Long-term outcome of children with acute cerebellitis.

BACKGROUND: Acute cerebellitis (AC) is characterized by cerebellar symptoms and magnetic resonance imaging (MRI) changes primarily confined to the cerebellum. OBJECTIVE: To analyze the neurological and cognitive long-term outcome of children with AC. METHODS: Children with AC diagnosed by typical clinical features and MRI findings were included in this retrospective study. Medical charts were reviewed and neurological deficits were assessed by neurological examination or by the expanded disability status scale telephone interview. Cognitive outcome was evaluated with a parental questionnaire (Kognitive Probleme bei Kindern und Jugendlichen). RESULTS: A total of 11 children (6 boys, 5 girls; age range: 3 years to 14 years and 10 months) were included. Of them, six children had a severe disease manifestation including mental status changes and neurological symptoms. Of the rest, two children had a moderate and three children had a mild form of AC. MRI of the cerebellum was obtained in the acute phase revealing signal alterations with different patterns. The average follow-up period was 4 years and 4 months. A complete recovery was observed in five children. Neurological sequelae were reported in five children ranging from ataxia to mild tremor. Cognitive deficits were found in six patients. The affected areas of cognition did include spatial visualization ability, language skills, and concentration. CONCLUSION: Neurological and cognitive sequelae are common in children with AC and underline the role of the cerebellum in cognition.

MR imaging findings in patients with secondary intracranial hypertension.

BACKGROUND AND PURPOSE: IH can alter the configuration of anatomic structures of the central nervous system. We determined the sensitivity and specificity of MR imaging to detect these changes in patients with secondary IH. MATERIALS AND METHODS: Patients (n = 36) with IH were prospectively investigated with MR imaging and were matched to 36 controls. MR images were evaluated for elongation and edema of the optic nerves, protrusion of the optic disc, flattening of the posterior sclera, height of the pituitary gland, and width of the optic nerve sheath. On MRV, we recorded venous sinus abnormalities and measured the luminal width of the superior ophthalmic veins. A grading score was introduced to define cranial venous outflow obstruction. RESULTS: Cranial venous outflow obstruction and ONS hydrops were the most valid signs indicating IH with a sensitivity of 94% and 92% and a specificity of 100% and 89%, respectively. Sensitivities and specificities were 56% and 97% for reduced pituitary height, 64% and 78% for flattening of the posterior sclera, 31% and 97% for widening of the superior ophthalmic veins, 33% and 100% for optic disc protrusion, 14% and 100% for optic nerve edema, and 6% and 100% for elongation of the optic nerve. At least 2 MR imaging findings could be demonstrated in each patient but in none of the controls. The number of positive MR imaging findings correlated with CSF pressure (r = 0.62, P = .01). CONCLUSIONS: The combination of cranial and orbital MR imaging and MRV can be highly sensitive and specific in the diagnosis of patients with IH.

MR imaging of the optic nerve sheath in patients with craniospinal hypotension.

BACKGROUND AND PURPOSE: Craniospinal hyper- or hypotension leads to morphologic changes in certain intracranial structures. We tested the hypothesis that the amount of CSF in the ONS visible in MR imaging is reduced in patients with CSH. MATERIALS AND METHODS: Nineteen patients with CSH were prospectively studied. Three readers assessed the width of the peri-optical CSF rim at 4 different anatomic positions by using coronal STIR sequences from a 3T MR imaging scanner. The height of the pituitary gland was also measured. Results were compared with normal values obtained with the same imaging technique. Qualitative signs of CSH also recorded were engorgement of venous sinuses, dural enhancement, subdural effusion, narrow ventricles, and sagging brain. RESULTS: CSF signal intensity surrounding the optic nerves was diminished in at least 2 of the 4 positions used for measurements so that decreased diameters of the ONSs were observed in all patients (sensitivity, 100%; specificity, 97%). The height of the pituitary gland was above normal limits in 12 of 19 patients (sensitivity, 63%; specificity, 97%). Frequencies of qualitative signs of CSH varied from 32% to 81%. CONCLUSIONS: The ISSON in patients with CSH is partially or fully collapsed due to reduced CSF content. In comparison with other anatomic markers, this sign showed the highest sensitivity for the diagnosis of patients with CSH in this study.

