Neurodevelopmental outcome related to cerebral risk factors in children after neonatal arterial switch operation.

BACKGROUND: Neurodevelopmental outcome after neonatal arterial switch operation for complete transposition of the great arteries is an important topic needing prospective assessment. METHODS: A group of 33 unselected children (3.0 to 4.6 years) operated on as neonates with combined deep hypothermic circulatory arrest and low flow cardiopulmonary bypass and a control group of 32 age-matched healthy children (3.0 to 4.8 years) underwent evaluation of socioeconomic and clinical neurological status and a standardized test comprising all areas of child development. Results of patients were related to those of the control group, to population norms, and to preoperative, perioperative, and postoperative cerebral risk factors. RESULTS: Clinical neurological status was normal in 26 patients (78.8%) and reduced in 7 (21.2%). Complete developmental score and the subscores for motor function, visual perception, learning and memory, cognitive function, language, and socioemotional functions were not different compared to population norms. Compared to the patients, the children of the control group scored higher on tests of complete development, cognition, and language, but also on socioeconomic status. Complete developmental score and the scores for motor, cognitive, and language functions were weakly inversely related to the duration of circulatory arrest, but not to the duration of bypass. Cerebral risk factors such as serum levels of the neuron-specific enolase, perinatal acidosis, perinatal asphyxia, peri- and postoperative cardiocirculatory insufficiency, or clinical seizures were not correlated to the test results. CONCLUSIONS: Neonatal arterial switch operation with combined circulatory arrest and low flow bypass is associated with neurological impairment, but not with reduced development as assessed by formal testing of motor, cognitive, language, and behavioral functions. Perioperative serum level of the neuron-specific enolase is not a valid marker for later developmental impairment.

Cerebellar ataxia. Clinical and CT findings in two cases of rare etiology.

Two low density lesions of the cerebellum which did not occupy space are reported. The neuropathological diagnosis of one case was subacute leucencephalitis. In the other case, the diagnosis of a Pelizaeus-Merzbacher's disease is discussed, taking into consideration the clinical observation, development and the CT findings.

[Tuberous sclerosis and organic bipolar disorder in a 15-year-old adolescent]. / Tuberöse Sklerose und organische bipolare Störung bei einer 15jährigen Jugendlichen.

We present the case of a 15-year-old girl with tuberous sclerosis who developed rapid cycling bipolar disorder under treatment with the anticonsulvant oxcarbazepine. Because of first occurrence of grand mal-seizures combined treatment with Valproate became necessary, but failed to prevent relapses of bipolar disorder. Mood stabilization was noticed under additional treatment with lithium, which was complicated by an increase of serum potassium.

[Copper and zinc in cerebrospinal fluid of children with neurological diseases (author's transl)]. / Die Spurenelemente Kupfer und Zink im Liquor von Kindern mit neurologischen Erkrankungen.

We examined CSF copper and zinc concentrations in 30 children with acute febrile illness and meningism (control group) and in 37 patients with shortlasting seizures, febrile or not, and acute viral meningitis. The trace elements were quantitatively measured by means of atomic absorption spectrophotometry. 1. It was shown that the concentrations of copper and zinc in CSF remain constant during childhood.--2. No increase could be found in the concentrations of copper and zinc in CSF caused by the neurological diseases of our patients. These data suggest that permanent cerebral lesions as a consequence of shortlasting seizures and viral meningitis would be very unlikely and that a transient dysfunction of metabolism does not liberate copper- and zinc-metallo-proteins in CSF. 3. There was no correlation between the protein concentration in CSF and copper and zinc concentrations in CSF.

[Spinal epidural abscess in an 11-year-old boy (author's transl)]. / Spinaler Epiduralabszess bei einem 11 Jahre alten Jungen.

Spinal epidural abscesses can occur in childhood. Neurological defects are late manifestations in the course of this disease. Recognition of the early symptoms followed by immediate neurosurgical intervention can prevent these defects. This report describes the course of an epidural abscess in an 11 year old boy. The painful febrile spinal syndrome of the cervical column was the outpointing initial symptom. Lateron disturbances of the autonomic nerve function appeared, such as acute abdomen. The differential diagnosis of the early symptoms is discussed.

[Computertomographical details of neurological syndromes in the newborn period (author's transl)]. / Computertomographische Detailhinweise bei neurologischen Funktionsstörungen des ZNS in der Neugeborenenperiode.

57 premature and full-term newborn infants suffering from a perinatal asphyxia were neurologically and computertomographically examined during their first two weeks of life. No pathognomonical morphological patterns were found comparing the neurological syndromes with the computertomographic results. However, characteristical morphologic findings were obtained related to gestational age and typical neurological syndromes of the newborn period. The cranial computerized tomography permits in many cases a localized topical diagnosis and early prognosis. These morphological findings can have therapeutical consequences.

