Clinical experience with open-label topiramate use in infants younger than 2 years of age

To assess the efficacy, safety, and tolerability of topiramate in infants younger than 24 months of age, we conducted an open-label, multicenter chart review study of infants who received topiramate. Twenty-eight patients were evaluated. All had refractory epilepsy. The mean age of seizure onset was 3.8 months (range 0-10 months). Refractory infantile spasms were the most common epilepsy syndrome. Among infants without infantile spasms, complex partial seizures were the prominent seizure type in eight, followed by simple partial seizures in six. Topiramate was prescribed as add-on therapy in 25 cases and a s monotherapy in 3 cases. Seven of the eight infantile spasms cases improved on topiramate therapy, attaining topiramate monotherapy in three infants. Half of the infants with other seizure types responded to topiramate. The average treatment duration among topiramate responders was 11 months. Topiramate was prescribed after a mean of 3.3 antiepilepsy drugs had been used in these infants. In no case was topiramate the first prescribed antiepilepsy drug. Adverse effects occurred only in five patients, leading to topiramate discontinuation in two patients. Topiramate was efficacious and well tolerated in infants younger than 24 months of age with refractory epilepsy. Prospective data are needed to corroborate this observation.     

Efficacy and tolerability of topiramate in children younger than 2 years old

To evaluate the efficacy and tolerability of topiramate in children with epilepsy younger than 2 years of age, we retrospectively reviewed the records of patients treated at our institution between 2001 and 2003. Thirteen children ages 5 to 23 months, five boys and eight girls, were identified. Seizure types were partial (five), generalized tonic-clonic (three), myoclonic (one), and infantile spasms (four). The mean age at seizure onset was 9.7 months. Topiramate was started at a mean age of 11.4 months (4-23 months). The number of antiepileptic drugs prior to topiramate therapy ranged from zero to four. One patient had been on the ketogenic diet. Topiramate was used as monotherapy in seven children and as polytherapy in six children. Mean follow-up was 14 months. The mean dose of topiramate was 8.8 mg/kg/day (2.5-18 mg/kg/day). The degree of seizure reduction was as follows: > 75% in five (38.5%) children, 50% to 75% in three (23%) children, and 0 to 25% in five (38.5%) children. Three of four (75%) patients with infantile spasms had a > 75% reduction in seizures. Adverse effects occurred in two children, including lethargy, hyperthermia, and anorexia. In children younger than 2 years of age, for whom the antiepileptic drug armamentarium is limited, topiramate appears to be an efficacious and safe therapeutic alternative for a variety of seizure types.     

Treatment of infantile spasms with zonisamide.

We determined the efficacy of and tolerability to zonisamide (ZNS) in newly diagnosed patients with infantile spasms. ZNS, 4-20 mg/kg per day, was introduced as an add-on therapy or monotherapy in 27 children with infantile spasms (cryptogenic, 2; symptomatic, 25). The dosage was initially 2-4 mg/kg per day, and then was increased by 2-5 mg/kg every 2-4 days until the seizures disappeared. Nine (33.3%) out of the 27 patients who were administered ZNS exhibited the disappearance of seizures. ZNS was effective in all the cryptogenic cases and seven (28.0%) of the symptomatic cases. The effective daily doses were 5-12.5 mg/kg (mean, 7.8 mg/kg), and the daily dosages were 40-100 mg (mean, 61.1 mg). The steady-state plasma ZNS concentrations were almost within the therapeutic range. The mean time interval between the start of ZNS therapy and the seizure disappearance was 5.0 days. Six (75.0%) of eight effective cases, the exception being one for whom EEG was not performed after the therapy, showed the disappearance of hypsarrhythmia. The recurrence of seizures were observed in four of the nine cases. No adverse reaction to ZNS was noted in any patient. In conclusion, ZNS treatment is considered to be worthwhile trying, for early stage therapy for infantile spasms.     

