pointed out by Naffziger to one of us, without doubt erroneous, since the arteriography can do no harm and since our own material seems to stress the importance of hurry: The patients succumbing to the initial attack did not die until, on an average, the 9th day, and there might have been time to interfere before, had we realized the importance of so doing. It goes without saying that a certain delay may be necessary in hospi- tals where no neurosurgeon is available, since, after all, it is a bit unsympathetic

Fig. 4. The conditions in the case of fig. 2 and 3, as seen during the operation. This operation was performed and the sketch drawn by our friend Dr. Sjoqvist.

to subject these severely ill patients to a transportation. Perhaps Muhammed could come to the mountain instead, provided that a flying neurosurgical squad could be organised for such purposes.

Summary arid Conclusionn.

1. A follow up examination was performed of 138 cases of subarachnoid hemorrhage, observed in the medical clinics of the Swedish universities (Upsala and Lund). 65 were males, 73 females. Most instances were to be encountered a t the age between 30 and 60 (table I, p. 17). Whilst the subarachnoid hemorrhage in


many cases was spontaneous in the true sense of the word it was felt that in a considerable number of cases the rupture occurred in connection with undue physical strain or elevation of the blood pressure.

2. In 47 cases a post mortem was performed. In no less than 9 cases malformations of the kidneys were to be registered (in 5 cases polycystic kidneys). I n 28 cases detailed information was to be obtained about the localization of the aneurysm responsible. In one case only was there more than one aneurysm (two). The vast majority, or 27 out of 29 aneurysms belonged to the carotid segment of the circle of Willis. In 7 cases the localization was the anterior communicating artery, which for various reasons occupies a special position when these aneurysms are concerned. At the post mortems pulmonary oedema was strikingly often met with and this matter is discussed. The predilection for females is discussed as well.

3. The clinical symptomatology is briefly reviewed and the attention is fo- cussed on the occurrence of migraine in the history.

4. Out of 138 cases 38 died during the initial attack. The 100 survivors were followed up. Recurrencies were to be registered in 39 cases (10 males with 4 fatalities, 29 females with 19 fatalities). 10 men and 3 women have died from causes unrelated to the subarachnoid hemorrhage. At the time of the follow up examination there were still alive 33 males (6 of whom had had recurrent attacks) and 31 females (10 recurrencies). 41 cases were traced for 5 years or less, 27 cases for more than 5 up to 10 years, 32 cases for more than 10 years.

5. The cases dying from their subarachnoid hemorrhage did so on an average a t the 9th day. The mortality during an initial attack was very much the same with males and females but recurrencies are more frequent with females and more deadly as well. These observations have to be considered when judging the indi- cations for neurosurgical intervention.

6. Since several survivors on the one hand may expect a fatal recurrent attack in the future, on the other hand may be seriously crippled neurologically there can be no doubt but that a more extensive use of neurosurgery should be done in these cases, not least so in young individuals, and perhaps especially in women. Without operation only about one case out of five is to be expected to make a good recovery and become able to take up his old occupation; one case out of five remains crippled and the three cases die sooner or later from SH. The mortality in recurrent attacks is about twice as high as in the initial attacks. Arteriography should in appropriated cases be performed as soon as ever possible.

References.

Amnsus, A. 5.: Upsala Universitets Arsskrift 1865. - Ask-Upmark, E.: Acta Path. et Microbiol. Scand. 1929, 6, 383. Nord. Med. 1939, 1, 698. Klin. Wochenschr. 1937, p. 897. Acta Psych. et Neurol. Scand. Suppl. VI, 1935. Unpublished observations 1929-1949. - Dandy, W. E.: Intracranial Arterial Aneurysms. Comstock. Itaka. N. Y. 1945. - Ehren- berg, L.: Till kannedomen om s. k. spontan subarachnoidalblodning. Diss. Upsala 1924. - Hamby, W. B.: J. A. M. A. 1948, 136, 522. - Ingvar, S.: Nouv. Iconograph. de la Sal- petriere 1918, 28, 313. - Jirasek, A., K. Henner and H. Sikl.: MBm. de 1’Acad. de Chir. 1937, 63, 577. - Krayenbiihl, H.: Schweiz. Arch. f. Psychiatrie und Neurol. 1941, 47,


155. - Lassen, H. C. A. and T. Vanggard: Acta Med. Scand. 1941, 107, 391. - Luft, R.: Hygiea 1938, 100, 177. - Mc Donald, C. and M. Corb: Arch. New. and Psych. 1939, 42,298. - Magee, C. G.: Lancet 1943,2,497. - Naffziger, H. C.: Personal communication 1947. - PetrBn, K.: Deutsch. Zeitschr. f. Nervenheilkunde 1928, 101, 308. - Sands, I. J.: Trans. Am. Neurol. Ass. 1938, p. 144. - Wolf, G. A., H. Goodell and H. G. Wolff 5. A. M. A. 1945, 129, 715. - Wolff, H. G.: Headache. Oxford University Press. New York. 1948.

Since this paper was presented there have appeared two more papers on arterial in-

Fernstrom, U.: Nord. Med. 1949, 41, 799. - Suter, W.: Schweiz. Med. Wochenschr. tracranial aneurysms:

1949, 79, 471.

A Follow-up Examination of 138 Cases of Subarachnoid Hemorrhage

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