Migraine with visual aura in developing age: visual disorders.

Abstract

Visual disorders are an important symptom in the migraine of developing age. Different kinds of visual disturbances can precede, accompany or follow a migraine attack. These visual disturbances can be grouped into negative (hemianopsia, quadrantopsia, scotoma) and positive (phosphene, teicopsia, metamorphopsia, macropsia, micropsia, teleopsia, diplopia, dischromatopsia, hallucination disturbances) disorders. The pathogenetic mechanism of the visual phenomena of migraine has not yet been clarified. Various hypotheses have been proposed: vasospasm with consequent ischemia of some cerebral areas, the opening of arteriovenous shunts between the intra and extra cerebral circulation, the formation of microthrombi in arterioles and dopaminergic hypersensitivity of some nervous centers. We have studied 1787 children, affected by migraine with (13%) or without (87%) aura. Among the patients, 211 (12%) referred visual disorders, especially scotoma and phosphene. These data let us hypothesize that a relationship between migraine and visual disorders is present also in pediatric age. However this relationship is less important than in adults.

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These disorders can alter vision through a vi-
sus impairment (negative phase) or the ap-
pearance of excitatory phenomena (positive
phase). These two phases can follow each
other as in flittering scotoma or appear sepa-
rately3. Negative visual disorders are amau-
rosis (a transient mono or bilateral total
blindness) and hemianopsia (absence of the
visual function in half of the visual field).
Positive visual disorders are: phosphenes, te-
icopsias, metamorphopsias, macro or mi-
cropsia and teleopsias. When there are mul-
tiplied images we have diplopia or poliplop-
ia. The aim of our study was to identify the
possible association between migraine and
visual disorders in evolutive age and to de-
fine which of the visual disturbances is more
common in childhood.
Materials and Methods
We have examined 1787 children referred
to our Unit between 1981 and 1995. The ex-
amined group consisted of 943 males and
844 females; age range was 3-15 years (mean
age 6.6 years). Two hundred thirty patients
(13%) were affected by migraine with aura
and 1557 (87%) by migraine without aura.
In order to rule out secondary cephalalgia, a
series of laboratory and instrumental exams
were requested; biohumoural and hemato-
chemical tests, urinalysis, X-rays of the skull
and of the paranasal sinuses, visual examina-
tion with visual field, EEG. All examina-
tions resulted within normal limits.
Moreover we have considered the relation-
ship of any visual disorder with the follow-
ing risk factors: headache index, familiar
European Review for Medical and Pharmacological Sciences
71
Abstract. – Visual disorders are an impor-
tant symptom in the migraine of developing age.
Different kinds of visual disturbances can pre-
cede, accompany or follow a migraine attack.
These visual disturbances can be grouped into
negative (hemianopsia, quadrantopsia, sco-
toma) and positive (phosphene, teicopsia, meta-
morphopsia, macropsia, micropsia, teleopsia,
diplopia, dischromatopsia, hallucination distur-
bances) disorders. The pathogenetic mecha-
nism of the visual phenomena of migraine has
not yet been clarified. Various hypotheses have
been proposed: vasospasm with consequent is-
chemia of some cerebral areas, the opening of
arteriovenous shunts between the intra and ex-
tra cerebral circulation, the formation of mi-
crothrombi in arterioles and dopaminergic hy-
persensivity of some nervous centers. We have
studied 1787 children, affected by migraine with
(13%) or without (87%) aura. Among the pa-
tients, 211 (12%) referred visual disorders, espe-
cially scotoma and phosphene. These data let
us hypothesize that a relationship between mi-
graine and visual disorders is present also in
pediatric age. However this relationship is less
important than in adults.
Key Words:
Visual disorders, Migraine.
Introduction
Visual disorders are considered as the
most representative symptoms of migraine
with aura and can accompany the pain and
exceptionally persist after the end of the cri-
sis1. Such disorders can last from a few min-
utes to one or two hours; their persistance
can be the first expression of an involvement
of the optical nerves and/or of the retina sec-
ondary to phlogistic or invasive processes2.
Migraine with visual aura in developing age:
visual disorders
D. LENDVAI, R. CRENCA, P. VERDECCHIA, A. REDONDI, E. TURRI,
S. PITTELLA, C. ANANIA
Department of Pediatrics Division, “Diagnosi e Cura Cefalee Infantili”
“La Sapienza” University - Rome (Italy)
1999; 3: 71-74

