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PurposeTo establish the efficacy and safety of methylphenidate (MPH) treatment for attention deficit hyperactivity disorder (ADHD) in a group of children and young people with learning disability and severe epilepsy.MethodsThis retrospective study systematically reviewed the case notes of all patients treated with methylphenidate (MPH) for Diagnostic and Statistical Manual of Mental Disorders, Fourth Edition (DSM-IV) ADHD at a specialist epilepsy center between 1998 and 2005. Treatment efficacy was ascertained using clinical global impressions (CGI) scores, and safety was indexed by instances of >25% increase in monthly seizure count within 3 months of starting MPH.Key FindingsEighteen (18) patients were identified with refractory epilepsies (14 generalized, 4 focal), IQ <70, and ADHD. Male patients predominated (13:5) and ADHD was diagnosed at a median age of 11.5 years (range 6–18 years). With use of a combination of a behavioral management program and MPH 0.3–1 mg/kg/day, ADHD symptoms improved in 61% of patients (11/18; type A intraclass correlation coefficient of CGI 0.85, 95% confidence interval [CI] 0.69–0.94). Daily MPH dose, epilepsy variables, and psychiatric comorbidity did not relate to treatment response across the sample. MPH adverse effects led to treatment cessation in three patients (dysphoria in two, anxiety in one). There was no statistical evidence for a deterioration of seizure control in this group with the use of MPH.SignificanceMethylphenidate with behavioral management was associated with benefit in the management of ADHD in more than half of a group of children with severe epilepsy and additional cognitive impairments. Eighteen percent had significant side effects but no attributable increase in seizures. Methylphenidate is useful in this group and is likely to be under employed.

SummaryObjectiveTo explore the structure–function relation of the temporal lobe in newly diagnosed West syndrome of unknown cause (uWS).MethodsQuantitative magnetic resonance imaging (three‐dimensional [3D] structural MRI and diffusion tensor imaging [DTI]) was analyzed using voxel‐based morphometry (VBM) and tract‐based spatial statistics (TBSS) in 22 patients and healthy age‐matched controls. The electrophysiologic responsiveness of the temporal lobe was measured using the N100 auditory event‐related potential (aERP) to a repeated 1,000 Hz tone. Neurocognitive function was assessed using the Bayley Scales of Infant Development, Second Edition (BSID‐II). Tests followed first‐line treatment with vigabatrin (17 patients) or high‐dose oral prednisolone (5 patients).ResultsTotal temporal lobe volume was similar in patients and controls. Patients had a smaller temporal stem (TS) (p < 0.0001) and planum temporale (PT) (p = 0.029) bilaterally. TS width asymmetry with a larger right‐sided width in controls was absent in patients (p = 0.033). PT asymmetry was present in both groups, being larger on the right (p = 0.048). VBM gray matter volume was increased at the left temporal lobe (superior and middle temporal gyri, the peri‐rhinal cortex, and medial temporal lobe) (p < 0.005, family wise error‐corrected). VBM gray matter volume correlated with the duration of infantile spasms (Pearson's r = −0.630, p = 0.009). DTI metrics did not differ between patients and controls on TBSS. Mean BSID‐II scores were lower (p < 0.001) and auditory N100 ERP attenuated less in patients than in controls (p = 0.002).SignificanceThe functional networking and white matter development of the temporal lobe are impaired following infantile spasms. Treatment may promote structural plasticity within the temporal lobe following infantile spasms, manifest as increased gray matter volume on VBM. It remains to be investigated further whether this predicts patients' long‐term cognitive difficulties.

ObjectiveThis study investigates auditory processing in infants with West syndrome (WS) using event-related potentials (ERPs).MethodsERPs were measured in 25 infants with mainly symptomatic WS (age range = 3–10 months) and 26 healthy term infants (age range = 3–9 months) using an auditory novelty oddball paradigm. The ERP recordings were made during wakefulness and repeated in stage II sleep.ResultsThe obligatory components (P150, N250, P350) and novelty response components (P300, Nc) were recordable during both sleep and wakefulness in patients and controls. All ERP latencies decreased with age in controls but not in the WS group (age × group interaction, F = 22.3, p < 0.0001). These ERP latency alterations were not affected by pharmacological treatment for WS.InterpretationThis study demonstrated a persistently altered ERP signature in patients with a recent history of infantile spasms. The prolongation of auditory obligatory and novelty ERPs in WS patients indicates a severe failure of temporal lobe maturation during infancy. It remains to be investigated whether this predicts long-term cognitive impairments characteristic for this epileptic encephalopathy.