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Brain & Development 19 (1997) 148-151

Case report

Acute confusional migraine and migrainous infarction in childhood
Atsuo Nezu a'*, Seiji Kimura a, Noriyuki Ohtsuki a, Miyabi Tanaka a, Shigeo Takebayashi b
aDepartment of Pediatrics, Urafune Hospital of Yokohama City University, 3-46 Urafune-cho, Minami-ku, Yokohama 232, Japan
bDepartment of Radiology, Yokohama City Kowan Hospital, Yokohama, Japan

Received 15 March 1996; accepted4 September 1996

Abstract
We report two children with acute confusional migraine (ACM) and another with migrainous infarction (MI), aged 7-12 years. There
was a family history of migraine in all patients. The patients, who were all right-handed, all manifested sudden onset of consciousness
disturbance and other neurological deficits as the first aura in their life. The symptoms in all cases almost completely resolved
spontaneously within 24 h, but transient occipital slowing on EEG with laterality corresponding to the side of migrainous origin lasted
more than 24 h. In the cases of ACM in the critical phase, although MRI and MR angiography showed no abnormal findings, IMPSPECT performed within 48 h of migraine attacks revealed a regional change in cerebral blood flow, which in one particular case
demonstrated hypoperfusion in the left posterior cerebral artery (PCA) territory. Therefore, although ACM was diagnosed clinically by
exclusion, SPECT was thought helpful for the diagnosis of ACM. We speculated that transient hypoperfusion affecting the dominantsided PCA territory involving the medial temporal structures was responsible for the confusion with amnesia in ACM, in contrast to the
lack of confusion or amnesia in the case of MI showing cystic encephalomalacia in the right thalamic and hippocampal regions. ©
1997 Elsevier Science B.V.
Keywords: Acute confusional migraine; Migrainous infarction; SPECT; MR angiography

1. Introduction
Transient abrupt alteration of consciousness in childhood is most commonly caused by non-convulsive epileptic status, and is rarely associated with drug intoxication,
encephalitis, head trauma, toxic metabolic encephalopathies, or hysteria attack. Acute confusional migraine
(ACM), reported by Gascon and Barlow, is considered a
much rarer cause in childhood [1-3]. In spite of the relatively high prevalence of migraine in children which
ranges from 1.1 to 5.0%, serious auras such as altered
consciousness, paresthesias and paresis are rarely seen,
since more than 90% of migraine in childhood comprises
common migraine [4-6]. Migrainous infarction (MI) is
likewise uncommon in children [7,8]. Riikonen and Santavuori reported that migrainous stroke was diagnosed in
six of 42 children suffering from arterial stroke [9]. For the
diagnosis of MI, it is important to exclude other causes

* Corresponding author. Tel.: +81 45 2615656; fax: +81 45 2433886.

0387-7604/97/$17.00 © 1997 Elsevier Science B.V. All rights reserved
PI1 S0387-7604(96)00551-7

such as Moyamoya disease, cerebral angiitis, antiphospholipid syndrome, and mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes (MELAS).
In this paper, although ACM was diagnosed by exclusion,
the diagnostic value of EEG, MRI, MR angiography
(MRA), and single-photon emission computed tomography (SPECT) was evaluated in the critical phase of
ACM; the genesis of confusion with amnesia in ACM is
also discussed.

2. Methods
EEG was performed with scalp electrodes according to
the international 10-20 system. Both MR/and MRA were
simultaneously carried out on a Signa 1.5 Tesla system
(General Electric, USA) without contrast enhancement.
Spin-echo Tl-weighted images (TR 440 ms, TE 15 ms)
and T2-weighted images (TR 2500 ms, TE 110 ins) were
obtained in the axial and coronal planes. MRA was performed using both time of flight (TR 28 ms, TE 8 ms) and

A. Nezu et aL /Brain & Development 19 (1997) 148-151

phase contrast (TR 24 ms, TE 8 ms) methods. SPECT was
carried out using Multi-SPECT (Siemens, Germany). A
peripheral arm vein was injected with 111 MBq (3 mCi)
123I-iodoamphetamine (IMP), and data acquisition was
started 20 min after injection. Images were acquired into
a 128 x 128 matrix with 3.3 rnrn slice thickness. Images
were analyzed qualitatively for defects in IMP uptake.

