Biomedical subjects

Betül Baykan

Publications and source records attributed to Betül Baykan.

11 recordsLinked to original sources

PubMed2006

Autoimmune thyroid encephalopathy presenting with epilepsia partialis continua.

We report the first case of an autoimmune thyroid encephalopathy presenting with multifocal motor status epilepticus. A 37-year-old female patient was admitted with multifocal motor seizures intractable to intravenous status epilepticus treatments, asymmetrical quadriparesis, truncal ataxia and continuous semi-rhythmical jerks. Pathological signal alterations were detected in both precentral cortices in MRI examination. Autoimmune thyroiditis was diagnosed after radiological examinations of the thyroid gland and thyroid function tests. Seizures promptly ceased following intravenous steroid treatment. Immunohistochemistry studies showed mild to moderate neuronal staining with the plasma and CSF samples. Remarkably, autoimmune thyroiditis may present with migrating focal motor status epilepticus. We recommend anti-thyroid antibody screening for multifocal motor status epilepticus cases of unspecified cause.

Adult↗

PubMed2005

No association of anti-GM1 and anti-GAD antibodies with juvenile myoclonic epilepsy: a pilot study.

PURPOSE: The presence of anti-ganglioside (GM1) or anti-glutamic acid decarboxylase (GAD) antibodies has been reported in association with therapy-resistant epilepsy mostly of focal origin. Our aim was to detect GM1 and GAD autoantibodies in patients with juvenile myoclonic epilepsy (JME) and to evaluate their association with rarely encountered therapy-resistant cases in this idiopathic generalised epilepsy syndrome. METHODS: Ninety-six consecutive JME patients and 25 healthy normal control subjects were included to the study. We investigated anti-GM1 and anti-GAD antibodies with enzyme-linked immunosorbent assay (ELISA) and radioimmunoassay (RIA), respectively. RESULTS: We found anti-GM1 antibodies in one of 96 (1.04%) JME patients and in one out of 25 (4%) healthy controls, without reaching a significant difference between the titres. Anti-GAD antibodies were detected in 5.8% of the patients and in 4% of the healthy controls. Both antibodies did not have any association with therapy-resistant cases and with any other relevant parameters. CONCLUSIONS: The results of our pilot study suggested that anti-GM1 and anti-GAD antibodies are rare and they did not associate with therapy-resistance and other parameters in JME syndrome.

Adolescent↗

PubMed2005

Add-on therapy with topiramate in progressive myoclonic epilepsy.

We evaluated the clinical responses to add-on therapy with topiramate in eight patients with progressive myoclonic epilepsy (PME). Severe myoclonic seizures disturbing daily activities were persistent despite adequate trials of various combinations of antiepileptic drugs in all patients. After the initiation of topiramate therapy, five patients had a marked decrease in myoclonic seizure frequency, prominent improvement of daily functioning, and recovery from previous drug-related side effects such as weight gain and irregularities of menstruation due to polycystic ovary syndrome. However, we had to discontinue topiramate in three patients because of side effects. Topiramate seems to be a useful alternative agent in the treatment of PME. The antimyoclonic effect provides some independence in daily activities and decreases the side effects related to other antiepileptic drugs, which are clear benefits for this grave disease, although having a short-term effect in some patients.

Adolescent↗

PubMed2005

Perioral myoclonia with absences: an overlooked and misdiagnosed generalized seizure type.

OBJECTIVE: The goal of this report is to draw attention to perioral myoclonia with absences, a rare and underrecognized generalized seizure type. METHODS: We describe a 31-year-old, mildly retarded patient who had absence seizures with prominent perioral movements and rare generalized tonic-clonic convulsions. He had an attack of status epilepticus consisting of asymmetrical perioral myoclonic jerks and was misdiagnosed with epilepsia partialis continua. RESULTS: The perioral myoclonia were associated with brief lapses of consciousness and with a generalized EEG pattern as shown by the synchronous video/EEG investigation with polygraphic recordings. The patient was diagnosed as having the syndrome of perioral myoclonia with absences described by C.P. Panayiotopoulos, C.D. Ferrie, S.E. Giannakodimos, and R.O. Robinson (in: Wolf P, editor. Epileptic seizures and syndromes. London: John Libbey; 1994, p. 143-53). CONCLUSION: We emphasize that this rare and peculiar seizure type associated with generalized epilepsy can be misdiagnosed as focal epilepsy.

Adult↗

PubMed2005

Comparison of classifications of seizures: a preliminary study with 28 participants and 48 seizures.

