Long-term safety and effectiveness of zonisamide in the treatment of epilepsy: a review of the literature

Zonisamide (ZNS) efficacy and safety in epilepsy have been demonstrated in four double-blind, placebo-controlled studies. In the present article, we examined all long-term studies performed with this drug. Nine open-label studies, in which ZNS had been administered as an add-on or as monotherapy to epileptic patients for at least 6 months, were selected for our analysis. Four outcome measures were searched. Retention of this drug after 1 year varied between 45% and 65%. The percentages of patients achieving a ≥50% seizure reduction, with respect to baseline, ranged between 37% and 65%. In patients with drug-resistant forms of epilepsy, the percentage of patients reaching a 6-month seizure freedom period was 9%. The percentages of patients who discontinued the experimental drug due to adverse effects ranged between 4% and 24%. Somnolence and dizziness were the most frequently reported adverse effects. Long-term studies demonstrate that ZNS has a good efficacy and tolerability profile, and support its use as adjunctive therapy for epileptic patients

Successful treatment of hypnic headache with topiramate: a case report.

Hypnic headache is a rare cause of benign headache in the elderly. This is a clinical report of a 67-year-old housewife, suffering from hypnic headache, in which topiramate yielded a successful prophylactic effect at 100 mg/da

A novel de novo SCN1A missense mutation in Severe Myoclonic Epilepsy Borderland

In this report we describe a novel missense SCN1A mutation in a patient affected by Severe Myoclonic Epilepsy Borderland (SMEB). This three and a half year-old female patient experienced prolonged febrile seizures at the age of 14 months, followed by generalized tonic-clonic seizures, atonic seizures, atypical absences almost in a cluster and triggered by fever. Cognitive and motor development was normal. The case was suggestive for SMEB. SCN1A analysis revealed an unknown de novo point mutation: a heterozygous replacement of nucleotide G with nucleotide T in position 4183 of the coding region of the gene (c.4183 G>T) in exon 21. This mutation causes the replacement of aspartic acid with tyrosine in 1395 (p.D1396Y). Even if other SCN1A missense mutations localized in the same region are associated to SMEB, a definite genotype-phenotype correlation has not yet been found, probably because other factors are involved in the pathogenesis of this type of epilepsy

Stress, mood disorders and memory in headache

Comorbidity between headache and other disorders such as psychological or memory problems is a topic of increasing scientific interest both for us diagnostic and therapeutic implications but also for pathogenetic advances. A central neurogenic mechanism such as a dysregulation of some neurotransmitter system might underlie not only headache but also other coexistent disorders; findings highlight the role of serotonin pathway

Electroencephalographic features in Dravet syndrome: five-year follow-up study in 22 patients

The aim of the study was to evaluate interictal electroencephalogram features in 22 patients with Dravet syndrome from the onset of the disease through the next 5 years. Electroencephalogram was abnormal in 5 patients (22.7%) at onset, and in 17 (77.3%) at the end of the study. Epileptiform abnormalities (focal, multifocal, or generalized) were seen in 6 patients at the onset and in 14 (27% vs 64%) at the end of the study. Photoparoxysmal response was present in 41% of patients at the end of follow-up. No statistical differences were found between mutated and nonmutated groups regarding evolution of background activity, interictal abnormalities, and presence of photoparoxysmal response. Electroencephalogram findings seemed to be age dependent, variable among different patients, and not influenced by the presence of sodium channel, voltage-gated, type I, alpha subunit (SCN1A) mutation. The lack of specific epileptiform abnormalities contributes to the difficulty of patients' management in Dravet syndrome

Diagnosing photosensitive epilepsy: Fancy new versus old fashioned techniques in patients with different epileptic syndromes

