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Behavior and learning problems in epileptic children.

In this modern age of rapidly advancing medical knowledge and technology there are few conditions as wrapped up in ignorance and prejudice as that known as epilepsy. A large part of the reason for this lies in the concept of the "epileptic person," by which is really meant the epileptic personality. In an attempt to assess behavior and learning in children with seizures, behavior and learning being regarded as opposite sides of the same coin, a study was designed in which totally objective neurological and psychological data was obtained from such a group of children. The evaluation was carried out without any prior knowledge of the nature of the presenting clinical picture, so as to prevent bias from pre-conceived notions. The results show that 70% of the first 200 children showed significant learning defects sufficient to make special educational placement mandatory. Of the remaining 30% some still showed minor learning problems enough to give rise to difficulties in regular classroom situations. Associated with these learning problems were varying behavioral reaction types, varying from the classical hyperactive child with minimal cerebral dysfunction to many environmentally-produced behavior difficulties resulting from faulty school placement due to failure to recognize learning problems. Faculty parental handling due to similar failure to realize the child's limitations also contributed to this. The significance of these findings with respect to the behavior disturbances of the so-called epileptic child will be discussed.

Adolescent↗

[Epilepsies with complex focal seizures in childhood. Seizure semiology and routine EEG, localization, neuropathology and etiology, follow-up forms and prognosis with anticonvulsant therapy].

Focal epilepsies of childhood with complex partial seizures, with or without secondarily generalized seizures are reviewed. Particular emphasis is placed on the semiology of seizures, which are subdivided into: aura, disturbance of consciousness, speech disturbances, automatims, and other motor and vegetative seizure symptoms. The significance of routine ictal and interictal EGGs and their importance for the localization is discussed. Recent neuropathological findings and etiology are presented and the courses epilepsies can take (especially those involving febrile convulsions), prognosis and long-term therapy with standard antieleptic drugs. A definition of pharmacoresistance is given, a prerequisite for surgical therapy, which in turn necissitates special presurgical diagnostic procedures [corrected].

Amygdala↗

[Magnetic resonance tomography in comprehensive examination in epilepsy in children].

The paper presents the results of examining 100 children suffering from various epilepsies by magnetic resonance tomography, which determined a morphological substrate of the disease in 26%. The findings were compared with the clinical picture and the results of routine study tools (skull X-ray, EEG, EchoEG, REG). The diagnostic value of the techniques applied was defined. It is concluded that a set of diagnostic tools rather than individual techniques should be applied to reveal etiological and pathogenetic factors of the disease.

Adolescent↗

[The duration of antiepileptic treatment].

110 patients at the age of 18 years and older with different forms of epilepsy and epileptic syndromes were observed during 2-15 years after the first fits. These patients did not follow medical advice. All of them were either treated for short time, irregularly or were not treated at all after their first hospitalisation after beginning of epileptic attacks. In spite of violation of the treatment regimen, 80 patients experienced improvement, 60 patients achieved long-term remissions. It is suggested that even short-term antiepileptic therapy may activate the patient's own antiepileptic system. Negative prognostic criteria were involvement of the temporal structures, multifocal disease.

Adolescent↗

[The diagnosis of epilepsy by using blood analysis for the content of autoantibodies to glutamate neuroreceptors].

The glutamate receptor autoantibody level was studied in blood of 170 epileptic patients as well as of 114 control donors (64 patients with nonepileptic cerebral pathology and 50 healthy individuals). The elevation of autoantibodies level (higher than 140%) was observed in 63.5% of patients with paroxysmal epileptic disturbances. This elevation was observed more frequently in the initial period of the disease as well as in generalized (convulsive or nonconvulsive) fits irrespective of disease etiology. Combined application of the test with EEG method permitted to confirm the epileptic nature of the disease in 83.5% of cases. The results of repeated analysis of glutamate receptor autoantibody level in blood proved to be a significant criterion of the effectiveness of anticonvulsive therapy. It also allowed to predict the development of posttraumatic epilepsy in individuals with craniocerebral trauma in the past.

Adult↗

Protective effects of TRH and its analogues in chemical and genetic models of seizures.

TRH shows strong influence on neuronal excitability and may participate in the regulation of seizures. We investigated the effect of TRH and its stable analogues on seizures induced by intravenous (i.v.) infusion of pentetrazole in rats. The data showed that i.v. administration of TRH (10 and 20 mg/kg), RGH-2202 (0.1 mg/kg) and Z-p-Glu-His-Pro-NH2 (10 mg/kg) increased threshold for pentetrazole-induced clonic seizures, but did not affect the tonic ones. Another stable analogue of TRH, 1p-Glu-Tyr-Pro-NH2 (0.1-10 mg/kg), had no effect on pentetrazole-induced seizures. In further study, effects of TRH and RGH-2202 were examined in WAG/Rij rats, a genetic model of absence epilepsy. TRH (25 and 50 micrograms i.c.v.) decreased dose-dependently the number and mean duration of spike-wave discharges in cortical EEG of WAG/Rij rats at 90 and 120 min after the peptide administration. On the other hand, RGH-2202 (1 microgram i.c.v.) significantly decreased only the number of spike-wave discharges at 60 min post-injection. These results confirm that TRH and some of its analogues have moderate antiepileptic activity.

