Jean Holowach, M.D., Donald L. Thurston, M.D., and James L. O’Leary, M.D.
Department of Pediatrics and Department of Psychiatry and Neurology, Washington University School of
Medicine, and the St. Louis Children’s Hospital
PETIT MAL
EPILEPSY
893
M
OST STUDIES on petit mal have been concerned with pathophysiology,electroencephalographic correlates, or an
evaluation of new drugs. This paper is de-voted to a clinical review of the natural life histories of 88 children with petit mal
epilepsy. It emphasizes aspects of this dis-order which have seldom been documented -the frequency of other seizures, the
pres-ence of organic brain damage, and diii-culties in diagnosis and treatment.
DEFINITION, DESCRIPTION, AND
DIFFERENTIAL DIAGNOSIS
Gowers1 defined petit mal as a “transient loss of consciousness without conspicuous
convulsion.” Unfortunately, there is a
tend-ency to diagnose any brief nonconvulsive
seizure as petit mal without sufficient re-gard to the state of consciousness. If a
pa-tient is conscious, if he sees, hears, smells, tastes, or “feels” during a seizure, it is not petit mal. Two other aspects of petit mal are just as important in the diagnosis as tile
transient loss of consciousness and the lack of conspicuous convulsion. These are the lack of aura and the lack of postictal phenome-non. Particularly since the level of conscious-ness is sometimes difficult to ascertain, we would like to extend Gowers’ definition to include “without warning and without after
effect.” The addendum would promote more frequent differentiation of petit mal from
other brief seizures “without conspicuous convulsion.” Many patients with psycho-motor epilepsy, particularly those who
ex-hibit only mastication, arrest of activity, or
brief automatism, are given a misdiagnosis of petit mal. In children with psychomotor
seizures, aura is almost always present. Even the youngest make clear an awareness of their predicament. They cry out, reach
for support, or run to their parents. By contrast, in petit mal there is always sudden onset without warning or premoni-tion. Whereas there is no “conspicuous con-vulsion” in petit mal, movement may occur,
and in fact we have found it to be more usual than immobile staring. Classically this is described as eye blinking or eye “rolling.” Far more characteristic in our experience is the rhythmic sway of the
head and neck or the upper part of tile
body forwards, laterally or backwards-this may be barely perceptible, or occur to an extreme degree. Nevertheless, maintenance of posture is usual. Patients seldom fall
dur-ing a petit mal attack. This fact is of tre-mendous assurance to anxious parents and
teachers. Only 4 of tile 88 children had a history of occasional falls. Most of these occurred during attacks while climbing or descending stairs. Although they have been described, we have never seen color
changes or sweating in petit mal. There is no drooling. Incontinence occurs infre-quently. Fifteen of the 88 patients had
in-voluntary micturition during attacks. This
is probably the most embarrassing feature
of all in petit mal. Highly characteristic but certainly not peculiar to petit mal alone is sllortness of duration. Though parents esti-mate a duration of “just a few minutes,” it is really only a matter of seconds, seldom
more than 15 or 20. If a seizure lasts very mucil longer than this, a diagnosis of petit mal should be seriously questioned.
There are no postictal effects with petit
(Submitted August 21, 1961; accepted for publication July 2, 1962.) ADDRESS: (J.H.) 500 South Kingshighway, St. Louis 10, Missouri.