[Computerized tomography in neurodegenerative diseases in childhood (author's transl)]. / Computertomographische Befunde bei neurodegenerative Erkrankungen im Kindesalter.

The early diagnosis of neurodegenerative diseases in childhood is of great interest for genetic counseling. The development of cranial computerized tomography (CCT) has led to an improvement in the recognition of progressive neurological disorders. On one hand CCT is of importance in distinguishing the neurodegenerative diseases from perinatally acquired cerebral lesions. On the other hand CCT permits the differentiation from degenerations of cerebral gray and white matter. In this connection repeated controls with CCT are of great significance. 4 characteristic case studies are described.

Chromosomal investigations in children with pyknolepsy on dipropylacetate monotherapy.

We analyzed chromosome preparations obtained from leukocytes of ten children with pyknolepsy and treated with dipropylacetate (DPA) as monotherapy. Structural or numerical aberrations were not found more frequently in our patients than in five normal children (control group).

[Benign intracranial hypertension in childhood following mastoiditis (author's transl)]. / Benigne intrakranielle Drucksteigerung bei Mastoiditis im Kindesalter.

Case report concerning a three years old boy with benign intracranial hypertension. The typical symptoms are results of increased intracranial pressure, associated with headache, vomiting, choked disk, occasionally dehiscence of cranial sutures and sixth nerve paresis. In computed tomography there were no tumor signs. In childhood a possible cause in thrombosis of the lateral sinus following an occult mastoiditis after antibiotic therapy. Mastoidectomy, intensive antibiotic therapy as well as serial lumbar punctures led to complete recovery in most cases.

Hypoxic ischaemic encephalopathy: correlation between ultrasound and computed tomography.

Hypoxic ischaemic encephalopathy (HIE) is a major cause of motor and mental retardation. There ist no doubt that the haemorrhagic form of HIE can be detected by cerebral sonography, but it is of great interest to recognize non-haemorrhagic HIE as well. We demonstrate the follow-up of three patients with different types of non-haemorrhagic HIE: Periventricular leukomalacia in a critical ill premature infant 35 weeks gestational age, multicystic encephalopathy in a term newborn with severe perinatal asphyxia and circumscript ischaemic leukomalacia in a five month old infant with near miss event for sudden infant death. Correlation between ultrasound and computed tomography proved that non-haemorrhagic HIE produces global or circumscript high echogenicity in the first week after the hypoxic event whereas computed tomography shows pathologic hypodensity in the same areas. Cerebral sonography is a very helpful and harmless method for the difficult diagnosis HIE. In the case of periventricular leukomalacia, CT scan gives no further information and can be avoided. Concerning HIE of term newborns and small infants, CT scan remains necessary, to evaluate the extension of cerebral injury.

Predominant sensory Guillain-Barré syndrome in hereditary sensory neuropathy--case report of a three-year-old boy.

We observed a three-year-old boy with increasing ataxia over a period of two weeks, which cleared up completely within four months. The electroneurographic features and their course in the child and the father allow us to suspect the additional existence of a hereditary sensory neuropathy. The authors put forward the hypothesis that the genetic disturbance of the sensory nerves is responsible for this clinical variant of Guillain-Barré syndrome.

[The significance of cranial computerized tomography for diagnosis and therapy of inflammatory diseases of the brain and meninges in children (author's transl)]. / Die Bedeutung der kranialen Computertomographie für die Diagnostik und Therapie entzündlicher Erkrankungen des ZNS im Kindesalter.

The significance of cranial computerized tomography (CCT) for the diagnosis and therapy of inflammatory diseases of the central nervous system in children is discussed in connection with five characteristic case studies. CCT is shown to be superior to classical neuroradiological approaches, and to allow important diagnostic insights: 1. the early recognition of diffuse brain edema and the resulting possibility of an early begin of therapy--2. the pathological expansions of the cerebral ventricles of various etiology before a pathological enlargement of the head can be detected, and the size of the ventricles after neurosurgical therapy can be measured--3. the early recognition of space-occupying inflammatory complications.

[Cranial computerized tomography of neonatal encephalopathies. Initial and follow-up studies (author's transl)]. / Computertomographische untersuchungen bei enzephalopathien in der neugeborenenperiode: ausgangsbefunde und verlaufsbeobachtungen.

