transient remission in intractable localization-related epilepsies of childhood onset

2

Click here to load reader

Upload: junko-nakai

Post on 19-Jul-2016

214 views

Category:

Documents


0 download

TRANSCRIPT

Page 1: Transient Remission In Intractable Localization-Related Epilepsies of Childhood Onset

PLATFORM AND POSTER SESSIONS 63

Photosensitivity According to Types of Epilepsy and Epileptic Syn- dromes. Hideaki Shiraishi, Tateki Fujiwara, Yushi Inoue, and Kazui- chi Yagi (National Epilepsy Center, Shizuoka Higashi Hospital, Shi- zuoka, Japan).

Purpose: Despite various reports about photo-paroxysmal response (PPR) among patients with epilepsy, few such studies utilize the clas- sification of epileptic syndromes defined by the International League against Epilepsy (ILAE) in 1989. We analyzed the features of patients with positive PPR according to the type of epilepsy and epileptic syn- dromes in Japanese patients.

Pcirients: We investigated 2,187 patients with epilepsy who were treated in The National Epilepsy Center for I year between February 1997 and February 1998. The mean age was 24.2 years old, 1,226 patients were male (56%), and 961 female (44%). We diagnosed the epileptic syndrome by electroclinical analysis and investigation of sei- zure phenomenon. The number of patients for each epileptic syndrome was 232 ( I I % of all patients) with idiopathic generalized epilepsy (ICE), 512 (23%) with symptomatic generalized epilepsy JSGE), I ,i99 (55%) with symptomatic partial epilepsy (SPE), 23 ( I % ) with idio- pathic partial epilepsy (IPE), 105 (5%) with undetermined epilepsy (UDE), and 110 (5%) with unclassifiable epilepsy (UCE).

Methods: We used a Grass PS33 Plus photic stimulator to deliver intermittent photic stimulation (IPS) with the following parameters: flash intensity 8 (about 1000 lux at the nasion), placed 30 cm from the nasion in a brightly illuminated room, 18 flashes per second, and eyes open then closed. If the PPR was elicited, 6, 10, 12, 15, 33, and 20 flashes per second stimulation were executed. The stimulation period was 4 seconds for screening at first, but stimulation was stopped if a positive response was obtained. The definition of positive PPR was based upon Binnie's criteria of bilateral diffuse spike-wave complexes or polyspike-wave complexes outlasting the stimulus for at least 100 msec.

Results: Thirty-seven ( I .7% of all patients) patients showed a posi- tive PPR in our analysis. The distribution was 19 male patients (1.5% of all the male patients) and I8 female patients ( I .9% of all the female patients), Mean age was 17.0 years old and ranged from 4 to 44 years old. The subpopulation of patients with positive PPR findings accord- ing to the type of epilepsy was 10 with SGE (2.0% of all patients with SGE), 13 with IGE (5.6% of all patients with IGE, p < O.Ol), 9 with SPE (0.7% of all patients with SPE), and 5 with UDE (4.8% of a11 patients with UDE, p < 0.05). Juvenile myoclonic epilepsy (JME) patients with positive PPR accounted for 17.4 % of all the patients with JME, p < 0.01. Positive PPRs occurred in 5 patients with GTCS on awakening (7.6% of all the patients with GTCS on awakening,

The subpopulations of SPE were: 2 with TLE (0.4% of all patients with TLE), 5 with OLE (4.5% of all patients with OLE, p < 0.05) and 1 with FLE. The exact epileptic focus could not be determined in 1 patient with positive PPR findings. Incidence of positive PPR gradually increased with age to 15 years old and suddenly decreased after 20 years old. The maximum incidence was 4.3% at the age range between I 1 and IS years old and this high incidence remained until 20 years old.

Conclusions: ICE was the most characteristic type of epilepsy with positive PPR findings, and the patients with JME and GTCS on awak- ening elicited PPR most frequently. OLE occurred most frequently among the patients with SPE. We also confirmed that the incidence of PPR showed a characteristic dependency upon age.

p < 0.01).

Clinical Course and Prognosis

Clinical and Electroencephalographic Study of Patients with Be- nign Rolandic Epilepsy from the Standpoint of the Total Number of Seizures. Takashi Kajitani, Motonori Kaueko, Takafumi Kimura, and Yohko Takeda (Department of Pediatrics, Kawasaki Hospital, Ka- wasaki Medical School, Okayama City, Japan).