894
mal. One never sees the fatigue, drowsi-ness, or deep sleep so common after even the briefest of psychomotor or jacksonian
seizures. Two of the older children claimed sometimes to know when they had had an attack. Their perception could not be de-scribed further. Some acted “sheepish” or
“embarrassed” afterwards. These reactions
could not always be ascribed to the child’s awareness of the attention being given by observers or to his loss of contact. Yawning
immediately after seizures, induced by hy-perventilation, has been recorded several times. Though for several days in a row, a subject may be seizure-free, petit mal most
often occurs daily, varying between ex-tremes of single attacks and epileptic status. In the untreated patient, the individual’s frequency pattern can be so constant as to
serve as an ancillary criterion in diagnosis. Petit mal is so characteristic that a
dif-ferential diagnosis is hardly necessary. Ex-cept in the rare instances where it is mis-taken for tic or behavior disorder (and once
in our experience for a urinary disorder characterized by persistent incontinence), petit mal can be recognized by the least
ex-perienced of observers. However, prevalent use of the term “petit mal” for other brief nonconvulsive attacks without careful dis-crimination has led to much confusion. Since anti-petit-mal therapy is often
spe-cffic, seizure differentiation is desirable on therapeutic as well as nosologic grounds. Not rarely during a petit mal seizure, phe-nomena somewhat reminiscent of psycho-motor or jacksonian epilepsy may be
seen-fumbling with clothes, mouth movements, licking, twitching, pursing (17 cases), hum-ming or singing (4 cases), mumbling (4
cases), or jerking of an arm or shoulder. In each of these situations, the clinical diag-nosis was unequivocally petit mal-loss of consciousness for only a few seconds, with-out aura, without color changes or
drool-ing, without postictal effects, and repro-duced repeatedly by voluntary
overbreath-ing.
Hyperventilation in particular, the
elec-troencephalogram, and a response to
spe-cific therapy are additional aids in the
diag-nosis of these cases.
The remaining members of Lennox’s
“petit mal triad,” myoclonic and akinetic seizures, although also without aura and
postictal effects unlike petit mal, are char-acterized by “conspicuous convulsion.”
The myoclonic seizure classically con-sists of a sudden single violent momentary jerk of a body part. This is often the head (head nodding) but may involve the whole
body (salaam). Repeated episodes over a 5-to-lO-minute period, or longer, are dis-tinguished from grand mal by eliciting a
return to consciousness or the crying which occurs between successive massive spasms. After prolonged myoclonic jerks, sleep may
occur. Onset of sleep is more likely due to
exhaustion than to a postictal state. It could be coincidental, since these seizures are often related in occurrence to the begin-ning or the end of normal sleep. We agree with Gastaut,2 who believes that on both
clinical and laboratory grounds it is not
possible to distinguish between astatic or akinetic seizures and myoclonic epilepsy.
Distinctions made between pykolepsy and petit mal are unwarranted.
INCIDENCE
Using strict criteria of diagnosis as de-scribed above, the occurrence of petit mal
either alone or combined with grand mal is not common. This series comprises 88 cases. Twenty-eight were private cases. The remaining 60 were culled from a group of 1,054 children registered in the
convul-sive clinic of St. Louis Children’s Hospital, a patient incidence of only 5.6%. Two
hun-dred thirty-eight of the 1,054 epileptic
chil-dren experienced two types of seizures; 38, three types; and 2, four types. The
fre-quency distribution of seizure types is shown in Table I. The relative rarity of petit mal is evident. Myoclonic and
psycho-motor epilepsy are each twice as common.
SEX AND RACE DISTRIBUTION
Both Lennox3 and Livingston4 found
I I I I I I I I I I I
3 5 7 9 II 13
AGE IN YEARS
895
FIG. 1. Age of onset of petit mal in 88 patients.
TABLE I
TOTAL SEIZURES
Type of Seizure
Cases
Number Per Cent
Psychomotor
Grand mal
“Syncopal”
Petit mal
Myoclonic (akinetic)
Jacksonian
Febrile
Breath-holding
Headaches
Abdominal pain
Visual
Combinations of above
three types
1l0
40
67
60
101
204
265
68
26 19 1
1
8.7
4.9
4.4
7.3
14.8
4.9
1 .9 1 .4 1.5
1.5
Total 1,374 ..
* Aura, loss of consciousness with limpness or tonus without clonus and with postictal effects.
No preponderance was evident in this
group. Forty-two were boys, 46 were girls. Seventy-seven were white, 11 were Negro
children.
AGE OF ONSET
The age of the children at the time of onset of petit mal is shown in Figure 1. Only three had a time of onset under 2
years of age; one at 9 months; two at 22
months. Eighty per cent began between 23
and 9 years of age, with a peak incidence at age 6.
7
5
13
(I, 10 U)
4
U
ui7
z
3
DURATION OF PETIT MAL PRIOR TO FIRST VISIT
The refractory nature of petit mal is
evident from the duration of symptoms prior to the first visit. Fifty-seven children, almost two-thirds of the group, had had
petit mal for over 1 year; 37 (42%) for 1 to 3 years; 14 (15.9%) for 3 to 6 years; and
6 (6.8%) for more than 6 years.
