J. Neurol. Neurosurg. Psychiat., 1953, 16, 172.

FAMILIAL HEMIPLEGIC MIGRAINEBY

C. W. M. WHITTYFrom the Department of Neurology, Radcliffe Infirmary, Oxford

The clinical phenomena of migraine are generallyexplained as due to vasoconstriction in some part ofthe internal carotid vascular system followed byvasodilatation in the territory of the externalcarotid vascular system and possibly of the internalalso. On such a view the aura of migraine issupposed to be due to ischaemia and consequentdysfunction of part of the cerebral cortex, and theheadache to tension in the walls of distendedarteries. The evidence for vasodilatation as thecause of the headache is considerable, and rangesfrom such simple observations as its relief bypressure on the carotid artery and its branches to itsreproduction by vasodilator drugs or artificialdilatation of vessel walls and its relief by vaso-constrictors (Wolff, 1948). The role of precedingvasoconstriction is not so well established, althoughthe occasional direct observation of retinal arteryspasm, the prolongation of the aura symptoms byvasoconstrictors, or the increase under their influenceof electroencephalographic changes considered torepresent cortical ischaemia, as well as the rarelyobserved relief of the aura by vasodilators, arearguments in favour. Such a theory does notattempt to explain the cause of migraine in thesense of what initiates the vascular changes them-selves.Some of the unusual forms of migraine are

interesting both for the clinical picture they presentand as a test of the validity of such a hypothesis asthe above. Hemiplegic migraine is one such variety.Here the common visual aura may proceed to or bereplaced by a hemiplegia (with aphasia if on thedominant side) and a partial hemi-anaesthesia,which is followed by a severe hemicranial headache.The occurrence " in some few cases ... of a transienthemiplegia" was mentioned by, Liveing in 1873 inhis extensive review of migraine, and since then anumber of cases have been reported. The term hasat times been rather loosely used to refer to anycase in which the usual phenomena of migraine areassociated with hemiplegia, even when not occurringas an aura to the attack. Some of these cannot be

regarded as simple migraine, but appear to beexamples of a migrainous syndrome symptomaticof an underlying structural pathology. ThusRamsay Hunt (1915) discusses a patient who hadhad migrainous attacks since childhood. At theage of 32 she had one of her usual attacks withheadache and vomiting, but this was accompaniedby a brief period of loss of consciousness, andthereafter left hemiparesis with bilateral extensorplantar responses, severe neck stiffness, and papil-loedema. At lumbar puncture the fluid containedfresh blood on repeated examinations. The picturesuggested a leak from an angioma. The questionof whether the preceding attacks of " normal "

migraine were also linked with such a lesion mustremain open in this case. However, the occurrenceof recurrent focal neurological signs associated withsevere lateralized headaches in known cases ofcerebral angioma is recognized. Such cases mayat times cause diagnostic confusion, and it seemsprobable that they gave rise to the suggestion in theolder literature that subarachnoid haemorrhagecould occur as part of a simple migraine. The useof cerebral angiography has recently helped toclarify this point.

In other cases, though the initial signs andsymptoms can be adequately explained by simplevasoconstriction, the subsequent course of theattack may suggest or even prove that a morepermanent change such as thrombosis has occurred.In these cases the neurological signs may follow theheadache or become aggravated rather than lessenas it develops. Oppenheim (1890) gives the case ofa woman who had had migrainous headaches formany years. Four months after childbirth she hadone of her usual attacks, which was accompanied byaphasia. Instead of clearing, a hemiplegia develo-ped, and coma and death ensued. At necropsythere was extensive thrombosis of the left middlecerebral artery. Infeld (1901) describes a patient inwhom an apparently typical attack was followed byhemiplegia and hemi-anaesthesia. These signscleared partially but left a residue bf paresthesiae,

