675
ALTERNATING
HEMIPLEGIA
IN
CHILDHOOD:
A
REPORT
OF
EIGHT
PATIENTS
WITH
COMPLICATED
MIGRAINE
BEGINNING
IN
INFANCY
Simon Verret, M.D., and John C. Steele, M.D., F.R.C.P.(C)
From the Division of Neurology and the Department of Paediatrics, The Hospital for Sick Children, Toronto
ABSTRACT. In this report eight patients are de-scribed who suffer an unusual symptom complex of
intermittent, alternating hemiplegia. Symptoms have begun in early childhood and in one child at age 3 months. Typically, there is a brief vasomotor
prodrome and then the abrupt onset of hemiplegia.
Contralateral headache tends to develop soon after
the onset of neurological symptoms. Recovery is
usual within several hours but may be prolonged over several days. Recurrence during early
child-hood is frequent.
Serial electroencephalograms have shown fluctu-ating and inconstant slow wave abnormalities as
occur with cerebral ischaemia, but radiological in-vestigations have failed to indicate structural or
dynamic changes in cerebral blood vessels. The
treatment of the disorder is symptomatic and
anti-serotonin compounds have not been found to be
effective.
As these children have matured, attacks have
be-come less frequent and in some a more characteris-tic picture of migraine has emerged. In several
patients residual neurological signs including de-mentia and dyskinesia are now present.
Because convulsions occasionally occur with the hemiplegia, differentiation from a primary seizure disorder is often difficult. The profile and evolution
of symptoms, however, suggests that these patients
suffer a primary vascular disorder within the cate-gory of migraine, but they are unusual in the early
onset of symptoms and the fixed neurological
defi-cits that may ensue. Pediatrics, 47:675, 1971,
MI-GRAINE, INFANTILE HEMIPLECIA, CHILDREN, IN-FANCY, ANGIOCRAPHY.
T
HE migraine sindrome encompasses abroad group of paroxysmal vasomotor
symptoms which may accompany or occur
independently of headache.” The
caus-ative mechanism in the majority is
un-known. Analgesics, sedatives, and
vasospas-tic drugs ameliorate the headache but
specific therapy must await the establish-ment of etiology.
Headache is a common symptom in
childhood. Bille’ surveyed 9,000 Swedish
school children and found that 75% had
ex-perienced headache by 15 years of age,
a!-though only 5% of these had what he
con-sidered to be typical migraine. The Mayo
Clinic reported an incidence of only 92 in
60,000 children examined. The incidence
depends on the criteria for diagnosis.
Many patients experience brief and
tran-sient neurological symptoms as an aura
prior to the onset of headache. The
corn-monest are scintillating scotornata; less
corn-mon are focal cerebral and brain stem
symptoms. Rarely, more protracted cerebral deficits such as hemiplegia occur.5’#{176} This
form of the migraine syndrome is termed
“herniplegic,” or complicated migraine, and is usually familial. Whitty,1#{176} Rosenbaum,1’ and Islerl2 described such patients with
symptoms beginning in late childhood and
adolescence.
This report concerns eight patients who
have suffered intermittent hemiparesis of
varying severity since infancy. Four have
residual neurological deficits of mental
re-tardation and movement disorder. Some
never experienced headache but all had rel-atives who suffer migraine. This observa-tion broadens the spectrum of the migraine syndrome, extending it to the very young.
(Received August 3; revision accepted for publication November 9, 1970.)
Presented at the Fifth Canadian Congress of Neurological Sciences, Toronto, Canada, June 1970.
ADDRESS FOR REPRINTS: (J.C.S.) Division of Neurology, The Hospital for Sick Children, 555 University Avenue, Toronto 101, Ontario, Canada.
676
]-Complicated (lieniiplegic) form.
O-Alternating.
CLINICAL MATERIAL
The patients were seen in the
Depart-ment of Neurology at The Hospital for Sick
Children between 1953 and 1969. Most
have been reexamined here at regular inter-vals. All were recalled and their
neurologi-cal condition reevaluated in the spring of
1970.
