PubMed Health⌕ Search

Biomedical subjects

A Kaminska

Publications and source records attributed to A Kaminska.

30 records · Page 2Linked to original sources

Delineation of cryptogenic Lennox-Gastaut syndrome and myoclonic astatic epilepsy using multiple correspondence analysis.

PURPOSE: To distinguish various types of childhood severe cryptogenic/idiopathic generalised epilepsy on the basis of reproducible diagnostic criteria, using multiple correspondence analysis (MCA). METHODS: We applied MCA to a series of 72 children with no evidence of brain damage, starting epilepsy between 1 and 10 years, with two or more types of generalised seizures. We excluded patients with infantile spasms or typical absences. MCA was performed on all clinical and EEG parameters, first throughout follow-up, then restricted to the first year of the disease. RESULTS: When including all follow-up variables, there were three groups: (1) Thirty-seven children with male predominance, familial history of epilepsy, simple febrile convulsions, massive myoclonus, tonic-clonic fits. Outcome was favourable, with no seizures and mildly affected cognitive functions. Interictal EEG showed short sequences of irregular 3-Hz spike-waves. (2) In 18 children, clinical characteristics were similar to those of the first group at the early stage, but 95% exhibited myoclonic status and vibratory tonic seizures, with persisting seizures on follow-up. EEG showed long sequences of generalised irregular spike and slow waves. Those two groups meet the characteristics of childhood onset myoclonic-astatic epilepsy (MAE) with respectively, favourable and unfavourable outcome. (3) Eleven children had later onset, atypical absences, tonic and partial seizures, and no myoclonus, or vibratory tonic seizures. All had mental retardation and persisting seizures. EEG showed long sequences of slow spike-wave activity and half the patients had spike and slow wave foci. These patients met the major characteristics of Lennox-Gastaut syndrome. Initial parameters failed to distinguish the first two groups, but Lennox-Gastaut syndrome (the third group) was distinct from both groups of myoclonic astatic epilepsy from the onset. Within MAE groups combined, clinical and EEG risk factors for mental retardation could be identified. CONCLUSION: It is possible to validate statistically the distinction between discrete epileptic syndromes. Myoclonic astatic epilepsy is therefore distinct from Lennox-Gastaut syndrome, and the distinction appears from the first year of the disorder.

Adolescent↗

Use of subtraction ictal SPECT co-registered to MRI for optimizing the localization of seizure foci in children.

UNLABELLED: Ictal SPECT studies are increasingly used to localize seizure foci in children with refractory epilepsy, but few studies have reported on ictal-interictal subtraction images co-registered to MRI at this age. METHODS: Twenty-seven children with partial epilepsy (aged 3 mo-18 y) underwent ictal ethyl cysteinate dimer (ECD) SPECT (20 mCi/1.73 m2) combined with video-electroencephalography (EEG) and interictal ECD SPECT followed 2 d later by three-dimensional MRI. Ictal-interictal and interictal-ictal subtraction images were computed by registering and normalizing the ictal to the interictal SPECT scans for each child. The ictal, interictal SPECT and subtraction images were registered to each child's MRI. Difference images (ictal-interictal) were then superimposed on MRI for anatomic localization of the perfusion changes. Intra- and interobserver reproducibility and "facility of interpretation" of overlay images were compared with standard analysis of the non-coregistered ictal and interictal scans. RESULTS: Overlay images allowed the detection of at least one hyperperfused focus in 93% of the children, compared with 74% using ictal and interictal scans separately. Seizure onset was suspected clinically, on EEG or on MRI in 20 children. Overlay images were concordant (n = 11) or larger (n = 7) than the suspected focus in 18 of 20 (90%), whereas these images failed to show any abnormality in 1 child and were discordant with MRI in another patient. In the remaining 7, images showed cortical localization in 6 patients. Among the 5 patients who underwent electrocorticography, overlay images were concordant in 3, larger in 1 and absent in 1. The intra- and interobserver reproducibility and facility of interpretation were significantly higher using overlay images than standard analysis, even when ictal and interictal SPECT were co-registered. CONCLUSION: The co-registration of ictal-interictal subtraction SPECT images to MRI seems to be a helpful technique in localizing the onset of seizure and guiding the intracranial recording in childhood epilepsy. Moreover, this method improves sensitivity, enhances intra- and interobserver reproducibility and makes interpretation easier.

Adolescent↗

[Ictal SPECT in the epileptic child. Contribution of subtraction interictal images and superposition of with MRI].

