MINOR
MOTOR
EPILEPSY
Diagnosis,
Treatment
and
Prognosis
By Samuel Livingston, M.D., Victor Eisner, M.D., and Lydia Pauli, M.D.
Department of Pediatrics, Johns Hopkins University School of Medicine and the
Epilepsy Clinic of Johns Hopkins Hospital
PEDIATRICS, June 1958
916
E
PILEPTIC seizures in children may bemanifest in a variety of forms. While
it may be difficult to classify some attacks,
most seizures may be roughly divided into four groups : Major motor (grand mal); petit mal; minor motor; and psychomotor. This paper presents the authors’ experience with minor motor seizures.
PATIENT
MATERIAL
The study concerns 698 children with minor motor epilepsy who were seen at the Children’s
Epilepsy Clinic of The Johns Hopkins
Hos-pital. The ages of these patients at the time
of their initial visit ranged from 3 weeks to
53 years; 369 were males and 329 were fe-males; 418 were white and 280 were Negro. All socio-economic groups were included in
this series.
The subsequent courses of 622 of these patients were studied either by regular visits to the clinic or through information obtained by a follow-up questionnaire. Data, other than that obtained at the initial visit, are not availa-ble on the remaining 76 patients. Of the 622 patients, 220 have been followed for 3 to 10 years, and the remaining 402 for intervals ranging from 10 to 22 years.
CLINICAL
MANIFESTATIONS
Types of Seizures
MASSIVE MyocLoNIc: This type of
sei-zure varies somewhat in clinical manifesta-tions, but most commonly consists of a sudden flexion of the head with simultane-ous extension of the upper extremities and flexion of the thighs on the abdomen. It is frequently preceded or followed by a short
cry, laugh or giggle. A common variation
(Submitted October 21, accepted December 26, 1957.)
ADDRESS: (S.L.) Baltimore 5, Maryland.
consists of a sudden generalized tonic spasm with slight trembling movements of out-stretched arms, which persists for several
seconds.
The duration of an individual massive myoclonic attack is very brief, lasting a few seconds. However, they frequently re-cur in rapid succession (series) lasting for
5 to 10 minutes. Some patients have as high
as 50 to 100 individual attacks or many clusters of spells daily.
Massive myoclonic seizures often occur just prior to the onset of natural sleep and immediately after awakening. Usually the individual attacks are so short that it is difficult to determine whether or not
con-sciousness is impaired.
HEAD NODDING: This type of spell
con-sists of a sudden nod or dropping of the head, usually forward. It frequently occurs many times daily and also may recur in rapid succession (series).
AKINETIC I This spell consists of an abrupt loss of all muscle control with consequent fall, usually forward, and often with injury to the knees, forehead or chin. The patient is usually able to get up almost
immedi-ately, and generally the return of conscious-ness is immediate. In some instances, how-ever, he may appear disoriented for 1 to 2
minutes.
Although these three types of spells differ in their clinical manifestations, the authors classify them all as minor motor seizures for
the following reasons: (a) each of them is
of short duration and consists of brief motor manifestations; (b) the different types may
electroencephalo-Intellectual Derelopment No. of Patients
AGE OF ONSET OF MINOR MOTOR SEIZURES
Age of Onset No. of Patients
Total 698
Age of Onset and Incidence
Minor motor seizures most often make an initial appearance early in life, usually
be-tween 3 and 12 months of age, with the
peak incidence between 3 and 6 months. Occasionally they begin at a later age, but
rarely after 4 or 5 years of age. The earliest age at onset in this series was 8 days and
the latest was 5% years. Table I shows the
age of onset of minor motor seizures in the
698 patients.
Major motor seizures frequently precede
or accompany minor motor seizures. One
hundred two of the six hundred ninety-eight
patients had one or two major motor sei-zures prior to the initial appearance of the minor motor seizures. In most instances the minor motor seizures appeared within several days after the onset of the major motor seizures. Eighty-six of these one hundred two patients continued to have
both major and minor motor seizures for a
period of time thereafter; the other 16 had no recurrence of major seizures.
Up until 3 years prior to this writing,
7,832 children with epilepsy were studied in this clinic; approximately 9% suffered with
minor motor seizures. As this figure of 7,832
includes children up to 15 years of age, and since minor motor seizures are far more common under 1 year of age, they consti-tute a relatively common type of seizure pattern in the first year of life.