Case 1
CASE REPORT
From 3 months of age this boy experienced
epi-sodes of hemiplegia involving one side or the other. The attacks, sometimes precipitated by excitement or frustration, were usually preceded by periorbital
redness, crying, and scratching of the head. Within 2 to 5 minutes he would become suddenly henri-plegic, sometimes on the right side, sometimes on the left. Occasionally there was double hemiplegia with difficulty in swallowing and speaking. The weakness usually resolved within 24 to 48 hours
though, when severe, it persisted for as long as 3
weeks. The frequency and severity of the episodes
were uninfluenced by anticonvulsants, sedatives, or methysergide.
At 3 years of age he still has five to six episodes per month. In the past year he has had four pro-longed seizures which occurred at 4 A.M., involved
the right side predominantly, and were followed by generalised weakness with difficulty in swallowing for 2 days.
He has moderate psychomotor retardation, a vo-cabulary limited to four words, and is just begin-fling to walk. He exhibits hypotonia, restlessness, and choreo-athetotic movement of the upper limbs with intermittent dystonic posturing. Repeated electro-encephalograms, performed during several hospital admissions, have been normal on occasion but at other times have shown fluctuating and variable slow-wave activity in relation to his attacks. Bilat-eral carotid arteriograms were normal. A right
ret-rograde brachial arteriogram performed Ui hours
following the onset of profound left hemiplegia was normal. Cerebrospinal fluid was normal.
His mother suffers intermittent generalised head-ache and at age 21 had an episode of right hemi-plegia associated with left hemicrania which disap-peared within 2 days. She has had no further
neurological symptoms. There is no family history
of seizure disorder.
Case 2
A 4-month-old girl suddenly developed weak-ness of the right limbs followed shortly by a focal convulsion lasting 2 minutes. Thereafter she expe-rienced frequent recurrent episodes of hemiplegia involving one side or the other, lasting 30 to 60 minutes and accompanied frequently by abdominal pain. With minor attacks she has choreo-athetotic movement and dystonic posturing of the involved limbs. By 4 years of age a characteristic pattern of attacks emerged. The hemiplegic episodes would increase in number for 5 to 6 days and culminate in a brief generalised seizure after which she would be relatively well for 2 or 3 weeks.
Occa-sionally she experienced a different type of episode
when she abruptly became ataxic, was vertiginous, and exhibited nystagmus and gaze palsy.
Now at age 18 years she has slurring dysarthria,
hypotonicity of limbs, symmetrical choreo-atheto-sis, and intermittent dystonic posturing of face and limbs. Her intelligence quotient measured on the L.M. Stanford-Binet scale is 80. Numerous electro-encephalograms have been normal though occa-sionally there has been diffuse slow activity during or immediately following an attack. Pneumoen-cephalograms, bilateral carotid arteriograms, and
cerebrospinal fluid were normal. Various anticon-vulsants have failed to prevent attacks but mepro-bamate seems to have reduced their frequency and severity.
A female sibling experienced episodic left hemi-crania and transient right-sided weakness between
7 and 11 years of age. In the past 5 years she has had no further episodes of weakness though she
TABLE I
SUMMARY OF EIGHT CAnER
Cases 1 .9 4 5 6 7 8
Family History [+1 [+1 + [+] + + + +1
Age at Onset (months) 3 4 7 9 18 4 36
Hemiplegia + ffl +
Seizures + + -
-Headache + - - + + - - +
Retardation + + + - - - - +
does have headaches. The father has intermittent headaches occasionally accompanied by confusion. The mother suffers infrequent headaches without neurological symptoms. There is no family history of seizure disorder though many members are al-lergic.
Case 3
At 7 months of age this patient developed epi-sodes of alternating hemiplegia lasting from several minutes to several hours once or twice a month. During attacks he had choreo-athetotic movement of the involved limbs and occasionally, when the attack was bilateral, he had dysphagia and failure of lateral gaze.
\Vhen first seen at 3 years of age, he was moder-ately retarded but had no focal neurological abnor-mality detectable by physical examination, electro-encephalogram, bilateral carotid arteriogram, or cerebrospinal fluid examination.
