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Biomedical subjects

H Doose

Publications and source records attributed to H Doose.

At least 55 records · Page 3Linked to original sources

Childhood epilepsy in a German city.

The incidence of epilepsy in children between 0 up to 8 years in the city of Kiel in Northern Germany was calculated. The study is based on a case documentation of the Kiel Epilepsy Center from 1957 to 1975. In this period 235 children were registered who were born in the years 1957 to 1966 and suffered from a single or recurrent epileptic seizures prior to their 9th birthday. Based upon the 37 691 children born in Kiel in the years 1957 to 1966, the mean cumulative risk for a single or recurrent epileptic seizures through the age of 8 is 6.23% (7.27% for boys, 5.12% for girls). Sixty percent of the children had their first seizure prior to the third birthday. The incidence rate for children below the age of 9 was 71.9/100.000 in 1965 and 72.4/100.000 in 1966. The maximum incidence rate was found in the first year of life (201.6).--The prevalence could only be estimated by summation of the cumulative incidence and was 4.5% in 1965. The clinical symptomatology was dominated by grand mal (68%), followed by partial seizures with complex (17%) and elementary symptomatology (16%), nonconvulsive generalized seizures (13%), infantile spasms (8%). Remarkably frequent is the benign epilepsy with centro-temporal sharp waves comprising 8% of the total group.

Age Factors↗

Spectral analysis of EEG in the late course of primary generalized myoclonic-astatic epilepsy. I. EEG and clinical data.

The EEG of 38 patients suffering from primary generalized myoclonic astatic epilepsy since early childhood is studied in late stages of the disease. Spectral analysis shows that parietal 4-7 cps rhythms (theta rhythms) which are typical of the EEG in the early stages of the disorder can still exist in the EEG of the adult. The rhythms seem to be related to the course of the epilepsy. In the EEG of patients who still have seizures rhythms occur more often than in the EEG of patients who are free of seizures in the two years before the reexamination of their EEG. The functional anomaly producing a 4-7 cps rhythmization of the parietal EEG seems to be one pathogenetic factor in some epileptic disorders of early childhood, especially in primary generalized myoclonic-astatic epilepsy.

Adolescent↗

Spectral analysis of EEG in the late course of primary generalized myoclonic-astatic epilepsy. II. Cluster analysis of the power spectra.

Cluster analysis is applied to power spectra of the EEG of 38 patients with a primary generalized myoclonic astatic epilepsy (Gundel et al. 1981). The tendency of the data to form clusters within this group is indicated by a random experiment which has been performed with the data. The clustering algorithm divided the material in seven distinct groups which may be combined to three main types of power spectra. These types are power spectra with a 10 cps peak, a 4-7 cps peak and power spectra without remarkable rhythmization. The comparison of EEG types and clinical data shows a correlation of 4-7 cps rhythms with the occurrence of seizures. 4-7 cps rhythms are interpreted as a symptom of "centrencephalic" convulsibility.

Adolescent↗

Petit mal status in early childhood and dementia.

The course of 117 children with so-called centrencephalic myoclonic-astatic petit mal (Doose et al. 1970) was evaluated with the question, whether petit mal status can be the cause of dementia frequently seen in these patients. A dementia is found definitely more frequently in patients with petit mal status. With regard to the animal experimental studies of Wasterlain (1978, Lit.) and Meldrum (1978, Lit.) it seems possible that the prlonged generalized hypersynchronous discharges of the petit mal status lead to a serious disturbance of the neuronal energy metabolism and thus to cerebral organic damages or disturbances of the brain development.

Adolescent↗

Occipital 3-4/s-rhythms in childhood EEG.

Report of follow-up studies in 65 children with occipital slow rhythms in the EEG (287 records). Two types of occipital rhythms can be differentiated: Paroxysmal bursts of 3--4/s-rhythms with high amplitude (I) accentuated by closing eyes and continuous usually regular occipital 3--4/s-rhythms (II). Type I is identical with the sinusoidal occipital 3/s-rhythms, which are observed especially frequently in childhood epilepsies. It could be demonstrated by follow-up studies, that the phenomenon usually disappears at latest during puberty. --The second type may also disappear during puberty or may even persist until the adult age. This persisting type is identical with what is quoted as "Grundrhythmusvariante" in the German literature. The continuous occipital rhythms could be demonstrated at earliest at age 3. The rhythms change their shape, amplitude, frequency and localisation during growth: In children prior to age 10 the rhythms show more often occipital accentuation in monopolar leads (against ear as reference); the amplitudes are higher on average; the frequency is often less than 4/s, and alpha groups preceding the rhythms after closing the eyes are less pronounced than in older children. Subharmonic waves are frequent. In the same manner as in adults a mostly right sided lateralisation can be seen. Genetic factors may be involved in the development of the occipital rhythms, although a simple mendelian transmission could not be confirmed. The EEG phenomenon is correlated with symptoms of psychic and vegetative lability. There is no correlation to epilepsy. The high incidence of anamnestic risk factors suggests that exogenous factors are at least contributing to the development of the rhythms. The continuous occipital 3--4/s-rhythms must be understood as the symptom of a disturbed development of central pacemaker systems. It can lead to persisting abnormalities or only occur transitorily in certain stages of the brain maturation.

Adolescent↗

Galvanic skin response in photosensitive children.