The three types of minor motor seizures
TABLE I
0-3mo 86
3-6mo 441
6-12 mo 106
12-24 mo 32
24 mo-5 yr 21
Over 5 yr 12
Normal 14
Slightly retarded 21
Moderately retarded 248
Severely retarde(l 388
Total 671
are encountered in the following order of
frequency: Massive myoclonic, head nod-ding and akinetic. The massive-myoclonic and head-nodding types generally occur in the very young infant and the akinetic type
obviously does not appear until the child is
able to stand.
Physical and Neurologic Findings
Brain damage is a very prominent part of the general clinical picture of patients with minor motor epilepsy. Clinical evi-dence of brain damage is usually present
prior to the onset of minor motor seizures,
especially in those patients in whom the seizures begin after 3 or 4 months of age.
Six hundred one (86%) of the six hundred
ninety-eight patients exhibited clinical
evi-dence of varying degrees of brain damage
before the onset of the seizures, such as de-velopmental retardation, cerebral palsies, impairment of vision, etc.
There were sufficient data to estimate the intelligence of 671 of the 698 patients when they were approximately 1 year of age.
Mental retardation of some degree was
ap-parent in 657 (96%) of the 671 patients. (Table II).
A demonstrable motor defect of central
origin, the most common of which was of
the spastic type, was apparent in approxi-mately 50% of the patients by the time they
reached 2 years of age. Impairment of vision or hearing was present in approxi-mately 20%.
Laboratory Findings
Blood counts and chemistry of the blood
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Fic. 1. Age, 23 years; light sleep (Seconal#{174}); clinical diagnosis, minor motor epilepsy. Note high-voltage, random, slow waves and spikes (interseizure record).
ferric-chioride test for phenylpyruvic acid was positive in three instances. Serologic
tests for syphilis were positive in two cases. Neutralization tests for toxoplasmosis were positive in two cases. Routine roentgeno-graphic examinations of the skull revealed abnormal calcifications within the brain in several instances, which gave additional evidence for diagnosing disorders such as tuberous sclerosis and toxoplasmosis. Other-wise, these examinations contributed little
or no information regarding the diagnosis.
Electroencephalographic examinations were performed in 594 of the 698 patients at the time of the initial study.
Abnormali-ties referred to by Gibbs et al.I as
“hypsa-rhythmia” (Figs. 1 and 2) were present in 511 (86%) of the tracings. The brain-wave pattern was normal in the remaining 83 patients.
Pneumoencephalographic studies were performed in 128 of the 698 patients. Of these, 112 revealed generalized cortical
atrophy or bilateral dilatation of the
yen-tricular system, or both, similar to that
shown in Figures 3A, 3B. 4A and 4B; 4 showed porencephalic cavities; 2 revealed
unilateral cortical atrophy; and in the re-maining 10 the air studies were non-revealing.
ETIOLOGY
While the etiology of minor motor sei-zures cannot be definitely stated, the records disclose an association with cerebral injury or damage in 445 (64%) of the 698
patients. The presumptive causes of damage in these Patients are shown in Table III. The most common was a cerebral birth
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‘Fota I 698
in 225 (32%) of the 698 patients; 14% of the
225 patients were born prematurely. The second most common presumptive cause
was a congenital defect in cerebral de-velopment (Table IV), and the next was
some variety of meningitis or
encephalo-pathy (Table V).
A presumptive etiologic factor could not
be determined in the other 23 patients. Pneumoencephalographic studies were
per-formed on only five of these patients. These tests were nonrevealing.
PROGNOSIS
The data obtained from the fo1lov-up
study made on 622 of the 698 patients
mdi-cate that the outlook for patients with minor motor epilepsy is exceedingly poor. All of the 622 patients have been followed for at least 3 years, many for as long as 15 to 22 years.
Twenty-three of the six hundred twent-two patients are known to be dead. Death
occurred l)efOre the sixth year of life in each instance. Four of these deaths were due to
traumatic injury associated with seizure.
Three patients died in status epilepticus. Ten patients were found dead in bed by the parents. It was thought that death was
due to suffocation (aspiration of vomitus).
Six died of acute infections.
One hundred forty-two of tile six hun-(fred twenty-two patients were in
institu-tions. These children were institutionalized between tile ages of 3 and 12 years.
