Folia Psychiatrica et Neurologica Japonica, Vol. 31, No. 3, 1977
The Long-Term Prognosis of Minor Seizures in Childhood Epilepsy Tohru Seki, M.D., Yuhji Kawahara, M.D. and Makoto Hirose, M.D.* Department of Pediatrics, Keio University School of Medicine, Tokyo * Department of Pediatrics, Yokosuka Ky6sai Hospital, Yokosuka
INTRODUCTION The studies comparing the long-term prognosis of various seizures included in minor seizures of childhood epilepsy have so far been poor in Japan2* For the purpose of elucidating some of these problems, this paper presents the authors’ experience with regard to the long-term prognosis of minor seizures in childhood epilepsy.
Table 1: Classification of Minor Seizures Minor Seizures
Sex Male Female
npical absences Infantile spasms Lennox syndrome
11 14 7
Myoclodc seizures
6
Atonic seizures Total
3 41
14 11 1 3 2 31
Total 25 25 8 9 5 12
MATERIALS This paper was based on 72 cases with minor seizures, which were followed for more than three-to-thirteen years at the Department of Pediatrics of Keio University from January 1963 to January 1973. The minor seizures were classified into the following groups: typical absences (25 cases), infantile spasms (25 cases), Lennox syndrome (8 cases), myoclonic seizures (9 cases) and atonic seizures ( 5 cases) (Table 1). The term “typical absence” is defined as follows: The attack is characterized by a sudden and brief disturbance of consciousness lasting 5-20 seconds without aura and postparoxysmal symptoms, either with or without minor motor, automatic and autonomic components, accompanying diffuse rhythmic 3 c/s spike and wave discharges in EEG during the attack. The termination of the attack is abrupt and the attack Received for publication June 20, 1977.
itself occurs frequently. The term “infantile spasms” is defined as follows: ( 1) The type of spasms consists of sudden, brief myoclonic jerks with flexion of the neck, trunk and legs. Extensor spasms are sometimes combined with flexor spasms. The spasms may be repeated frequently to form a series, which may consist of 5-20 spasms, sometimes even more, and (2) the interictal EEG pattern consists of hypsarhythmia including modified hypsarhythmia and periodicity. Cases that satisfied (1) and (2) are defined as infantile spasms. The term “Lennox syndrome” is defined as follows: (1) the clinical seizure types consist of either tonic seizures or atypical absences, or both, and (2) diffuse pseudorhythmic slow spike and wave discharges, in the range of 1.5 to 2.5 c/s, are noticed in interictal EEG. Cases that satisfied (1) and (2) are defined as Lennox syndrome. Myoclonic seizures and atonic seizures
T. Seki et al.
316
Table 2: Minor Seizures Typical absences Infantile apasma Lennox syndrome
Myoclonic seizures Atonic seizures Total
Materials
leases 40 (100%) 49 (100%) 10 (100%) 15 (100%)
Cases of Follow-up
Cases of Dropout
25 (63%) 25 (51%) 8 (80%) 9
15 (37%) 24 (49%) 2
(60%)
(40%)
9
(100%) 123 (100%)
are defined according to the international classification of epileptic seizures.* The details of each group are as follows: Typical absences occurred without the combination of other fits in 16 cases (64%), grand ma1 or febrile convulsions (mixed form by Fukuyama or epilepsy triggered by fever (Rodin)) before the onset of typical absences in eight cases (32%) and combination of grand ma1 after the onset of typical absences in one case (4%). Infantile spasms occurred firstly without the combination of other fits in most of the children (21 cases, 84%), a combination of grand ma1 and infantile spasms about the same time in one case (4%) and fits (grand ma1 and unilateral seizures) other than spasms before infantile spasms in three cases (125%). I n Lennox syndrome, the combination of various types of seizures were as follows: tonic seizures only (2 cases), tonic seizures plus atypical absences (2 cases), tonic seizures plus myoclonic seizures plus grand ma1 (2 cases), tonic seizures plus grand ma1 (1 case) and tonic seizures plus myoclonic seizures (1 case), Myoclonic seizures occurred without the combination of other fits in all cases. Atonic seizures occurred without the combination of other fits in four cases (80%) and the combination of focal motor seizures after the onset of atonic seizures in
(20%)
6
5
(56%) 72 (59%)
4
(44%) 51 (41%)
Table 4: Length of Follow-up Period (yr.)
SOX
Total
Male
Female
3-4 5-9 10-13
19 19 3
17 10 4
36 29 7
Total
41
31
72
one case ( 20%) . Table 2 shows all cases and cases of drop-out during the period of observation. Cases of drop-out occupied about 50% of each group except Lennox syndrome. Table 3 shows the age of onset in each group. The peak of onset of infantile spasms is between four and seven months of age, typical absences between four and nine years, and Lennox syndrome, myoclonic seizures and atonic seizures in early childhood. Table 4 shows the length of follow-up. The length of follow-up varied from three to 13 years, but most children (65 cases, 90%) were observed for three to nine years. Various anticonvulsant drugs, ACTH-Z and steroid hormones were appropriately prescribed in each case. Ketogenic diet was performed in cases that were resistant to
Minor Seizures in Childhood Epilepsy
317
Table 3: Age of Onset in Each Group Age of Onset of Infantile Spasms Age b . 1
1
2
3
4
5
6
7
8
9
No. of cases
1
0
2
3
3
4
4
1
1
10-14
28-32
3
3
Age of Onset of Other Minor Seizures ~
Minor seizures
The Long-Term Prognosis of Minor Seizures in Childhood Epilepsy Tohru Seki, M.D., Yuhji Kawahara, M.D. and Makoto Hirose, M.D.* Department of Pediatrics, Keio University School of Medicine, Tokyo * Department of Pediatrics, Yokosuka Ky6sai Hospital, Yokosuka
INTRODUCTION The studies comparing the long-term prognosis of various seizures included in minor seizures of childhood epilepsy have so far been poor in Japan2* For the purpose of elucidating some of these problems, this paper presents the authors’ experience with regard to the long-term prognosis of minor seizures in childhood epilepsy.