Therapeutic efficacy of ACTH in symptomatic infantile spasms with hypsarrhythmia

The records of twenty-six infants with both symptomatic infantile spasms and classic hypsarrhythmia were reviewed to determine the efficacy of various ACTH dosages and time of initiation of therapy. Mean age of infantile spasm onset was 6.4 months. Most patients (13) had sustained perinatal hypoxic-ischemic insults. Seventeen patients (65%) had complete cessation of spasms. Between these responders and the 9 nonresponders there was no difference in duration of spasms prior to treatment (2.6 and 2.0 months) or mean ACTH dose (87.4 and 84.5 U/m², respectively). Infants treated with high-dose ACTH (> 100 U/m²) did not have an improved response rate. The most favorable outcomes were associated with spasm onset at > 8 months of age (all of whom were responders, regardless of dose) or when treatment was started within 1 month of onset of infantile spasms with > 80 U/m² ACTH (88% responders). Infants treated more than 2 months after onset often did not respond (57%) regardless of dose. Nonresponders with spasm onset at < 4 months of age had the worst prognoses; all had poorly controlled seizures and regressed developmentally. Although all infants in the study were neurologically abnormal, development either improved or did not deteriorate in most responder infants following spasm resolution and one-half remained seizure free. Nonresponder infants continued to have infantile spasms or other seizure types. These data suggest that ACTH is valuable in the treatment of significantly impaired infants with symptomatic infantile spasms, but the most important determinants of outcome may be age of onset and rapidity of treatment rather than dosage.     

Double-blind substitution of vigabatrin and valproate in carbamazepine-resistant partial epilepsy. 012 Study group

Patients from 12 countries reporting two or more partial seizures per month despite treatment with optimal doses of CBZ were randomised to additional vigabatrin (VGB, 2-4 g daily) or sodium valproate (VPA, 1-2 g daily) using a double-blind, double-dummy design. The study included a 6 month retrospective baseline on unchanged CBZ dosage, a month's prospective baseline, a short titration phase, and an assessment period lasting 3 months on duotherapy. CBZ was withdrawn over a further 2 months in responders ( > or = 50% monthly seizure reduction compared with baseline), who continued on alternative monotherapy for 3 or more months. If seizure control deteriorated, CBZ was reinstated and these patients were also followed up for 3 months. A total of 215 patients (108 VGB, 107 VPA) reporting a mean of seven partial seizures per month fulfilled the criteria for the intention-to-treat analysis. 53 and 51% of patients in the VGB and VPA group respectively achieved a monthly reduction in seizure numbers > or = 50%, respectively. 27 and 31% maintained alternative monotherapy. Overall, 17% (7% monotherapy, 10% duotherapy) of the VGB treated patients and 19% (8% monotherapy, 11% duotherapy) of the VPA group remained seizure-free during the final 3 month treatment period. VGB and VPA, which increase neuronal inhibition mediated by gamma aminobutyric acid, can be added to or substituted for CBZ when this Na+ channel blocker fails to control partial seizures. This lends credence to the hypothesis in support of a mechanistic approach to the management of epilepsy.     

Lamotrigine in add-on therapy: assessment of efficacy in drug resistant epilepsy

The authors presented the results of treatment with lamotrigine (LTG, Lamictal) in 13 patients with drug resistant epilepsy (add-on therapy). There were 8f, 5m. aged 16-60 years, mean age 28.8 years. Generalized seizures occurred in 8 patients (62%). In this group there was 1 patient (aged 16 years) with the Lennox-Gastaut syndrome and 1 patient (aged 20 years) with valproate resistant juvenile myoclonic epilepsy. Complex partial seizures and complex partial with secondary generalization occurred in 5 patients (38%). Before LTG addition mean seizure frequency was from 3/month to several times/day. The mean duration of epilepsy was 16.6 years. The 8 patients were treated with CBZ and VPA, one with PHT and VPA, one CBZ and VGB. Monotherapy with VPA was introduced in 3 patients. After 6 months of treatment with LTG the efficacy was evaluated. 12 patients took LTG with VPA, 1 LTG with CBZ. Complete reduction of seizures was achieved in 3 cases (23%), at least 50% reduction in 3 patients (23%), reduction below 50% in 4 patients (31%). In 3 cases (23%) the results of treatment were negative (increase or no change in seizure frequency). Beneficial psychotropic effect was observed in 9 patients (69%). Adverse effects occurred in 2 patients (15%). Headache, vertigo, sleepness were observed in one case. Rash occurred in 1 patient (treated with LTG and VPA). After 6 months 3 patients were excluded from the study because of negative effects of treatment. LTG is helpful and well tolerated in drug-resistant epilepsy.     

