Effect of levodopa on pathological gait in Dravet syndrome: A randomized crossover trial using three-dimensional gait analysis.

OBJECTIVE: Individuals with Dravet syndrome (DS) exhibit progressive gait disturbance. No quantitative studies have been conducted to evaluate the effectiveness of medication for gait disturbance. Therefore, the aim of this study was to evaluate the effectiveness of levodopa for pathological gait in people with DS using three-dimensional gait analysis (3DGA). METHODS: Nine individuals with DS, ages 6-20 years, participated in a crossover study of levodopa and were randomly assigned to the levodopa precedence or no levodopa precedence group. Levodopa/carbidopa hydrate was prescribed at a dose of 5 mg/kg/day (body weight <60 kg) or 300 mg/day (body weight ≥60 kg). The medication was taken for 4-6 weeks (4-week washout period). 3DGA was performed three times before the study, with and without levodopa. A mixed-effects model was used to evaluate the effectiveness of levodopa. The primary outcome was the change in the Gait Deviation Index (GDI). In addition, spatiotemporal gait parameters, 6-minute walking distance (6MD), and balance were evaluated. The correlation between the effectiveness of levodopa and age or gait performance before starting levodopa was analyzed. RESULTS: Levodopa improved the GDI by 4.2 points, (p = .029), 6MD by 52 m (p = .002), and balance test result by 4.1 mm (p = .011) in participants with DS. No severe adverse events were observed, with the exception of one participant, who exhibited fever and consequently stopped taking levodopa. Levodopa was more effective in younger participants with a higher baseline gait performance. SIGNIFICANCE: Our randomized crossover trial showed that levodopa has the potential to improve gait disturbance in people with DS.

Hippocampal diffusion abnormality after febrile status epilepticus is related to subsequent epilepsy.

OBJECTIVE: To assess hippocampal signal changes on diffusion-weighted imaging (DWI) during the acute period after febrile status epilepticus (FSE) and to examine the relationship between DWI and subsequent epilepsy. METHODS: A prospective, multicenter study of children with a first episode of FSE was performed. The patients underwent magnetic resonance imaging (MRI) within 3 days of FSE, and signal intensity was evaluated on DWI. Electroencephalography studies within 3 days of FSE were also assessed. Nine to 13 years after FSE, information on subsequent epilepsy was obtained. RESULTS: Twenty-two children with FSE were evaluated. DWI showed unilateral hippocampal hyperintensity in six patients (27%). Three of six patients with hippocampal hyperintensity had ipsilateral thalamic hyperintensity. On EEG within 3 days of FSE, five of six patients with hippocampal hyperintensity had ipsilateral focal slowing, spikes, or attenuation. Nine to 13 years later, the outcomes could be determined in five patients with hippocampal hyperintensity and in 10 without. All 5 patients with hippocampal hyperintensity had hippocampal atrophy and developed focal epilepsy, whereas only 1 of 10 patients without hippocampal hyperintensity developed epilepsy (P = 0.002). Ictal semiology was concordant with temporal lobe seizures in all patients. Ipsilateral temporal epileptiform abnormalities were seen on EEG in four of five at last follow-up. SIGNIFICANCE: Acute DWI hippocampal hyperintensity was seen in 27% of patients with FSE. Acute DWI hyperintensity suggests cytotoxic edema caused by prolonged seizure activity. Hippocampal DWI hyperintensity is related to mesial temporal lobe epilepsy and can be a target of neuroprotective treatments to prevent the onset of epilepsy.

Microarray and FISH-based genotype-phenotype analysis of 22 Japanese patients with Wolf-Hirschhorn syndrome.

Wolf-Hirschhorn syndrome (WHS) is a contiguous gene deletion syndrome of the distal 4p chromosome, characterized by craniofacial features, growth impairment, intellectual disability, and seizures. Although genotype-phenotype correlation studies have previously been published, several important issues remain to be elucidated including seizure severity. We present detailed clinical and molecular-cytogenetic findings from a microarray and fluorescence in situ hybridization (FISH)-based genotype-phenotype analysis of 22 Japanese WHS patients, the first large non-Western series. 4p deletions were terminal in 20 patients and interstitial in two, with deletion sizes ranging from 2.06 to 29.42 Mb. The new Wolf-Hirschhorn syndrome critical region (WHSCR2) was deleted in all cases, and duplication of other chromosomal regions occurred in four. Complex mosaicism was identified in two cases: two different 4p terminal deletions; a simple 4p terminal deletion and an unbalanced translocation with the same 4p breakpoint. Seizures began in infancy in 33% (2/6) of cases with small (<6 Mb) deletions and in 86% (12/14) of cases with larger deletions (>6 Mb). Status epilepticus occurred in 17% (1/6) with small deletions and in 87% (13/15) with larger deletions. Renal hypoplasia or dysplasia and structural ocular anomalies were more prevalent in those with larger deletions. A new susceptible region for seizure occurrence is suggested between 0.76 and 1.3 Mb from 4 pter, encompassing CTBP1 and CPLX1, and distal to the previously-supposed candidate gene LETM1. The usefulness of bromide therapy for seizures and additional clinical features including hypercholesterolemia are also described.

