J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.16.3.172 on 1 August 1953. Downloaded from J. Neurol. Neurosurg. Psychiat., 1953, 16, 172. FAMILIAL HEMIPLEGIC MIGRAINE BY C. W. M. WHITTY From the Department ofNeurology, Radcliffe Infirmary, Oxford The clinical phenomena of migraine are generally regarded as simple migraine, but appear to be explained as due to vasoconstriction in some part of examples of a migrainous syndrome symptomatic the internal carotid vascular system followed by of an underlying structural pathology. Thus vasodilatation in the territory of the external Ramsay Hunt (1915) discusses a patient who had carotid vascular system and possibly of the internal had migrainous attacks since childhood. At the also. On such a view the aura of migraine is age of 32 she had one of her usual attacks with supposed to be due to ischaemia and consequent headache and vomiting, but this was accompanied dysfunction of part of the cerebral cortex, and the by a brief period of loss of consciousness, and headache to tension in the walls of distended thereafter left hemiparesis with bilateral extensor arteries. The evidence for vasodilatation as the plantar responses, severe neck stiffness, and papil- cause of the headache is considerable, and ranges loedema. At lumbar puncture the fluid containedguest. Protected by copyright. from such simple observations as its relief by fresh blood on repeated examinations. The picture pressure on the carotid artery and its branches to its suggested a leak from an angioma. The question reproduction by vasodilator drugs or artificial of whether the preceding attacks of " normal " dilatation of vessel walls and its relief by vaso- migraine were also linked with such a lesion must constrictors (Wolff, 1948). The role of preceding remain open in this case. However, the occurrence vasoconstriction is not so well established, although of recurrent focal neurological signs associated with the occasional direct observation of retinal artery severe lateralized headaches in known cases of spasm, the prolongation of the aura symptoms by cerebral angioma is recognized. Such cases may vasoconstrictors, or the increase under their influence at times cause diagnostic confusion, and it seems of electroencephalographic changes considered to probable that they gave rise to the suggestion in the represent cortical ischaemia, as well as the rarely older literature that subarachnoid haemorrhage observed relief of the aura by vasodilators, are could occur as part of a simple migraine. The use arguments in favour. Such a theory does not of cerebral angiography has recently helped to attempt to explain the cause of migraine in the clarify this point. sense of what initiates the vascular changes them- In other cases, though the initial signs and selves. symptoms can be adequately explained by simple Some of the unusual forms of migraine are vasoconstriction, the subsequent course of the interesting both for the clinical picture they present attack may suggest or even prove that a more http://jnnp.bmj.com/ and as a test of the validity of such a hypothesis as permanent change such as thrombosis has occurred. the above. Hemiplegic migraine is one such variety. In these cases the neurological signs may follow the Here the common visual aura may proceed to or be headache or become aggravated rather than lessen replaced by a hemiplegia (with aphasia if on the as it develops. Oppenheim (1890) gives the case of dominant side) and a partial hemi-anaesthesia, a woman who had had migrainous headaches for which is followed by a severe hemicranial headache. many years. Four months after childbirth she had The occurrence " in some few cases ... of a transient one of her usual attacks, which was accompanied by hemiplegia" was mentioned by, Liveing in 1873 in aphasia. Instead of clearing, a hemiplegia develo- on September 30, 2021 by his extensive review of migraine, and since then a ped, and coma and death ensued. At necropsy number of cases have been reported. The term has there was extensive thrombosis of the left middle at times been rather loosely used to refer to any cerebral artery. Infeld (1901) describes a patient in case in which the usual phenomena of migraine are whom an apparently typical attack was followed by associated with hemiplegia, even when not occurring hemiplegia and hemi-anaesthesia. These signs as an aura to the attack. Some of these cannot be cleared partially but left a residue bf paresthesiae, 172 J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.16.3.172 on 1 August 1953. Downloaded from FAMILIAL HEMIPLEGIC MIGRAINE 173 weakness, and athetotic movements in the affected the attacks detailed the signs were right-sided with hand. Von Schroeder (quoted by Hunt, 1915) aphasia, though in two cases attacks on the other mentions a patient who had severe hemicranial side were mentioned. The attacks lasted two to headaches for some years, and after a typical attack