Table 1: Classification of Minor Seizures Minor Seizures
Sex Male Female
npical absences Infantile spasms Lennox syndrome
11 14 7
Myoclodc seizures
6
Atonic seizures Total
3 41
14 11 1 3 2 31
Total 25 25 8 9 5 12
MATERIALS This paper was based on 72 cases with minor seizures, which were followed for more than three-to-thirteen years at the Department of Pediatrics of Keio University from January 1963 to January 1973. The minor seizures were classified into the following groups: typical absences (25 cases), infantile spasms (25 cases), Lennox syndrome (8 cases), myoclonic seizures (9 cases) and atonic seizures ( 5 cases) (Table 1). The term “typical absence” is defined as follows: The attack is characterized by a sudden and brief disturbance of consciousness lasting 5-20 seconds without aura and postparoxysmal symptoms, either with or without minor motor, automatic and autonomic components, accompanying diffuse rhythmic 3 c/s spike and wave discharges in EEG during the attack. The termination of the attack is abrupt and the attack Received for publication June 20, 1977.
itself occurs frequently. The term “infantile spasms” is defined as follows: ( 1) The type of spasms consists of sudden, brief myoclonic jerks with flexion of the neck, trunk and legs. Extensor spasms are sometimes combined with flexor spasms. The spasms may be repeated frequently to form a series, which may consist of 5-20 spasms, sometimes even more, and (2) the interictal EEG pattern consists of hypsarhythmia including modified hypsarhythmia and periodicity. Cases that satisfied (1) and (2) are defined as infantile spasms. The term “Lennox syndrome” is defined as follows: (1) the clinical seizure types consist of either tonic seizures or atypical absences, or both, and (2) diffuse pseudorhythmic slow spike and wave discharges, in the range of 1.5 to 2.5 c/s, are noticed in interictal EEG. Cases that satisfied (1) and (2) are defined as Lennox syndrome. Myoclonic seizures and atonic seizures
T. Seki et al.
316
Table 2: Minor Seizures Typical absences Infantile apasma Lennox syndrome
Myoclonic seizures Atonic seizures Total
Materials
leases 40 (100%) 49 (100%) 10 (100%) 15 (100%)
Cases of Follow-up
Cases of Dropout
25 (63%) 25 (51%) 8 (80%) 9
15 (37%) 24 (49%) 2
(60%)
(40%)
9
(100%) 123 (100%)
are defined according to the international classification of epileptic seizures.* The details of each group are as follows: Typical absences occurred without the combination of other fits in 16 cases (64%), grand ma1 or febrile convulsions (mixed form by Fukuyama or epilepsy triggered by fever (Rodin)) before the onset of typical absences in eight cases (32%) and combination of grand ma1 after the onset of typical absences in one case (4%). Infantile spasms occurred firstly without the combination of other fits in most of the children (21 cases, 84%), a combination of grand ma1 and infantile spasms about the same time in one case (4%) and fits (grand ma1 and unilateral seizures) other than spasms before infantile spasms in three cases (125%). I n Lennox syndrome, the combination of various types of seizures were as follows: tonic seizures only (2 cases), tonic seizures plus atypical absences (2 cases), tonic seizures plus myoclonic seizures plus grand ma1 (2 cases), tonic seizures plus grand ma1 (1 case) and tonic seizures plus myoclonic seizures (1 case), Myoclonic seizures occurred without the combination of other fits in all cases. Atonic seizures occurred without the combination of other fits in four cases (80%) and the combination of focal motor seizures after the onset of atonic seizures in
(20%)
6
5
(56%) 72 (59%)
4
(44%) 51 (41%)
Table 4: Length of Follow-up Period (yr.)
SOX
Total
Male
Female
3-4 5-9 10-13
19 19 3
17 10 4
36 29 7
Total
41
31
72
one case ( 20%) . Table 2 shows all cases and cases of drop-out during the period of observation. Cases of drop-out occupied about 50% of each group except Lennox syndrome. Table 3 shows the age of onset in each group. The peak of onset of infantile spasms is between four and seven months of age, typical absences between four and nine years, and Lennox syndrome, myoclonic seizures and atonic seizures in early childhood. Table 4 shows the length of follow-up. The length of follow-up varied from three to 13 years, but most children (65 cases, 90%) were observed for three to nine years. Various anticonvulsant drugs, ACTH-Z and steroid hormones were appropriately prescribed in each case. Ketogenic diet was performed in cases that were resistant to
Minor Seizures in Childhood Epilepsy
317
Table 3: Age of Onset in Each Group Age of Onset of Infantile Spasms Age b . 1
1
2
3
4
5
6
7
8
9
No. of cases
1
0
2
3
3
4
4
1
1
10-14
28-32
3
3
Age of Onset of Other Minor Seizures ~
Minor seizures