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weakness, and athetotic movements in the affectedhand. Von Schroeder (quoted by Hunt, 1915)mentions a patient who had severe hemicranialheadaches for some years, and after a typical attacksuddenly lost consciousness and on coming roundhad a hemiplegia and hemianopia. The paresiscleared but the hemianopia remained. Thomas(1907) also describes two cases of long-standingmigraine with only visual aura, in each of which oneattack was accompanied by hemiparesis, hemi-anaesthesia, and hemianopia. In each case allsigns resolved except the hemianopia. Althoughsome of these cases may represent the manifestationsof a fixed lesion, such as an angioma, the occurrenceof a permanent neurological deficit without such astructural vascular abnormality has been clearlydemonstrated by Symonds (1952) in a case ofmigraine with visual aura. And the findings ofOppenheim leave no doubt that thrombosis inotherwise normal vessels can at times complicate anattack.Apart from these cases, there are others in which

a dense but completely reversible hemiplegiaaccompanies a migrainous attack. The neurologicalsigns may be present for a few hours only or maypersist for as long as a week, outlasting the headache,though they ultimately clear completely. Thesesymptoms may alternate with attacks in which themigraine presents with some more usual andlimited aura or simply as hemicrania and vomiting.There is also usually a family history of the com-plaint. Between attacks there are no neurologicalsigns and health is normal. For all these reasonssuch. cases have justifiably been regarded as truehemiplegic migraine.

Jelliffe (1906) described two cases in some detail.In one, his second case, the possibility of a structurallesion arises, but his first case illustrates the syn-drome. The patient had had attacks since child-hood. The onset was with visual symptoms whichproceeded to a right hemiparesis with sensory loss.Headache was severe, though described as mainlyright-sided. All symptoms subsided completely inthree to four days, and he was able to return to hisnormal routine. In 1910 Clarke published thehistory of a family of which three generations hadsuffered from the complaint. The attacks had astriking similarity from case to case. He gavedetails of six cases, though no less than 11 personsamongst 23 siblings were known to be affected.In all six the onset was with visual symptoms whichproceeded to a hemiplegia with some degree ofhemi-anaesthesia, and was followed in a few hoursby seyepre hemicrania and vomiting when theneurological signs would begin to abate. In all

the attacks detailed the signs were right-sided withaphasia, though in two cases attacks on the otherside were mentioned. The attacks lasted two tothree days and recovery was complete. They hadoccurred since childhood with a tendency to be lessfrequent as age advanced, his oldest patient being 54years at his death from pneumonia. This descriptionillustrates two points which are common in hemi-plegic migraine; its strong familial tendency andthe similarity in the pattern of attack within thefamily tree. Dynes (1939) also described thecondition in a mother and daughter. Here thehemiparesis was either right- or left-sided, andagain the course of the attack was the same in eachcase: signs would begin in the hand (and speech inright-sided attacks), proceed to a hemiparesis withsome anaesthesia, but visual signs occurred rela-tively late. Symonds (1952) has added two furthercases. In both there was a family history of similarattacks. In one, where the family history showedthree generations affected, the patient had had acarotid angiogram done during the height of anattack. This had shown no abnormality. The electro-encephalogram (E.E.G.) gave evidence of markedelectrical abnormality throughout the right hemi-sphere. (The paresis was on the left.) The cerebro-spinal fluid contained three polymorphs per c.mm.but was otherwise normal, though late in a previousattack there had been 185 polymorphs per c.mm.An air encephalogram done shortly after this hadbeen normal. The case is of special interest as thefirst record of cerebral angiography done during anattack. As Symonds (1952) remarks, the evidenceof its normality must exclude arterial spasm in anybut the smallest arteries. Indeed, it is difficult toenvisage a primary arterial spasm sufficient to causean ischaemia which results in motor and sensoryloss which may persist for days and yet recovercompletely. In this. sense the " cause " of hemi-plegic migraine remains a problem.Four cases of the condition are briefly reported in

this paper. Three illustrate the strongly familialtendency. In one, the opportunity arose to doboth angiographic and E.E.G. studies, and theresults are discussed. A fifth case, in which anisolated attack of hemiplegia followed by hemicraniaoccurred, is also described. Although it is doubtfulwhether the case could properly be considered oneof hemiplegic migraine, it illustrates a possiblemechanism whereby symptoms are produced, andseemed relevant and interesting.