Action Potentials↗

Altered calcium channel currents in Purkinje cells of the neurological mutant mouse leaner.

Mutations of the alpha1A calcium channel subunit have been shown to cause such human neurological diseases as familial hemiplegic migraine, episodic ataxia-2, and spinocerebellar ataxia 6 and also to cause the murine neurological phenotypes of tottering and leaner. The leaner phenotype is recessive and characterized by ataxia with cortical spike and wave discharges (similar to absence epilepsy in humans) and a gradual degeneration of cerebellar Purkinje and granule cells. The mutation responsible is a single-base substitution that produces truncation of the normal open reading frame beyond repeat IV and expression of a novel C-terminal sequence. Here, we have used whole-cell recordings to determine whether the leaner mutation alters calcium channel currents in cerebellar Purkinje cells, both because these cells are profoundly affected in leaner mice and because they normally express high levels of alpha1A. In Purkinje cells from normal mice, 82% of the whole-cell current was blocked by 100 nM omega-agatoxin-IVA. In Purkinje cells from homozygous leaner mice, this omega-agatoxin-IVA-sensitive current was 65% smaller than in control cells. Although attenuated, the omega-agatoxin-IVA-sensitive current in homozygous leaner cells had properties indistinguishable from that of normal Purkinje neurons. Additionally, the omega-agatoxin-IVA-insensitive current was unaffected in homozygous leaner mice. Thus, the leaner mutation selectively reduces P-type currents in Purkinje cells, and the alpha1A subunit and P-type current appear to be essential for normal cerebellar function.

Animals↗

Synthesis of a new homologous series of p-chlorophenyl alcohol amides, their anticonvulsant activity and their testing as potential GABAB receptor antagonists.

The anticonvulsant activity of a homologous series of p-chlorophenyl alcohol amides is described. The new compounds (+/-)-2-hydroxy-2-(4'-chlorophenyl)-butyramide (2), (+/-)-3- hydroxy-3-(4'-chlorophenyl)pentanamide (4) and (+/-)-4-hydroxy-4-(4'-chlorophenyl)-hexanamide (6), were prepared and tested for their anticonvulsant activity. Compounds 2, 4, and 6 exhibited significant activity in seizures provoked by pentylenetetrazol. Chlorine in the para position of the phenyl ring increased both their potency at the peak effect and the duration of their anticonvulsant activity. The anticonvulsant activities of (+/-)-3-hydroxy-3-phenylpentanamide (3) and compound 4 were antagonized by DL-baclofen. This, and the protecting activity of 3 in the genetic-absence-epilepsy rats of the Strasbourg model suggest that the phenyl alcohol amides could be GABAB receptor antagonists.

Amides↗

Trisomy 12p and epilepsy with myoclonic absences.

We report the case of a 6-year-2-month-old female affected by trisomy 12p syndrome. Seizures were typical myoclonic absences from both the clinical and EEG points of view. Our patient and other sporadic reports in the literature seem to support the hypothesis that, at least in some cases, myoclonic absences can be a direct or indirect effect of a chromosomopathy.

Brain↗

Electroclinical features of idiopathic generalised epilepsy with persisting absences in adult life.

OBJECTIVES: To describe the electroclinical features of typical absences persisting in adult life. METHODS: Twelve adult patients (aged 21 to 56 years) with idiopathic generalised epilepsy featuring typical absences as the prominent clinical feature were studied. All patients underwent a full clinical and neurophysiological investigation including ictal documentation of seizures. RESULTS: Neurological examination and neuroradiological investigations were normal in all cases. Clinical findings included a median age at onset of absences of 14 (range 4-32) years, almost constant tonic-clonic seizures (in 83% of patients), frequent episodes of absence status (in 33% of patients), and associated cognitive or psychiatric disturbances. Interictal EEG findings showed normal background activity, generalised paroxysms of spike waves or polyspike waves, and inconstant focal spikes (in five patients); runs of polyspikes were seen during non-REM sleep. Ictal EEG findings showed generalised spike waves at 3 Hz, sometimes preceded by multiple spikes, or more complex EEG patterns with sequences of polyspikes intermingled with spike waves or polyspike waves, showing discharge fragmentation or variation of intradischarge frequency. CONCLUSION: The results of the present study show that absences persisting in adult life may show particular clinical and EEG patterns, distinct from those in childhood or adolescence.

Adult↗