60 premature and newborn infants with clinical evidence of hypoxia or traumatic encephalopathy were examined by cranial computerized tomography (CCT) during the first fortnight of life and their findings compared with those of a "control group", consisting of 7 infants with malformations. 48 patients showed pathologic findings in the initial CCT. With regard to type, topography and extension, two groups with two subgroups could be outlined: 1. lesions with low density due to hypoxic-necrotizing alterations. a) Bilateral in the white matter around the frontal and occipital horns of the lateral ventricles in both, premature and fullterm newborn infants. b) Corresponding to vascular distribution, focal or global, involving both gray and white matter in both, premature and newborn infants. 2. lesions with high density due to hemorrhages. a) Subependymal and intraventricular, mainly in asphyxiated premature infants. b) Subdural and intracerebral, probably of traumatic origin, involving premature and fullterm newborn infants. The morphological findings in the initial CCT were compared with the outcome in each case. Thus, it was possible to distinguish certain morphological patterns significantly associated with prognosis. 14 patients (23.3%) died in the newborn period. The surviving 46 children (76.7%) were at least once re-examined by CCT and followed up during 6-24 months. 16 patients (26.7%) had a normal development. 12 (20%) showed developmental retardation. 18 (30%) suffered from neurological sequela. Frequently the early follow-up CCT showed characteristic patterns. We believe, that the great number of pathologic findings with essential information warrant the application of CCT in premature and fullterm newborn infants with persistent neurological signs. Perhaps our CCT observations will lead to the consequence of a more controlled high care regimen.

[Early prognosis of cerebral haemorrhage in premature infants with birth weights less than 1500 g. A clinical and computed tomographic study (author's transl)]. / Zur Frühprognose von Hirnblutungen bei Frühgeborenen unter 1500 g. Eine klinische und computertomographische Untersuchung.

22 premature infants with a birth weight less than 1500 g were studied by computed tomography. All of them needed intensive care therapy and had symptoms, which may have been caused by cerebral haemorrhage. In 15 cases (= 68%) we found subependymal and intraventricular bleeding. Clinical data showed that ventilated premature infants with subependymal and intraventricular haemorrhage needed respiratory support in the first hours p.p. because of hyaline membrane and aspiration syndroms. Premature infants without cerebral haemorrhage were ventilated much later (mean 96 h. p.p.). All patients with intraventricular haemorrhage and ventricular enlargement with and without parenchymal haemorrhage (grade III and IV) died. Prematures with subependymal haemorrhage (grad I) and intraventricular haemorrhage without ventricular dilation (grade II) may survive, depending on eventually present pulmonary complications. The introduction of computed tomography in the examination of the CNS on prematures can therefore help to give an early prognosis.

[Computed tomography in subdural effusions of infancy (author's transl)]. / Die Computertomographie in der Diagnostik des subduralen Ergusses.

31 out of 128 infants with abnormal neurological signs show areas of decreased radiodensity of computed tomography (CT) over the cerebral hemispheres, primarily frontal. It is emphasized, that the diagnosis of subdural effusions is permitted in cases with decreased density over the frontal lobes and hardly visible cerebral sulci as well as wedge-shaped widening of the interhemispheric fissure. But in cases with decreased density over the frontal regions and prominent cerebral sulci a subdural effusion cannot be excluded. In association with signs of increased intracranial pressure these CT findings are yet indicative of subdural effusion. Are there to delimitate two spaces of different density between skull and cortex the diagnosis of subdural effusion is certain already by means of CT-morphological criteria. The therapeutic regimen is dependent upon the clinical symptoms.

[Fibromuscular dysplasia of the internal carotid and intracerebral arteries (author's transl)]. / Fibromuskuläre Dysplasie der Corotis interna und der intrazerebralen Gefässe

Fibromuscular dysplasia was found in six patients (four women over 50 years, a 20-year-old man and a five-year-old girl). Angiography (in five cases performed because of a cerebrovascular accident) gave the typical appearance of "knotted string" changes in the large and medium sized arteries. With marked localized arterial narrowing treatment is surgical. Conservative treatment remains sympotomatic because the cause of the disease is still unknown. Even surgical treatment brings only sympotomatic relief and, because of the slowly progressive nature, is indicated only when there are neurological signs.

[Endogenous foreign body meningitis due to a spinal epidermoid]. / Endogene Fremdkörpermeningitis bei spinalem Epidermoid.

We present a two year old child with recurrent aseptic meningitis due to a spinal epidermoid in the subdural space. A dermal sinus had been operated earlier in this child. The connection between dermal sinus and spinal epidermoid is demonstrated. Computed tomography (CT) examination should be used as a screening in all patients with congenital dermal sinus with special reference to visualizing anatomical details.

[How effective is the treatment schedule in children with infantile spasms?]. / Wie begründet ist die Stufentherapie bei Kindern mit BNS-Leiden?

A fatal incident during combined anticonvulsive therapy with ACTH and Dexamethason prompted us to reevaluate our treatment results in 19 children suffering from infantile spasms. We followed Doose's recommendation using Clonazepam as a means of first choice. Failing that, than Dexamethason and Primidone was used. A combination of ACTH, Dexamethason and Primidone was administered to patients failing both treatment courses. Our results question the necessity, the tolerance limit and the efficacy of this treatment schedule. Therefore we propose a prospective multicentric study to reinvestigate the therapeutic guideliness for infantile spasms.