Case 1 (R.I. 135977).-Familial hemiplegic migrainewith prolonged confusion afterwards; pattern of attacksalways the same with left-sided paresis.A woman aged 52 had had " attacks since the age of

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15, always of the same pattern. These were initiated by on the second day of the attack. Pressure was 200 mm.,flashing lights in the left half-field, and normal vision in protein 50 mg. per 100 ml., and there were 10 poly-this field would be abolished. Soon afterwards numb- morphs per c.mm. The Wassermann reaction wasness first of the left hand and then of the leg developed. negative and the Lange curve 0000000000. Electro-She had difficulty with her speech; would use wrong encephalograms were done on the second, fourth, andwords and "couldn't think of the words, though she eighth days after the onset of the attack. The firstknew what she wanted to say ". In about two or three showed slow (less than 1 c.p.s.) medium voltage waveshours right-sided headache would develop and gradually from the right hemisphere with no alpha rhythm; onincrease in intensity; at this stage she occasionally the left side some 8 c.p.s. alpha waves were present, butvomited. The hemiplegia would clear slowly over a this was interrupted by occasional high voltage 2 to 3period of two or three days. As it was clearing she c.p.s. activity with some spikes. The further recordswould often have visual hallucinations in the hemianopic showed a gradual return to the appearance in that madefield. She would see something moving and turn to before the attack.find nothing there; occasionally she had formed The family history was of interest. Her father hadhallucinations of a cat or someone's hand. She recog- had similar attacks of left-sided paralysis with severenized them as hallucinatory but they worried her. She headaches throughout his life. In these he had occa-had never lost consciousness during an attack. Fre- sionally lost consciousness and been incontinent. Hequency of attack had varied, and she had been free for was said to have died in an attack at the age of 61. Heras long as five years. During the last nine years frequency elder sister had also had frequent attacks of left hemi-had increased, and she would have them every two or plegia with headaches but had never lost consciousness.three months. Two years before she was seen, on the A younger brother had not been affected at the time ofassumption that the attacks were epileptic, she had been his death in the 1914-18 war at the age of 25. An elderput on to a regime of phenobarbitone, " epanutin ", and brother is Case 2 below. Of her four daughters aged" tridione ". This had not reduced the attacks but had 29, 26, 23, and 11, none have so far had attacks like hers.changed their character slightly. The initial visual The second and youngest are entirely healthy, but thephenomena persisted, but these were followed rapidly by eldest and third have had " fainting fits " since childhood,severe headache, and the numbness and weakness of the sudden loss of consciousness with falling and markedlimbs was much less prominent and of shortened dura- pallor, but no involuntary movements or incontinence.tion. However, as this receded she would find that for Electroencephalographic records were obtained from theas long as two weeks she was in a curious mental state in second and third daughters. Both showed an unstablewhich she felt muddled and confused, could concentrate alpha rhythm with much 3 to 4 c.p.s. activity and someon nothing, had great difficulty in doing her housework, random spiking from the temporal areas. They resembledand was noticed by others to be irritable and argumen- that of their mother between her attacks. This patienttative. This would clear slowly, and she would feel, showed a weak positive blood Wassermann reaction,and seem to others, her normal self. She was admitted but the test in her brother and all the children wasto hospital some five weeks after her last attack. negative.