The ages of the remaining 457 patients varied from 3% to 24 years. Only 11 of these
457 patients had normal intelligence. The
remainder were retarded mentally, tile vast majority being severely retarded. Three
patients who appeared to have normal
in-‘FABLE III
PRESUMPTIVE CAUSES FOR BRAIN I)AMAGE IN
I’ATIENTS WITII MINOR MOTOR SEIZURES
Cerebral birth injury (trauma and/or anoxia) 225 Congenital defects of cerebral development 142
Meningitis or encephalopathy 44
Subdural hematoma . 6
Congenital toxoplasmosis 2
Phenylpyruvic oligophrenia 3
Syphilis of CN 2
Kernicterus 3
Cerebral vascular lesions 6
Trauma not related to birth 12
TABLE V
MENINGITIS OR ENCEPHALOPATIIY IN PATIENTS
WITH MINOR MOTOR SEIZURES
CONGENITAL DEFECTS OF CEREBRAL DEVELOPMENT
IN PATIENTS WITH MINOR MOTOR SEIZURES
920 EPILEPSY
Fic. 3. A and B. Pneumoencephalogram of a 9-month-old girl showing marked cortical atrophy. The lateral
ventricles are slightly dilated. There is atrophy of cerebral convolutions resulting in wide sulci. Patient’s
birth was normal. Development was delayed. She held up her head at 4 months of age. She was unable
to sit up at time of the examination (9 months of age). At 83 months of age, she began having general-ized, major and minor motor seizures of the massive myoclonic type. (Interpreted by Dr. Webster Brown.)
telligence at 1 year of age (Table II) now exhibited evidence of retardation.
The follow-up studies made on these 457 patients indicate that the frequency of
minor motor seizures tends to decrease spontaneously with increasing age; and that these spells disappear completely, in most
cases, by the time the patient reaches 5
TABLE IV
Anomalies of gyri and cerebral cortex* 112
Hydrocephalus 8
Porencephaly 4
Microencephaly 16
Tuberous sclerosis 2
Total 142
* This group embraces a great number of defects of the gyri and cerebral cortex which are designated in the
literature by a variety of terms: Cerebral agenesis,
cerebral aplasia, cerebral hypoplasia and cerebral atro-phy. This diagnosis was made on the basis of the
pneu-moencephalographic finding which revealed generalized cortical atrophy and/or bilateral dilatation of the ven-tricular system. Of course, it is realized that the cerebral changes in these cases could also have been caused by
cerebral birth injury (trauma and/or anoxia).
Pertussis 18
Measles 4
Meningococcus 3
Influenza 2
Pneumococcus 2
Mumps 3
Chicken pox 4
Tuberculosis 2
Lead 3
Postvaccinia 3
‘Fotal 44
years of age. These studies also showed that patients with minor motor epilepsy fre-quently develop other types of seizures, particularly major motor seizures, as the child grows older.
Of these 457 patients there were 285
who were 5 years or older at the time of
Fic. 4. A and B. Pneumoencephalogram of a 2-year-old boy showing bilateral marked cerebral atrophy. There is symmetrical marked dilatation of the lateral ventricles and slight dilatation of the third ventricle. In addition there is a considerable subarachnoid accumulation of air, most marked over the left parietal hemisphere, suggesting especially marked atrophy on this side. Pregnancy was coniplicated by
hemor-rhage in the sixth month and also the day before delivery. Birth was by cesarian section at term after
the fetal heart had not been heard for 1 hour. Convulsions occurred on first day, and minor motor spells of the massive myoclonic type probably started during the first month. Development was retarded: At 2 years of age he could lift his head but could not hold it up. (Interpreted by Dr. Gunter Schultze.)
psychomotor attacks. One hundred forty-three had been free of all seizures for pen-ods ranging from 2 to 18 years. It is of inter-est to note that none of these patients de-veloped petit mal spells.
The results of the electroencephalo-graphic follow-up studies are in close
ac-cord with those previously reported by Gibbs et al.1 They obtained serial
electro-encephalograms on a group of patients with minor motor seizures and observed that the hypsarhythmic pattern “tends to disappear with increasing age and is usually
replaced by either a normal EEG or one
with focal electroencephalographic
abnor-mality.”