FAMILY HISTORY OF SEIZURES
In six cases there was no reference to a genetic history of epilepsy, or it was
un-known, uncertain, or remote. In the remain-ing 82 cases, the family history was
posi-tive in 35, or 42.6%. Parents or siblings were affected in 17, or one-half, of these cases.
Limiting a “positive” family history to
af-fected parents and siblings only, there is a 20.7% incidence in our series. Familial
in-cidences of children with Jacksonian5 and
psychomotor compared with petit mal epi-lepsies in our series are 14.7 and 23.0% re-spectively.
PATHOLOGY OF THE CENTRAL NERVOUS SYSTEM
Only nine patients, 10%, had brain dam-age of recognized etiology-one congenital microcephaly, one tuberous sclerosis, one
Sturge-Weber syndrome, one craniosynosto-sis, one neonatal intracranial hemorrhage, and four post-encephalomyelitis (three
poli-oencephalomyelitis). Whereas this inci-dence of apparent central nervous system lesions is considerably less than that found
in our reviews of jacksonian (34%) and psy-chomotor epilepsy (33%) the over-all
in-cidence of brain damage and mental re-tardation for the group is quite high, 24% (21 cases), and practically identical to that
found in our cases of jacksonian and psy-chomotor epilepsy.
ASSOCIATION AND SIGNIFICANCE OF OTHER SEIZURES
5 An important aspect of petit mal is the
high incidence of other seizures, and
fre-quently alluded to, this fact has seldom been documented. Fifty-four of our chil-dren (61%) experienced non-petit-mal sei-zures; 37% occurred prior to, 16% during,
and 46% after the onset of petit mal. Of
the 20 patients with antecedent
convul-sions, there were 10 with febrile, 6 with
grand mal, 3 with breath-holding, 3 with
jacksonian, and 1 with myoclonic seizures. Three had had two types of seizures pre-viously. The interval before the onset of petit mal ranged from 3 months to 7% years
-less than 1 year in 6 cases, and more than 4 years in 6 cases. Other seizures
appear-ing at about the same time as the petit mal
attacks in nine cases were grand mal, six; grand mal and phychomotor, one; jackso-nian and psychomotor, one; and febrile, one.
Of tile 25 children who first experienced other seizures after the onset of petit mal,
22 had grand mal, 1 psychomotor, and 2 both grand mal and phychomotor attacks.
Two of the children with grand mal also had febrile seizures. Tile interval before
the onset of later convulsions varied from 3 months to 93 years. In 7 the time was
less than 1 year, in 10 it exceeded 4 years. These data merit comment. In the case of psychomotor epilepsy it has been sug-gested that previous convulsions, particu-larly febrile seizures of infancy may dam-age the temporal lobe resulting either in cerebral dysrhythmia or psychomotor
at-tacks. Our own experience supports this contention. In a group of 120 children with
psychomotor epilepsy the incidence of pre-ceding febrile seizures was 22.5%. By con-trast only 10 children (8.89) had febrile
sei-zures prior to the onset of petit mal.
Con-sidering the frequent association of other
seizures with petit mal, particularly grand mal (42% of the total series), it is more rea-sonable to consider previous febrile sei-zures as an early manifestation of epilepsy
“triggered” by fever and infection.
Fur-thermore, in 62.9% of the children who had
other seizures these first appeared coinci-dent with or after the onset of petit mal
and so could hardly have been responsible
for either previous brain injury or
conse-quent petit mal.
INTELLIGENCE
Several authors’ have remarked on the
high intelligence of petit mal patients.