She was a cheerful, rather obese, woman of poorintelligence. She was right-handed for writing and Case 2 (R.I. 142521).-Familial hemiplegic migrainesewing, though probably ambidextrous for cooking. with occasional associated attacks ofloss ofconsciousness;One brother was considered to be left-handed. Her pattern of attacks always similar with left-sided paresisblood pressure was 200/110 mm. Hg, but fundal and but occasional right-sided involvement also.peripheral vessels appeared healthy and urinary concen- A man aged 56, the brother of Case 1, had sufferedtration was within normal limits. Radiographs of the from severe right-sided headaches since the age of 17.skull were normal. An E.E.G. showed an alpha They occurred only about once a year, and were alwaysrhythm of 9 to 10 c.p.s. reduced on opening the eyes. associated with left-sided paresis. He would first noticeThere were paroxysms of high-voltage, 3 c.p.s. waves some numbness and weakness of the left leg, this spreadfrom both temporal areas, more marked on the right to involve the hand, and there was accompanyingside and with some isolated spike discharges. Three blurred vision, though he did not describe a clear-cutdays after admission a right carotid angiogram was done. hemianopia. Headache then developed and built upThis was normal. Some hours after this procedure she gradually over some hours. During this time the weak-was noticed to be drowsy, irritable, and confused. In ness of the limbs increased, and very occasionally thereresponse to questions she appeared dysphasic. She had was some weakness and clumsiness of the right hand also.a left homonymous hemianopia, but denied any positive He had noticed no speech difficulties, but felt drowsyvisual phenomena in the half field. There was a left and muddled and others had remarked on this. Thehemiparesis, more marked in the arm, and anaesthesia to headache usually cleared in 24 hours, but the weaknesspin-prick and light touch. She complained of headache persisted for two or three days. On four occasions onlyfor the first time some 24 hours later, though she had he had had a sudden " blackout " in which he had lostprobably had it for longer. During the next three days consciousness, probably for a few minutes only, but hethe signs slowly subsided, though some hemianopia had never been incontinent. On coming round he hadpersisted for five days. She remained somewhat con- had the weakness noted above, and a headache soonfused for a week. The cerebrospinal fluid was examined developed. He regarded these as similar to his other

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attacks. On examination some months after his lastattack there were no abnormal signs in the centralnervous system. His blood pressure was 150/80 mm.Hg. Radiographs of the skull were normal. Theblood Wassermann reaction was negative. An electro-encephalogram showed an unstable alpha rhythm withsome bitemporal 6 c.p.s. activity and random spiking.Overbreathing caused the temporal waves to slow to3 c.p.s., more markedly on the left side.

Case 3 (R.I. 165615).-Familial hemiplegic migrainewith other forms of migraine also occurring in the patientandfamily.A girl aged 16 had had episodic hemicranial headaches

either right- or left-sided for three years. She had somedifficulty in reading just before an attack, but the des-cription did not suggest a localized field defect. Forsome hours before she would feel dull and lethargic, andwould not go to work feeling that an attack was pending.These were her usual attacks, but on two occasions in theprevious eight months, following the vague generalsymptoms, numbness and clumsiness of movement haddeveloped in the left hand, spreading to the face andthen to the leg. She could not walk properly or pickthings up with that hand, and speech was slurred thoughnot dysphasic. Right-sided headache developed inabout half an hour, but the left-sided signs persisted forsome hours. By the following morning she would becompletely recovered. When examined some 10 daysafter her last attack there were no abnormal signs in thenervous system. A radiograph of the skull was normal.The E.E.G. record was abnormal. It showed diffuse2, 4, and 6 c.p.s. waves in both hemispheres with occa-sional spike discharges. The abnormality was increasedby overbreathing, but did not become paroxysmal.There were no localized abnormalities. Her mother, towhom she bore a striking physical resemblance, had hadsimilar attacks for many years.

Case 4 (R.I. 133116).-Hemiplegic migraine with otherforms of migraine also occurring in the patient, and afamily history of the latter.A woman university student aged 19 had suffered

from migraine since the age of 12. Her usual attackswere initiated by a general blurring of vision. Thingsappeared to be rippling as if seen through water. Head-ache, usually left- but sometimes right-sided, wouldfollow in about half an hour and would increase inseverity and lead to vomiting. Sometimes, thoughrarely, after the visual symptoms, paresthesiae in theright hand and aphasia would develop; weakness ofthe arm and leg would follow. These symptoms wouldpersist after the onset of headache, but gradually de-creased during the next few hours. On one occasionsome weakness and numbness of the hand had persistedfor six days. When seen at this time there was a signi-ficant reduction in tendon jerks in the right arm, somechange in the appreciation of light touch and pin-prickin the hand, but no actual sensory loss. The conditioncleared completely in the next few days. Radiographs ofthe skull were normal. The E.E.G. record showedsome 4 and 5 c.p.s. activity in both temporal regions.