Clinical Effects of Thyrotropin-Releasing Hormone for Severe Epilepsy in Childhood: A Comparative Study with ACTH Therapy

We investigated the effects and side effects of thyrotropin-releasing hormone (TRH) on severely epileptic children to evaluate the clinical usefulness of TRH in the treatment of epilepsy and compared them with the results of ACTH therapy. The subjects were 64 patients admitted consecutively between 1980 and 1986. Their seizures were frequent, more than one a day or more than one a week. The subjects were divided into two groups; 33 patients treated with ACTH and 31 treated with TRH. The mean follow-up periods in TRH and ACTH therapy were 8 months and 3.0 years, respectively. The daily dose of TRH-t 0.5-1 mg was administered intravenously (i.v.) or intramuscularly (i.m.) for 1-4 weeks. The follow-up periods were 3-12 months (mean 6 months). In the TRH group, complete control of seizures was achieved in 7 of 13 (53.7%) of those with infantile spasms, and marked improvement of EEGs were observed in 8 of 13 (61.5%) of them. In the ACTH group, seizure cessation was observed in 75% of infantile spasms. Of the patients who received ACTH, 66.7% had various side effects, including pneumonia, huge subcutaneous abscess, hypokalemia, cataracts, and brain shrinkage as shown on computed tomography (CT), whereas only 16.7% of the patients treated with TRH had transient reduction of urine volume without other laboratory and physical abnormalities. The results of the study indicated that some patients who received TRH had cessation of infantile spasms and improved EEG findings with no serious side effect. Because of the untoward side effects of ACTH therapy, TRH is considered a possible new treatment for children with infantile spasms.     

Historic, clinical, and prognostic features of epileptic encephalopathies caused by CDKL5 mutations

Mutations within the X-linked cyclin-dependent kinase-like 5 (CDKL5) gene are important causes of early-onset epileptic encephalopathies. We sought to determine the historic, clinical, and prognostic features of epilepsy secondary to CDKL5 mutations. We performed retrospective chart reviews of children at our institution with epilepsy and CDKL5 mutations. Six children were identified. One manifested a deletion in exons 10-15 of the CDKL5 gene, another manifested a single base-pair duplication in exon 3, and the rest manifested base-pair exchanges. The mean age of seizure onset was 1.8 months (range, 1-3 months). Although the majority (4/6, 67%) presented with partial-onset seizures, all children developed infantile spasms. All children demonstrated developmental delay and visual impairment. Although such mutations are X-linked, two children were boys. They did not present with more severe phenotypes than their female counterparts. Despite trials of antiepileptic drugs (mean, 5; range, 3-7), steroids/adrenocorticotropic hormone (4/6; 67%), and the ketogenic diet (6/6; 100%), all children manifested refractory seizures at last follow-up. Although no treatment eliminated seizures, topiramate, vigabatrin, and the ketogenic diet were most helpful at reducing seizure frequency.     

ACTH Therapy for Infantile Spasms: A Combination Therapy with High-Dose Pyridoxal Phosphate and Low-Dose ACTH

Summary: Combination therapy consisting of high-dose pyridoxal phosphate (40–50 m/kg/day) and low-dose synthetic ACTH (0.01 mg/kg/day) was prescribed in 28 children with infantile spasms. Monotherapy with pyridoxal phosphate provided excellent seizure control in 3 of the 28 (11%) patients. ACTH was subsequently added to the regimen of the remaining 25 patients. As of 1 month after discontinuing the ACTH treatment, 21 of the 25 (84%) patients had experienced no seizures. The mean interval until seizure control was achieved was 4.1 days after the start of treatment with ACTH. The 21 patients have been monitored for a mean of 34.9 months (range 2–81 months); 6 patients (29%) have had recurrences of infantile spasms, and 10 (48%) have experienced normal development. Fourteen of the 28 patients (50%) have had transient increases in liver enzymes, but none of the patients developed more serious side effects.     