Eighty patients with benign rolandic epilepsy who had discontinued medication without the recurrence of seizures for 1.8-21.8 years (av-

erage, 8.7 years) were classified into 4 groups according to the total numbers of epileptic seizures. Group A consisted of 10 patients (12%) with a single seizure; group B, 28 (35%) with 2-4 seizures; group C, 22 (28%) with 5-9 seizures; and group D, 20 (25%) with 210 seiiures during the entire course.

Boys predominated, with 60% boys and 40% girls. Males predomi- nated in group A (70%). A positive family history of convulsive dis- orders (febrile convulsions, epilepsies, etc.) within third degree rela- tives was noted in 60%, 50%, 5096, and 60% of groups A, B, C, and D, respectively. A preceding history of febrile convulsions in infancy or early childhood was found in 30%, 1896, IS%, and 5% of groups A, B, C, and D, respectively. The main clinical seizure types were partial seizures, secondarily generalized seizures, and hemiconvulsive sei- zures. Partial seizures occurred in 3 cases (30%) in group A and in 19 cases (95%) in group D. The difference between these 2 groups was statistically significant (P < 0.001). As a whole, seizures occurred in 69 cases (86%) only during sleep, in 9 ( 1 I "r) in both sleep and the awake state and in only 2 (3%), i s . one in groups A and B, respeclively, i n the awake state only. The peak ages at onset i n groups A, B, C, and D were 7.7, 7.0, 7.5, and 6.4 years, respectively. The period of active epilepsy (time between the first and last seizures) was <2 years in 21 (75%), 7 (32%), and 4 (20%) cases in groups B, C, and D, respectively. Seizures persisted for >4 years in 6 cases in group D only. The interval between the first and second seizures was <6 months in 10 cases (36%) in group B and in 18 cases (90%) in group D (P < 0.00 I ). The interval was > I2 months in 12 cases (43%), I case (5%), and I case (5%) in groups B, C, and D, respectively. There were significant differences between groups B and C (P < 0.01), and groups B and D (P < 0.025). The last seizure occurred at age 3-12 years (peak age, 8.2 years) in group A, 3-12 years (peak age, 8.7 years) in group B, 7-12 years (peak age, 10.2 years) in group C, and 5-13 years (peak age, 10.1 years) in group D.

Rolandic discharges (RD) observed on EEG persisted <3 years in 6 (60%), 17 (61%), 9 (41%), and 2 (10%) cases in groups A, B, C, and D, respectively. There were significant differences between groups A and D (P < 0.025) and groups B and D (P < 0.005). One-third of the 80 cases showed generalized spike-wave discharges without any concomi- tant absence seizure in addition to the RD in the course of the illness. In 40% of group A, 25% of group B, 32% of group C, and 50% of group D, generalized spike-wave discharges were observed.

Among these 4 groups, there were somewhat different findings re- garding the preceding history of febrile convulsions, main clinical sei- zure types, age at onset, period of active epilepsy, interval between the first and second seizures, age at the last seizure, period of persistence of the RD on EEG, etc. Thus, we can predict that the total number of seizures during the entire course will be low when the interval between the first and second seizures is >1 year.

Transient Remission In Intractable Localization-Related Epilepsies of Childhood Onset. Junko Nakai, Kazuyoshi Watanabe, Akihisa Okumura, Tamiko Negoro, and *Kosaburo Aso (Department of Pedi- atrics, Nagoya University School of Medicine, Nagoya; and *Aichi Prefectural Hospital for Disabled Children of Dai-Ichi Aoitori Gakuen, Nagoya, Japan).

Purpose: About 10% to 20 % of patients with epilepsy have medi- cally intractable seizures. In some patients, the intractable course may be interrupted by seizure-free periods. Transient remission was re- ported in mesial temporal sclerosis (MTS) or focal cortical dysplasia (FCD), but few studies have examined this phenomenon in detail. We retrospectively reviewed 99 patients with intractable cryptogenic or symptomatic localization-related epilepsy and studied the clinical fea- tures of those with transient remission lasting for >2 years.