Whereas at least three of this group are in
classes of advanced education (1.9., 130), overt evidence of mental retardation in
other children is disturbing. Twenty-one patients (24%) were retarded-an incidence
equal to that found in our children with
jacksonian and psychomotor epilepsy. Psy-chologic evaluations were available in 14 of these 21 patients. Their intellectual
func-tion varied from marked mental deficiency
to the dull normal intellectual range. Ten were tested with the Wechsler Intelligence Scale for Children and scored I.Q.’s ranging
from 45 to 87. Six scored within the “de-fective” range, one in the “borderline” range, and three in the “dull” normal
in-tellectual range. The remaining four were
tested with the Stanford Binet Intelligence
Scale, Form L. Their I.Q.’s ranged from 65 to 80. This distribution is similar to that
seen on the W.I.S.C. The age range in both
groups was 7 to 14 years. Six children were tested at least twice by different examiners, without showing significant changes in
in-tellectual functioning. Almost all of the re-tarded children, 19 of the 21, experienced other non-petit-mal seizures. In 12 of the
19, these first appeared at the same time as
or after the onset of petit mal. It is
there-fore apparent tilat associated convulsive
sei-zures are responsible for neither the exist-ing brain damage nor the petit mal. In
con-trast to grand mal, reputedly, frequently recurring petit mal seizures do not produce brain injury. Granting this premise one can only conclude that petit mal can occur as a result of or without relation to brain
dam-age.
Only 5 of the 21 retarded children had
a recognized background (1 congenital mi-crocephalic, 1 subarachnoid hemorrhage, 1 tuberous sclerosis, 1 Sturge-Weber
ARTICLES 897
With regard to intelligence it is
interest-ing that several children were reported to be “brighter” during periods of increased
seizure frequency.
HYPERVENTILATION AND OTHER INFLUENCES
Witil few exceptions, a diagnosis of petit mal should be seriously questioned in the
untreated patient who does not have an at-tack on hyperventilation. Seizures were pre-cipated by this means in 70 of 75 patients
(
93%). In the remaining 13 cases, there was no mention of hyperventilation, or it wasconsidered impractical. An inverse relation-ship between ease of activation by this means and seizure control is suggested.
We prefer to test the patient standing.
Truncal sway is then more apparent. The children are asked to hold a piece of paper and to blow it away from them as far as possible. (Rapid or shallow respirations are
ineffective.) Few patients are too young to understand what is expected of them. Hy-perventilation is continued for 3 minutes, if no response is elicited before this. Since
seizures sometimes first appear shortly after hyperventilation, observation during this period too is recommended. Occasionally spontaneous seizures are observed in a pa-tient who has a negative HV response. In
these instances, the provocative effect of hyperventilation may be counteracted by
the inhibiting influence of attention and concentration.8’#{176}
During hyperventilation patients often
blink, close their eyes, lick their lips, or swallow. It is essential to distinguish these physiological manifestations from paroxys-mal phenomena.
We recall but one instance in which
non-petit ma! epilepsy (jacksonian) was preci-pitated by hyperventilation.
Of the various types of epilepsy, petit mal is particularly influenced by environ-mental factors. It is aggravated by
emo-tional states-pleasant or otherwise, by dis-cipline, by fatigue, by fever, and by
infec-tion.
The relation of petit mal to puberty is discussed under tile section on “Prognosis.”
One of the most fascinating features of petit ma! is the exquisite light sensitivity
exhibited by some patients. In three cases from the series and in one other since,
sei-zures were greatly exaggerated by bright sunshine, particularly with alternate light
and shade. All four children appeared to seek such flicker excitation. While staring at the sun, they were often observed to be rapidly fluttering a hand in front of their
eyes. Though dark glasses are prescribed, the children may remove them and stare at
the sun as if hypnotized while having re-peated paroxysms.
THE ELECTROENCEPHALOGRAM
Of 80 electroencephalograms available
for study from this series of 88 cases, 33
(41%) showed classic 3 per second petit
ma! spike and dome formations in 1 or more tracings. Of these, two also showed
focal spikes, one of occipital and another
of mid-temporal origin. Beside the 33
classic petit ma! records, 18 showed spike
waves occurring singly, in short runs, or as poly spike formations. This made a total of 51 (64%) of petit mal cases in which spike and wave patterns appeared. Other convul-sive patterns were noted in an additional 22
cases distributed as follows: paroxysmal slow, 12; spikes, 3; focal patterns, 2;
paroxys-mal trends (pace irregularity and
hypersyn-chrony), 5. Seven tracings were indetermi-nate, no form of convulsive activity being noted. Thus in 1 or more tracings, 90% of cases showed seizure formations in at least one tracing. Considering those children having petit ma! seizures only, there was no
significant difference between the incidence of the classic versus non-classic seizure pat-terns. Nor was it significant whether or not the basic pattern of tile tracing showed
evidence of disorder since the incidences of such disorder were approximately equal
es-tablish the incidence in which spike waves
appeared in 1 tracing but not in another.