Serial electroencephalograms were ob-tained on 389 of the patients in the present series at approximately 6-month intervals
until they were 7 years old. The initial tracings showed hypsarhythmia in 351 pa-tients and normal readings in the remaining 38; all of these latter were under 3 months
of age when the initial electroencephalo-gram was obtained. Subsequent
electro-encephalograms revealed the hypsarhythmic pattern in 30 of the 38 patients by the time
they reached 1 year of age. This makes a total of 381 of these 389 patients who had hypsarhythmia.
The hypsarhythmic pattern disappeared
from the electroencephalograms of 330 of
the 381 patients by the time they reached 5 years of age, and from the tracings of the
remaining 51 patients before 7 years of age. At 7 years of age 227 (58%) of the 381
patients had normal electroencephalograms
and the tracings of the remaining 154 pa-tients revealed various types of abnormali-ties, other than hypsanhythmia.
Two of the eight patients who did not
have hypsarhythmic patterns at any time
had normal tracings when they were 7
years old and the other 6 had abnormal brain waves.
922 EPILEPSY
the typical 3-per-second spike and wave discharge of petit mal epilepsy. Gibbs et al.1
also reported that none of the patients they studied “had developed seizure (lischarges of the classical petit mal tYpe.”
TREATMENT
Minor motor seizures are most difficult to control with anticonvuisant drugs. Each of the anticonvulsant drugs currently availa-ble has been prescribed, alone or in
com-bination, and the results have been essen-tially poor with all of them. There were
occasional good results with the drugs shown in Table VI; treatment should begin with one of these. Other drugs should be prescribed, if necessary, only after the maximal tolerated dosage of the starting drug has failed to produce a satisfactory clinical response.
In the authors’ experience, a ketogenic diet is the best therapy for minor motor epilepsy; complete control of spells was
TABLE ‘I
l)Iu;s TISEI) FOil TIlE ‘FREATMENT 01’ \IINOII MOTOR 5:IzIuEs
(‘l’/,e.vt’ (lrlJgs are Iiste(I in order of relatire effectiveness in I he aut/wrx experience. .\one of them provided control in more than a small piumber of patients.)
Pheunbarbital or mephiobarbital (Mebaral#{174})*
Meprobamate (Equanil#{174}, Miltown#{174})
Amphetamine sulfate or dextro-aniphetamine sulfate
Bromides
Methsuximide (Celontill5)
Pheusuxilnide (Milontin#{174})
.5,5-diethyl-1 -Inethylba rbiturie acid (Genionil#{174})
* Mebaral#{174} was given to those PittielIts who
Iiiaui-fest untoward react ions to phenobarbital.
obtained in 91(49%) of 186 of the patients
with this form of therapy.
DIFFERENTIAL DIAGNOSIS
In the past many physicians have not
made a clear distinction between minor motor seizures and petit mal spells. Lennox2
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Fic. 5. Age 7 years; awake; clinical diagnosis, petit mal epilepsy. Note bursts of
transient lapses of consciousness, lasting
usually 5 to 30 5CCOI1(l5, associated with
bilateral synchronous 3-per-second spike
and wave forms in the electroencephalo-gram (Fig. 5).
Brief seizures that occur many times a
day are an outstanding characteristic of
both minor motor and petit mal epilepsy. However, in addition to the difference in the clinical manifestations of these two
types of attacks, there are many other
differ-ences that make possible a correct
diag-nosis. Petit mal spells usually make their
initial appearance between 4 and 8 years
of age. They rarely, if ever, occur during the first few years of life. Petit mal spells are seen almost entirely in children who do not manifest (lemonstrable evidence of brain damage. Classic 1)tlrsts of 3-per-second spike and wave formations (Fig. 5) are present in tile electroencephalograms
of all patients who suffer with this form of
epilepsy.
The minor motor seizure with head nod-ding should present little difficulty in diag-nosis. To the authors’ knowledge, this type of spell is not encountered in any other disorder. A patient with amyotonia con-genita was referred to the clinic with a questionable diagnosis of niinor motor head-nodding spells; when 7 months of age, in a sitting position tile head would wobble and frequently drop forward. The clinical
differentiation between these two disorders
was not difficult.