Cerebrovascular diseases in two patients with entire NSD1 deletion.

We describe two patients with NSD1 deletion, who presented with early-onset, or recurrent cerebrovascular diseases (CVDs). A 39-year-old female showed developmental delay and abnormal gait in infancy, and developed slowly-progressive intellectual disability and movement disorders. Brain imaging suggested recurrent parenchymal hemorrhages. A 6-year-old male had tremor as a neonate and brain imaging revealed subdural hematoma and brain contusion. This report suggests possible involvement of CVDs associated with NSD1 deletion.

SCN8A-related developmental and epileptic encephalopathy with ictal asystole requiring cardiac pacemaker implantation.

INTRODUCTION: SCN8A-related epilepsy has various phenotypes. In particular, patients with developmental and epileptic encephalopathy (DEE) are resistant to antiepileptic drugs and may present with autonomic symptoms, such as marked bradycardia and apnea during seizures, and thus have an increased risk of sudden death. Herein, we report a case of very severe SCN8A-related epilepsy necessitating cardiac pacemaker implantation because of repetitive ictal asystole. CASE REPORT: The patient was a 14-month-old girl. Tremor and generalized tonic seizure occurred after birth. During seizures, bradycardia and perioral cyanosis occurred, and then, after developing tachycardia and apnea, marked bradycardia and generalized cyanosis occurred, which sometimes resulted in ictal asystole requiring cardiopulmonary resuscitation. Her seizures were refractory to antiepileptic drugs. As the seizures requiring resuscitation did not decrease, cardiac pacemaker implantation was performed four months after birth. Exome sequencing revealed a heterozygous de novo variant in SCN8A (NM_014191.3:c.4934T>C,p.(Met1645Thr)). Even though phenytoin was effective, seizures with bradycardia remained approximately once a month, and pacemaker activity was observed. CONCLUSIONS: This is, to our knowledge, the first reported case of SCN8A-related DEE in whom pacemaker implantation was performed. Pacemaker implantation should be considered as a treatment option for critical patients with SCN8A-related DEE as in the present case, because the incidence of sudden unexpected death in epilepsy is reported to be approximately 10% in patients with SCN8A-related DEE.

Natural histories of patients with Wolf-Hirschhorn syndrome derived from variable chromosomal abnormalities.

Wolf-Hirschhorn syndrome (WHS) is a subtelomeric deletion syndrome affecting the short arm of chromosome 4. The main clinical features are a typical craniofacial appearance, growth deficiency, developmental delays, and seizures. Previous genotype-phenotype correlation analyses showed some candidate regions for each clinical finding. The WHS critical region has been narrowed into the region 2 Mb from the telomere, which includes LETM1 and WHSC1; however, this region is insufficient to cause "typical WHS facial appearance". In this study, we identified 10 patients with a deletion involving 4p16.3. Five patients showed pure terminal deletions and three showed unbalanced translocations. The remaining patients showed an interstitial deletion and a suspected inverted-duplication-deletion. Among 10 patients, one patient did not show "typical WHS facial appearance" although his interstitial deletion included LETM1 and WHSC1. On the other hand, another patient exhibited "typical WHS facial appearance" although her small deletion did not include LETM1 and WHSC1. Instead, FGFRL1 was considered as the candidate for this finding. The largest deletion of 34.7 Mb was identified in a patient with the most severe phenotype of WHS.

Successful corpus callosotomy for post-encephalopathic refractory epilepsy in a patient with MECP2 duplication syndrome.