Valproate Metabolites in Serum and Urine During Antiepileptic Therapy in Children with Infantile Spasms: Abnormal Metabolite Pattern Associated with Reversible Hepatotoxicity

The purpose of this study was to identify abnormal metabolite patterns of valproate (VPA) as possible early indicators of VPA-induced liver toxicity. In a prospective study, we determined serum and urine levels of VPA metabolites by gas chromatography-mass spectrometry (GC-MS) during the course of therapy in 25 children treated for infantile spasms with high VPA doses (less than or equal to 100 mg/kg body weight/day). Most patients had similar metabolite profiles: The main metabolites in serum were the beta-oxidation products (2-en-VPA and 3-keto-VPA) and the major diunsaturated metabolite 2,3'-dien-VPA. Glucuronide conjugates and the oxidation products represent the most abundant metabolites in urine. Other metabolites, including the potential hepatotoxin 4-en-VPA, were detected only in low concentrations. Two children had transiently aberrant metabolite profiles, indicating altered beta-oxidation, (levels of 2-en-VPA, 2,3'-dien-VPA, and 3-en-VPA were markedly increased) in connection with hepatomegaly and increased liver enzyme activities at a time when both had febrile infections and were receiving dexamethasone comedication. At no time were increased levels of 4-en-VPA or its derivatives detected. Establishing the VPA metabolite profile may aid in evaluation of patients who show signs and symptoms of liver dysfunction during VPA therapy. The present study shows that initial stages of hepatotoxicity reactions to VPA may be accompanied by characteristic changes in VPA metabolism; early detection of such abnormal metabolite patterns might decrease the risk of severe hepatic injury.     

The role of vigabatrin in childhood seizure disorders: results from a clinical audit

PURPOSE: The emergence of visual field defects attributed to vigabatrin (VGB) treatment and intramyelinic edema in animal experiments has raised concerns about its future role in the treatment of childhood seizures. METHODS: We evaluated our experience with this antiepileptic agent with retrospective analysis of database and chart audit. RESULTS: Of 73 patients, 43 girls and 33 boys were treated with VGB over a 7-year period. The mean age of patients at the introduction of VGB was 87 months (range, 5-257 months). In 12 of 73 cases, VGB was used as monotherapy; in 61 of 73 cases, it was used as an add-on drug. Seizure types included secondarily generalized seizures (21), mixed seizures (21), partial seizures (18), and generalized seizures (13). Seizure etiology was idiopathic/cryptogenic in 22 patients, symptomatic in 50, and undetermined in a single patient. The mean duration of therapy was 16 months (median, 10 months; range, 1-144 months). VGB was effective in 30 (seven seizure free, 23 with >90% reduction in seizures), partially effective in four (50-90% reduction in seizures), and ineffective in 38 (<50% reduction in seizures). Nearly 50% of patients with infantile spasms responded to VGB. All patients underwent ophthalmic evaluation; two (16%) of 12 patients who could undergo static threshold perimetry were demonstrated to have the characteristic visual field constriction. CONCLUSIONS: VGB is effective in producing a significant reduction in seizure frequency in nearly half the patients with childhood seizures, including refractory epilepsy. Despite emerging concerns regarding visual side effects, this drug retains an important role in the medical management of childhood epilepsy.     