[Gaussian distribution of intelligence in VLBW preterm infants at age 5: very low correlation with very low birth weight]. / Normalverteilung der Intelligenz von VLBW-Frühgeborenen mit 5 Jahren: sehr geringe Korrelation mit sehr geringem Geburtsgewicht.

BACKGROUND: Preterm infants with very low birth weight < 1500 g (VLBW) have a higher risk of developmental disorders. In addition to the common estimation of the mean intelligence values, we studied the distribution of intelligence at preschool age in VLBW infants and the risk factors influencing this distribution. PATIENTS AND METHODS: A prospective cohort study of 277 VLBW infants < 32 weeks born in 1991-1995 and treated according to a standardized regimen in one Perinatal Center was carried out, including measurement of intelligence (Kaufman-Assessment Battery for Children) at age 5. Statistical methods employed were: explorative data analysis, correlation, chi (2)- and t-tests; the tested variables were: small for gestational age (< third percentile), perinatal acidemia (umbilical arterial pH < 7.10), perinatal hypoxia (BE < - 10), hypothermia (< 36 degrees C), hypoglycemia after the first day of life (< 30 mg / dL), bronchopulmonary dysplasia (FiO (2) > 0.21 > or = 36 weeks), intraventricular hemorrhage, ventricular dilation, periventricular leukomalacia, seizures, abnormal acoustic evoked potentials, and hyperexcitability at discharge. RESULTS: The distribution of intelligence in 137 VLBW infants < 32 weeks (60 % follow-up rate) was similar to a symmetrical Gaussian bell curve. The intelligence increased very slightly with birth weight (Pearson correlation: 0.172; p = 0.045) and was significantly lower in children with hypoglycemia after the first day of life (- 13.35; 95 % confidence interval: - 20.08 to - 6.63; p = 0.002), hyperexcitability at discharge (- 16.28; 95 % confidence interval: - 25.26 to - 7.31; p = 0.005), and bronchopulmonary dysplasia (- 7.00; 95 % confidence interval - 11.71 to - 2.29; p = 0.039). CONCLUSIONS: At preschool age, the intelligence of VLBW infants is normally distributed and correlates only slightly with the very low birth weight. Hypoglycemia after the first day of life and bronchopulmonary dysplasia are risk factors for lower intelligence. Hyperexcitability at discharge seemed to represent a promising prognostic factor for a later intelligence reduction.

[Caudal regression syndrome - caudal agenesis]. / Kaudales Regressionssyndrom - kaudale Agenesie.

BACKGROUND: Neural tube defects are caused by complex genetic and environmental factors. The congenital anomaly most specific to pregnant women with diabetes mellitus is caudal regression syndrome. PATIENT: A 4-year-old boy with a history of mild delay in motor development presented with primary enuresis and encopresis. On physical examination, he had no sensory and motor deficits, but a short anal cleft. On questioning, the mother reported insulin-dependent diabetes mellitus during pregnancy. MRI of the spinal cord demonstrated a thoracic syringomyelia, a dysplastic conus medullaris, and an absence of coccyx and distal sacrum, called caudal regression syndrome or caudal agenesis. CONCLUSION: The caudal regression syndrome refers to sacral agenesis associated with spinal cord anomalies, e.g. syringomyelia. Sacral agenesis is marked by total absence of the coccyx and total or distal absence of the sacrum. An abnormal backside combined with a history of maternal diabetes mellitus in pregnancy is highly suggestive for the presence of caudal regression syndrome.

[Non-haemorrhagic subependymal pseudocysts: ultrasonographic, histological and pathogenetic variability]. / Nichthämorrhagische subependymale Pseudozysten: sonografische, histologische und pathogenetische Variabilität.

