Three cases ofruptured intracranial aneurysm in a family G. Dierssen. E Montiaga. A. Vazquez. B. Paternin a Depa rt men t ofNeurosurgery, "Marques de v ald ecilla" Natio nal Hosp ital , Un iversity ofCantabria, Santander

A fam ily is reported in which three member s had an an eurysm at the junction of th e posterior cerebra l artery and the carotid artery . Two patients harbonred symmetrically placed bilat eral aneurysm each . The cases suggest a hereditary basis for its cau sation.

Since Chambers et al. reported a familia l incidence of intracraneal aneurysm in 1954 (7) there have been a number of reports of cases (1, 2,4, 5, 6, 9, 11, 13, 14, 16,1 8,19,20,21 ,22,2 5,26), most ofthem have occurre d in pairs and mor e than two proven cases in a family is rare (5, 8, 15, 17, 24); it is unusual to encounter multiple an eurysms in familial cases.

Drei Fälle von rupturi erten intrakra niellen Ane urys me n in einer Familie

The real significance of familial an eurysm can only be known if the re are furth er reports of observed cases which would thus allow for an application of statistical analysis as ha s been suggested by Andrews (3).

Es wird über eine Familie berichtet , in der drei Angeh örige ein Aneurysma am Abgan g der A. cerebri posterior von der A. carotis hatten . Bei zwei Patienten waren symmetrische bilat erale Ane urys men vorhanden . Die Fälle lassen eine erbliche Basis dieser Veränderungen vermuten.

This is the report of a family in which three members , brother , sister and a cousin of th em had a ruptur ed intracraneal an eurysm, two of whom harboured two aneurysms each.

Key-Words Cere bral aneurysm - Famili al ane urys m Multiple intracraneal ane urysm

Case I A 54 years-old man was admitted 10the . Marques de Valdecilla" National Hospital in Ju ne 1980 with a suba rachnoid hemorrh age which occurre d about two hours prior to admission. Upon adm ission neurologie examination showed a drowsy patient with anisocoria and neck rigidity. The lumbar punctur e showed bloody fluid and a moderately elevated intrac ran ial pressu re . The angiographic study showed a bilobulated a neurysm of the left carotid artery with marked spas m of the artery and also a small ane urysm at the junction of the right carot id a nd posterior communicating ar tery. The clinical course of the patient was poor: his consciousness decreased progressively and the patient died flve days after the bleeding.

Case11 A 50 years old normotensive woman , sister of case I, developed a sudden headache, vomiting and diplopia twc hours prior to admlsslon: soon afterwa rds she became drowsy. A month previously she had had a simila r episode of sudden headache accompanied by stiffness of the neck which had lasted for some hours. There was no past history ofv ascular hypertension or diabetes. On adm ission neurological examination showed a drowsy patient with a stiffneck and a third nerve paresis. Lumba r puncture showed bloody fluid and increased intracran ial pr essure. The CT scan showed only evidence cf suharac hnc idal bleedin g. Complete cerebral angiography demonstrated a large aneurysm ar asing at thejunction ofth e left carotid arte ry and the posterior communic at ing art ery. The patient was opera ted on a nd the a neurysm clipped four days after admission to hospital. The postoperative course was satisfactory and the patient left the hospita l having completcly recovered ten days after surgery.

Neurochirurgia 33 (990) 85-87 © Georg Thieme Verlag Sta ttga rt - NewYo rk

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G. Dierssen. I·: Mont iaqc. .4 . Vazquez, B. Patemine

,'\/eurochim rgia 33 (/ 990)

Case fIl A 59 yea rs-old rlght -handed normotensive woman . cousin of cases nr. 1 a nd 11, wa s ad mitted to the hos pita l one hou r a fter a sudden hea da ch e followed by un conscious ness . On adm ission the patie nt wa s in a coma with a pa res is of the third right cranial ne rve a nd a Je ft he mipa resia , there was also a stitTness ofthe neck. A CT scan showe d o nJy evi de nce of suba ra chnoida l blcc dtng. Th e lumbar pun cture dem onstra ted inten se ly bloo dy fluid a nd a moder adl y incr ea sed intracranial pre ssure . A com plete angiogra phic st udy show ed sym metrica lly pla ce d sa ccular a neurysm s in both int ernal ca rotid a rte r ies at the j unctions wit h the posterior co mmun icating a rte ries. The patient recover ed co nsciousness afte r 24 hour s but 40 hours a fter the fir st episode the pa tie nt su ffered from re newed bleeding and dled .