THERAPY
Phenobarbital can be effective in petit
mal. For this and reasons discussed below,
all patients are started on phenobarbital alone. Eight children, roughly 10%, were controlled completely-four on phenobarbi-tal alone, and four by the addition of the drug to previous ineffective therapy. Four
others had sufficient improvement with phenobarbital alone to not warrant addi-tional drugs for significant periods of time. Mustard and Livingston10 found that with
trimethadione alone, grand mal appeared for the first time in 11 of 66 patients within 2 months of onset of therapy. Since 9 of our children had a simultaneous onset of other
seizures prior to any therapy, another 20 had convulsions before the onset of petit mal, and 1 developed his first grand mal attack within 10 days of stopping trimetha-dione therapy, the relationship suggested
by Mustard and Livingston is not certain.
The
high
incidence of other seizures (61%) illustrates the strong tendency for thesechildren to develop non-petit-ma! seizures sooner or later. One might then ask, “Could phenobarbital serve as prophylaxis against this eventuality?” Of the 25 patients who de-veloped other seizures after the onset of petit mal, 18 did so prior to their first clinic visit. Only seven developed grand mal on our service.
A review of these cases is of interest.
Patient S.R. had an onset of grand mal on
an effective dose of phenobarbital at the time of menarche 5 years after the begin-nings of her intractable petit mal. Patient E.R. confessed omitting his phenobarbital at the time of his first generalized convul-sion. Patient B.M., a 14-year-old girl, was
receiving only 3 grain of phenobarbital daily at the time of her first seizure. Two children, W.W. and W.H., had their first major seizure on specific petit mal therapy
only. Patient E.Y. had his first convulsive seizure 10 days after stopping
trimetha-dione while still on adequate phenobarbital.
The last child, T.B., had her first major
seizure 2 months after discontinuation of phenobarbital, which she had been taking
for 4 years without previous seizures. It is not unreasonable that prophylactic pheno-barbital in adequate dosage might prevent
non-petit ma! seizures in these patients. The important question is, “Does this treatment for a limited period, such as 4 years as is
our practice, alter the ultimate prognosis?” Only a long-term follow-up can answer this important question. Phenobarbital dosage
ranges from 3 to 1% grains at bedtime. This dose is seldom exceeded, despite the
ab-sence of side-effects. In none of our patients
was the control of grand ma! seizures a
problem.
In at least two patients, a deleterious effect of dilantin on petit ma! frequency was
man-ifest. Dilantin is used only when pheno-barbital fails to control grand mal or other non-petit ma! seizures.
The effectiveness of benzedrine or
dexe-drineh12 in petit ma! epilepsy has been
reported by several authors. Because this effect is inconstant and often short-lived,
we prefer to use these drugs as adjuvants when more specific medication fails.
Long-acting preparations dispensed in granule form are prescribed in doses from 2% mg
to tolerance. One referred patient was
re-ceiving 60 mg daily without any effect on her or her seizures. Paradoxically the side-reactions of dexedrine range from mania to
deep sleep. Most undesirable to parents is the anorexia and weight loss.
If in a period of 3 to 6 weeks, no re-sponse to phenobarbital is evident the next
choice is trimethadione or acetazolamide (Diamox). Acetazolamide precludes the serious risks of oxazoladine therapy and
the necessity for routine blood and urine
examinations. Nevertheless, the established effectiveness of trimethadione in a high percentage of patients with petit ma! seem-ingly justifies this liability. Over the past
ARTICLES 899
manifested a fulminating exudative and
ex-foliative erythema multiforme with intense mucous membrane involvement 10 days
after onset of therapy. Survival in this case was apparently related to massive steroid
therapy.
