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At least 181 records · Page 10Linked to original sources
PubMed2006

Extended, one-stage callosal section for treatment of refractory secondarily generalized epilepsy in patients with Lennox-Gastaut and Lennox-like syndromes.

PURPOSE: We report on the efficacy and safety of extended one-stage callosal section performed in a large and homogeneous series of patients. METHODS: Seventy-six patients with Lennox-Gastaut (n = 28) and Lennox-like (n = 48) syndrome were studied (42 male patients; mean age, 11.2 years). All patients had multiple seizure types per day, including atonic, tonic-clonic, atypical absence, myoclonic, and tonic seizures. All of them were developmentally impaired. The EEG showed intense secondary bilateral synchrony in all of them. All patients were submitted to an extended, one-stage, callosal section, leaving only the splenium intact. Mean follow-up time was 4.7 years. RESULTS: Worthwhile improvement (>50%) was noted in 69 of 76 patients; 52 patients had a > or =90% reduction in seizure frequency. Seven patients were seizure free after surgery. The seizure patterns most responsive to surgery were atonic (92%), atypical absence (82%), and tonic-clonic (57%) seizures. All patients had some degree of a transient acute postoperative disconnection syndrome. A consistent increase in attention level was observed postoperatively. CONCLUSIONS: We report one of the larger homogeneous series of patients submitted to callosotomy and are the first to report on the effectiveness and safety of performing extended callosal section in a single stage in this patient group. Extended callosal section should be considered a good palliative surgical option for suitable candidates. The increase in attention level was as useful as seizure control in improving quality of life of these patients.

Attention↗
PubMed2005

[Frontotemporal dementia non familial and generalized epilepsy].

UNLABELLED: A 62 year-old patient is presented, without family antecedents of dementia who begins with 45 years of age with sudden crisis of dyspnea, blurred vision, fall to the floor and repetitive jerks of arms. Tried by epilepsy with phenytoin and valproate repeats similar crisis sporadically. Four years ago their relatives began to notice changes of personality, irritability and obsessive behaviors. Later on, are added episodes of disorientation of days of duration, some with auditory hallucinations and also convulsive manifestations. Finally appear polymorphic crisis, some with continue alteration of consciousness, catatonic states and sphincteric incontinence. Physical and neurological examination without abnormalities. Neuropsychological evaluation evidenced consistent defects in frontal functions. EEG showed widespread slowness and sporadic irritative activity in frontotemporal regions. Anterior cortical atrophy in CT scan and bilateral frontotemporal hypoperfusion in SPECT. Labs exams and CSF were normal. CONCLUSION: The association of FTD and epilepsy, in non-family form, suggests a different neurodegenerative cortical syndrome.

Atrophy↗
PubMedDate not supplied

[Generalized epilepsy with febrile seizures plus: clinical and genetic analysis of three Serbian families].

The results of clinical and genetic analysis of three Serbian families (pedigrees) with autosomal dominant inheritance, incomplete penetrance and phenotypic features of GEFS+ are presented in this study. Mutation analysis of the SCN1A, SCN1B and GABRG2 genes was performed in all affected and some unaffected members of these three families. Twenty-six exons of SCN1A, five exons of SCN1B and nine exons of GABRG2 were individually amplified using primers based on intronic sequence. PCR products were sequenced in both forward and reverse directions. Subsequently, the samples were run and analyzed using 377 DNA automated sequencer. No consanguinity was noticed. The MM and OM family members live in Republic of Srpska while KS family originates from the central Serbia. No mutations of the exons of SCN1A, SCN1B and GABRG2 genes were found in tested subjects. Obligate carriers in MM family (III-1, III-2, and III-4) exhibit variable expressivity or incomplete penetrance rather than proof of polygenetic inheritance. OM pedigree follows autosomal dominant pattern despite reduced penetrance. Bilinear transmission may assume the possibility of multigenetic mode of inheritance in KS family. The fact that all affected members in three Serbian families were negative for mutations in SCN1A, SCN1B and GABRG2 genes strongly supports the hypothesis of significant genetic heterogeneity of GEFS+. Recognizing GEFS+ on clinical grounds contributes to more precise integration of this syndrome into already existing classification of epileptic syndromes.

Adolescent↗