Intravenous Methylprednisolone for Intractable Childhood Epilepsy

BackgroundSteroids have been used for the treatment of certain epilepsy types, such as infantile spasms; however, the use in the treatment of other intractable epilepsies has received limited study. We report our experience with intravenous methylprednisolone in children with epilepsy refractory to multiple antiepileptic drugs.MethodsA series of consecutive children were analyzed retrospectively. Patients with infantile spasms, progressive degenerative, or metabolic disorders were excluded.ResultsSeventeen children aged 2-14 (mean 5.3) years were included. Associated cognitive and motor deficits were recognized in 82%. Most children (88%) had daily seizures and 13 (76%) were admitted previously with status epilepticus. The epilepsy was cryptogenic (unknown etiology) in 47% and the seizures were mixed in 41%. Intravenous methylprednisolone was given at 15 mg/kg per day followed by a weaning dose of oral prednisolone for 2-8 weeks (mean 3 weeks). Children were followed for 6-24 months (mean 18). Six (35%) children became completely seizure free; however, three of them later developed recurrent seizures. At 6 months posttreatment, improved seizure control was noted in 10 (59%) children. Children with mixed seizures were more likely to have a favorable response than those with one seizure type (49% vs 31%, P = 0.02). No major side effects were noted, and 35% of the parents reported improvements in their child's alertness and appetite.ConclusionAdd-on steroid treatment for children with intractable epilepsy is safe and may be effective in some children when used in a short course.     

Long-term outcome of symptomatic infantile spasms established by video-electroencephalography (EEG) monitoring

In this study, we examine the long-term clinical outcome of children with symptomatic infantile spasm. The children between 2 and 18 years of age diagnosed with symptomatic infantile spasms were reviewed. Sixty-eight children (age range, 2-13 years; mean, 4.5 years) met the inclusion criteria. Children who underwent epilepsy surgery were excluded. Age of onset for infantile spasms ranged from 1 to 24 months (mean, 7.1 months). Developmental delay was noted in all; there was seizure freedom in 14 children (20.5%). Infantile spasms were reported as the only seizure type in 10 (14.5%) children older than age 2 years. During the follow-up; symptomatic generalized epilepsy was diagnosed in 23 children (34%) and focal epilepsy in 21 (31%). The long-term outcome of these children remains unchanged in the majority of the children with symptomatic infantile spasms. We could not establish any risk factor that might be related to favorable or adverse outcome.     

Vigabatrin as add-on therapy in children and adolescents with refractory epilepsy: an open trial

Seventy-seven children and adolescents with drug-resistant epilepsies received vigabatrin as add-on therapy for a median of 18 months (range 4–36 months) at a dose of 50 mg/kg/day divided in two doses; patients with spasms were given a maximum dose of 100 mg/kg/day. In 23 patients (29.9%), seizure frequency decreased by 50–100% and in 12 patients (15.6%) by 25–50%. The number of seizures remained unchanged in 34 patients (44.1%) and increased in seven (9.1%). Vigabatrin was most effective in cryptogenic and symptomatic partial seizures (39% and 43%, respectively), and in infantile spasms (25%). Adverse events occurred in 20 patients (26%), though they were generally mild and transient, suggesting that vigabatrin is well tolerated.     

The efficacy and side effects of topiramate on refractory epilepsy in infants and young children: a multi-center clinical trial.

OBJECTIVES: This study has been conducted to assess the efficacy and safety of topiramate in refractory epilepsies in infants and young children. METHODS: A prospective clinical trial was performed in three tertiary care hospitals, on 47 children aged 6-60 months with refractory epilepsy. Topiramate was added to at least two baseline anti-epileptic drugs. The efficacy was rated according to seizure type, frequency and duration. RESULTS: Children with refractory epilepsy were classified according to their clinical, neuro-imaging, and neurophysiological profile into infantile spasms (IS) (9 cases, 19%), Lennox-Gastaut syndrome (LGS) (25 cases, 53%) and other epilepsies (13 cases, 28%). Children were also classified into cryptogenic and symptomatic epilepsy. Topiramate was introduced as add-on therapy in a daily dose of 1 mg/kg/day for 2 weeks, followed by increments of 1-3 mg/kg/day at 2-week intervals, up to a maximum of 10 mg/kg/day. After a minimum treatment period of 6 months, 28 (60%) of the children had a satisfactory response (completely seizure free, or more than a 50% seizure reduction). The remaining 19 children (40%) had an unsatisfactory response (50% or less reduction in seizure frequency, no change or increased seizure frequency). Topiramate appeared to be equally effective in infantile spasms, Lennox-Gastaut syndrome and children with other types of epilepsy, with no significant difference between those with a satisfactory and an unsatisfactory response (p=0.089). There was also no significant difference in response between patients with cryptogenic and symptomatic epilepsy (p=0.360). Mild to moderate adverse effects, mainly somnolence, anorexia and nervousness, were present in 25 (53%) of children. One of the children developed hypothyroidism. CONCLUSION: Although the long term safety and possible adverse effects of topiramate have not been fully established in infants and young children, this study has shown that it is a useful option for children with frequent seizures unresponsive to standard anti-epileptic drugs.     