PURPOSE: Our aim was to compare three available seizure classifications (SCs), namely, the international classification of epileptic seizures published in 1981 (ICES; Epilepsia 1981;22:489-50); the semiological seizure classification (SSC) by H. Luders, J. Acharya, C. Baumgartner, et al. (Epilepsia 1998;39:1006-13; Acta Neurol Scand 1999;99:137-41); and the proposal of a new diagnostic scheme for seizures (PDSS) by J. Engel, Jr. (Epilepsia 2001;42:796-803) published in 2001. The three SCs were compared with respect to diagnostic success rates, usefulness, and consistency by a large group of neurologists in this preliminary study. METHODS: After a training period, 28 blindfed participants with different levels of experience with epilepsy classified videos or written descriptions of 48 randomly selected seizures according to the three SCs. Definite diagnoses of the seizures were established based on all clinical, ictal/interictal EEG, and MRI data. All the participants answered a questionnaire concerning their preferences for SCs after the study. RESULTS: The overall diagnostic success rates were 81.4% for ICES, 80.5% for PDSS, and 87.5%, for SSC. Various parameters concerning experience with epilepsy affected success rates positively, without reaching statistical significance, whereas experience with epilepsy surgery appeared to be a parameter significantly affecting the success rate in all SCs. In reliability analysis, Cronbach's alpha was 0.94 for ICES, 0.88 for PDSS, and 0.70 for SSC, all showing good agreement in the group. Nineteen reviewers chose SSC, eight chose ICES, and one chose PDSS as their preference in the questionnaire, completed after the end of the study. CONCLUSION: The results of this preliminary study demonstrate that with proper training, physicians treating epilepsy patients can handle new SCs, and emphasize the need for revision of the current classification.

Adolescent↗

PubMed2005

Typical absence seizures triggered by photosensitivity.

PURPOSE: To describe the characteristics of patients with typical absence seizures (TASs), consistently triggered by photosensitivity. METHODS: Consecutive patients having TAS induced by intermittent photic stimulation were included in the study. All clinical parameters, EEG, and video-EEG data were assessed during the long-term follow-up. Statistical analyses were performed with SPSS 10.0 software. RESULTS: Nine female and two male patients with a mean age at onset of 14 +/- 5.9 years (range, 7-27 years) and with a mean follow-up of 9 +/- 7.56 years had photosensitive TASs. They constituted 7.64% of absence epilepsies and 0.4% of all patients seen in our tertiary center. The seizures were usually subtle and had a reported frequency of 1 to 9 times daily. Seven patients were clinically photosensitive and reported that some of their TASs were induced by photic stimuli in daily life. All patients also had spontaneous TASs, and four of them had generalized tonic-clonic seizures. EEG results did not show any distinctive features when compared with those of other cases with TASs. Remission could not be achieved in five patients with antiepileptic drug treatments, and we always observed relapses after drug discontinuation or dose reduction in the remaining six cases in remission. Spontaneous remission did not occur even in the five patients older than 30 years. CONCLUSIONS: TASs triggered by photosensitivity are a rare and heterogeneous clinical condition with a marked female preponderance. It is notable that TASs do not remit in these cases.

Adolescent↗

PubMed2004

Obsessive-compulsive disorder after epilepsy surgery.

It is a well-known fact that after epilepsy surgery (ES) preexisting psychopathology may deteriorate or de novo psychopathological syndromes, mainly of a depressive and psychotic nature, may appear. Previously, recovery of obsessive-compulsive disorder (OCD) after ES has been reported in patients who had comorbid OCD preoperatively; however, there have been no reports on the appearance of de novo OCD interfering with daily living activities post-ES. This is the first report of OCD after ES in patients with mesial temporal lobe epilepsy (MTLE). Five patients with MTLE were identified with obsessive personality traits before surgery. Within the first 2 months after ES, two of these MTLE patients fulfilled OCD diagnostic criteria. These OCD patients were not any different from the other three patients with respect to age, age of onset of epilepsy, seizure types, and seizure frequency. All patients stopped having seizures postoperatively, but the OCD patients had worse quality of life postoperatively than preoperatively. Our findings show that those patients with obsessive traits preoperatively should be carefully monitored after ES.

Adult↗

PubMed2004

Autosomal recessive idiopathic epilepsy in an inbred family from Turkey: identification of a putative locus on chromosome 9q32-33.