He is now 13 years of age and continues to have shorter, less severe attacks, often triggered by
frus-tration or apprehension. He occasionally experi-ences diffuse headache unrelated to attacks of hemiplegia. He has never had a seizure.
Except that his father, a doctor, has suffered
al-ternating hemicrania for many years, there is no
family history of neurological dysfunction. Two younger siblings are asvmptomatic and of normal
intelligence.
Case 4
This 9-month-old boy developed sudden epi-sodes of left hemiplegia commonly accompanied by severe vomiting, head pain, drowsiness, and dys-tonic posturing of the head toward the left. The
attacks occurred spontaneously once per month
and the weakness slowly disappeared over 8 hours. He has never had a seizure. Neurological examina-tion, electroencephalogram, and skull roentgeno-grams are normal at 33 months of age.
Several members of his father’s family suffer
headache and his father and paternal grandfather
are liable to intermittent neck pain with torticollis to one or the other side.
Case 5
This boy was 2 years old when he began to have episodes of right hemiplegia. They lasted for from 5 hours to 2 weeks, occurred once a month, and were not mitigated by anticonvulsants. When age 3 he experienced headache, vomiting, and epigastric pain in relation to attacks. He is now 5 years old and he continues to have headache, but with only occasional transient hemiparesis.
Neurological examination of this boy is negative except for bilateral orbital bruits. Three electroen-cephalograms, skull roentgenograms, a pneumoen-cephalogram, and bilateral carotid arteriogram are all normal.
His mother suffers from intermittent headache
without neurological deficit and a maternal aunt has a seizure disorder.
Case 6
Between 2 and 43 years of age this boy had re-current episodes of nocturnal alternating hemiple-gia preceded by moaning and crying but
unat-tended by seizure. Episodes occurred three to four
times per month and lasted about 20 minutes. Ten months ago they ceased spontaneously. He is of
average intelligence and physical and neurological
examination are negative. Three
electroencephalo-grams were normal but a fourth performed shortly
after an episode of right hemiplegia showed an ex-cess of slow activity in the left mid-head region. Skull roentgenograms, bilateral carotid
arterio-grams, and cerebrospinal fluid examination were all
normal.
His brother (Case 7 ) suffers a similar disorder and the father has intermittent headaches. There is no other family history of neurologic deficit, sei-zures, syncope, or allergy.
Case 7
At age 18 months a younger sibling of Case 6 had five nocturnal episodes of alternating hemiple-gia similar in all respects to his brother’s and
oc-cursing over a 3-week period. He is now 33 months
of age and though mildly hyperactive he exhibits normal psychomotor development.
Case 8
At age 3 years this girl awoke complaining of
right-sided headache and inability to move her left
arm and leg. The arm and leg twitched for about 3 minutes. Six months later she fell striking her head on the kitchen door, cried out, became apnoeic, lost consciousness, and was cyanotic. She recovered within a minute but had a right hemiplegia which disappeared slowly during the next 2 days. From 4
to 83 years she had 10 episodes of right hemiplegia
preceded by transient paresthesiae of the right hand and face and slurring of speech. For 12 hours before an attack she was irritable and during the 24 hours the hemiplegia lasted she complained of mild headache. She is now 11 years old and has had no further attacks for 2 years although she continues to have occasional headache.
She was born 1 month before term. Her psycho-motor development was normal. From 10 months to 3 years of age she had 10 breath holding spells without seizure or hemiplegia, all precipitated by
minor injuries. She has always shown mild
spastic-itv of the legs, incoordination of the extremities, choreo-athetosis of the hands, and dystonic postur-ing of the arms. Her intelligence is normal though she is dyslexic. Repeated electroencephalograms
and a pneumoencephalogram have been normal.
On one occasion, shortly after an attack, the cere-brospinal fluid protein was elevated to 70 mg/100
TABLE 11
INVESTIGATIONS
(axes
Elect roencephalograimi normal seizure activity
slow activity or asyimimimitry related to attacks
(‘erehral Arteriography
[‘neunmoencephalogramu (‘erebrospinal 1’ltli(l
1 2 3 4 5 6 7 8
+ + + + + + + +
o 0 0 0 0 0 0 0
+ + 0 0 0 + 0 +
N N N - N N
-N N -
---
N - - NN N N - N N - *
* ‘l’ransient elevation of eerelirospinal fluid protein (see text).