This was increased by overbreathing, but did not becomeparoxysmal. Her father had migraine, but with avisual aura only.

This patient, an intelligent and reliable observer, hadtaken ergotamine tartrate for a time with considerablerelief of the headaches, but had abandoned it in favourof aspirin and codeine because she considered thatergotamine prolonged the aura.

Case 5 (S. M. S/1532).-A clincal syndrome of hemi-plegic migraine associated with an allergic reaction tofish: no family history.A man aged 34 eight days before he was seen had

eaten some fish which disagreed with him. During thefollowing day he had nausea and diarrhoea. Thefollowing morning, 36 hours after, he developed a veryirritating urticaria over the chest and arms, but heproceeded to work as usual. Shortly after starting outhe felt sick and vomited once or twice. He had a slightgeneralized headache, and then noticed that he couldnot see clearly to the right. The right arm felt numb andtingling, the leg was also affected, and on attemptingto walk he fell down. His speech became thick and hecould not say what he wanted to. There was no loss orclouding of consciousness, and he was fully aware thathis mates helped him to an ambulance and that he wastaken to hospital. He noticed at this time a graduallyincreasing, throbbing, left-sided headache. This largelycleared in the next two days but the hemiparesis persistedfor four to five days, clearing slowly. When first examinedin hospital a right hemiparesis including the face, withincreased reflexes and extensor plantar response, wasnoted. There was diminished sensation to pin-prick andlight touch, and a slight dysphasia. The cerebrospinalfluid was of normal pressure with 30 mg. protein per100 ml. and 1 lymphocyte per c.mm. Seven days laterthere was some increase of reflexes on the- right side butno other abnormality. When examined three weeksafter the attack the nervous system was entirely normal.At this time radiographs of the skull were normal. TheE.E.G. record showed a stable 8 c.p.s. alpha rhythm.There were a few sharp waves from both occipital regionswhich were increased but did not become paroxysmalon overbreathing. The record was almost within normallimits.

Previous history revealed a severe urticarial reactionfollowing an A.T.S. inoculation eight months earlier,and frequent attacks of asthma and bronchitis as a child.He had occasional retro-orbital throbbing headaches,sometimes severe, but never sufficient to keep him fromwork. There was no family history of migraine or unusualallergic manifestations

DiscussionA survey of the cases reported here and those in

the literature suggests that they fall into two ratherdissimilar groups. In one the course of events ismore like that of the more usual forms of migraine,an aura' phase in which the hemiplegia appearsand is followed shortly by headache, when theparesis slowly subsides. Attacks may be either

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right- or left-sided, and migraine with a more limitedaura may also occur in the same patient. Thefamily history usually reveals other cases of migraine,but they may not be always of the hemiplegicvariety. In the other group the attacks are morestereotyped. Often they occur constantly on oneside, and other varieties of attack in a given caseare unusual. The signs of the aura are prolonged,sometimes for days, and may persist or increaseafter the onset of headache. The cerebral distur-bance is more widespread; confusion, drowsiness,and even coma may be noted; sometimes motorsigns may for a while be bilateral. The familyhistory is very striking. Not only are many indivi-duals in several generations affected, but thedetailed pattern of attack is unusually constant fromcase to case. It is for this group particularly thatthe term familial hemiplegic migraine seems appro-priate. The syndrome is well illustrated in Clarke'spatients, and in the first, second, and possibly thirdpatients of this series.The first two cases and their families raise points

of special interest. They provide evidence bothclinical and electroencephalographic of a hereditary"cerebral dysrhythmia" which may show itself asattacks of loss of consciousness. There is alsoevidence of a hereditary factor responsible for aremarkably stereotyped pattern of migraine. Itwould be tempting to associate the two and suggestthat the vascular spasm element of the migraine alsoprecipitates an attack of loss of consciousness.This, however, is clearly not so. For in Case 2and his father the two types of attack are associatedin the same individual, while in Case 1 and hersister only migrainous attacks occurred, and in twoof her children only loss of consciousness. Further,in one child a "'familial " electroencephalographicabnormality was shown, though neither type ofattack had as yet occurred. The two tendenciesseem to be quite separate here, and capable ofindependent inheritance, even if some more intimatelink between epilepsy and migraine in general isindicated by other cases.

