[Academic achievement of children born with very low birth weights at the age of 10 years].

OBJECTIVE: The academic achievement in Japanese and arithmetic of children born with very low birth weights (VLBW) was examined by means of academic skill tests at the age of 10 years. METHODS: The participants were 14 VLBW children who could be followed up in our hospital at the age of 10 years, and 6 men and 8 women. They had a mean gestational age of 27 weeks and 6 days and a mean birth weight of 988 g. The tests of the fourth grade of Japanese and arithmetic were performed during the waiting time of outpatient. The results were compared with those in typical development (TD) children. RESULTS: Concerning the comprehension of Japanese, the correct answer rate as to the problems answered in their own word was 42.9 ± 51.4% in the VLBW children and 69.7 ± 46.3% in the TD children, respectively. The correct answer rate as to composition was 28.6 ± 46.9% in the VLBW children and 72.7 ± 44.9% in the TD children, respectively, that in the VLBW children being obviously low. The correct answer rate as to calculation laws of arithmetic was 55.4 ± 14.7% in the VLBW children and 66.3 ± 15.5% in the TD children, respectively. The sentence resolving as to arithmetic was 42.9 ± 50.4% in the VLBW children and 52.9 ± 50.1% in the TD children, respectively. CONCLUSIONS: The VLBW children had difficulty in Japanese and arithmetic which required the sentence understanding, compared with the TD children.

Idiopathic childhood occipital epilepsy of Gastaut: report of 12 patients.

This study sought to present clinical and outcome data of patients with idiopathic childhood occipital epilepsy of Gastaut, to validate previously reported characteristics of this epilepsy. The study group was comprised of 12 affected children (three boys and nine girls), with a median age of onset at 10.3 years. Common ictal manifestations included elementary visual hallucinations (75.0%), blindness or blurring of vision (50.0%), headache (50.0%), and secondarily generalized tonic-clonic seizures (58.3%). Interictal electroencephalography revealed occipital spike-wave paroxysms reactive to eye closure and opening in all patients, accompanied by spike-wave activity in the extra-occipital areas in four (33.3%), and by generalized spike-wave discharges in two (16.7%). One patient exhibited the onset of occipital lobe seizures 1 year after manifesting absence epilepsy. Seizure remission occurred in 81.8% of cases, in half of which medication was discontinued by late adolescence. This study confirmed the previously delineated electroclinical features of epilepsy syndrome, with additional aspects including the frequent association of generalized tonic-clonic seizures and atypical evolution from childhood absence epilepsy.

[Dysphagia and videofluoroscopic examination of swallowing in patients with severe motor and intellectual disabilities, and the results of dysphagia rehabilitation for these patients].

A dysphagia rehabilitation team at National Ehime Hospital treated fifty-seven patients with severe motor and intellectual disabilities suspected of having dysphagia. The rehabilitation team graded the degree of dysphagia by observing the patients at mealtimes. The grades were compared with the videofluoroscopic examination of swallowing results. There was some correlation with the dysphagia grades and videofluoroscopic examination of swallowing results (solid food:p = - 0.434, n =54, p < 0.0001, liquid:p = - 0.482, n = 54, p = 0.005). There were cases in which manifestations of severe dysphagia such as retention of solid food in the pharynx could only be detected on videofluoroscopic examination of swallowing. Dietary manipulation allowed shortening of the time taken at mealtimes for some patients. The dysphagia rehabilitation was useful in many cases in decreasing the number of days with fever, and improving the eating functional level, but the results as a whole were not statistically significant.

Benign angiopathy of the central nervous system associated with phenytoin intoxication.

We report a patient with epilepsy who presented with acute onset of left hemiparesis associated with phenytoin intoxication due to interaction with clobazam. Magnetic resonance angiography of the head revealed stenosis of the M2 segment of the right middle cerebral artery, whereas an erythrocyte sedimentation rate and cerebrospinal fluid analysis were normal, being consistent with a diagnosis of benign angiopathy of the central nervous system. The patient exhibited an elevated plasma level of thrombin-antithrombin III complex along with a marginally increased plasma concentration of soluble E-selectin. The present case suggests that phenytoin intoxication can cause cerebral vasospasm, which may be associated with some inflammatory endothelial injury accompanied by activated intravascular coagulation.

[Long-term follow-up of a case of eyelid myoclonia with absences].

We reported a case of eyelid myoclonia with absences treated for 10 years. Seizures of eyelid myoclonia accompanied by absence, precipitated by sunlight, began at the age of 4 years and disappeared at the age of 11 years. Non-convulsive status epilepticus, occurring shortly after awakening, began at the age of 10 years and have occurred intermittently until the age of 18 years. While generalized EEG abnormalities disappeared after initiation of treatment, the patient still had occipital spikes on interictal EEG at the age of 18 years, suggesting occipital hyperexcitability as the basis of photosensitivity. Along with poor drug compliance due to her living apart from her parents, it was difficult to completely suppress non-convulsive status epilepticus, posing serious concern in her adult life.

Clinical investigation of genetic contributions to childhood-onset epilepsies and epileptic syndromes.

Although there have been major advances in the understanding of the molecular bases of certain inherited epilepsy syndromes, clinical studies are still needed to verify the possible genetic contributions to common epilepsies. We examined the proportions of positive family histories of epilepsy (within second-degree relatives) and consanguinity (within first-degree relatives) in 311 probands with childhood-onset epilepsy, and found that they had high family history rates of epilepsy (19.3%) and consanguinity (6.1%). A positive family history of epilepsy was found more in probands with generalized epilepsy than in ones with localization-related epilepsy, and more in probands with idiopathic/cryptogenic epilepsy than in ones with symptomatic epilepsy. However, on analysis after the symptomatic epilepsies had been divided into two categories, probands with pre- or perinatal symptomatic generalized epilepsy and ones with postnatal symptomatic localization-related epilepsy showed high positive family history rates, similar to ones with idiopathic/cryptogenic epilepsy. On the other hand, a positive family history of consanguinity was noted more in probands with generalized epilepsy than in ones with localization-related epilepsy, but there was no significant difference between probands with idiopathic/cryptogenic epilepsy and ones with symptomatic epilepsy. These findings suggest that in addition to the hereditary effect on idiopathic/cryptogenic epilepsy, a genetic susceptibility may contribute to the development of pre- or perinatal symptomatic generalized epilepsy, and to that of postnatal symptomatic localization-related epilepsy. Furthermore, a genetic predisposition seems to have an influence through consanguinity on the etiologies of both idiopathic/cryptogenic and symptomatic generalized epilepsies.