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BACKGROUND: Porencephalic cysts are brain cavities resulting from perinatal vascular occlusion and are commonly associated with severe neurological deficits and medically intractable epilepsy. Thirty-seven children presenting to the University of Münster with intractable seizures because of a porencephalic cyst were treated by uncapping and fenestration of these cysts to the lateral ventricle between 1978 and 1992. We conducted the following study to determine the efficacy and safety of the uncapping and fenestration procedure for the treatment of seizures. METHODS: We reviewed all cases retrospectively and assessed the outcome of these patients with regard to seizures, paresis, and perioperative complications. RESULTS: Of 37 children, 23 (62%) were seizure-free postoperatively. Nine patients (24%) had a reduction of seizures and five children (14%) remained unchanged. Of 30 patients with preoperative hemiparesis, 11 (30%) improved after the operation. The leading postoperative problem was a subcutaneous/subgaleal cushion of CSF, which affected 12 children (12 of 37). A dural patch covering the iatrogenic dural defect could not prevent or reduce postoperative CSF leakage, but prolonged the postoperative fever period. Postoperative fever occurred in 36 children (36 of 37) and was caused by an aseptic meningitis. CONCLUSIONS: Children with intractable seizures and porencephalic cysts benefit from uncapping and cyst fenestration to the lateral ventricle. Concomitant perioperative complications are mild and are easily treated.

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Arterio-venous malformations (AVM) of the CNS are considered uncommon lesions in childhood, but is a main cause for subarachnoid haemorrhage (SAH) of children. We report a case of a 14-year-old girl with an AVM localized in the cervical spine: after an acute event with SAH she shows the clinical features of a cervical myelopathy-syndrome. MRI and selective angiography show an AVM, originating from the right vertebral artery. Patients with AVM have a higher incidence of recurrent bleeding with SAH, hematomyelia or infarction and even cord compression. This grave sequel makes it necessary to treat AVM e.g. with embolization.

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We report a boy with benign familial seizures of the sixth month of life. At the age of six months he suffered his first afebrile grand mal seizure. Up to his ninth month several seizures of the same type occurred. EEGs were always normal. The history yielded a paternal hereditary trait: five members of the family had also suffered grand mal seizures in the sixth month of life. All but one had no therapy with antiepileptic drugs. One girl was treated with phenobarbital for two years. All members of the family show normal neurological development. Under low dose therapy with phenobarbital our patient has remained seizure-free up to now.

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We report clinical and neurophysiological findings in six children (three female, three male) with type I lissencephaly and three children (all female) with type II lissencephaly (Walker-Warburg syndrome). In type I lissencephaly the diagnosis is based only on electroencephalographic (EEG) signs, whereas in type II lissencephaly the diagnosis rests on clinical signs. In type I lissencephaly the EEG typically shows high alpha-beta activity, which is not seen in type II lissencephaly.

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In an infant with typical pyridoxine-dependent seizures, CSF GABA level, was determined before treatment with pyridoxine. Before onset of treatment, level of GABA in CSF was highly lowered (16 pmol/ml), pyridoxine level in serum was within normal range. Immediately after application of 80 mg pyridoxine fits stopped and the EEG was without seizure activity. The data substantiate previous findings in brain tissue from a patient with pyridoxine-dependent seizures. They are proof of a disturbed GABA metabolism in pyridoxine dependent seizures.

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Prolactin levels were measured immediately after the seizure in some, and 15 to 20 minutes later in all of 67 children aged between 6 months and 17 years. Values were determined after grand mal, complex partial and petit mal seizures and psychogen seizures. A more than 2 to 3 fold prolactin increase over the baseline value occurred almost always after grand mal and regularly after complex partial seizures. No hyperprolactinaemia was observed after petit mal seizures. Also after psychogenic seizures a rise in serum prolactin failed. The neurophysiological basis underlying this phenomenon is a decrease of gaba- und dopaminergic systems associated with the seizure. The described method is useful in the differential diagnosis of epileptogenic versus psychogenic seizures.

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We report the case of a female patient with a progressive therapy-resistant extrapyramidal movement disorder. Cranial computed tomography showed symetrical hyperdensities in the globus pallidus. The same areas are of decreased signal intensity in magnetic resonance imaging. These findings suggest an increased iron accumulation. The clinical symptoms and the radiological findings of the cerebrum are highly indicative for a Hallervorden-Spatz disease.

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Alternating hemiplegias in children suggest the possibility of a Moyamoya syndrome. In the absence of clinical symptoms it is the EEG which will offer the decisive clue in childhood Moyamoya syndrome. The pertinent finding, which consists in progressive frequency decrease and amplitude activation after hyperventilation, has only been described in pediatric patients. It has been termed re-build-up phenomenon. Magnet resonance imaging and magnet resonance angiography (MR angiography) are able to confirm the tentative diagnosis of a Moyamoya syndrome based on the EEG.

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Corticomotoneuronal latencies after non-invasive stimulation of the motor cortex were studied in two patients with adrenomyeloneuropathy (AMN), in one patient with adrenoleukodystrophy (ALD), in two obligate heterozygotes, and in three clinically not affected sisters of the patients with AMN. Corticomotoneuronal latencies were prolonged in the patients as well as in obligate female carriers, but normal in the clinically and biochemically not affected relatives. The noninvasive cortex stimulation was found to be a sensitive method to detect clinical and subclinical deficits in patients and heterozygous carriers.

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In the last years the Kearns-Sayre-Syndrome has been defined with the typical trias of chronic external ophthalmoplegia, pigmentary retinal dystrophy and cardiac conduction defects. Today it is no longer believed to present an entity but a variant of the multiple plussymptoms of the ophthalmoplegia-plus group. In pediatrics the existence of this clinical disorder is not yet well acknowledged. The case of a 16-year-old patient is used as an example for the impressive clinical symptoms and the involvement of several organs in this clinical disorder. The combination with a Klinefelter-Syndrome has not previously been reported.

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We report on a case of autosomal dominant periodic ataxia. So far, fourteen families have been described with this rare disease. An early diagnosis is important; the patients can be treated effectively with acetazolamide.

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The effect of Valproat on plasma prolactin level in epileptic children with long term medication was investigated. Valproat was able to decrease plasma prolactin level significantly. This result is a sign of an increase in the central GABAergic system activity.

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Prolactin concentration was measured 20 minutes after each seizure in 8 patients with grand mal, 2 patients with complex partial seizures and 5 patients with petit mal seizures. In the group of grand mal and complex partial seizures serum prolactin showed markedly increased levels. After petit mal seizures there was no change in serum prolactin concentration. The possible causes for changes in serum prolactin after a seizure in children are discussed.

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