An akinetic minor motor seizure
some-times simulates another type of epileptic
seizure which the authors classify as an atonic major motor seizure. This latter type of spell consists of a sudden loss of
con-sciousness. The nlusculature remains flaccid throughout tile entire spell; there is no apparent tonic or clonic phase. The main clinical difference between these two types
of seizures is as follows: An akinetic minor motor seizure is usually of brief duration,
seldom lasting longer than 2 or 3 seconds,
time, but seldom less than 2 or 3 minutes,
all(I the return of consciousness is gradual. Furthermore, patients with akinetic minor
motor seizures also almost always have or
have had other types of minor motor seizures.
The physician may mistakenly interpret an akinetic minor motor seizure as a simple attack of syncope or a cataleptic spell. These latter attacks, particularly the
cata-ieptic spells, rarely occur in the very young child. In some instances it may be necessary to utilize the electroencephalographic find-ings to make the correct diagnosis. This is particularly true in those cases where there
are no other clinical manifestations of an epileptic disorder. The electroencephalo-gram is normal ill patients with svncopal
attacks and also in catalepsy.
Myocionic jerks such as are seen in pa-tients with minor motor epilepsy of the
massive myociollic type are to be
differenti-ated from ordinary myoclonic epilepsy. This latter term is used here for the type of
epilepsy which consists of contractions of
isolated flexor muscles or muscle groups. Clinically it frequently simulates tics or habit spasms. It occurs most frequently in older children who usually do not manifest evidence of brain damage. In the authors’ experience, the electroencephalogram is not specific in simple myoclonic epilepsy.
Likewise, it is necessary to differentiate
minor motor epilepsy from the myoclonic states seen in cerebral degenerative diseases
in infants, such as described by Ford et al.,3
Alpers,4 Somoza,5 and Christensen and
Krabbe.6 These workers reported cases of degeneration of the cerebral gray matter in which convulsions and myoclonic jerks appeared before the first year of life. Only one of tile four infants with myoclonic jerks who were reported in these series
lived to the age of 2% years. The clinical
features of this condition include major
924
in status epilepticus. In this type of case
the myoclonic jerks persist continuously for
days and weeks, sometimes with short re-misssions, to death. In contrast, in minor motor epilepsy, myoclonic jerks may recur
in series which seldom last longer than 5
to 10 minutes. In the degenerative diseases progressive mental deterioration is a promi-nent feature, but minor motor epilepsy is
usually nonprogressive.
The massive myoclonic types of minor
motor seizure may simulate the Moro reflex in being precipitated by external stimula-tion, but this type also occurs
spontane-ously and often in series, thus differing clinically from the
‘
typical Moro reflex. The occurrence of a loud cry in association witha massive myoclonic type of minor motor seizure may be mistakenly interpreted as
intestinal colic.
Clinical syndromes characterized by
myoclonic movements, and sometimes
designated as myoclonic epilepsy, are: The
paramyoclonus multiplex of Fniedreich, the
intermittent myoclonus of Lundberg, and the progressive familial myoclonus of
Unvernicht. These are probably variants of
a single disorder and occur in older
chil-dren and adults.
DISCUSSION
Although the minor motor seizure is a relatively common type of seizure pattern encountered in infancy and early childhood,
the medical literature contains little about this type of attack. The paucity of reports dealing with minor motor epilepsy as a specific entity is probably due to the fact that most physicians in the past, and even many today, designate minor motor seizures as petit mal spells, probably because they are of short duration.
The salient clinical manifestations of minor motor seizures were reported as far back as 1841 by West7 and 1849 by
Newn-ham.8 In 1924, Asal and Moro9 referred to these spells as “Blitzkrampfe” (lightning
seizures). Subsequently a few additional re-ports dealing specifically with minor motor
seizures have appeared in the literature.b0h5
Minor motor seizures are also referred to by a variety of other terms: Petit-mal variant seizures, abortive grand mal,
inhibi-tory attacks, astatic attacks, salaam spells,
organic petit mal, jackknife convulsions,
massive seizures, infantile spasms and
in-fantile myocionic seizures.
All those who have written on the
sub-ject agree that minor motor seizures occur almost entirely in children with organic brain damage and that the vast majority of patients with these seizures are mentally retarded. It has been the authors’
experi-ence that, in many instances, demonstrable
evidence of brain damage is present prior to the onset of the seizures. Recently Illingworth’#{176} and Baird and Borofsky’4 re-ported a small group of children who ap-peared to be developing normally during early infancy until the onset of minor motor seizures, following which mental deteriora-tion soon was observed. The authors also
have seen some children who appeared to
be developing normally up until the onset of this type of seizure. Cerebral pathology could have been present in the very young children in spite of apparent normal de-velopment (Figs. 6A and 6B). It is also possi-ble that an unrecognized insult to the brain, such as encephalitis or hemorrhage, could
have been the basis for the mental
retarda-tion and the seizures.