EEG and neuroimaging studies in young children having epilepsy surgery

The aim of this study was to evaluate the yield of electroencephalography and structural and functional neuroimaging in children having resective epilepsy surgery before 5 years of age. Charts of all 28 children (54% male) having resective surgery before 60 months of age at the Mayo Clinic between January 2002 and June 2009 were reviewed. Mean age at seizure onset was 9.6 months (S.D. 12.7); mean age at surgery was 28.8 months (S.D. 17.7). Sixteen children (57%) had partial-onset seizures, 8 (29%) had partial-onset seizures and spasms, and 4 (14%) had spasms alone. Initial surgery type was hemispherectomy in 6 cases, multilobar resection in 8, temporal in 7, and extratemporal in 7. Only 10 of the 25 children (40%) with recorded seizures preoperatively had a well-localized, single ictal focus. Ictal discharge was generalized in 8/25 cases (32%), both generalized and focal in 1 case (4%), hemispheric in 4 cases (16%), and absent in 1 case (4%). Findings from magnetic resonance imaging were abnormal in 27 cases, and revealed focal pathology in 20. Surgical outcome was favorable, with 18 of the 27 survivors (67%) being free, or nearly free, of disabling seizures. In?summary, electroencephalography frequently failed to indicate a single ictal focus in young children having epilepsy surgery. In contrast, magnetic resonance imaging was more helpful, revealing focal abnormalities in 74% of patients.     

Ganaxolone for treating intractable infantile spasms: a multicenter, open-label, add-on trial

This is a multicenter, open-label, add-on trial, investigating the safety and efficacy of ganaxolone (GNX) in a population of children with refractory infantile spasms, or with continuing seizures after a prior history of infantile spasms. A total of 20 children aged 7 months to 7 years were enrolled in this dose-escalation study, after baseline seizure frequencies were established. Concomitant antiepilepsy drugs were maintained throughout the study period. The dose of GNX was progressively increased to 36 mg/kg/d (or to the maximally tolerated dose) over a period of 4 weeks, then maintained for 8 weeks before tapering and discontinuation. Seizure diaries were maintained by the families, and spasm frequency was compared with the baseline period. The occurrence of adverse events was clinically monitored, and global evaluations of seizure severity and response to treatment were obtained. A total of 16 of the 20 subjects completed the study, 15 of whom had refractory infantile spasms at the time of study enrollment. Spasm frequency was reduced by at least 50% in 33% of these subjects, with an additional 33% experiencing some improvement (25-50% reduction in spasm frequency). Ganaxolone was well tolerated, and adverse events attributed to GNX were generally mild. Ganaxolone was safe and effective in treating this group of refractory infantile spasms patients in an open-label, add-on trial. Further investigation with randomized, controlled study design is warranted.     