PURPOSE: Differentiation of non-haemorrhagic subependymal pseudocysts from subependymal pseudocysts after cerebral haemorrhage in the preterm infant. MATERIALS AND METHODS: Selective ultrasonographic screening in 2200 neonates and retrospective analysis in 72 infants with subependymal pseudocysts, the full-term infants being analysed from birth, and the preterm infants after the second week of life, thus avoiding the gestational age at which cerebral haemorrhage occurs in the preterm infant. RESULTS: Three variants of pseudocysts were identified: the caudothalamic germinolysis is a leukomalacic and pseudocystic gliosis. Pseudocysts in the anterior choroid plexus of the lateral ventricle could be distinguished from caudothalamic germinolysis by their location, form and movement. Pseudocysts lateral of the frontal horns are the result of regression of germinal matrix remains. CONCLUSION: Differentiation of non-haemorrhagic from post-haemorrhagic germinolysis is necessary to clarify the aetiology and pathogenesis of non-haemorrhagic pseudocysts. Caudothalamic germinolysis possibly is the result of infection with stenotic intima proliferation following vasculitis. The results are thalamostriatal vasculopathy and germinal necrosis. Anterior plexus cysts might be the result of folding faults of the ependyma in the growth period of the choroid plexus. Pseudocysts lateral of the frontal horns should not be mistaken for ventricular ligaments.

Infant with unusually large choroid plexus papilloma undergoing emergency surgery. Case report with special emphasis on the surgical strategy.

Choroid plexus papillomas are one of the most common tumors of the nervous system in infants. The most frequent early symptoms, megalocephalia and vomiting, caused by elevated intracranial pressure often lead to a diagnosis only at a critical clinical stage. This study describes a case of a 3-month-old infant with a choroid plexus papilloma measuring 7 x 8 x 6 cm originating in the right lateral ventricle. The infant underwent emergency surgery in an acutely deteriorated state, i.e., acute herniation symptoms with fixed and dilated pupils. Despite of the size of the tumor, the proximity to eloquent cortex, and clinically deteriorated state, the infant recovered completely.

Prenatal diagnosis of isolated foramen ovale obstruction. A report of two cases.

Premature obstruction of the foramen ovale is a rare but serious clinical entity. Obstruction can be associated with right ventricular failure, fetal hydrops, tricuspid regurgitation, left heart obstructive defects and supraventricular tachycardia. In most cases, the diagnosis is made at the postmortem examination. The etiology is unknown. We discuss 2 prenatal cases with the sole diagnosis of ventricular asymmetry. The right ventricle was dilated and hypertrophic, and the foramen ovale ballooned into the left atrium without color Doppler detection of right-to-left flow. After delivery, the infants had no hydrops or symptoms of cardiac or respiratory distress. Echocardiography demonstrated a structurally normal heart with increased right ventricle dimensions until the 7th day of life. Delivery should be induced if possible in cases of foramen ovale obstruction with signs of cardiac decompensation.

[Neonatal chylothorax with trisomy 21]. / Neonataler Chylothorax bei Trisomie 21.

Neonatal chylothorax is an uncommon cause of respiratory distress in the newborn. It may result from anomalous lymph drainage with or without association with aneuploidy syndromes (trisomy, Turner's syndrome, Noonan's syndrome), from injury to the thoracic duct, and from obstruction of the superior vena cava. We report a term newborn and a premature infant with neonatal chylothorax, both associated with trisomy 21. In the case of the term infant, bilateral pleural effusions were diagnosed immediately before birth. The baby suffered from respiratory distress. The physical findings were characteristic of trisomy 21. In the premature infant (gestational age 24 weeks, 735 g) the ductus arteriosus was ligated on day nine after birth. Four days after surgery a central venous line was inserted via the left vena mediana cubiti into the left vena subclavia. Nine days after surgery a left-sided chylothorax occurred. The infant had subtle signs of a trisomy 21 (slightly enlarged tongue, brachycephalic head. questionable simian crease). In both children, cytogenetic studies were done and confirmed the diagnosis of trisomy 21. Infants with neonatal chylothorax should be carefully evaluated for dysmorphic signs of a trisomy or monosomy. Premature infants may present with only subtle clinical signs requiring cytogenetic studies to confirm an aneuploidy syndrome.

Mathematical modelling of growth and substrate conversion of Zymomonas mobilis at 30 and 35 degrees C.

Zymomonas mobilis was grown in continuous cultures at 30 and 35 degrees C. The specific substrate consumption rates at 35 degrees C were higher than those at 30 degrees C. An unstructured mathematical model based on the linear equation for substrate consumption provided a statistically adequate description for cultures grown at 35 degrees C but not for cultures grown at 30 degrees C. A structured two-compartment model described growth and substrate consumption well at both temperatures. Some theoretical and practical aspects of the two-compartment model are discussed.