The extent of the mental defect in pa-tients with minor motor seizures certainly suggests that this type of seizure is associ-ated with severe organic brain damage in nearly every case. Studies of the causes of such damage have not been too fruitful. In many patients, cerebral birth injuries or encephalitis appear as probable causes; Gibbs et al.1 attributed 32% of their 237 cases to such disturbances, and in the pres-ent series these are considered as probable causes in 53%. Other presumptive causes
of the lateral and third ventricles; and air in the cisterna magna. Impression: Generalized cerebral
atrophy, most marked in right fronto-temporal area. (Interpreted by Dr. David Gould.)
anoxia) was tile most likely cause. Pneu-moencephalographic examinations were
performed on only 5 of these 253 patients; more of such studies might have been
re-veaiing. Gibbs et al.’ did not state whether
pneumoencephalographic studies were per-formed on their patients.
Baird and Borofsky14 recently suggested that, in some patients, pertussis immuniza-tions may be a factor in the production of
infantile myoclonic seizures. In 8 of 24
patients with a previously normal
develop-mental history, the seizures developed within ito 5 days after
diphtheria-pertussis-tetanus immunizations; and in another in-stance within 15 days. Twenty-seven other
patients in their report had abnormal de-velopmental histories prior to the onset of
tile seizures.
This supposition is of interest in view of the previous report of Byers and MoTh7 of a group of 15 children who developed severe cerebral symptoms following the
adminis-tration of prophylactic pertussis vaccine. In 14 of these patients the cerebral
symp-toms appeared within 24 hours; 13
subse-quently developed epilepsy.
In 1955 Low18 published electroen-cephalographic studies of 83 children be-fore and after their first pertussis immuniza-tion. The electroencephalograms of one
child showed development of marked,
diffuse slowing, which disappeared 1 week
following the injection. The tracing of an-other showed less marked slowing, with
fever as the only symptom. There were no
changes in the electroencephalograms of the remaining 81 patients. It was concluded
that “mild, but possibly significant”
cere-bral reactions may occur following
prophy-lactic pertussis inoculations.
Tile present authors also have seen
pa-tients in whom minor motor seizures
de-veloped after DPT immunizations, but had
in all probability one of coincidence. Minor motor seizures (classified by Baird and
Borofsky as infantile myocionic seizures)
make their initial appearance in the vast majority of instances before 12 months of age (Table I) with the peak incidence be-tween 3 and 6 months of age; 527 (75%) of the 698 patients had their first minor motor seizures before they reached 6 months of age. Approximately 30% of these 527
pa-tients were seen in the clinic prior to 10
years ago, at which time pertussis
immuni-zations generally were not administered until after 6 months of age.
It is generally agreed that minor motor
seizures are exceedingly difficult to control with anticonvulsant drugs-in the authors’ experience, the most difficult of all the epi-leptic seizures to control. Judging from the many patients with minor motor epilepsy referred from other physicians, it appears that the “methadione” drugs (Tridione#{174} and Paradione#{174}) are most commonly employed for the control of these spells. This is
proba-bly due to the fact that most physicians
con-sider minor motor spells as petit mal. The authors have found the methadione drugs are of no avail in controlling minor motor spells. Gibbs et al.1 and TaylorU have re-ported similar experiences.
Only an occasional satisfactory result has been achieved with the drugs listed in Table VI. Gibbs et al.1 reported that phenacetylcarbamide (Phenurone#{174}) is more effective than phenobarbital, diphenyl-hydantoin (Dilantin’), or
metilyl-phenyl-ethyl-hydantoin (Mesantoin#{174}). Stamps et al.’ reported good control of seizures and electroencephalographic improvement in some patients with chlortetracycline (Aureo-mycin#{174}), but Peterman2#{176} treated 18 patients with this antibiotic and observed no favora-ble results. Aureomycin#{174} failed to give con-trol in 11 children in the present series. Perlstein2l and Baird and Borofsky re-ported some good results with meproba-mate. The authorsl2 also had favorable re-suits with this compound in a few patients.
