Hippocampal malrotation with normal corpus callosum: a new entity?

Among 527 MRI examinations of patients with a suspicion of epilepsy in 5 years, we found 32 cases of hippocampal malrotation (HIMAL). The characteristic features are: incomplete inversion of the hippocampus with and abnormally round shape; unilateral involvement of the whole hippocampus; normal signal intensity and size; blurred internal structure; an abnormal angle of collateral sulcus; abnormal position and size of the fornix; normal size of the temporal lobe; enlargement and particular configuration of the temporal horn, typical of corpus callosum agenesis; and a normal corpus callosum. In 7 cases (22%) HIMAL occurred together with developmental disorders. It was predominantly seen in men. The clinical features were varied. Based on some MRI features, the presence of developmental disorders, the male predominance, the frequently positive family history, and a review of the literature, we think HIMAL may be the consequence of a mild hemisphere developmental disorder. It is probably not the basic cause of epilepsy in such varied clinical setting, but may be a sign of a developmental disorder and can help in selecting patients for more meticulous investigation. It also may give some new understanding of brain development.

Slit-ventricle syndrome in shunt operated children.

Chronic intracranial hypotension is considered as a frequent complication in shunted hydrocephalus, besides obstruction and shunt-infections. In the last twenty years 32 cases of slit-ventricle were diagnosed among the more than one thousand operations on hydrocephalic children at the Paediatric Department of the National Institute of Neurosurgery, Budapest, Hungary. Most of them have been operated on in infancy. Time from the first operation to the development of slit-ventricle ranged from one to twelve years, the mean was 6.5 years. Seven patients were symptomless (22%), while 25 patients (78%) had more or less severe slit-ventricle syndrome with headache (25 cases), nausea/vomiting (23 cases), altered consciousness (21 cases), brainstem signs (12 cases), and epileptic fits (2 cases). Ten patients with moderate clinical signs improved under conservative treatment. In 15 cases an anti-siphon device (ASD) was implanted. In five of them the clinical result was good, but in the remaining 10 cases typical hypertensive signs were seen. In these cases low flow rate valves were implanted instead of the middle flow rate valve and ASD. In one case the intracranial hypertension persisted, so a middle flow rate shunt system was "reimplanted" and finally the patient improved. In this study the experiences with these 32 cases will be analysed and discussed. The authors stress the primary use of combined valves to avoid the slit-ventricle syndrome.

The treatment of malignant scala posterior tumors in children: II. Preliminary result of the pre- and postoperative adjuvant chemotherapy of scala posterior tumors.

Ten children with posterior scala tumor infiltrating the surrounding brain substance and/or the brain stem entered in the present study with preoperative chemotherapy. In 8 of the 10 cases regression and necrosis of the tumor were seen by CT examination after the preoperative therapy. The diameter of the tumor decreased on the average by 35.6% (14.0-74.3%). The main side effect was granulocytopenia. According to our observation, the preoperative therapy enables a more radical surgery in some cases of medulloblastoma and ependymoma. Further observations are necessary to confirm these preliminary results.

The second report of Knobloch syndrome.

Two sibs affected with Knobloch syndrome comprising severe visual handicap (high myopia and retinal detachment with other defects), atypical occipital cephalocele, and unusual plantar creases are described.

On early surgery of hydrocephalus in low-birth-weight infants.

The paper weighs the indication and the timing of surgery in hydrocephalus mostly due to ventricular haemorrhage in premature infants with an average of 31 weeks of gestational age and of 1.409 g average birth weight. The authors suggest to wait with the intervention until the intraventricular haemorrhage has subsided because the hypertension may often cease when the CSF is not bloody anymore. In case of a progressive process they gained favourable results with early surgery. They give a detailed account about the choice of the various shunts, list eventual complications, the US and CT controls.

Treatment of malignant scala posterior brain tumors in children: the chemotherapy of relapsed medulloblastoma with a dibromdulcitol containing drug regime and pharmacokinetic studies of dibromdulcitol in children.

Dibromdulcitol (Elobromol) has favorable pharmacokinetic parameters for the treatment of brain tumors: high spinal fluid/plasma ratio and long half-life in spinal fluid. Oral application makes its administration easy. The drug combination vincristine, procarbazine, and dibromdulcitol proved to be effective in a pilot trial on relapsed medulloblastomas: 8 complete and 4 partial remissions were achieved from 16 cases. The main side effect was granulocytopenia, which was in some cases severe. However, in the dose-schedule we used it did not delay the treatment longer than 1 week.

Scintigraphic study of cerebrospinal fluid shunts.

This paper deals with experience gained in the study of patency of different types of shunt. Results of subsequent surgery are considered. Findings of 79 scintigraphic studies with radiotechnetium on 68 patients are presented. The method is simple, quick, easy to reproduce and means only moderate radiation load for the patient. Results obtained suggest that this study is a reliable diagnostic tool which can be used in a routine way.

Laminectomy or laminotomy?

After laminectomy in infants and children vertebral deformities often develop. To prevent these the author applied the reanchoring of the dissected arches and ligaments (laminotomy) with success in four children. The details of the procedure and the reanchoring of the vertebral arches are described. This treatment modality should be favourized instead of the destructive laminectomy in this age group in the majority of cases.

[Vaginal delivery following active transabdominal treatment of a prenatally diagnosed fetal hydrocephalus]. / Vaginale Entbindung nach aktiver transabdominaler Behandlung eines pränatal diagnostizierten fetalen Hydrozephalus.

In a case of fetal hydrocephalus the authors repeatedly removed cerebrospinal fluid from the ventricles, transabdominally, by continuous pressure monitoring. Their aim was partly to improve the future functional prospects of the fetus, partly, by reducing the head circumference, to make possible a spontaneous and rapid deliver. After the period of adaptation a ventriculo-peritoneal shunt was implanted.

Ultrasound control of progressive hydrocephalus in infancy.

The authors conducted multidimensional echotomography on 119 infants to determine hydrocephalus and other disorders. The process proved quite reliable in showing changes in ventricle size. They raise the idea of conducting broad scale preventive screening among infants in danger groups to achieve early diagnosis.

Cerebrovascular occlusion in childhood.

Among 2493 patients undergoing cerebral angiography 43 children were found to have cerebrovascular occlusion. The diagnosis, aetiology, management and care of the condition is outlined. It is stressed that angiography is indispensable in diagnosis.

[Rhythmic correlations between regional intracranial blood volume and intracranial pressure within minutes during continuous simultaneous recording in infants]. / Rhythmische Korrelationen des regionalen intrakraniellen Blutvolumens (rICBV) mit dem intrakraniellen Druck (ICP) im Minutenbereich während kontinuierlicher Simultanregistrierung beim Säugling.

In infants with severe neurologic illnesses, rhythms found during continuous simultaneous recording of the rICBV and the ICP and their correlation with each other were described and supported by representations of the original curves. There were relations of the rICBV with the cardiac and respiratory rhythmus, with the so-called C- and B-waves, and clinical modes of behaviour. By using different electronic "frequency windows" and different recording speeds, a variety of relations with the clinical behaviour could be established. As a rule, several rhythms occurred at the same time. Problems and possibilities of the use of rhythmic processes for clinical diagnosis were discussed.