Ictal SPECT is a highly sensitive method to localize the epileptogenic focus in refractory temporal lobe epilepsy in adults. In extratemporal epilepsy, sensitivity can be improved by subtracting interictal from ictal images and superimposing subtraction images on MRI. In children, such a procedure is potentially interesting because most epilepsies are extratemporal and ictal SPECT not yet routinely developed. The aim of this study was to test the feasibility of ictal SPECT with subtraction image processing in a pediatric population. Twenty-six children with refractory partial epilepsy and aged from 3 months to 18 years underwent ictal ECD-SPECT (20 mCi/1.73 m2) combined with video-EEG and interictal ECD-SPECT plus 3D-MRI two days later. Ictal-interictal subtraction images were computed by registering and normalizing the ictal to the interictal SPECT scans for each child. The ictal, interictal SPECT and subtraction images were registered to the children's MRI. Difference images were then superimposed to MRI for anatomical localization of the perfusion changes (overlay images). Looking for perfusion changes, overlay images allowed to detect at least one hyperperfused focus in 92 p. 100 of the 26 children compared to 73 p. 100 visually comparing ictal and interictal scans separately. Seizure onset was suspected on clinical and/or EEG and/or MRI in 19 children. Positive overlay images were concordant (n = 11) or larger (n = 7) than the suspected focus in 17/19 (90 p. 100), whereas they failed to show any abnormality in 1 child and were discordant with MRI in another one. In the 7 remaining patients, images showed cortical localization in 6 cases. Ictal SPECT is therefore faisable in very young children. Ictal-interictal subtraction SPECT images co-registered to MRI improves sensitivity compared to classical visual analysis. It seems therefore to be a helpful technique to localize the onset of seizure and to guide the intracranial recording in childhood epilepsy.

Adolescent↗

Lamotrigine and seizure aggravation in severe myoclonic epilepsy.

PURPOSE: In severe myoclonic epilepsy of infancy (SME), multiple drug-resistant focal and generalized seizure types occur. Lamotrigine (LTG), found effective in many generalized and partial seizures, has been little used in severe childhood epilepsy syndromes with multiple seizure types. We studied the effects of LTG in SME. METHODS: Twenty-one patients with SME, aged 2-18 years, were treated with LTG, 20 in add-on and one in monotherapy. LTG was started at 0.2-2.5 mg/kg/day and increased to 2.5-12.5 mg/kg/day. For each seizure type, excluding atypical absences, >50% variations compared with the 2 months preceding LTG were considered indicators of response, also taking into account the degree of disability each seizure type produced. RESULTS: LTG induced worsening in 17 (80%) patients, no change in three, and improvement in one. There was >50% increase in convulsive seizures in eight (40%) of 20 patients. Myoclonic seizures worsened in six (33%) of 18 patients. Of five patients improving in at least one seizure type, four had concomitant worsening of more invalidating seizures. Clear-cut worsening appeared within 3 months in most patients but was insidious in some. LTG was suspended in 19 patients after 15 days-5 years (mean, 14 months) with consequent improvement in 18. CONCLUSIONS: The pronounced seizure deterioration during LTG treatment was not attributable to the natural course of the disease and could be a direct effect of therapeutic LTG doses. LTG treatment seems inappropriate in SME.

Acute Disease↗

Use of lamotrigine in Lennox-Gastaut and related epilepsy syndromes.

Lennox-Gastaut syndrome a combination of various generalized seizures including atypical absences and tonic seizures with generalized slow spike waves and mental deterioration, is often difficult to distinguish from a subgroup of myoclonic-astatic epilepsy, other generalized epilepsy syndromes, and various symptomatic generalized epilepsies. Conventional antiepileptic medication is poorly effective in this condition, particularly because various types of seizures respond differently to each given drug. Lamotrigine is effective in the various types of generalized seizures and efficacy has been demonstrated in Lennox-Gastaut syndrome. Given the potential of major mental deterioration within a matter of months in this condition, and the need of slow dose escalation in order to prevent skin rash, lamotrigine should be administered as soon as the diagnosis of Lennox-Gastaut syndrome is suspected. In addition, there is growing evidence that lamotrigine is also most useful in the subgroup of myoclonic-astatic epilepsy beginning in childhood, and that these patients should benefit from the drug like those affected by Lennox-Gastaut syndrome, as soon as the diagnosis is suspected. However, this drug may worsen other cases of myoclonic-astatic epilepsy beginning in infancy. These clinical observations add to the evidence for the need of clear diagnostic work-up before appropriate drug therapy is decided in pediatric epilepsy.

Anticonvulsants↗

Muscle stiffness and continuous electromyographic activity in old rats; an animal model for spasticity?

A mechanomyographic response of the hind foot to passive straightening and bending, as well as an electromyographic activity of the gastrocnemius and tibialis anterior muscles were recorded in old (35-44-month-old) and young female rats. In old rats, spontaneous, tonic electromyographic activity patterns were concurrently observed in both antagonistic muscles; they were low-amplitude, dense tonic activity and continuous, high-amplitude, sparse electromyographic activity. The tonic electromyographic activity was correlated with a decline in the strength and mass of muscles, as well as with motor disturbances, including paresis of the rigidly straightened backward hind legs, dragged behind by an animal. In muscles of old rats, morphological features of a chronic denervation atrophy were found. Baclofen (10 and 15 mg/kg, i.p.) diminished the spontaneous tonic electromyographic activity and potently decreased the whole body muscle tone, whereas Madopar (50 mg/kg of L-DOPA+12.5 mg/kg of benerazide) was ineffective. It is suggested that old rats in which the above-described pathologic alterations are observed might be a useful animal model in the search for basic etiopathological mechanisms of spasticity and similar disturbances found in humans.

Aging↗