In suitable cases the ketogenic diet is the best of all treatments for minor motor
51)ellS. This diet is exl)ensive and requires
very close supervision by the child’s
par-ents. For these reasons its application is limited to children of falnilies who are
co-operative, and are willing to go to
con-siderabie expense and effort to control the
seizures. It was possible to treat 186 of the patients with a ketogenic diet, and complete control of spells was obtained in 91 (49%), although in some instances anticonvulsant medication was still necessary after the diet was established. The management of this
dietary regimen has already been presented
in detail.12
It is important to note that by and large the most serious handicap in minor motor
epilepsy is not the seizures themselves but
the associated mental retardation. Minor motor seizures disappear spontaneously as the child grows older, rarely recurring after 5 or 6 years of age. Although it is true that many of these patients subsequently de-velop other types of seizures, particularly major motor seizures, the latter are more amenable to anticonvulsant therapy.
Therefore, the major treatment of minor motor epilepsy is general care and manage-ment of the mentally retarded child. This differs little from the care of mentally re-tarded children in general. In many in-stances institutionalization is the only re-course. However, in many cases the management of the child at home has been both feasible and emotionally rewarding for the parents. No general advice can be given
on this point, as itis a matter for individual
decision in each case.
SUMMARY
The diagnosis, treatment and prognosis of
minor motor epilepsy, based on study of 698 children, are discussed; 622 were followed
for intervals ranging from 3 to 22 years. Minor motor seizures begin most often between 3 and 12 months of age.
Brain damage is prominent in tile general clinical manifestations of patients with
minor motor epilepsy.
pres-Typic es le constatation
electroencephalo-or 6 years of age.
These spells are exceedingly difficult to control with anticonvuisant drugs. A ketogenic diet is the most effective therapy.
The most serious aspect of minor motor
epilepsy is the associated mental retarda-tion.
Presumptive etiologic factors are dis-cussed.
ACKNOWLEDGMENT
We are indebted to Drs. Frank R. Ford
and A. Earl Walker for advice and sugges-tion in tile preparation of this paper.
REFERENCES
1. Gibbs, E. L., Fleming, M. M., and Gibbs, F. A. : Diagnosis and prognosis of
hyp-sarhythmia and infantile spasms.
PEDI-ATRICS, 13:66, 1954.
2. Lennox, W. G. : Petit mal epilepsies; their treatment with tridione. J.A.M.A., 129: 1069, 1945.
:3. Ford, R. R., Livingston, S., and Prvles,
C. V. : Familial degeneration of the
cerebral gray matter in childhood.
J.
Pediat., 39:33, 1951.
4. Alpers, B.
J.
: Diffuse progressivedegen-eration of the gray matter of the cere-brum. Arch. Neurol. & Psychiat., 25: 469, 1931.
5. Somoza: Quoted by Alpers, B.J.4 6. Christensen, E., and Krabbe, K. H. :
Polio-dystrophia cerebri progressiva (infan-tilis). Arch. Neurol. & Psychiat., 61:28,
1949.
7. West, W.
J.:
On a peculiar form of infan-tile convulsion. Lancet, 1:724, 1841.8. Newnham, W.: History of four cases of eclampsia nutans, or the “Salaam” con-vulsions of infancy. Brit. Rec. Obst. Med., 1849, p. 1.
9. Asal, B., and Moro, E.: Uber bosartige
Nickkrampfe im fr#{252}hen Kindesalter.
Jahrb. Kinderh., 107:1, 1924.
10. Buchanan, D.: Convulsions in infancy and childhood. M. Gun. North America, 30: 163, 1946.
ii. Taylor, F. M.: Myoclonic seizures in in-fancy and childhood. Texas
J.
Med., 48:647, 1952.
12. Livingston, S.: The Diagnosis and
Treat-spasms in infancy and childhood. Epi-lepsia, 4:61, 1955.
14. Baird, H. W., III, and Borofsky, L. G.: Infantile mvoclonic seizures.
J.
Pediat., 50:332, 1957.15. Ghao, D. H. C., Taylor, F. M., and
Druck-man, R. : Massive spasms.
J.
Pediat., 50:670, 1957.
16. Illingworth, R. S.: Sudden mental
deteri-oration with convulsions in infancy.