BACKGROUND: Patients with MECP2 duplication syndrome present with distinct facial anomalies and clinical features such as global developmental delay, recurrent respiratory infections, and epileptic seizures. Approximately half of all patients develop epileptic seizures which are refractory in most cases despite active medical management. Furthermore, no previous reports have discussed the efficacy of surgical treatment for seizures in patients with MECP2 duplication syndrome. CASE REPORT: In the present report, we describe a case of MECP2 duplication syndrome in a 15-year-old boy who developed epileptic seizures following influenza-associated acute encephalitis. His frequent epileptic spasms, tonic, atonic, and partial seizures were refractory to multiple antiepileptic medications. Electroencephalography revealed continuous diffuse epileptic discharge, resulting in regression. A total corpus callosotomy (CC) was performed at the age of 14 years and 7 months. His seizures markedly decreased following CC, although he continued to experience brief partial seizures approximately once per month. Post-operative examination revealed that his epileptic discharges had disappeared, and that his developmental state had returned to pre-encephalopathy levels. CONCLUSION: Our findings indicate that CC may represent a valuable surgical option for children with medically refractory generalized seizures following acute encephalopathy, irrespective of genetic disorders such as MECP2 duplication syndrome.

Ictal electroencephalographic findings of neonatal seizures in preterm infants.

The aim of this study is to clarify the characteristics of ictal EEG findings of neonatal seizures in preterm infants. Seizures associated with ictal EEG changes were recognized in nine infants with gestational age of less than 37 weeks. Propagation, migration, shifting, changes in morphology of ictal EEG discharges were evaluated. Seizure manifestation was divided into the following categories; motor seizure, apneic seizure, automatic seizure and seizure without clinical symptoms. The types of the seizures were motor seizures in five infants, apneic in two, automatic in one and those without clinical symptoms in five. All seizures were of focal onset. The foci of seizures were temporal in six infants, occipital in two, central in one, and frontal in one. The morphology of ictal discharges was low voltage spikes or sharp waves in six infants, spikes in two, theta waves in one and high-voltage spiky theta in one. The propagation of ictal discharges was focal in five infants and regional in five. The migration of ictal discharges was observed in two infants and a shift in two. There was no clear relation between seizure manifestation and ictal EEG foci, duration of seizures and morphology or propagation of ictal discharges.

Subacute encephalopathy: clinical features, laboratory data, neuroimaging, and outcomes.

We sought to clarify the clinical, laboratory, neuroradiologic, and neurophysiologic features of the "subacute" subtype of encephalopathy. We retrospectively identified nine patients with subacute encephalopathy out of 97 patients diagnosed as manifesting acute encephalopathy. Neurologic symptoms, clinical course, laboratory data, neuroradiologic and electroencephalographic findings, and outcomes were reviewed through medical records. The median age of patients was 44 months (range, 28-156 months). The initial neurologic sign was a brief seizure in 4, a prolonged seizure in 3, delirious behavior in 1, and a loss of consciousness in 1. Loss of consciousness the next day was subtle in 4, and mild in 5. However, a worsening of consciousness was observed 3-7 days after onset. Laboratory data were unremarkable, and electroencephalography during the early phase found abnormalities in 4 of 7 patients. Magnetic resonance imaging revealed no abnormalities during the early phase, and mild cortical atrophy during the late phase. All but one patient had various degrees of neurologic sequelae. Subacute encephalopathy was characterized by a delayed worsening of neurologic symptoms, mild cortical atrophy on late magnetic resonance imaging, and poor neurologic outcomes. Recognition of this type of acute encephalopathy is important, and a method to promote early diagnosis is desirable.

Mild oliguria in preterm infants who later developed periventricular leukomalacia.

The aim of this study is to determine whether or not renal involvement was present during the early neonatal period in preterm infants with PVL. We conducted a case-control study. The following items were evaluated; urine output, serum levels of sodium (Na), potassium (K), chloride (Cl), urea nitrogen (UN), and creatinine (Cr). The factors that could influence the urine output were also compared between the PVL and the control group. The mean urine output during the first 24h in the PVL group was 19.8ml/kg/day, and was significantly lower than in the control group (28.8ml/kg/day, p<0.05). The mean UN and Cr were not significantly different between the two groups. The minimal serum Na and Cl levels in the PVL group were significantly lower (128.3 and 94.3mEq/l) than those in the control group (134.8 and 100.7mEq/l, p<0.01 each). The maximal serum K level was significantly higher in the PVL group (6.47mEq/l) as compared to the control group (5.57mEq/l, p<0.05). There were no differences in any postnatal variables between the two groups. The preterm infants who later developed PVL had mild but significant oliguria during the first 24h of life. This suggests that preterm infants with PVL will have renal involvement immediately after birth.