Case 1 also provides some suggestive evidence infavour of arterial spasm as responsible for theonset of her attacks. Angiography is known attimes to cause arterial spasm in the territory of theinjected vessel, especially if hypertension be present,and angiography in this case clearly precipitated anattack. Why the spasm should start, as the clinicalonset of visual symptoms suggests it so frequentlydoes, in the posterior cerebral circulation, remains aproblem. It has been shown by Moore and Stern(1938) that the anatomical course of the posteriorcerebral artery allows it to be readily compressed

against the edge of the tentorium. But this requiressome rise in supratentorial pressure to bring itabout, of which there is no clear evidence in thecases under discussion. Such an anatomical factor,though possible, must therefore remain hypothetical.Indeed, it is difficult to see how simple spasm couldbe responsible for signs which persisted for five daysand then disappeared. That relative ischaemiacan cause loss of neuronal function short of cellnecrosis is accepted, but if the vascular spasmresponsible for this is brief, then recovery should bemore rapid. If it lasts for days, then permanentdamage should result. The explanation put forwardby Symonds (1952) for such a prolongation ofsigns is that small thromboses may occur in theterminal arterioles during the spastic phase, and insupport of this he cites the polymorph pleocytosisin the cerebrospinal fluid of one of his cases. Butsuch a hypothesis also requires that some permanentdamage should occur. Some slight reduction inthe cerebral cell population must result on eachoccasion. He recognizes this difficulty, and suggeststhat the gradual mental deterioration recorded inone such case does, in fact, represent this permanentdeficit. However, if the recurrent signs are alwayshemiplegic and on the same side, it seems sur-prising that some slight permanent lateralizeddisability does not result. In one of Clarke's casesand in two of those described here attacks hadbeen occurring mainly on one side for decades, butno neurological sequelae could be found. It ispossible that after arterial spasm there is someconsequent oedema in the area supplied when bloodflow is restored just as in a limb temporarily deprivedof its arterial blood. This could account for aprolongation of signs, and would suggest a mecha-nism similar to that envisaged in " angioneuroticoedema" of the brain. Such a process wouldexplain the more widespread disturbances some-times noted, and would lead to less permanentdamage than recurrent small thromboses.Some such mechanism is probably illustrated in

Case 5. He would hardly qualify as one of hemi-plegic migraine, though the symptoms are strikinglysimilar. In him, their mechanism of productionwas presumably "allergic ". A condition ofoedema similar to the urticaria of the skin may besupposed to have occurred also in parts of the leftcerebral hemisphere. Such a " cause " for migrainehas frequently been postulated. Osler (quoted byRussell, 1909) cites the case of a boy aged 12 whohad occasional attacks of right hemiplegia withaphasia lasting for some days but showing completerecovery. The attacks diminished in severity andat the age of 26 he developed severe migraine, and

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at 28 started to have attacks of urticaria andangioneurotic oedema. Osler suggested that theearlier hemiplegic attacks and the migraine werecaused by " angioneurotic oedema of the brain ".Foster Kennedy (1926, 1936) assembled a numberof cases of episodic focal neurological abnormalitiesin which the evidence for an allergic precipitationseemed convincing. In some, for instance, elimi-nating certain articles of diet, for which positiveskin sensitivity tests had been obtained, completelyabolished the attacks. Amongst them were caseswhich presented as migraine with a focal aura, andat least one which could be regarded as a typicalhemiplegic migraine. That increased intracranialtension can accompany such supposedly allergic andreversible neurological lesions seems clear from theobservation of a case where an urticarial reaction toinjected serum was accompanied by headaches,neck stiffness, papilloedema, hemiparesis, andincreased C.S.F. pressure. As the urticaria sub-sided, so the neurological picture cleared (FosterKennedy, 1929). However, that such increase isdue to local or general brain swelling, rather than toextracerebral vascular factors, remains an assump-tion. It seems unlikely that all cases are of thistype. Nevertheless it may be the explanation insome. If so, then evidence of raised intracranialpressure might be expected. This was apparentlynot so in cases 1 and 5 of this series, where cerebro-spinal fluid pressure was measured during anattack, nor in Symonds' second case. It is possiblethat brain swelling may occur at the expense of the