The anti-epileptic effect of trimethadione
is very prompt, usually immediate or with-in a few weeks or months on increasing dosage. All except the youngest children are started on 0.6 or 0.9 gm/day in
three divided doses. If there is less than
complete control, increments of 0.3 gm/ day are made at weekly intervals up to a
maximum of 1.8 gm daily. On numerous occasions a daily dose of 1.2 or 1.5 gm is of limited or no value and increasing the
dose by only 1 (0.3 gm) capsule achieves complete control. We have found para-methadione (Paradione) to be effective when trimethadione has failed and each to
be useful at one time and not at another in
the same patient. Lennox15 has suggested that trimethadione be discontinued after freedom from petit mal for 6 months.
Be-cause of relapses following even longer in-tervals of control we have adopted the routine of continuing anti-petit mal
medi-cations for at least 1 year after the last at-tack.
In a previous publicationl6 the value of acetazolamide in the treatment of
intract-able petit ma! was reported. Eleven of 16 such patients were completely controlled, and the remainder improved (a reduction of 50% or more in seizure frequency). The
action of acetazolamide is also prompt. In three cases where petit ma! occurred daily
for periods of 3, 5, and 7 years respectively, the addition of acetazolamide stopped tile seizures immediately. Rarely is it necessary to wait a week or two to see results. We prefer to start with a large daily dose, 500
to 1,000 mg, irrespective of the age or weight of the patient, given in fractional amounts, and then make reductions as
in-dicated. This regimen is unquestionably more effective than starting with small
doses and making increases later. The agent is always added to previous therapy. It is
possible that in some cases its action is synergistic. If the addition of acetazolamide
to phenobarbital is ineffectual, the drug should be reconsidered in combination with trimethadione or other specific medications
which had failed previously to give
corn-plete control. Toxic side effects of aceta-zolamide are few. Rarely are they serious.
Succinimide derivations have been shown to have anti-petit ma! action. We have used methsuxamide (Celontin) with
success. An initial dose of 0.3 gm daily is increased at weekly intervals to 3 or 4 capsules as tolerated. Phensuxamide
(Mi-lontin) has seldom been of value. Our ex-perience with the new analogue,
ethosuxa-mide (Zarontin), is limited.
ChloraquinelS has been used in seven difficult cases, with complete control in
two, significant improvement in two and no effect in three. The recommended dos-age is 125 mg twice daily. Duration of
therapy is undecided.
The ketogenic diet has been tried in a
few intractable cases with satisfactory
re-sults. Considering the physiologic basis for
the diet, a carbonic anhydrase inhibitor such as acetazolamide should achieve the same or a more satisfactory result, an
anti-convulsant action independent of metabolic acidosis.
In summary, the objective of therapy is complete control of petit ma! for 1 year on phenobarbita! and specific drugs if re-quired. At the end of this time, petit ma! medications, oxazolidone, acetazolamide, or
succinimides as the case may be are dis-continued over a 4-week period and the
patient is maintained on phenobarbital alone for another 3 years. At the end of this time phenobarbital is discontinued cautiously over a 9-to-18-month interval.
RESULTS OF THERAPY
In 16 cases therapy could not be
evalu-ated because of failure to keep return
ap-pointments or an inadequate trial of medi-cation. All of these had been followed less
than 6 months.
(62.5%) were completely controlled for at
least 1 year. Only 8 children (17.7%) had re-lapses (all within 5 months) after discon-tinuation of petit ma! drugs at the end of 1
year of freedom from seizures. Five promptly regained control on re-adding medication. All antiepileptic drug therapy has now been discontinued in 16 children following 4 years of therapy after tile last
petit ma! or other seizures. Seizure control was credited primarily to trimethadione in 24 of these 45 cases, to acetazolamide and
trimethadione in 10, to acetazolamide alone in 2, to phenobarbital in 8, and to
meth-suxamide in 1. Over one-half of tile group
(27 patients) obtained this control more or less immediately or within 3 months after adding effective medication. However, in
many children control required 1, 2, or more years of assiduous trial and error.
Twelve children (16.6%) were considered improved. Each had seizure-free periods lasting from a few weeks to as long as 8 months, then reverting to daily episodes.
Tile period of medical observation in this group was from 1 year to 12 years (over 3
years in six cases).