Purpose: To demonstrate the clinical importance of using a high quality photic stimulator for recording EEGs to diagnose photosensitivity. Methods: We performed EEG examinations on 2 adult and 2 paediatric patients with a history of visually induced seizures; routinely we used a Grass PS 40 photic stimulator (rectangular Xenon lamp giving flashes of 10 mu s duration, 0.7 J, 1-30 Hz, width 7 cm, length 12 cm). We repeated the IPS with a Grass PS 33 plus stimulator (round Xenon lamp giving flashes of 10 mu s duration, 1 J, 1-60 Hz, diameter 14 cm). Results: Patients were affected by both benign and catastrophic epilepsies. They complained about episodes of dizziness (case 1), dizziness accompanied by a sensation in the arms and fear (case 2), absences (case 3), and myoclonic jerks (case 4). These symptoms occurred when working with neon lights, computers or ironing striped clothes (case 1), while driving (case 2), whenever there was sunlight (case 3 and 4). Only IPS performed with the Grass PS 33 plus stimulator evoked PPRs accompanied by their typical complaints. In all cases, the revised diagnosis led to changes in their treatment and the disappearance or diminishment of their complaints and PPR range. Conclusion: A PPR can occur in various types of epilepsy, can have a different meaning, and requires a different therapeutic intervention. Only an appropriate photic stimulator with diffuse white light and a flash intensity level of 1 J/flash, can reliably demonstrate whether a patient is photosensitive, or equally important exclude it. (C) 2010 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved

Electroencephalographic features in dravet syndrome: five-year follow-up study in 22 patients

: The aim of the study was to evaluate interictal electroencephalogram features in 22 patients with Dravet syndrome from the onset of the disease through the next 5 years. Electroencephalogram was abnormal in 5 patients (22.7%) at onset, and in 17 (77.3%) at the end of the study. Epileptiform abnormalities (focal, multifocal, or generalized) were seen in 6 patients at the onset and in 14 (27% vs 64%) at the end of the study. Photoparoxysmal response was present in 41% of patients at the end of follow-up. No statistical differences were found between mutated and nonmutated groups regarding evolution of background activity, interictal abnormalities, and presence of photoparoxysmal response. Electroencephalogram findings seemed to be age dependent, variable among different patients, and not influenced by the presence of sodium channel, voltage-gated, type I, alpha subunit (SCN1A) mutation. The lack of specific epileptiform abnormalities contributes to the difficulty of patients' management in Dravet syndrome

Effetti indotti dall'apomorfina sui movimenti oculari di lento inseguimento in pazienti con corea di Huntington

Nei disturbi extrapiramidali è stata descritta una compromissione dei movimenti oculari di lento inseguimento. Lo studio ha valutato l'interferenza delle vie dopaminergiche sul sistema dei movimenti oculari di lento inseguimento, mediante la somministrazione di apomorfina, sostanza ad attività dopaminergica, in pazienti con corea di Huntington. In condizioni di base i movimenti di lento inseguimento sono apparsi compromessi per la presenza di movimenti erratici e correzioni con frequenti saccadici. La somministrazione di apomorfina ha indotto un peggioramento della capacità di produrre i movimenti di lenti inseguimento, nonostante un netto miglioramento della sintomatologia coreic

INSOMINA, QUALITY OF LIFE AND PSYCHOPATHOLOGICAL FEATURES

Most of the studies about chronic insomnia focused only on specific features, providing in this way a partial outlook of the problem. The aim of this study was to examine a sample of chronic insomniacs from different points of view, by estimating the prevalence of stress, psychopathological symptoms, psychiatric disorders, changes in quality of life and illness behaviour. Forty-three patients (54.4%) experienced some psychosocial stressors in the last 6 months preceding the enrollment in the study. More than 55% of insomniacs reported symptoms of psychopathology. One or more than one psychiatric disorder was diagnosed in 61.5% of insomniacs. Life events could play a role in facilitating the transformation of a psychopathological symptom in a psychiatric disorder, since patients with a psychiatric diagnosis showed a higher percent of stress. The more noticeable disability in the group of insomniacs with psychiatric disorders and the absence of a corresponding worsening in illness behaviour suggests that insomniacs feelings about their health status are so negative to be not fatherly worsen by comorbidity in spite of a worsen disability. The evaluation of insomniacs should be as much detailed as possible, without neglecting tests for the evaluation of disability and illness behaviour, which are important not only for a correct diagnosis but also for monitoring the effects of therapies