PubMed HealthSearch

Biomedical subjects

A Arzimanoglou

Publications and source records attributed to A Arzimanoglou.

16 recordsLinked to original sources

Hippocampal developmental changes in patients with partial epilepsy: magnetic resonance imaging and clinical aspects.

Developmental disorders of the hippocampal formation (HF) have been described in epileptic syndromes associated with lissencephaly, but HF malformations can be found without widespread cortical changes. We report 19 patients with partial epilepsy and abnormal HF patterns on magnetic resonance imaging (MRI). The changes consisted of incomplete folding with abnormal medial location along the choroid fissure, globular shape and/or verticalization, and were observed in the following three contexts: (1) diffuse disorder of neuronal migration (n=1); (2) temporal lobe malformation (n=5), including heterotopia, abnormal gyration, and, in 2 cases, reduced HF volume; and (3) apparently isolated HF changes (n=13, bilateral in 3 cases). The clinical features were heterogeneous in terms of severity of epilepsy and, when the focus could be determined, in localization (temporal or extratemporal). In 4 patients with apparently isolated HF changes, MRI was suggestive of both abnormal development and hippocampal sclerosis. In these patients, presurgical investigation and postoperative results suggested multiple epileptogenic foci, involving the frontal lobes in 2 cases. One HF specimen was large enough for the observation of developmental abnormalities corresponding to the changes seen on MRI. HF changes in shape and/or position should be included among the structural abnormalities associated with partial epilepsies. They may represent the visible part of a more extensive or more distant disorder of brain development.

Adolescent

Gabapentin add-on therapy with adaptable dosages in 610 patients with partial epilepsy: an open, observational study. The French Gabapentin Collaborative Group.

The objectives were to evaluate gabapentin add-on therapy in a large population under conditions close to real practice and to determine the therapeutic doses as reached with adaptable dosages. A 6-month multicentre, open-label study, involved addition of gabapentin to pre-existing treatment at the initial dosage of 1200 mg and subsequent adjustment between 900 and 2400 mg/day according to efficacy and tolerability. A study group of 610 adult patients, with partial epilepsy, persistent seizures and a median seizure frequency with a baseline of 7.2 per month were recruited; one-third had less than four seizures per month. Polypharmacy was frequent, with a mean of 2.3 concomitant drugs. After 6 months, 368 patients (62%) continued on gabapentin, at a mean dosage of 1739 mg/day with 44% of responders. On an intention-to-treat basis, median reduction in frequency was 21.2%, and the responder rate was 33.9%. The responder rate increased to 40.7% in the less severe subgroup receiving only one concomitant drug. Seventy-nine patients (13.4%) remained without seizures during the last evaluation period, versus nine (1.5%) during the baseline. Most of them had initially less than four seizures per month. The most frequent adverse effects, somnolence (29.3%), asthenia (14.6%), nausea (7.9%), ataxia (7.7%) and vertigo (7.2%), occurred rapidly after initial titration to 1200 mg/day, and were usually transitory. Weight gain (8.8%) seemed to be related to gabapentin dose. The combination of two recent drugs, vigabatrin and gabapentin, in 190 patients led to similar efficacy levels, with a tendency for more frequent somnolence and asthenia.

Acetates

Is the underlying cause of epilepsy a major prognostic factor for recurrence?

BACKGROUND: We investigated the prognostic value of the type of epilepsies and epileptic syndromes for seizure recurrence. In patients with partial epilepsy, we focused on the prognostic value of any structural brain abnormality and of the location of the epileptogenic region. METHODS: A total of 2,200 adult outpatients were included in a hospital-based observational survey, with a follow-up of 1 to 7 years. Twenty-two percent of the patients exhibited generalized epilepsy, 62% partial epilepsy, and 16% undetermined epilepsy. RESULTS: Seizure control (>1 year without seizure) was achieved in 82% of patients who had idiopathic generalized epilepsy, 35% of those with symptomatic partial epilepsy, 45% of those with cryptogenic partial epilepsy, and 11% of those with partial epilepsy associated with hippocampal sclerosis (HS). Temporal lobe epilepsy (TLE) was the most refractory partial epilepsy, with only 20% of such patients remaining seizure free, compared with 36% of extra-TLE patients. In partial epilepsy, HS, cerebral dysgenesis, and dual pathology (HS and another lesion) were associated with a low rate of seizure-free patients (11%, 24%, and 3%, respectively). No significant difference in seizure control was found between patients with extra-TLE and those with TLE and no HS. CONCLUSIONS: In adults, partial epilepsy is more difficult to treat than idiopathic generalized epilepsy. In patients who have partial epilepsy, the location of the epileptogenic zone does not seem to be a determining factor. Brain abnormalities--especially HS, either alone or associated with another lesion--are a major prognostic factor.