Methods: We investigated 99 patients with cryptogenic or symptom- atic localization-related epilepsies who were treated in Nagoya Uni- versity Hospital for >5 years and had medically intractable seizures. The underlying diseases of these 99 patients consisted of mesial tem- poral sclerosis ( 1 3 patients), tuberous sclerosis (9), hypoxic ischemic encephalopathy (9). central nervous system infection (7), iieuronal mi- gration disorder (7), cerebrovascular disease (4), and periventricular leukomalacia ( I ) . No underlying disease was determined in the remain- ing 49 patients. The epileptic focus was located in the temporal lobe in 37 patients, frontal lobe in 29, occipital lobe in 5 , parietal lobe in 2, and was not determined in the remaining 26. The age of onset ranged from

Epilqniu, Vol. 41, SuppI. 9, 2000

Page 2: Transient Remission In Intractable Localization-Related Epilepsies of Childhood Onset

PLATFORM AND POSTER SESSIONS

0 month to 14 years (mean 4 years and 3 months). The duration of follow-up ranged from 5 to 33 years (mean 15 years). The age at the last visit ranged from 9 to 34 years (mean 21 years). Eight (8%) patients had a family history of febrile convulsions, and 13 ( I 3%) had a family history of epilepsy. Forty-nine (50%) patients had mental deficits.

Result: Among 99 patients with intractable cryptogenic or symptom- atic localization-related epilepsies of childhood onset, 10 (10%) pa- tients had transient remission that lasted for 2 years or more. The underlying diseases of these 10 patients consisted of mesial temporal sclerosis (3 patients), tuberous sclerosis ( I ) , cortical dysplasia ( I ) , in- tracranial hemorrhage (I), head trauma ( I ) , and porencephalic cyst (1). No underlying disease was determined in the remaining 2 patients. The epileptic focus was located in the temporal lobe in 5 patients, frontal lobe in 2, and was not determined in the remaining 3 patients. Transient remission occurred within a few months after initiation of medical treatment if an appropriate anticonvulsant was given and lasted for 28 to 99 months. Epileptiforin discharges on the EEG disappeared in 7 patients during transient remission, and reappeared in 5 after recurrence of intractable seizures. Two (20%) patients had mental deficits, and 5 (50%) had a family history of epilepsy. Among 89 patients without transient remission, 57 (64%) patients had mental deficits, and 8 (9%) had a family history of epilepsy.

Conclusions: Transient remission in intractable localization-related epilepsy occurs in a variety of pathological conditions and may reflect the progressive nature of some types of epileptic foci.

Psychiatry and Psychology

VIQ-PIQ Discrepancies in Partial Epilepsy: On the Relation to Lat- eralities of Focal MRI Lesions, P3 Peaks, and Focal Spikes. Osamu Kanazawa, Mihoko Yoshino, Mutsuo Sasagawa, Manabu Wachi, Masafuini Fukuda, and Shigeki Kameyama (Epilepsy Center, Nishi- Niigata Central National Hospital, Niigata, Japan).

Purpose: Subtest IQ such as verbal IQ (VIQ) and performance IQ (PIQ) in WAIS or WISC are thought to represent neuropsychological functions of the left and right hemispheres, respectively. The P300 (P3) event-related potential reflects cognitive processes. We do not ye1 know the brain site of P3 origin or how epileptogenic foci (EF) influ- ence P3 potentials. To examine neuropsychological influence by partial epilepsy (PE), we studied VIQ-PIQ discrepancies in PE in relation to lateralities of focal MRI lesions, P3 peaks, and EF.

Methods: Thirteen patients showed VIQ-PIQ discrepancies signifi- cant at the p<O.O5 level, represented by a>l2-point spread for the WAIS in adults, and a 15-point spread in the WISC in children. We evoked P3 potentials in the individuals with discrepant IQ differences by asking them to keep a mental count of rare tones, including intro- duction of oddbail tones. EEGs were recorded by the international 10-20 system and P3 peaks were shown in a topographical view by offline analysis. Patients were divided into normal and abnormal groups according to MRI findings, and were examined for the laterali- ties of the dominant side in subtest IQ (conventionally, we regarded higher VIQ as left hemisphere dominant and higher PIQ as right hemi- sphere dominant), P3 peaks, and EF. We did not correlate results with lert or right handedness.

Resulrs: Five patients (38.5%) were in the normal group and 8 pa- tients (61.5%) were in the abnormal group. Concordance of the later- alities in P3 peaks and dominant side in subtest IQ was shown in I patient (20%) in the normal group and 5 patients (62.5%) in the ab- normal group. In the normal group, all patients showed contralateral P3 peak shift to EF, and all except I patient showed contralateral P3 peak shift to the dominant side in subtest IQ. The other 3 patients in the abnormal group showed unilateral focal cortical dysplasias (FCD), ip- silateral P3 shift, and contralateral dominant side in subtest IQ to the focal MRI lesions.