Efficacy and tolerability of adjunctive therapy with zonisamide in childhood intractable epilepsy

Purpose: This study investigated the efficacy and safety of zonisamide (ZNS) adjunctive therapy in children with intractable epilepsy to existing antiepileptic drugs (AEDs). Methods: A clinical retrospective study was performed from 2003 to 2005 at two tertiary epilepsy centers. We reviewed the data from 163 children (107 boys and 56 girls) who experienced more than four seizures per month, whose seizures were intractable to an initial 2 or more AEDs, and could be followed up for at least 6 months after ZNS adjunctive therapy initiation. Efficacy was estimated by seizure reduction rate according to seizure types including infantile spasms, and adverse events were also measured. Results: Seventy-nine patients (48.5%) out of 163 patients experienced a reduction in seizure frequency of more than 50%, and 25 patients (15.3%) became seizure-free. The rate of seizure reduction greater than 50% in children with partial seizures was 40.5% (17/42) and in children with generalized seizures was 51.2% (62/121). Of 36 patients who manifested mainly myoclonic seizures, 20 patients (55.6%) showed a seizure reduction of more than 50% and 9 patients (25.0%) were seizure-free. Mean maintenance dosage of drug was 8.2 mg/kg/day (range 5.0-16.0 mg/kg/day). Adverse events were documented in 15 children (9.2%), including somnolence (8 patients), fatigue, and anorexia, but all were transient and successfully managed. One patient discontinued ZNS therapy due to acute pancreatitis. Conclusion: ZNS adjunctive therapy is an effective and safe treatment in various childhood intractable epilepsy.     

Epilepsy in children with delayed presentation of perinatal stroke

A subgroup of children with perinatal stroke do not present clinically until after the perinatal period. Detailed epilepsy outcomes in these children have not been well studied. A retrospective cohort study of 45 children with delayed presentation of perinatal stroke identified by review of pediatric stroke clinic records, physician referral, and International Classification of Diseases, Ninth edition, code searches of hospital records, was performed at a tertiary pediatric hospital in Indianapolis, Indiana. A modified version of the Engel scale was used to grade epilepsy outcomes. The chi(2) test, Fisher's exact test, and relative risks were calculated to examine the association of epilepsy at time of last follow-up with initial presentation with seizures, infantile spasms, radiographic findings, and initial abnormal electroencephalogram (EEG). These tests were also used to examine the association of epilepsy with cognitive or motor disability and the association of initial abnormal EEG with motor disability. Patients presented with hemiparesis (40; 89%), seizures (4; 9%), or headaches (1; 2%). All had unilateral infarcts on cranial imaging. Four children (9%) had infantile spasms, 2 at presentation and 2 later. Nineteen children received at least 1 EEG for suspicious spells or frank seizures; initial EEG was abnormal in 16 patients (84%). At last follow-up, 17 patients (38%) had epilepsy, which was severe in 4 (24% of those with epilepsy). Initial presentation with seizures (relative risk = 3.2; 95% confidence interval, 2.0-4.9) and infantile spasms (relative risk = 3.2; confidence interval, 2.0-4.9) were associated with epilepsy at last follow-up. Infantile spasms were also associated with moderate-to-severe epilepsy at last follow-up (relative risk = 10.3; confidence interval, 1.9-54.4). Epilepsy at last follow-up was associated with cognitive disability (P = .05). Initial abnormal EEG was not associated with cerebral palsy (P = .30). Epilepsy is frequent in children with delayed presentation of perinatal stroke and is associated with initial presentation with seizures and infantile spasms at any point in time. Cognitive disability often accompanies epilepsy in these children.     

West syndrome: a university hospital based study from Oman.

Forty-four children (20 male: 24 female) with West syndrome (infantile spasms, mental retardation/regression and hypsarrhythmia) diagnosed at Sultan Qaboos University Hospital (Pediatric Neurology Division of the Department of Child Health) are reported, with thirty-four (77.3%) children constituting the symptomatic group. All children were followed up for an initial 1 year at this hospital. Thirty-seven cases (84%) still continue their follow-up with us. The age of onset ranged from 1 to 14 months (mean, 6.0 months). Developmental delay before the onset of infantile spasms was noted in 29 (65.9%) children. Brain computed tomography was abnormal in 29 (65.9%). Sodium valproate and vigabatrin were the most often used drugs, though other antiepileptic drugs were also used. Nine (24.5%) children achieved good seizure control, out of which five have normal development. Only one child could be weaned off antiepileptic drugs completely. There was one death in the whole series, related to aspiration pneumonia.