3. Case reports
3.1. Case 1

A 7-year-old boy complained of minimal pain behind
the ears. One hour later, confusion suddenly developed
following visual blurring and vomiting, and he was
admitted to our hospital. Physical findings on admission
consisted of pallor, confusional disorientation, and mild
signs of amnesia. Severe pulsating headache in the occipital area developed 3 h after the onset. The next morning,
he was completely oriented, and no symptoms other than
slight headache were present. EEG on admission revealed
posterior slowing with left-sided dominance. MRI and
MRA, which were simultaneously.performed within 24 h
of the onset, revealed no abnormal findings. 123I-IMPSPECT carded out within 48 h of the onset showed
decreased cerebral blood flow (CBF) in the left posterior
splenium region (Fig. 1A). No further headache recurred
over the following 6 months since starting dihydergot at a
daily dose of 1 mg.

149

3.2. Case 2

A 12-year-old girl developed abrupt onset of photophobia, confusion, aphasia and right hemiplegia, followed by
severe pulsating headache in the left temporal region.
Aphasia and right hemiplegia subsided 2 h after the
onset. Although she manifested confusion and amnesia
on admission, they also improved spontaneously 6 h
after the onset. EEG performed the next day demonstrated
left occipital slowing (Fig. 2). MRI and MRA performed
within 24 h revealed no abnormal findings. She was treated
with propranolol at a daily dose of 10 mg. Nevertheless,
she experienced two occurrences of aura consisting of
numbness of the left hand and left-sided common migraine
over the following month, lz3I-IMP-SPECT carried out
two days after the last attack of common migraine showed
hyperperfusion of the left temporal region (Fig. 1B). Dihydergot was added at a daily dose of 1 mg, and there was no
recurrence of migrainous episodes over the next 18
months.
3.3. Case 3

A 7-year-old girl, who continued to suffer from common migraine occurring almost twice a week over 6
months, developed a severe pulsating headache of the
right temporal region accompanied by vomiting and drowsiness, starting in the evening. The next morning, she
noticed left hemiparesthesia despite relief of headache.
She promptly underwent CT scan of the head (Fig. 3A

Fig. 1. Early images of 123I-IMP SPECT. A: Case 1 shows decreased cerebral blood flow (CBF) in the left part of the splenium of the corpus callosum
(arrow). B: In case 2, CBF is increased in the left temporal region (arrows).

150

A. Nezu et al. / Brain & Development 19 (1997) 148-151

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symptoms such as nausea, pallor, and abdominal pain are
more pronounced, while auras, especially paresthesias and
motor paresis, are rarely seen, since migraine with aura
comprises less than 10% of migraine in childhood
[5,6,10,11]. On the other hand, although drowsiness is
occasionally noted as a manifestation of aura in adults,
most patients with ACM characterized by auras of confusion, disorientation, and amnesia are reported to be
younger than 21 years of age [2,3]. It was also reported
that ACM can occur as the initial episode of aura. Cases 1
and 2, who had no history of migraine, were also admitted
with the first auras in their life of confusion and amnesia
preceding severe pulsating headache. Although case 3 had
suffered from common migraine over the previous 6
months, infarction lesions developed during the initial
aura, and a relationship between migraine attack and
infarction was strongly suspected. The clinical diagnoses
of ACM and MI were made by exclusion after the symptoms resolved spontaneously with sleep within 24 h, since
these episodes developed as the initial manifestation of
migraine with aura and the criteria of migraine with aura

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Fig. 2. EEG o f case 2 displays slowing aCtivities in the left posterior
areas.

and Fig. 3B), but no lesions were detected. Hemiparesthesia abated spontaneously the next day, but numbness on
the left hand lasted 2 more days. She was referred to our
hospital 3 days after the onset. EEG showed occipital
slowing with right-sided dominance. Infarction in the
right thalamic and hippocampal regions was detected on
MRI performed a week after the onset (Fig. 3C and Fig.
3D). MRA showed no abnormal findings. 123I-IMP-SPECT
w a s also performed a week after the onset and showed
reduced CBF in the same areas as the lesions revealed
by MRI. She was treated with propranolol at a daily
dose of 5 rag, and experienced no recurrence of episodes
during the following 12 months.
All cases were right-handed. No cases had previously
experienced a transient stroke, or an attack of migraine
with aura or epilepsy. There was a family history of
migraine in all cases. Blood and urine examinations
including levels of lactate, pyruvate and amino acids
yielded no abnormal results in all cases. There was no
evidence of hypercoagulable state, and anti-cardiolipin
antibody was negative.