Adolescent

Genetic mapping of a major susceptibility locus for juvenile myoclonic epilepsy on chromosome 15q.

The epilepsies are a group of disorders characterised by recurrent seizures caused by episodes of abnormal neuronal hyperexcitability involving the brain. Up to 60 million people are affected worldwide and genetic factors may contribute to the aetiology in up to 40% of patients. The most common human genetic epilepsies display a complex pattern of inheritance. These are categorised as idiopathic in the absence of detectable structural or metabolic abnormalities. Juvenile myoclonic epilepsy (JME) is a distinctive and common variety of familial idiopathic generalised epilepsy (IGE) with a prevalence of 0.5-1.0 per 1000 and a ratio of sibling risk to population prevalence (lambda(s)) of 42. The molecular genetic basis of these familial idiopathic epilepsies is entirely unknown, but a mutation in the gene CHRNA4, encoding the alpha4 subunit of the neuronal nicotinic acetylcholine receptor (nAChR), was recently identified in a rare Mendelian variety of idiopathic epilepsy. Chromosomal regions harbouring genes for nAChR subunits were therefore tested for linkage to the JME trait in 34 pedigrees. Significant evidence for linkage with heterogeneity was found to polymorphic loci encompassing the region in which the gene encoding the alpha7 subunit of nAChR (CHRNA7) maps on chromosome 15q14 (HLOD = 4.4 at alpha = 0.65; Z(all) = 2.94, P = 0.0005). This major locus contributes to genetic susceptibility to JME in a majority of the families studied.

Chromosome Mapping

[Therapeutic options provided by new antiepileptic drugs].

The introduction on the French market of vigabatrin, gabapentin and lamotrigine has considerably diversified our conventional therapeutical schemes in epilepsies, as will be as amplified by the arrivals of topiramate, tiagabine and oxcarbazepine. Compared to the conventional drugs, these new products present more favorable pharmacokinetics, no or very weak interactions and a better tolerability, specially regarding the cognitive field. They should be used according to their spectrum of activity, function of their modes of action. In add-on trials on partial epilepsy patients all these new products have shown efficacy on partial and secondarily generalized seizures. Seizure frequency is reduced by at least 50 p. 100 in 30 to 50 p. 100 of the patients. A substantial number of patients can be rendered seizure-free with vigabatrin. Lamotrigine has a broader spectrum, as it is also efficacious on the different seizure types of generalized, symptomatic or idiopathic epilepsies. Main adverse events are non-specific central nervous system disturbances such as dizziness, drowsiness, ataxia, tremor or diplopia. More specifically, vigabatrin may induce weight gain and requires closer supervision in case of psychiatric history; lamotrigine which has also probable antidepressant properties, may induce skin rashs, rarely severe. Further data are needed for gabapentin which is now used at daily dosages which are two to three times those used in the initial studies. Gabamimetic agents may be worsening in some cases of generalized epilepsies, more specially on absence and myoclonic seizures. The most obvious benefits, some patients becoming seizure-free, are obtained in cases of intermediate severity, with a bitherapy including one of these new drugs. Developments in children are often delayed. Nevertheless the prognosis, including cognitive outcome, is considerably improved in infantile spasms with vigabatrin and in Lennox-Gastaut syndrome with lamotrigine and felbamate, the latter being highly toxic. For the moment in France, authorities have limited the use of all these new antiepileptic drugs to adjunctive therapy in epilepsies resisting to conventional drugs. But recent monotherapy data show similar efficacy with better tolerability. Once the pivotal, controlled studies have enabled to obtain regulatory approval, all these compounds must undergo a large-scale evaluation phase in order to better define dosages, long-term tolerability, indications and eventual contra-indications in the various epileptic syndromes, including children.

Adult

[Rett syndrome. A well defined but mysterious encephalopathy].

The Rett syndrome is characterized by a progressive development of loss of intellectual functions and of motricity, including abnormal stereotypic hand movements and reduction of the motor skill. This syndrome is exclusively observed in girls. Its typical evolution is characterized by a normal initial development (until 6 to 18 months after birth) followed by a progressive installation of the clinical signs in 4 steps. There is currently no biological marker for the Rett syndrome and therefore the diagnosis is only based on clinical criteria. The most common erroneous diagnosis is infantile autism. In this review, the current status of clinical, genetic and pathogenetic knowledge of the Rett syndrome is presented.