Discussion It is unu sual for more th an two members of one family to be alTected by aneur ysm but severa l authors have repor ted families with three siblings har bonrin g intracranial an eur ysms (5, 8, 15, 17, 24). One family with aneurysms in four out of eight siblings has been described by Edelsan et al. (10) and Fox and Ko (12) studied a family in which the use of elective angiogra phy revealed aneu rysms in six out of 13 siblings . There is a considera ble discussion as to the role played by inheritence in the development of intr acranial aneurysm and th e significance of the occurrence of aneur ysm formati on within families. Some authors believe tha t the occurrence of int racra nial aneurysms within families is in most instances, fortuitous (20, 23); while others argue that the sharing of common genetic and environmental factors in siblings may explain their predisposition to aneurysm form ation (3). Inherited tendencies to developing hyper tension, polycystic kidneys (23) or mesench ymatou s diseases (21. 24, 25) have been believed to be causes offamilial aggregation of aneurysm (4) rat her th en a dir ect genetic elTect weakening th e arte rial wall and so directly causing the aneurysm formatio n. In our observation three relatives in a family are alTected by angiogra phically proven aneu rysms . An examination of the histor y of diseases in other memb ers of the family showed no known ar teri al hypert ension either in the thr ee memb ers affected by the ruptured an eur ysm. or in the rest of the member s of th e family. Nor was there a recorded history of mesenchymatous or neu rologie diseases in th e family with the exception of the father of case IIIwho died at the age of 70 from a stroke and a broth er of the alTected siblings (case l and Il) who died of meningitis in early childhood. One could speculate as to whether this meningitis might not have been a subaraehnoidal haemorrhag e but we were unabl e to obtain suflicient criteria from the recollections ofthe family in ord er to formulate a reasonable hypothe sis, specially considering th e high incidence of meningitis in our eountry at that time.



Fig.I Pedigree ofthe tarmly. Circles indicate females, rectangles males, 1, 2, 3 ruptured aneurysm. 4. died trommeningitisat the age of seven. 5. died from a stroke at the age of 70

Common environmental faetors (3) eould have played a role in the aneur ysm forma tion of the two siblings described, however these were not shared by their cousin who was raised and lived in a eompletely different environment. Ther e is reasonabl e evidence to suggest that genetic factors may have played a pre dominant role in th e formation of aneurysms in the patient s decribed by us. If this is the case an au tosomal domin ant inheritance eould be suspected according to the characteristics of the family chart (Fig. 1) as in some ofth e cases previously described in the literature (5,12 ,1 5,19). lt is perhaps import ant to point out that in all three of our observation s the aneurysm were located in the internal eharoti d artery and that in two oft hree case s there were bilateral aneurysms. The incidence of intracranial aneurysm oceurring at identical sites among siblings is seen to be more than twiee the expected frequency (3, 15). A1though cases of multiple aneurysm are very infrequ ent (2), these are, however , reported in some families (6,14 ,21. 18). Both cireumstanees seem to eonfirm a dir ect genetic influence in the ane urysm form ation in th e memb ers of the family described by us,

Refer ences Acosta -Rua. G. J.: Familial incidence of rup tured intrac ra nial a neurysm. Arch. Neurol. 35 (1978) 675 -677 2 Ambros etto . P., E. Galass i: Fam ilia l occurence ofmultiple intracranial a neurysm: case report a nd review of the literature. Acta Neu rochir. 56 (1981) 233-238 J A ndre tos. R. J.: Intracrania l aneurysm: characteristics of an eurysm in siblings. N. Eng!. J. Med. 297 (1977) 115 4 Banne rman . R. M.. G. B.lngalt, C. J. Graf- The familial occurrence of intrac ran ial an eurysm. Neurology 20 (1970) 283- 292 s Beaumont. P. J. V.: The fa milial occurrence of be rry aneu rysm. J. Neurol. Neurosurg . Psych ia try 31 (1968) 399-402 6 Brisman. R., K. Abbass ioun: Familial int racra nial a ne urysm. J. Neurosurg . 34 (1971)66 8-682 i Chambers. W. H., B. F. He rper j r, 1. R. Simps on: Famlllal incide nce of congenital a neurysm of cerebral a rterles . Report of cases of ruptured a neu rysm in fathe r and son. JAMA 155 (1954) 358-359 8 Donald, P. U.. O. S ugar: Fa milial cerebra l ane urysm including one extrac ra nial intern al ca rotid aneu rysm. Neurology 10 (1960) 288

Three cases of ruptured intracranial aneurysm in a family.

A family is reported in which three members had an aneurysm at the junction of the posterior cerebral artery and the carotid artery. Two patients harb...
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