Efficacy and adverse effects of rectal thiamylal with oral triclofos for children undergoing magnetic resonance imaging.

We studied the efficacy and adverse effects of rectal thiamylal in combination with oral triclofos in sedation for pediatric magnetic resonance imaging. Five hundred forty-six children underwent MRI examination from January of 1997 to December of 2001. Among them, 10mg/kg of rectal thiamylal was administrated after oral triclofos in 378 children. Successful sedation was obtained in 321 of 378 patients (85%) after a single rectal administration of thiamylal. Totally, 369 children (98%) could undergo MRI examination completely under successful sedation. Adverse effect was observed only in one patient showing respiratory depression. Rectal thiamylal is effective for sedation for MRI in children. Adverse effect was rare in our patients. Although the risk of side effect was considered to be rare, we should follow principles for the sedation of children.

The effects of co-medications on lamotrigine clearance in Japanese children with epilepsy.

PURPOSE: Although it has been reported that some antiepileptic drugs have inducing or inhibiting effects on lamotrigine (LTG) clearance, whether they have the same effects in Asian epilepsy patients as in those in other countries has not been clarified, especially in children. The aim of this study was to determine the effects of co-medications on LTG clearance in Japanese children with epilepsy. METHODS: A total of 342 routine serum concentration measurements of LTG in 102 Japanese epilepsy patients under 20years of age were reviewed. The dose-corrected concentration (DCC) of LTG was calculated as [concentration]/[dose/(body weight)], and the DCC of LTG was compared by co-medication. The difference in the DCC of LTG was compared between patients with and without valproic acid (VPA) and between those with and without drugs inducing glucuronic acid conjugation (phenytoin (PHT), carbamazepine (CBZ), and phenobarbital (PB)). RESULTS: The DCC of LTG was significantly higher in patients on VPA and significantly lower in patients on drugs inducing glucuronic acid conjugation than in patients on LTG monotherapy. The DCC of LTG was significantly higher in patients on CBZ than in patients on PHT or PB. There was no correlation between the DCC of LTG and the concentration of VPA or metabolic inducers within the therapeutic range. Other antiepileptic drugs including clobazam, clonazepam, zonisamide, and levetiracetam had little effect on LTG concentration. CONCLUSION: LTG concentration changes dramatically with concomitant antiepileptic drugs in Japanese children, as previously reported from other countries, and special attention is required. Although the dose of LTG should be adjusted when starting or discontinuing VPA or metabolic inducers, no adjustment is needed when changing the dose of VPA or metabolic inducers in the therapeutic range.

Popliteal angle in preterm infants with periventricular leukomalacia.

The aim of this study is to clarify the usefulness of popliteal angle in infants with periventricular leukomalacia. The popliteal angle was measured at 1, 4, 8, and 12 months of corrected age in 47 infants with periventricular leukomalacia and in 103 control infants with normal development. The popliteal angle was categorized into two groups; tight, when it was < or =120 degrees , and wide, when it was >120 degrees . The severity of diplegia was defined at 2 years as follows: mild, when an infant could walk; moderate, when an infant could sit but could not walk; severe, when an infant could not sit. Tight popliteal angle was more often observed in infants with periventricular leukomalacia than in normal infants at any corrected age. The rate of tight popliteal angle was significantly lower in infants with mild diplegia than in those with moderate or severe diplegia at 8 and 12 months of corrected age. Specificity and positive predictive value were high at 8 and 12 months of corrected age, whereas sensitivity was relatively low. The results of this study suggest that evaluation of popliteal angle is useful for detection of infants with periventricular leukomalacia, although false-negative rate is high during late infancy.

[Subacute encephalitis/encephalopathy with residual cognitive deficit].

We studied clinical features of 5 patients with subacute encephalitis/encephalopathy with residual cognitive deficit. In all patients, impairment of consciousness was mild at the onset, progressed between 4 and 15 days after the onset, and reached its peak at 6-18 days after the onset. Neuroradiological or electrophysiological examinations at the onset showed normal or mildly abnormal findings. In accordance with the clinical deterioration, brain atrophy was detected on cranial magetic resonance imaging (MRI) in 3 patients, and electroencephalogram revealed slowing of the background activities in all patients. Single photon emission computed tomography (SPECT) showed hypoperfusion in the fronto-temporal areas in all patients at the recovery stage. Three patients had severe mental deficits, and the other two had mild cognitive deficit. Motor impairment was observed in one patient.