Brain Diseases

[Rendu Osler disease revealed by ruptured cerebral arterial aneurysm in an infant].

A 6 week-old boy whose mother and sister present with hereditary hemorrhagic telangiectasia (HHT) presented suddenly with listlessness, hypotonia, and acute anemia. Cerebrospinal fluid was grossly hemorrhagic. Brain CT scan was compatible with subarachnoid and intracerebral hemorrhage. Operative investigation diagnosed a ruptured aneurysm of one branch of the right middle cerebral artery. A large clot was removed from the right frontal lobe. The ruptured artery was clipped. Further cerebral and abdominal angiographies did not show other aneurysms. The infant died 18 days later, with bilateral subdural hematoma. The family history and review of the literature suggest that the rupture of a cerebral aneurysm in this infant may have been an early manifestation of HHT. Brain CT scan study seems mandatory in every infant born to a mother with HHT.

Cerebral Hemorrhage

Vigabatrin in the treatment of epilepsy in children.

1. This study presents the results of the preliminary screening of vigabatrin as add-on therapy in an open, non-controlled multicentre study in children with refractory epilepsy. 2. There were 135 children, with an age range of 2 months-12 years. Main seizure type was partial in 42%, generalized in 29%, Lennox-Gastaut syndrome in 19% and West syndrome in 10%. 3. Vigabatrin was added onto current antiepileptic treatment in an initially recommended dose of 40-80 mg kg-1 day-1. However, the doses were frequently increased when tolerance allowed it, and the final mean dose used was 87 mg kg-1 day-1 (27-600). 4. A 75% to 100% reduction in seizure frequency was observed in 25% of patients (11 patients became seizure free) and 50 to 75% decrease in a further 13%. Efficacy was better in partial seizures, with good to excellent results in 49% of patients. The use of high doses, above 100 mg kg-1 day, was not associated with greater efficacy in this preliminary study. 5. No side effects were reported in 79% of patients. Agitation and insomnia were observed in 8.8% and somnolence in 6%. Other adverse events included ataxia (2.2%), nausea (2.2%) and increased appetite (1%). A moderate and transient decrease in haemoglobin was reported in six patients from the same centre; these patients were all receiving very high doses of vigabatrin (250 to 600 mg kg-1 day-1). 6. Vigabatrin thus appears to be a safe antiepileptic drug that may be effective in the treatment of severe epilepsy in children.

Adolescent

Spontaneous calcific cerebral embolus from a calcific aortic stenosis in a middle cerebral artery infarct.

Calcific emboli from a calcific aortic stenosis is an uncommon event, usually following local trauma, as from cardiac surgery or left heart catheterization or as a sequel to bacterial endocarditis. We report what we believe to be the first case of a spontaneous calcareous emboli demonstrated by cranial computed tomography. In this patient, systemic hypertension and mild aortic insufficiency may have caused increasing mechanical forces acting on the aortic cusps and may have precipitated embolism.

Aged

Aneurysm of the circle of Willis. Comparison between computed tomography and angiography.

The authors report their experience in 13 patients with unruptured non-giant aneurysms diagnosed at computed tomography (CT), who were without clinical deficits but who complained of chronic headache or cranial nerve palsy and had other members in their families with a cerebral vascular malformation. Screening with contrast-enhanced CT is discussed with respect to certain social groups. Surgical management before rupture of aneurysms could be envisaged in view of the bad prognosis when rupture has occurred.

Cerebral Angiography

[Computed tomographic examination in orbitofacial injuries].

Eighty patients with craniofacial injuries were investigated using a CT scan apparatus of the second generation. The advantages of this examination over conventional methods of exploration are discussed and details provided of projections used. Conventional tomography imaging has now been completely abandoned.

Facial Injuries

[Hurst-type acute leukoencephalitis with complete recovery in a child].

A case of acute haemorrhagic leukoencephalitis in an 11-year old girl demonstrates the usefulness of computerized tomography which shows large, asymmetric hypodense areas in the white matter and diffuse oedema. The causative role of a Mycoplasma pneumoniae infection is suggested by positive serology and by a high level of cold anti-l agglutinins. Treatment with corticosteroids in massive doses combined with a tetracycline resulted in complete cure without sequelae.

Acute Disease