Transcranial Doppler Sonography in familial hemiplegic migraine

Transcranial Doppler Sonography in familial hemiplegic migraine

Francesco Pierelli, Flavia Pauri, Letizia Maria Cupini, Giancarlo Fiermonte, Paolo Andrea Rizzo

Cephalalgia

Pierelli F, Pauri F, Cupini LM, Fiermonte G, Rizzo PA. Transcranial Doppler Sonography infamilial. hemiplegic migraine. Cephalalgia 1991;11:29-31. Oslo. ISSN 0333-1024

A patient affected by familial hemiplegic migraine underwent Transcranial Doppler Sonographytwice: the first during a spontaneous attack with right hemiparesis and aphasia, the secondduring a headache-free period. During the attack the following haemodynamic changes wereseen: (a) bilateral increase in the middle cerebral artery and anterior cerebral artery blood flowvelocities (this increase was more pronounced on the left side), (b) decreased systo-diastolicratio and pulsatility index on the right side, (c) increased systo-diastolic ratio and pulsatility indexon the left side. Our results indicate that during the attack in this familial hemiplegic migrainepatient a diffuse vasoconstriction of the basal cerebral arteries developed. Moreover,Transcranial Doppler Sonography data suggest that a prolonged vasoconstriction of theperipheral arterioles could play a role in determining the neurological symptoms in thissyndrome. • Familial hemiplegic migraine, Transcranial Doppler Son ography

Francesco Pierelli, Flavia Pauri, Letizia Maria Cupini, Giancarlo Fiermonte, Paulo AndreaRizzo, Istituto di Clinica delle Malattie Nervose e Mentali, Università "La Sapienza" Roma, Vialedell'Università 30, 00185 Roma, Italia; Accepted 11 October 1990

Familial hemiplegic migraine has been described as a "migraine with aura including hemiparesis and where,at least, one first degree relative has identical attacks" (1). The pathogenesis of the neurological signs is stillnot clear, if they are related to a prolonged arterial spasm, to angioneurotic oedema or to the occurrence ofsmall thromboses in the terminal arterioles (2, 3). It is now possible to obtain useful information aboutintracranial haemodynamics by the use of Transcranial Doppler Sonography. Trans-cranial DopplerSonography has been employed in studying cerebral flow velocity in other types of vascular headache suchas migraine and cluster headache (4-8). This report concerns a patient affected b y familial hemiplegicmigraine who underwent Transcranial Doppler Sonography recording during a spontaneous attack.

Case report

A 31-year-old, right-handed man experienced six attacks of familial hemiplegic migraine with right-sidedhemiparesis after the age of 10. The first two episodes occurred after minor head injuries during a soccergame, while the remaining four were characterized by spontaneous onset. His mother as well as his brotherhave both been affected by similar attacks of hemiplegic migraine during their life span. On admission thispatient complained of a right-sided throbbing headache with right hemiparesis and aphasia. The attack hadbegun 60 hours before with a right lower limb paraesthesia which had spread to the whole of the right side ofhis body, right hemiparesis, scintillating scotomata, and speech disturbances: "I knew what to say but I wasunable to find the right words". In about 30 min a severe throbbing right-sided headache developedaccompanied by nausea, vomiting, photophobia, and phonophobia. The neurological signs persisted duringthe pain period and cleared in four days with full recovery. An EEG showed the presence of theta-deltaactivity on the left temporal areas. CT scan was normal both before and after contrast enhancement. Asecond CT scan performed seven days after the onset of the migraine attack was normal. Doppler flowexamination of the intra-extracranial vessels, performed during a headache-free period, showed normalfindings. No flow velocity changes in the common, internal, and the external carotid arteries were seen duringthe attack. By contrast, Trans-cranial Doppler Sonography showed higher systolic, diastolic, and mean flowvelocities in the anterior and middle cerebral arteries (Fig. 1). The systolic flow velocity changes consisted ofan 87.5% increase at the level of the left middle cerebral artery and a 50% flow velocity increase in the rightone; flow velocity increase in the anterior cerebral arteries were respectively 76.6% (left) and 66.6% (right).The diastolic increases were 33.3% (left) and 100% (right) in the middle cerebral arteries, 36.8% (left) and72.2% (right) in anterior cerebral arteries.