Immediate suppression of seizure clusters by corticosteroids in PCDH19 female epilepsy.

PURPOSE: The pathomechanism and treatment of PCDH19 female epilepsy (PCDH19-FE) remain unclear. Here, we report that corticosteroids are effective for control of the seizure clusters or other acute symptoms of PCDH19-FE and argue for the possible involvement of a compromised blood-brain barrier (BBB) in its pathogenesis. METHODS: The efficacy of corticosteroids was retrospectively reviewed in five Japanese patients with PCDH19-FE. The results of antibody assays against the N-methyl-d-aspartate-type glutamate receptor (abs-NR) in serum/cerebrospinal fluid were also compiled. RESULTS: Corticosteroid treatments significantly improved the acute symptoms, including seizure clusters, in all cases, most often immediately after the initial administration. However, the effect was transient, and some seizures recurred within a few weeks, especially in association with fever. Serum and/or cerebrospinal fluid abs-NR were detected in all patients. Target sequences of the detected antibodies were multiple, and the titers tended to decrease over time. In one patient, immunohistochemical analysis using rat hippocampal slices also revealed serum antibodies targeting an unknown epitope in neuronal cytoplasm. CONCLUSION: Our findings imply an involvement of inflammatory processes in the pathogenesis of PCDH19-FE and therapeutic utility for corticosteroids as an adjunctive option in acute treatment. PCDH19 is well expressed in brain microvascular endothelial cells and thus its impairment may cause BBB vulnerability, which may be ameliorated by corticosteroids. The abs-NR detected in our patients may not indicate an autoimmune pathomechanism, but may rather represent non-specific sensitization to degraded neuronal components entering the general circulation, the latter process facilitated by the BBB vulnerability.

Combination of neonatal electroencephalography and ultrasonography: sensitive means of early diagnosis of periventricular leukomalacia.

This study was performed to assess the predictive value of ultrasonography and electroencephalography (EEG) in order to identify infants with periventricular leukomalacia (PVL) during the early neonatal period, especially non-cystic cases, and to clarify the combination of ultrasonographic and EEG findings that are the most useful. We studied 288 eligible infants, whose gestational ages ranged between 27 and 32 weeks. PVL was observed in 49 infants (26 cystic PVL and 23 non-cystic PVL). On ultrasonography, 31 infants with PVL were detected on the basis of definite periventricular echodensity (PVE). Thirty-seven infants had at least one of equivocal or definite PVE or cystic changes, but the other 12 did not have any of them. The sensitivity and specificity were 0.76 and 0.81, respectively. In EEG findings, acute stage abnormalities (ASA) of grade II or more were recognized in 31 infants with PVL. The sensitivity and specificity were 0.63 and 0.91, respectively. Equivocal or definite chronic stage abnormalities (CSA) were seen in 43 infants, the sensitivity and specificity being 0.88 and 0.84. The sensitivity of CSA was higher than that of ASA, and the specificity of ASA was higher than that of CSA. When these EEG findings were combined, 45 infants with PVL were detected. The sensitivity, specificity, positive predictive value, and negative predictive value were 0.92, 0.77, 0.45, and 0.98, respectively. Moreover, ultrasonographic and EEG findings were combined, 46 out of the 49 infants with PVL were detected with a sensitivity of 0.94 and a specificity of 0.64. The results indicated that EEG may be suitable for detecting infants at risk for development of PVL on the basis of its high sensitivity, and ultrasonography may be useful for confirming the presence of PVL on the grounds of its high specificity. Appropriate use of these measurements will make an early diagnosis of infants with PVL possible, even in non-cystic cases.

Unconsciousness and delirious behavior in children with febrile seizures.

The aim of this study is to clarify the incidence and clinical features of prolonged unconsciousness and delirious behavior in children with febrile seizures. We studied 213 consecutive febrile seizures during 208 febrile episodes in 203 patients. The seizure manifestations, the duration of seizures, the duration of unconsciousness, and the presence or absence of delirious behavior were determined on the basis of interviews with the parents with the assistance of medical records. The duration of seizures was less than 5 minutes in 90.2% of the seizures. The duration of unconsciousness was less than 30 minutes in 93% of the seizures. Delirious behavior was observed in 2.0% of the patients. Delirious behavior appeared before febrile seizures, and its duration was not long. On multiple regression analysis, nongeneralized seizures, seizures of >/=5 minutes, and intravenous diazepam were demonstrated to be independently associated with prolonged unconsciousness. In conclusion, prolonged unconsciousness and delirious behavior are rare in children with febrile seizures. Careful diagnostic evaluation is necessary when a child with febrile seizures has associated prolonged unconsciousness or delirious behavior.