N = Normal.
Her mother, maternal grandfather, and a
mater-nal great niece have intermittent hemicrania and
accompanying contralateral transient mono- or
hemiparesis. (See Tables I and II.)
DISCUSSION OF CLINICAL FEATURES
In all eight patients attacks began before 3 years of age and in 4, before 1 year.
In six patients there has been alternating hemiplegia, abrupt in onset. In the two
oth-ers it was always unilateral and on the same
side. While improvement was usual within
24 hours, in several patients the attacks
re-quired up to 3 weeks for complete
resolu-tion. In several patients there was restless-ness, choreo-athetosis, and dystonia of the hemiparetic limbs. In those several patients
with double herniplegia, hulbar symptoms of
dvsarthria and dysphagia occurred. In Cases 6 and 7 attacks occurred only during sleep. Only in Cases 1 and 4 were attacks clearly triggered by excitement or frustration. In
only two cases, 1 and 8, was there a definite
prodrorne. Four children experienced
headache in association with the
hemiple-gia. In two of these hemicrania was on the
contralateral side to the hemiparesis, but in two others the headache was diffuse. It was
never severe and usually resolved within
hours although the neurological deficit
might persist up to 3 weeks. Case 3 has
ex-perienced intermittent headache
indepen-dent of hemiplegia. Four of the children
have never suffered headache, but as
Whitty2 reported, neurological symptoms
due to migraine may occur without
accom-panying headache.
In all cases one or the other parent had a
history of intermittent headache and in four of the seven families, relatives had had
fo-cal neurological deficits in association with
headache. Cases 6 and 7 are brothers. 1 he
familial occurrence of complicated
m-graine has been stressed by Bradshaw and
Parson.6
In four of the patients residual neurologi-cal signs are apparent: two are moderately retarded, one is mildly retarded, one is
dys-lexic, and all four have a movement
disor-der such as restlessness, choreo-athetosis, or
dystonic posturing of the limbs. Two also
show hypotonia; and one shows mild
spas-ticity and ataxia. None have evidence of re-sidual herniparesis. Two have focal or
gen-eralised seizures infrequently. Case 2 had a
seizure with her initial attack and this may have been due to transient cerebral
ischae-mia. In Case 1 convulsions began 21
months after the onset of hemiparctic
epi-sodes and are independent of them.
Persistent neurological signs may follow attacks of migraine but are unusual. Hunt,7
Connor,1’ and Guest and Woolf” have
re-ported such complications in adults. These
deficits are likely a consequence of cerebral
infarction due to prolonged ischaemia
during arterial vasoconstriction. Sequelae
would be anticipated in an infant’s brain
which is more liable to ischaemic insult
during development. It is unusual that in
our patients the deficits, when present, took the form of dyskinesia rather than of
hemi-paresis. Involuntary movements following
attacks of migraine are rare but there are
\Ve were unable to correlate the occurrence
of persistent signs with the frequency and
severity of hemiparetic attacks, but seque-lae were prt’se1t 111 those whose symptoms began iii early infancy.
Electroencephalograms in all patients
sliovccl no constant focal abnormality or
evidence of seizure activity. In two patients
a single recording was nornial. In the six
others repeated electroencephalograms
l11\’( l)Cefl 1)erformed. The majority of
trac-ings ‘cre normal except that on occasion durmg or shortly after an attack asymmet-neal cerel)ral activity and slow waves were recorded froni the hemisphere contralateral to the herniplegia. Although the above
dcc-trociicephalographic findings do not
cx-elude epilepsy, they arc more compatible
with the alterations that occur with
ccre-l)ral iscbemia.
Cerebral arteriographv performed in five
pati(nts was normal. The families of Cases
4 asid 8 would not allow this procedure. Case 7 had a sibling
(
Case 6)
who sufferedsimilar cerebral symptoms and therefore we
decided arteriography was not justified.