Finally, the mean flow velocity changes consisted of a 59% increase at the level of the left middle cerebralartery and a 61.5% increase in the right middle cerebral artery; anterior cerebral artery increases wererespectively 82.35% (left) and 80.95% (right). In the left middle cerebral artery a marked increase in systolicpeak velocity was seen (150 cm/sec, basal value 80 cm/sec).

The diastolic and mean velocities in the left middle cerebral artery were slightly increased, whencompared with those obtained in the other arteries during the attack. The systo-diastolic ratio and thepulsatility index were decreased in the right anterior and middle cerebral arteries and increased in the leftanterior and middle cerebral arteries. The more pronounced change was represented by a marked increasein systo-diastolic ratio and pulsatility index in the left middle cerebral artery (Table 1).

A transfemoral carotid arteriography performed during a pain-free period showed normal findings.

Discussion

The Transcranial Doppler Sonography study of this patient with familial hemiplegic migraine showed that,during the attack, the following main changes of the cerebral haemodynamics developed:

1. diffuse increase of cerebral blood flow velocities with a greater extent on the left side;

2. decreased systo-diastolic ratio and pulsatility index on the fight side;

3. increased systo-diastolic ratio and pulsatility index on the left side.

Some aspects of our results (increased blood flow velocities, decreased systo-diastolic ratio, and pulsatilityindex) are similar to those described by Thie et al. (5) in classic migraine patients during headache attacks. Inaccordance with this author these findings could be interpreted as a sign of vaso-constriction at the level ofthe larger cerebral arteries. Moreover, the flow velocity values, although increased, did not reach those levelsusually seen in arterial spasms as those occurring after sub-arachnoid haemorrhage (moderate spasm:mean flow velocity > 120 cm/sec) (9).

By contrast, on the left side, together with an increased blood flow velocity a marked increase ofsysto-diastolic ratio and pulsatility index was seen (middle carotid artery > anterior carotid artery). This lastfeature (increased systo-diastolic ratio and pulsatility index) is commonly considered to be related to a rise ofthe vascular resistance at the level of the more peripheral arterioles (10-12).

Thus, the Transcranial Doppler Sonography data indicate that in this patient not only a vaso-

Table 1. Doppler flow velocities (cm/sec), systo-diastolic (S/D) ratio, and pulsatility index (PI) during theattack of familial hemiplegic migraine and pain-free period.

Right MCA Left MCABasal Attack % Variation Basal Attack % Variation

Syst. 88 132 +50 80 150 +87.5Diast. 34 68 +100 36 48 +33.3Mean 52 84 +61.5 44 70 +59S/D 2.5 1.9 -24 2 3.1 +55PI 1.03 0.76 -26.2 1 1.45 +45

Right ACA Left ACABasal Attack % Variation Basal Attack % Variation

Syst. 72 120 +66.6 60 106 +76.6Diast. 36 62 +72.2 38 52 +36.8Mean 42 76 +80.9 34 62 +82.3S/D 2 1.9 -5 1.6 2 +25PI 0.85 0.76 -10.6 0.64 0.87 +35.9

MCA = middle cerebral artery.ACA = anterior cerebral artery.Basal = pain-free period.

constriction of the basal vessels but also an involvement of the distal vascular bed on the left side was present.This finding appears to be well correlated with the neurological symptomatology presented by the patient(right-sided hemiparesis, aphasia).

The persistence of the neurological symptoms during the Transcranial Doppler Sonography recording couldaccount for the different findings encountered in this case if compared with those obtained in classic migrainepatients recorded some hours after the disappearance of the neurological aura (5).

The CT scan findings observed in our case confirm the absence of cerebral oedema as previously observed byothers (13); this could lead to exclude a possible role of brain oedema in determining the neurologicalsymptomatology in this syndrome.

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