4. Discussion

The characteristics of migraine attacks in childhood
seem to differ somewhat from those in adults. Autonomic

Fig. 3. Axial CT scans in the critical phase and MR images a week after
the onset in case 3. The CT scans (A, B) reveal no abnormality. Infarction in the right hippocampal region (C, arrow head) and the right thalamus (D, arrow), both of which were fed by the posterior cerebral
artery, is seen in the Tl-weighted, 440/15, axial images.

A. Nezu et al. / Brain & Development 19 (1997) 148-151

of the International Headache Society were not completely
fulfilled because o f the absence of repeated attacks.
M I is defined as cerebral infarction consequently occurring during the course of a prolonged aura. To exclude
cerebral infarction due to other causes coexisting with
migraine attack, thorough investigation is necessary [7].
Several disorders associated with a hypercoagulable state
such as vasculitis, anticoagulant deficiencies, M E L A S ,
M o y a m o y a disease, fibromuscular dysplasia, and cardiac
diseases may be present, although the cause of infarction
in childhood is unknown in about 50% of cases [9]. During
the postictal phase in case 3, M R A and SPECT showed no
additional specific findings, but were helpful for excluding
expanding vasculopathies.
In the clinical evaluation of A C M in the critical phase,
visual aura and left-sided occipital slowing on EEG associated with migraine attacks, although not specific findings, suggested transient ischemic change in the left
posterior cerebral artery (PCA) territory [12,13]. For
example, generalized seizures may be followed by transient confusion with amnesia accompanied by occipital
slowing. However, in the present cases, the period of continued EEG slowing despite neurological improvement
was considered more prolonged than that in patients with
epileptic disorders. M R A was not able to detect laterality
in the size of the middle or posterior cerebral artery even
within 24 h of the onset. SPECT seemed to show the most
meaningful findings, although SPECT examinations were
unfortunately not performed with a consistent temporal
relationship. The finding in case l of a regional decrease
in CBF in the left part of the splenium of the corpus callosum during the critical phase, which suggested the relationship of A C M with transient focal ischemic change,
was more diagnostic than the findings in case 2. The finding in case 2, of hyperperfusion in the left temporal region
after an episode not of A C M but of c o m m o n migraine, was
consistent with the findings in c o m m o n migraine and suggested that case 2 had a migrainous constitution [14-16].
Although the pathogenic mechanism of A C M is inconclusive, Sheth et al. reported the relationship of A C M to
transient global amnesia [3]. Since ictal SPECT studies
had not been reported in ACM, they speculated that bilateral hypoperfusion affecting the occipital and hippocampal regions is causative. The present cases of A C M also
manifested not only confusion but also amnesic syndrome,
which is characterized by an inability to acquire new memories and some degree of retrograde amnesia but with
adequate intellectual function; A C M in case 1 evaluated
with SPECT performed in the critical phase may especially be linked to transient ischemic impairment in the
dominant-sided PCA territory involving the medial tern-

151

poral structures. In contrast, the case of MI showing cystic
encephalomalacia in the right thalamic and hippocampal
regions had no amnesic syndrome. Since amnesia due to
left P C A occlusion is considered to be more commonly
accompanied by confusion than that caused by other focal
cerebral lesions responsible for transient amnesic syndrome [17], we hypothesized that confusion with amnesia
in A C M originated from the involvement of the left PCA
territory and not the right PCA territory. All cases were
very responsive to low-dose prophylactic treatment. In
conclusion, SPECT findings in the critical phase were considered helpful for the diagnosis of ACM.

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