PURPOSE: The study describes the clinical features of an inbred family from Turkey with three members affected by seizures and tests possible autosomal recessive (AR) inheritance by means of linkage analysis. METHODS: Personal and family history was obtained from each subject, and general physical, neurologic, and EEG examinations were performed. A set of 382 fluorescence-labeled markers was used for the initial genome-wide search. A further set of 83 markers was used to map the locus precisely and to exclude the remaining genome. RESULTS: Twelve individuals from three generations were examined. Two subjects were affected by idiopathic epilepsy, whereas, their brother experienced a single unprovoked generalized seizure. Two siblings affected by idiopathic epilepsy and their unaffected sister showed a photoparoxysmal response to photic stimulation. Nine family members reported migraine. The genome-wide search led to the identification of a unique homozygous, 15.1-cM region shared by subjects with seizures on chromosome 9q32-33 and providing a lod score of 2.9. This locus, however, was not associated with migraine in this pedigree. CONCLUSIONS: The study suggests that idiopathic epileptic traits with AR inheritance might be underestimated in the general population and that inbred pedigrees may represent powerful tools for the identification of AR genes.

Chromosome Mapping↗

PubMed2002

Epileptic seizures in Behçet disease.

PURPOSE: To outline the clinical characteristics of seizures in our large series of Behçet disease (BD) patients with neurologic involvement. METHODS: All files of 223 patients with neuro-BD were evaluated retrospectively, and the group with clearly documented seizures was included in the current study. Clinical characteristics, EEG, neuroimaging and cerebrospinal fluid findings were reevaluated, and the seizures were classified according to the new proposed criteria of the International League Against Epilepsy. On excluding the patients in whom the seizures were due to possible seizure-provoking factors, the seizures that appeared during a neurologic exacerbation were noted. RESULTS: Seizures were seen in 10 (4.48%) of 223 patients. There were one female and nine male patients. In five of the patients, seizures occurred during neurologic exacerbation. Therefore, the actual prevalence of seizures due to BD in our group is 2.2%. In the remaining five patients, the seizures were not related to neurologic BD attacks, but probably were due to some seizure-provoking factors. The predominating seizure type was generalized tonic-clonic convulsions accompanied by focal motor seizures. It is notable that four patients died 1-5 years after the onset of the seizures. CONCLUSIONS: Our study showed that seizures are rare in BD. As the seizures due to some interventions and drugs are as frequent as neuro-BD-related seizures, seizure-provoking factors must be considered before attributing them to the pathogenetic mechanism of BD. The occurrence of seizures seems to be associated with a high mortality rate.

Adolescent↗

PubMed2002

Recurrent absence status epilepticus: clinical and EEG characteristics.

In order to outline the clinical and EEG characteristics of recurrent absence status epilepticus (ASE), eight cases with more than two attacks of ASE were studied. Their current ages were between 13 and 84 years, and five of the patients were women. There was a history of epilepsy in five of the patients before the first ASE episode. A varying degree of confusion was the main clinical symptom with associated mild motor signs like perioral, eyelid and generalised myoclonus, seen in one, two and four patients respectively. Two of the patients had juvenile myoclonic epilepsy. One patient had an atypical form of childhood absence epilepsy characterised by recurrent ASE attacks on awakening. There were two patients with phantom absences and late onset generalised convulsions, one patient with perioral myoclonia and absences, and finally two patients with eyelid myoclonia with absences, which are proposed syndromes. On the EEGs that revealed the diagnosis of ASE, there was a marked variability of the generalised multispike and wave discharges. The EEG findings appeared to be syndrome-related with some exceptions. IV Clonazepam lead to a dramatic improvement. Our study shows that the majority of recurrent ASE cases do not fit into the International syndrome classification.

Adolescent↗

PubMed2002

Subacute sclerosing panencephalitis: clinical and magnetic resonance imaging evaluation of 36 patients.

We studied 36 patients (24 males, 12 females), all of whom had definite subacute sclerosing panencephalitis with typical periodic complexes in their electroencephalograms and increased titers of measles antibody in serum and cerebrospinal fluid. Their clinical and laboratory findings on admission were reviewed retrospectively. The age at onset of symptoms varied from 4 to 23 years. The average age at onset of disease was 13.1 +/- 4.18 years. The mean of the duration from the infection to the onset of subacute sclerosing panencephalitis was 9 years. Unusual symptoms, especially in the early periods of disease, included hemiparesis (7 patients), headache (3), generalized tonic-clonic seizures (6), absence seizure (1), nausea (3), and vomiting (3). Twenty-six cranial magnetic resonance imaging (MRI) and 12 computed tomography examinations were performed. Nine patients had normal MRI. In the early stages, lesions usually involved parieto-occipital corticosubcortical regions asymmetrically. In time, symmetric periventricular white-matter changes became more prominent. In addition to the common clinical findings in cases of subacute sclerosing panencephalitis reported in the literature, there were some different clinical features of the disease. Eventually, we concluded that there seems to be no correlation between the clinical stages and either the duration from the onset of subacute sclerosing panencephalitis or the MRI findings.

Adolescent↗