Arch. Dis. Childhood, 30:529, 1955.
17. Bvers, R. K., and Moll, F. C. : Encephal-opathies following prophylactic pertussis vaccine. PEDIATRICS, 1:437, 1948. 18. Low, N. L. : Electroencephalographic
studies following pertussis immuniza-tions.
J.
Pediat., 47:35, 1955.19. Stamps, F. W., Gibbs, E. L., and Haase, E. : Epileptic patients treated with
aureomycin. Dis. Nerv. System, 12:227, 1951.
20. Peterman, M. G. : Present status of
idio-pathic epilepsy.
J.
Pediat., 44:624, 1954. 21. Perlstein, M. A. : Use of meprobamate(Miltown) in convulsive and related
dis-orders. J.A.M.A., 161:1040, 1956.
22. Livingston, S., and Pauli, L. : Meproba-mate in the treatment of epilepsy of children. A.M.A.
J.
Dis. Child., 94:277,1957.
SUMMARIO IN INTERLINGUA
Epilepsia Motori Minor
Iste articulo presenta un revista del casos
de 698 juveniles con epilepsia motori minor. Le serie include 622 casos que esseva tenite
Stil) observation durante periodos de inter 3 e 22 annos.
Sub le termino de epilepsia motori minor,
le autores gruppa tres typos de accesso: Mas-sive accessos myoclonic, accessos de nutation del capite, e accessos acinetic. Omne le tres es characterisate per (1) breve duration, (2)
declaration a basse etates-inter 3 e 12 menses,
e (3) disparition a un etate de circa 5 o 6 annos.
Lesiones cerebral es un aspecto prominente in infantes con iste attaccos. Quasi omne le
patientes suifreva de grossier retardation men-tal. Iste facto rende le prognose pauco
928 EPILEPSY
graphic de “hypsarrhythmia.” Isto
presente in 381 del 389 patientes pro qui encephalogrammas esseva obtenite. Le con-figuration de hypsarrhythmia dispare a un etate de 5 a 7 annos.
Patientes con accessos motori minor fre-quentemente disveloppa accessos motori o
psychomotori major in le curso del
avantia-mento de br etate. Nulle patiente in le presente serie disveboppava accessos de petit mal.
Le condition debe esser distinguite, de un latere, ab accessos de petit mal-in que be electroencephalogramma monstra triple formas de agulia e unda per secunda-e, del altere latere, ab acinetic accessos motori major-in que be periodos de inconscientia es plus longe, con retornos gradual plus tosto que instantanee al stato de conscientia. Altere conditiones que pote esser confundite con epilepsia motori minor es syncope, cataplexia, isolate
con-tractiones nlvoclonic, e degenerative morbos
cerebral.
Le etiobogia del condition es incognoscite. Causas possibibe es discutite.
Le tractamento es difficile. In be experientia del autores, barbituratos, meprobamato, bro-muros, e succinamidas ha omnes essite efficace
in certe casos. Ii pare que be melior tractamento es un dieta cetogenic. Ilbo provideva un
regu-lation del accessos in circa un medietate deJ patientes a qui illo esseva date.
Le principal probbema in le preoccupation
con iste patientes consiste in be associate re-tardation mental. Accessos de epibepsia motori
minor dispare usualmente quando be patientes
attinge un etate de circa 5 a 6 annos. Le altere typos de accesso que es frequentemente
disveloppate in iste patientes se monstra usual-mente plus impressionabile per therapias
anticonvulsive.
Ti GASTRO-ESOPHAGEAL REGION IN INFANTS, G. S. Muller Botha. (Arch. Dis.
Child-hood, 33:78, February, 1958.)
Improvements in surgical and roentgenographic technics have led to more frequent diagnosis of conditions due to defects in the closing mechanism between the esophagus
and the stomach in infants. This paper presents detailed anatomic observations on the components of the normal closing mechanism and rebates the findings in clinical con-ditions, such as partial thoracic stomach, hiatus hernia, congenital short esophagus, and cardio-esophageal relaxation or chalasia. The muscle fibers and the connective tissues of the diaphragm form an important mechanism to prevent regurgitation of the con-tents of the stomach into the esophagus. Normally, this mechanism is extremely
corn-petent. The present study is based upon detailed dissection of 115 subjects. In the
majority, the ages ranged from a few hours to 1 month. The clinical manifestations