Investigations of galvanic skin response under stress by different stimuli were carried out in 65 photosensitive and 70 non-photosensitive subjects aged 11--17 years. Photosensitive individuals at the age of 12--15 years showed a more pronounced skin reaction than non-photosensitive ones. The emotional reagibility (expressed by IE = (SEE TEXT), Traxel 1960, Stocksmeier and Langosch 1973) shows significantly higher values than in nonphotosensitive children. In addition to this, photosensitive subjects demonstrate a delayed habituation. Together with further results (Gross-Selbeck et al. 1976) the findings indicate that photosensitivity is not the isolated symptom of a genetically determined increased cerebral excitability, which can represent a factor in the pathogenesis of epilepsy for instance, but must be understood as a special characteristic of a particular constitution.

Adolescent↗

Sinal myoclonus.

A 5-year-old boy is reported with spinal myoclonus caused by cervical astrocytoma due to neurofibromatosis. The essential clinical and electromyographical signs of spinal myoclonus are the following: (1) Continuous rhythmical appearance in muscles innervated by the affected segments of the spinal cord. (2) Synchronous myoclonus in muscles innervated by the same spinal segments of one body side, asynchronous myoclonus in muscles of different segments as well as in contralateral muscles of the same segments. (3) Usually stable frequency, which may increase to a manifold under the influence of different stimuli. (4) Accentuation of the myoclonus under mental distress, disappearance during deeper sleep. The pathophysiological basis of spinal myoclonus might be a disinhibition in the area of the spinal formatio reticularis. Supraspinal stimuli can influence the myoclonus.

Astrocytoma↗

[Genetics and genetic counselling in childhood epilepsy (author's transl)].

According to electroencephalographical studies on the pathogenesis of epilepsy a hereditary predisposition plays a greater role than believed before. It is far more frequent than the manifest disease. The phenomenon of cerebral excitability is influenced by different genetically determined, differently age- and sexdependent but amongst each other independent factors. When defining the risk of epilepsy for siblings and descendents in practice, one is forced to proceed from the statistical empirical value with all its uncertainty. During the past few years, the figures have become more exact for special types of epilepsy as absence-epilepsy, myoclonic astatic Petit mal, and Impulsiv-Petit mal. Details of these results and their consequences for genetic advice are discussed.

Adolescent↗

Genetic factors in childhood epilepsy with focal sharp waves. I. Clinical data and familial morbidity for seizures.

203 epileptic children (127 boys, 76 girls), who had demonstrated at least once focal sharp waves in the EEG during the course were investigated regarding clinical and historical data as well as familial morbidity for seizures. Expectedly, the seizure symptomatology was multiform: focal seizures of different type, especially secondarily generalized, grand mal, psychomotor fits, infantile spasms etc. Organic brain lesions play an important role. In 23% of cases a familial seizure affliction can be registered. The morbidity (close family) is significantly lower than in families of patients with spike wave absences (2.5 and 4.4% resp., Doose et al. 1973). Like in spike wave absence epilepsies mothers and mothers' siblings are more often affected than fathers and fathers' siblings. Among the affected relatives grand mal epilepsies predominate. The increase of febrile convulsions and spike wave absences observed in the families of absence epileptics is not present. In probands with onset of epilepsy during early childhood a significantly increased familial affliction can be observed. A more extensive discussion of the results will follow in a second paper deeling with EEG findings in probands and siblings.

Child↗

Genetic factors in childhood epilepsy with focal sharp waves. II. EEG findings in patients and siblings.

Electroencephalographical investigations were performed in 203 epileptic children exhibiting focal sharp waves in the EEG and in 312 siblings. As controls served 685 brain-healthy children (Gerken 1971) as well as 252 children with spike wave absences and their 242 siblings (Doose et al. 1973). According to the EEG findings in probands so-called "centrencephalic" EEG-criteria (theta rhythms, spikes and waves, photosensitivity) as a symptom of a genetically determined seizure susceptibility can be expected in at least 60% of the probands. 18% of the siblings showed at least once one definite "centrencephalic" EEG pattern. These results must be regarded as minimal values, since in siblings only one electro-encephalographic study could be performed. The results make it evident, that in the pathogenesis of epilepsy with focal sharp waves a genetically determined seizure susceptibility plays a very important role. After comparison with the results of genetic investigations in patients with spike wave absences (Doose et al. 1973) the difference as for the genetic basis between "centrencephalic" and focal epilepsies apparently is only due to a different gene density.

Brain Damage, Chronic↗

Cranial computertomography in children with tuberous sclerosis.

20 children with tuberous sclerosis were examined by computertomography (CCT). The test is useful to detect cerebral involvement with great certainty. The method is especially helpful in the early diagnosis of the disease. This offers the possibility of giving an early genetic advice. Characteristic CCT scan abnormalities in tuberous sclerosis are multiple densities in the walls and the roof of lateral ventricles. This specific finding can already be seen prior to the occurrence of calcifications in standard roentgenograms.

Adolescent↗

EEG spectral analysis in children with febrile convulsions.

The abnormal theta-rhythm of the EEG can be identified by use of spectral analysis. The rhythms appear as peaks in the power spectrum and can be considered as given, if the intensity of this peak is significantly higher than the average intensity and if a left-right coherence is present. Abnormal theta-rhythms in the parietal region are significantly more frequent in children with a histroy of febrile convulsions than in controls. This difference becomes especially obvious in the records with open eyes. According to previous investigations, the abnormal theta-rhythm is correlated with a genetically determined susceptibility to convulsions.

Child, Preschool↗

[EEG in neuropediatrics (author's transl)].

After remarks about the history of pediatric electroencephalography some particularly important neurophysiological and especially electroencephalographical studies out-of the last ten years are reviewed: polygraphic studies in healthy and sick newborns, spectralanalytical investigations of the EEG in twins, infants and older children. The review is closed by some critical considerations about possibilities and limitations of the automatic analysis of the childhood EEG.

Bioelectric Energy Sources↗