three days and recovery was complete. They had suddenly lost consciousness and on coming round occurred since childhood with a tendency to be less had a hemiplegia and hemianopia. The paresis frequent as age advanced, his oldest patient being 54 cleared but the hemianopia remained. Thomas years at his death from pneumonia. This description (1907) also describes two cases of long-standing illustrates two points which are common in hemi- migraine with only visual aura, in each of which one plegic migraine; its strong familial tendency and attack was accompanied by hemiparesis, hemi- the similarity in the pattern of attack within the anaesthesia, and hemianopia. In each case all family tree. Dynes (1939) also described the signs resolved except the hemianopia. Although condition in a mother and daughter. Here the some of these cases may represent the manifestations hemiparesis was either right- or left-sided, and of a fixed lesion, such as an angioma, the occurrence again the course of the attack was the same in each of a permanent neurological deficit without such a case: signs would begin in the hand (and speech in structural vascular abnormality has been clearly right-sided attacks), proceed to a hemiparesis with demonstrated by Symonds (1952) in a case of some anaesthesia, but visual signs occurred rela- migraine with visual aura. And the findings of tively late. Symonds (1952) has added two further Oppenheim leave no doubt that thrombosis in cases. In both there was a family history of similar otherwise normal vessels can at times complicate an attacks. In one, where the family history showed attack. three generations affected, the patient had had a Apart from these cases, there are others in which carotid angiogram done during the height of an guest. Protected by copyright. a dense but completely reversible hemiplegia attack. This had shown no abnormality. The electro- accompanies a migrainous attack. The neurological encephalogram (E.E.G.) gave evidence of marked signs may be present for a few hours only or may electrical abnormality throughout the right hemi- persist for as long as a week, outlasting the headache, sphere. (The paresis was on the left.) The cerebro- though they ultimately clear completely. These spinal fluid contained three polymorphs per c.mm. symptoms may alternate with attacks in which the but was otherwise normal, though late in a previous migraine presents with some more usual and attack there had been 185 polymorphs per c.mm. limited aura or simply as hemicrania and vomiting. An air encephalogram done shortly after this had There is also usually a family history of the com- been normal. The case is of special interest as the plaint. Between attacks there are no neurological first record of cerebral angiography done during an signs and health is normal. For all these reasons attack. As Symonds (1952) remarks, the evidence such. cases have justifiably been regarded as true of its normality must exclude arterial spasm in any hemiplegic migraine. but the smallest arteries. Indeed, it is difficult to Jelliffe (1906) described two cases in some detail. envisage a primary arterial spasm sufficient to cause In one, his second case, the possibility of a structural an ischaemia which results in motor and sensory lesion arises, but his first case illustrates the syn- loss which may persist for days and yet recover drome. The patient had had attacks since child- completely. In this. sense the " cause " of hemi- hood. The onset was with visual symptoms which plegic migraine remains a problem. proceeded to a right hemiparesis with sensory loss. Four cases of the condition are briefly reported in http://jnnp.bmj.com/ Headache was severe, though described as mainly this paper. Three illustrate the strongly familial right-sided. All symptoms subsided completely in tendency. In one, the opportunity arose to do three to four days, and he was able to return to his both angiographic and E.E.G. studies, and the normal routine. In 1910 Clarke published the results are discussed. A fifth case, in which an history of a family of which three generations had isolated attack of hemiplegia followed by hemicrania suffered from the complaint. The attacks had a occurred, is also described. Although it is doubtful striking similarity from case to case. He gave whether the case could properly be considered one details of six cases, though no less than 11 persons of hemiplegic migraine, it illustrates a possible on September 30, 2021 by amongst 23 siblings were known to be affected. mechanism whereby symptoms are produced, and In all six the onset was with visual symptoms which seemed relevant and interesting. proceeded to a hemiplegia with some degree of Case 1 (R.I. 135977).-Familial hemiplegic migraine hemi-anaesthesia, and was followed in a few hours with prolonged confusion afterwards; pattern of attacks by seyepre hemicrania and vomiting when the always the same with left-sided paresis.