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72
trait, recurrent abdominal pain (RAP), limb
pains, cyclic vomiting, kinetosis, sleep disor-
ders, vertigo, hyperactivity. In the group of
patients with aura, 211 (12% of the total of
patients studied) referred visual disorders;
of these patients 96 (14%) were males and
118 (56%) females.
Results
Among the visual disorders present in
the migraine with aura group, we observed:
phosphenes, scotomata, foggings, teichop-
sias, amaurosis, diplopias, photopsias,
hemianospias, quadrantic hemianopsias,
metamorphosias. There were no hallucina-
tions and dyschromatopsias, which are
more frequent in adults. We recordered the
frequency of each single disorder: phos-
phene4were present in 74 patients (37,7%)
and scotoma in 49 (25%), thus demonstrat-
ing a possible concurrence between positive
and negative disorders. Furthermore,
among the patients positive for visual disor-
ders, 65% were positive for familiar trait,
and 50% had a positivity of cephalalgic risk
factors, such as hyperactivity. Finally, 70%
of visual disorders accompanied the mi-
graine attack while 30% were prodomic.
Scotoma showed a peculiar characterist,
since it appeared more frequently as a pro-
dromic symptom (64%) than as an epiphe-
nomenon (36%).
Discussion
No single etiopathogenic theory is able
to explain the visual phenomenon that ac-
company migraine attacks. Many authors
suggest that a vascular mechanism of is-
chemic type is at the origin of aura. The
first studies in this field are due to Wolff et
al4who demonstrated that visual disorders
regressed with amyl nitrite and appeared
with ergotamine. The evident reduction in
the cerebral flow during aura has been
shown by Skyhøj et al5and Olsen et al6by
intra-arterial infusion of Xenon 133 under
tomographic control and by Nattero et al
by Doppler ultrasound7. Some authors8
have shown that the opening of arterial-ve-
nous shunts between the intra-and-extrac-
erebral circulation can determine a flow re-
duction at the level of cerebral cortex with
consequent ischemia that could be sec-
ondary to the formation of arteriolar mi-
crothrombis9. In Leao’s Child Depression
Scale (C.D.S.)10visual disorders have been
correlated to an intense initial neuronal ex-
citation, provoked by different nociceptive
stimuli, followed by a wave of extremely
reduced electrical activity propagating in
the postero-anterior sense11,12. C.D.S. has
been experimentally reproduced both in
animal models and in man13,15; the most
employed experimental methods have been
the electrical or mechanical stimulation
and the injection of high KCl concentran-
tions. This latter technique shows the im-
portance of the biochemical activity of
some substances16-18. Different studies have
evaluated the role of ATP, pH, and phos-
phocreatine intracellular concentrations19.
N-methyl-d-aspartate (NMDA) has been
identified as one of the most important re-
ceptors for C.S.D. triggering and propaga-
tion20. Recently, it has been observed that
in patients affected by migraine with aura,
plasmatic glutamate levels are high21.
Some authors have correlated this finding
to low levels of magnesium which facili-
tates the development of a vasoconstric-
tion22. Anyhow, vasomotor activity is con-
nected to hormones and neurotransmitters
activity. According to Sicuteri23, essential
headache is the expression of a specific dis-
order of the nociceptive system on which
the pain-producing functions depend24.
Many substances act at this level: serotonin,
catecholamines, bradykinin, angiotensin,
endorphins, P-substance, etc25. Migraine
symptoms including visual ones, could be
related to a dopaminergic hypersensitivity
of some nervous centres19. It is believed
that visual disorders are due to an involve-
ment of the optic pathways (retina, optic
nerve, chiasm, optic tracts, cortex). When
the disorder is monolateral (scotoma,
amaurosis, hemianopsias) it originates from
the retina or the optic nerve or the lateral
portion of the chiasm. If the disorder is bi-
lateral, its most likely origin will be in the
central part of the chiasm; when the campi-
metric defect is bitemporal, or in the optic
D. Lendvai, R. Crenca, P. Verdecchia, A. Redondi, E. Turri, S. Pittella, C. Anania

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tracts of the cortex, then it will be lateral
and homonymous26. The common total
amauroses, and rarely foggings, originate
from an involvement of both the optic
tracts or occipital areas. As far as excitation
phenomena are concerned, phosphenes and
teichopsia originate from the occipital cor-
tex where they have also been experimen-
tally reproduced by electric stimulation27.
Perceptive distortions (both micro and
macro-metamorphopsias and teleopsias)
and visual hallucinations originate from the
tempoparietal cortex.When other disorders
such as vertigos, diplopia, ataxia, alternat-
ing paresis, mental confusion and nystag-
mus are associated to the visual symptoma-
tology of cortical origin, probably all areas
served by the vertebrobasilar arteries are
involved28. Data emerging from our study
suggest that in evolutive age visual disor-
ders appear both as prodromic and accom-
panying phenomena of migraine, thus
showing a diagnostic relevance. For this
reason,their presence does not indicate an
organic damage unless they persist for
more hours. However, it should not be for-
gotten that migraine and aura in particular,
often scare the small patient and his/her
parents. When aura appears a prodromic
sympton, it is experienced with anxiety
and fear and visual disorders as accompa-
nying factors worry both the patient and
parents. We believe that a better and his/
her deeper understanding of this sympto-
matology can prevent this syndrome in a
great number of small patients who, other-
wise, in the greater majority of cases, are
likely be destined to develop severe forms
of migraine over the years.
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