Popliteal angle of low birth weight infants during the first year of life.

The aim of this study is to clarify the evolutional changes of the popliteal angle in low birth weight infants during the first year of life. The popliteal angle was measured at 1, 4, 8, and 12 months of corrected age on routine physical examination of 204 appropriate-for-date infants who had achieved normal psychomotor development. The popliteal angle was visually determined and categorized into two groups; tight, when it was 120 degrees or less, and wide, when it was more than 120 degrees. The infants were divided into five groups according to their birth weights; Group E, birth weight or=2500 gm. At 4 months of corrected age, the rate of a tight popliteal angle was significantly higher in Groups E + V + L1 than in Groups L2 + N. A tight popliteal angle was rare in all groups at 8 or 12 months of age. The results of this study indicate that the physiologic muscle tone of lower extremities is higher in infants with birth weights of <2000 gm at 4 months of corrected age. One should carefully interpret a tight popliteal angle in low birth weight infants during early infancy. Serial assessment of the popliteal angle is necessary before judging that a low birth weight infant has spastic cerebral palsy.

[A case with frontal lobe epilepsy presenting with absence seizures as cardinal manifestation: ictal EEG findings].

We report here a 9-year-old boy presenting with absence and complex partial seizures. Absence seizures occurred several times a day, with sudden arrest of speech and gesture, alteration of consciousness, myoclonus of unilateral or bilateral angles of the mouth, occasional simple automatism and brisk recovery of consciousness. Complex partial seizures occurred once to three times a month with loss of consciousness, salivation, deviation of the head and eyes toward the left, elevation of upper limbs and tonic convulsion of the left upper and lower limbs. Interictal EEG showed right frontal pole-dominant high-voltage slow waves or spike-and-waves. Ictal simultaneous video-EEG recordings of absence seizures revealed a frontal dominant 3-3.5 Hz spike-wave burst lasting several seconds. A partial seizure never preceded the absence seizure. Transverse topographical analysis revealed that the first spike component of the spike-wave burst of absence seizure always showed phase reversal on the right anterior temporal electrode. The following ones, however, showed phase reversal on the left anterior temporal electrode. Ictal EEG of the complex partial seizure could not be detected because it rarely occurred. There was no abnormal finding on brain MRI. Interictal single photon emission tomography (SPECT) indicated hypoperfusion of the dorsal and medial cortex of the right middle frontal lobe. Interictal positron emission tomography (PET) also indicated hypometabolic areas in the dorsal and medial cortex of the right frontal lobe, together with those in the right temporal and parietal cortex. EEG evolution and neuroimaging studies suggested that the epileptic focus of the absence seizure might have originated at the dorsal cortex of the right middle frontal lobe and immediately spread to the medial cortex. Both the seizures were well controlled by the combination of phenytoin and high dose sodium valproate.

Characteristics of epilepsy occurring in the first four months.

INTRODUCTION: Epilepsies with an onset during the early infantile period are relatively rare and their characteristics are not well recognized. The aim of this study was to determine the clinical characteristics of epilepsies with an onset during the early infantile period. METHODS: Clinical information on 73 patients with the onset of epilepsy within the first four months was collected from hospitals affiliated with Nagoya University. Patients were categorized into three groups: the idiopathic (20 patients), cryptogenic (19 patients), and symptomatic groups (34 patients). RESULTS: Fourteen (70%) of the 20 patients in the idiopathic group, nine (47%) of the 19 patients in the cryptogenic group, and 10 (29%) of the 34 patients in the symptomatic group had their first seizure within the first month of life. All patients in the idiopathic group, 12 patients (63%) in the cryptogenic group, and 18 patients (53%) in the symptomatic group had partial seizures (PS) alone throughout their clinical course. Four patients in the cryptogenic group and nine in the symptomatic group had PS at the onset, but evolved into spasms later. All patients in the idiopathic group, 13 patients (68%) in the cryptogenic group, and 13 patients (38%) in symptomatic group had experienced no seizures for at least one year at the time of the last follow-up. CONCLUSIONS: In patients with non-idiopathic epilepsy, an age-dependent evolution of seizure types was often observed. Recognition of this subgroup of patients could be important for the identification of appropriate candidates for early epilepsy surgery.