Pneumoencephalograms were done in four latients and were normal. The cerebrospi-nal fluid, examined in six patients, was nor-mal in five and showed a transient elevation of cerebrospinal fluid protein in the sixth.
Homocystinuria was excluded by urine
amino acid chromatograms in four patients.
None had elevated blood pressure during
or l)etween attacks, or any abnormality of
the heart or cardiovascular system to
sug-gest a source of emboli.
TREATM ENT
Seven of the eight I)atients received
anti-convulsants but these have not altered the
frequency or severity of attacks. In Case 2,
rneprobamate seemed of some value.
Methy-sergide used in Case 1 had no effect.
An-giospastic drugs were not used in an’ of
these patieilts.
NATURAL HISTORY
It is 17 years since the first patient, Case 2, was seen. She is now 18 years of age and
continues to have frequent episodes of
hemi-paresis unattended by headache. Case 3,
age 13 years, also has persistent attacks
though these are becoming less prolonged
and severe. Two patients
(
Cases 5 and 8)have developed more typical migraine with
periodic headache and gastrointestinal
symptoms. Their hemiparetic attacks have
become less frequent and less severe.
At-tacks ceased spontaneously by 4 years of
age in two patients
(
Cases 6 and 7) andthey remain asymptomatic. The two
youn-gest patients,
(
Cases 1 and 4)
now 3 yearsof age, continue to have episodes of
hemi-paresis and headache.
The number of patients is too small and
the symptoms too varied to allow a definite statement of the natural history of the con-dition but the prognosis is clearly less
fav-ourable than in the usual migraine
syn-drome of older children.
COM MENT
The diagnosis of migraine as a cause of
intermittent hemiplegia is acceptable only
when electroencephalograms fail to show
seizure activity and cerebral arteriography is normal. All of our patients were initially regarded as suffering a convulsive disorder but the absence of seizure activity in
elec-troencephalograms, the arteriography
per-formed in all but three, the profile and
evo-lution of symptoms, the lack of response to
anticonvulsants, and the family histories in-dicate that symptoms were due to cerel)ral vascular ischaemia.
Otherslm1s have noted that when the
iiii-graine syndrome occurs in young children, it is usually manifest by periodic anorexia, cyclical vomiting, langour, pallor,
restless-ness, or head banging. A hemiplegic form
of migraine beginning in infancy seems to
have been unreported previously.
The prognosis of the uncomplicated
mi-graine syndrome in older children is
excel-lentme1 and mild sedation is of definite value. This report indicates that when
neu-rological symptoms occur in infancy the
dis-680
order may supervene. We have found no
effective treatment for this condition and
therapy must await a knowledge of the
etiology of migraine.
SUMMARY
This report describes eight children who
suffer intermittent hemiparesis as a likely
consequence of migraine. Symptoms began
in infancy. In six children the hemiparesis alternated and on occasion was bilateral. In four children residual neurological signs of mental retardation and dyskinesia are pres-ent. Seizures occur infrequently in two pa-tients.
The onset of hemiparesis is abrupt and in four cases has been followed by
contralat-era! or diffuse headache. Recovery, usual
within several hours, occasionally is
pro-longed for several days to weeks. In some
patients attacks recurred frequently during early childhood. Electroencephalograms
show inconstant and variable slow-wave
ab-normalities from cerebral hemispheres but
radiological investigations fail to indicate structural or dynamic changes in cerebral
blood vessels. As the patients matured,
at-tacks became less frequent and in some
cases more characteristic symptoms of
mi-graine have emerged.
Differentiation from a primary inhibitory seizure disorder is difficult but the profile
and evolution of symptoms, the family
his-tories, and electroencephalograms indicate that these patients suffer a periodic
vascu-lar disorder within the category of
mi-graine.
The prognosis is less favourable than that
of the usual migraine syndrome of
child-hood.
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AcknowIedgmen
We thank Drs. J. S. Prichard and Douglas Mc-Greal for allowing us to study their patients, and
The Department of Medical Publications, The
Hospital for Sick Children, for its help in the