volume of the cerebrospinal fluid so that the generalrise in intracranial pressure may be slight. However,a more likely explanation is that oedema, if present,is localized. In that case it is the strategic siting ofthe oedema which produces the signs of cerebraldysfunction, and total intracranial volume may belittle if at all increased. The phrase " hives of thebrain ", attributed to Osler, may be an apt descrip-tion of the intracranial state. In many ways theconception of a reversible focal oedema of thebrain seems to fit better than simple ischaemia fromvascular spasm, with a clinical picture in whichneurological dysfunction of fairly rapid onset maypersist for days, and yet finally resolve completely.

I am grateful to Sir Charles Symonds for some helpfulcomments on this paper. My thanks are also due toDr. J. D. Kidd and Dr. M. S. Good for the opportunityof seeing two of these cases.

REFERENCESClarke, J. M. (1910). Brit. med. J., 1, 1534.Dynes, J. B. (1939). Ibid., 2, 446.Hunt, J. R. (1915). Amer. J. med. Sci., 150, 313.Kennedy, F. (1926). Arch. Neurol. Psychiat., Chicago, 15, 28.

(1929). Amer. J. med. Sci., 177, 555.(1936). N. Y. St. J. Med., 36, 469.

Infeld, M. (1901). Wien. klin. Wschr., 14, 673.Jelliffe, S. E. (1906). N. Y. Med. J., 83, 33.Liveing, E. (1873). On Megrim, Sick-Headache, and Some Allied

Disorders. Churchill, London.Moore, M. T., and Stern, K. (1938). Brain, 61, 70.Oppenheim, H. (1890). Charite-Ann., 15, 298.Osler, W. (1909). Quoted by Russell, A. E. Clin. J., 34, 414.Schroeder, J. von (1884). Klin. Mbl. Augenheilk., 22, 351.Symonds, C. (1952). Trans. med. Soc. Lond., (1950-51), 67, 237.Thomas, J. J. (1907). J. nerv. ment. Dis., 34, 153.Wolff, H. G. (1948). Headache and Other Head Pain. Oxford

University Press, New York.

APPENDIXSince this paper was written, a further case (R. I.

167618) of the familial type has been seen in a man of39. The hemiplegia was always right-sided. A leftcarotid angiogram, which was normal, again appearedto precipitate a typical attack which followed about onehour after the procedure. This hemiparesis was markedfor 48 hours and cleared gradually in the next 24 hours.About 20 hours after its onset electroencephalograms,lumbar puncture, and air-encephalograms were done.The E.E.G. record, which had previously shown a stablealpha rhythm, blocked on opening the eyes, now showedalmost complete disappearance of alpha. There werealso short runs of 5 to 6 c.p.s. waves in the temporal andfrontal leads, more on the left than on the right, increasedon overbreathing. The cerebrospinal fluid, previously

showing 4 lymphocytes per c.mm. and a protein of 45 mg.per 100 ml. with a pressure of 140, now had 84 poly-morphs and 40 lymphocytes per c.mm. with a protein of35 mg. per 100 ml. Pressure was not measured as thepatient was in the sitting position, but rate of flow did notsuggest any marked rise. Air pictures showed nolocalized distortion or displacement of the ventricularsystem. The left lateral ventricle was slightly larger thanthe right, but this was within the range of physiologicalasymmetry occasionally seen. This finding seems toexclude any degree of generalized left hemisphereoedema, though it is not incompatible with focal.Indeed, the cerebrospinal fluid changes, similar to thoseobserved by Symonds (1952), must reflect some changeof an exudative nature in the cortex.

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