Conclusion: In our partial epilepsy series with VIQ-PIQ discrepan- cies, concordance of the lateralities in P3 peaks and dominant side in subtest IQ was shown in < half of the patients. Epileptogenic foci seem

to have 3 different grades of influence on P3 peak shift and dominant side in subtest IQ according to the severities of accompanying focal MRI lesions: 1. Without MRI lesions, EF can make P3 peak shift contralaterally, but the dominant side in the subtest IQ shift ipsilater- ally; 2. With less severe focal MRI lesions such as hippocampal atro- phy etc., EF can make not only P3 peaks but also the dominant side in the subtest IQ shift contralaterally; 3. With severe focal MRI lesions such as FCD, EF can make the dominant side in the subtest IQ shift contralaterally, but the P3 peak may shift ipsilaterally. Epileptogenic foci without MRI lesions seem to control ipsilateral P3 potentials. MRI lesions render a hemisphere unlikely to become dominant, but epileptogenic foci can coexist with apparently normal neuropsycholog- ical function.

A Multi-Institutional Study on a New Five-Axial Classification Sys- tem for Epileptic Psychoses. Masato Matsuura, Takuya Kojima, Naoto Adachi, Yasunori Oana, Yoshiro Okubo, Tunekatsu Hara, and Teiichi Onuma (Nihon University School of Medicine, National Lep- rosarium Tama Zenshoen, Tokyo Medical College, Tokyo Medical and Dental University, Komakino Hospital, National Saikata Hospital).

Purpose: The pathophysiology and clinical phenomenology of epi- leptic psychoses are wide and varied, and an internationally accepted classification system has yet to be established. A multi-axial classifi- cation should be developed to capture the complexity of clinicai situ- ations and to describe the heterogeneity of individuals presenting with the same diagnosis. We propose a new 5-axial classification system based on a review of the previous literature and evaluate its validity and applicability in Japan by a multi-institutional design.

Merhods: According to the ICD- 10 guidelines, psychosis is defined as the presence of hallucination, delusions, or a limited number of behavioral abnormalities, such as gross excitement and overactivity, marked psychomotor retardation, and catatonic behavior, without dis- turbance of consciousness. Among the patients with epilepsy and psy- choses treated by the authors at 6 epilepsy clinics, 128 patients were randomly selected to participate as subjects. Those patients who de- veloped epilepsy after the onset of their psychoses were excluded. The average age of the subjects was 39.4 years, average age of onset of epilepsy was 13.3 years, and average age of onset of psychoses was 27.9 years.

Results: The first axis codes the type of epilepsy and laterality of epileptic focus. Temporal lobe epilepsy (48%) and other localization- related epilepsies (14%) were prevalent, but all types of epilepsy were seen. Left-sided (29%) and right-sided foci (27%) were in equal pro- portion, and bilateral foci (9%) also were found. The second axis codes the type of psychoses and clinical course. Paranoid schizophrenia-like disorder (41%) and delusional disorder (19%) were prevalent; whereas acute transient psychotic disorder (9%), catatonic disorder (7%), hal- lucinosis (6%~), and hebephrenic schizophrenia-like disorder (4%) were relatively infrequent. Single episode (24%1), recurrent episodes (34%), and chronic course (34%) were found in almost equal proportion. Therc were several cases with chronic course after recurrent episodes (3%). The third axis codes the temporal relationship between seizure occur- rence and EEG changes during psychoses. lnterictal onset (69%) was most frequent followed by postictal (15%), para-ictal (6%), and other (5%). EEG changes during psychoses were seen in 38%, and normal- ization of the EEG was seen in 4%of patients. In 22%, EEG could not be recorded during psychoses (22%). The fourth axis codes the pre- cipitating factors of psychoses. Specific personality trait (20%), changes in drug-regimen (l4%), co-morbidity with other psychiatric disorders (12%), and psychosocial factors (10%) were seen. The fifth axis codes the organic background. Abnormal findings by brain imag- ing techniques (38%). mild intelligence deficiency (30%), and previous history of brain damage (27%) were noted.

Conclusions: The present results demonstrate the heterogeneity of epileptic psychoses. We have proposed a 5-axial classification that appears to provide a convenient format for organizing and communi- cating clinical information to assist clinical practice and facilitate re- search. Using those guidelines, we may be able to improve our under- standing of the pathogenesis, develop suitable treatment, predict the prognosis, and ultimately establish appropriate intervention strategies.