Eleven children (15.2%) were classified as unimproved, tile longest free seizure in-terval being less than 2 weeks. Each child
had been treated for over 1% years, nine
for more than 3 years.
When every available resource is ex-hausted without improvement, all drugs
save phenobarbital or other non-petit-mal
drugs as required are discontinued. In four sucii instances a subsequent spontaneous “cure” occurred. The duration of previous trials of therapy, which were considered
adequate, in these four cases ranged from 1 to 4 years.
PROGNOSIS
From a study of the data presented and from personal contact with parents and
educators, we have developed rather strong feelings concerning the prognosis in petit ma!, indicating that the condition is not
necessarily benign and unaccompanied by cerebral pathologic changes with effect on
mentality or personality. Nor does puberty terminate the attacks.
The minimal physical manifestations of petit ma!, as compared to those of a
gen-eralized or local motor seizure, are can-celled to a degree by the extreme
fre-quency of daily attacks often with variable periods of status. Patients do not fall dur-ing an attack in spite of the common and often extreme truncal sway. However, is is hard to convince parents, teachers, or even
medical students, that they will not. Tile incidence of cerebral pathology rec-ognized or suspected from mental
retarda-tion is as high among these children as with other types of epilepsy. Twenty-one (24%) of the group had apparent intellectual
deficits. Were psychologic and psychomet-nc testing a routine, this value would un-doubtedly be higher. It is commonly re-ported that petit ma! attacks do not cause
residual and progressive brain damage.
Several parents (including one physician) emphatically deny this assumption. Since
psychologic testing was not done prior to the onset of petit ma! in these retarded children, the question remains unanswered.
Petit ma! is not simple to treat. The pro-longed duration of symptoms prior to the first visit attests to the limitations of ther-apy. One-quarter to One-tilird of these chil-dren do not respond to standard therapy, or do so for only limited periods of time.
The prevailing concept that petit ma! will improve or cease during adolescence would, even if true, be small solace to
anx-ious parents of a 6 or 7-year-old youngster who is having a hundred or more spells a day. Exceptions are common enough. Four
girls of this series first manifested petit ma!
at menarche. Only one had a “spontaneous cure” at this time. At least four patients are still having daily petit ma! at ages 16, 16,
17, and 19 years respectively.
Though few patients with convulsive
sei-zures have petit ma!, the reverse is not true. Actually the majority of our children, 61%,
exhibited
other
seizures, predominately grandmal.
Anticipating
this
likelihood it901
with uncomplicated petit ma! with
anti-convulsive drugs for a minimal period of 4
years.
At any rate we consider the prognosis
for petit ma! no different than that for any
other type of epileptic manifestation, grand ma! or otherwise.
SUMMARY AND CONCLUSIONS
Eighty-eight cases of petit ma! epilepsy in children are reviewed. The clinical
char-acteristics and differential diagnosis of petit ma! are presented in detail. Aspects
dis-cussed and significant findings are as fol-lows: Petit mal is a relatively infrequent type of epileptic manifestation (4.4%). Petit ma! is rare before 2% years of age.
Twenty-three per cent of the children had attacks for over 3 years prior to their first visit sug-gesting the limitations of therapy. A history
of seizures in parents or siblings was pres-ent in 21% of the cases. Whereas, only 10% had apparent lesions of the central nervous
system, the incidence of intellectual retar-dation, 24%, is similar to that found in our
reviews of jacksonian and psychomotor epilepsy. Sixty-one per cent had non-petit mal seizures (chiefly grand mal). In 63%,
these first appeared with or after the onset of petit ma!. Petit ma! attacks were preci-pitated by hyperventilation in 93% of the
cases. Ninety per cent of the children had seizure formations in at least one electro-encephalographic tracing. Sixty-four per
cent showed spike and wave patterns; in 26%, other convulsive patterns were noted.
Recommendations in therapy and results are reported. The review suggests that petit ma! is not entirely benign and
unaccom-panied by cerebral pathologic changes with effect on mentality or personality, nor spontaneously cured at puberty. The high incidence of grand ma! and other seizures
in these children is emphasized and pro-phylactic therapy suggested.
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