Global taxonomic, functional, and phylogenetic diversity of bees in apple orchards.

An essential prerequisite to safeguard pollinator species is characterisation of the multifaceted diversity of crop pollinators and identification of the drivers of pollinator community changes across biogeographical gradients. The extent to which intensive agriculture is associated with the homogenisation of biological communities at large spatial scales remains poorly understood. In this study, we investigated diversity drivers for 644 bee species/morphospecies in 177 commercial apple orchards across 33 countries and four global biogeographical biomes. Our findings reveal significant taxonomic dissimilarity among biogeographical zones. Interestingly, despite this dissimilarity, species from different zones share similar higher-level phylogenetic groups and similar ecological and behavioural traits (i.e. functional traits), likely due to habitat filtering caused by perennial monoculture systems managed intensively for crop production. Honey bee species dominated orchard communities, while other managed/manageable and wild species were collected in lower numbers. Moreover, the presence of herbaceous, uncultivated open areas and organic management practices were associated with increased wild bee diversity. Overall, our study sheds light on the importance of large-scale analyses contributing to the emerging fields of functional and phylogenetic diversity, which can be related to ecosystem function to promote biodiversity as a key asset in agroecosystems in the face of global change pressures.

Congenital poisoning by polychlorinated biphenyls and their contaminants in Taiwan.

In 1979, a mass poisoning occurred in Taiwan from cooking oil contaminated by thermally degraded polychlorinated biphenyls. Because these chemicals persist in human tissue, children born to female patients after the outbreak were exposed in utero. In 1985, 117 children born to affected women and 108 unexposed controls were examined and evaluated. The exposed children were shorter and lighter than controls; they had abnormalities of gingiva, skin, nails, teeth, and lungs more frequently than did controls. The exposed children showed delay of developmental milestones, deficits on formal developmental testing, and abnormalities on behavioral assessment. These findings are most consistent with a generalized disorder of ectodermal tissue. This syndrome is one of very few documented to result from transplacental exposure to pollutant chemicals.

Postinfectious encephalomyelitis: etiologic and diagnostic trends.

Fifty cases of postinfectious encephalomyelitis admitted to our Pediatric Department during the period 1980 to 1997 were consecutively collected and reviewed. There were 28 males and 22 females. The age of onset ranged from 9 months to 14 years. The antecedent infections included measles (6 cases), rubella (5 cases), mumps (4 cases), chicken pox (4 cases), Epstein-Barr virus infection (11 cases), mycoplasma infection (6 cases), and unknown etiology (14 cases). The cessation of measles, rubella, and mumps as causes for encephalomyelitis in our patients corresponds with the introduction of a measles-mumps-rubella nationwide vaccination program in Taiwan commencing in 1992. The main clinical symptoms were fever, headache, and/or vomiting, seizure, and motor weakness. The presenting signs included altered consciousness, meningeal signs, cranial nerve palsy, brainstem signs, involuntary movement, and cerebellar signs. Computed tomography scans were abnormal for 14 (56%) of 25 patients studied, whereas magnetic resonance imaging (MRI) disclosed lesions in 14 (82%) of 17 patients, with abnormal signals in various parts of the cerebral hemisphere, as well as in the basal ganglion, diencephalon, midbrain, brain stem, and cerebellum. Of the three patients with negative MRI findings, an abnormal finding on somatosensory evoked potential was noted for one patient, and a focal decrease in tracer uptake on single photon emission computed tomography (SPECT) was found for the other two patients. This study demonstrates that the causative agents of postinfectious encephalomyelitis in Taiwan have changed from those of traditional exanthematous diseases to nonspecific respiratory infections and suggests that this may also be the case in other parts of the world. MRI remains the imaging method of choice, whereas other neurofunctional studies such as evoked potentials and SPECT are complementary for the diagnosis.

Cranial ultrasound in the detection of postmeningitic complications in the neonates.

Serial cranial ultrasound examinations were performed through the anterior fontanel to detect and follow the complications of bacterial meningitis in 16 neonates. The final results included normal findings in 9 patients, and abnormal in the other 7 cases. Among the latter, 5 patients with hydrocephalus were sequentially found after the second week of the disease and the earlier the onset, the larger the ventricular dilation. One ventriculitis showed polycystic loculi with abnormal septa in the advanced stage. Cystic low attenuation lesion with mass effect at a later stage of meningitis specified one patient with brain abscess. Progressive dilatation of ventricular systems without associated growth of head girth disclosed a process of brain atrophy. They had neither obvious neurological signs nor specific CSF findings clinically, but their sonograms showed the abnormal changes which were finally proved by CT scans. The potential value of cranial ultrasound in the detection of post-meningitic complication besides CT scan is stressed.

Auditory brainstem responses in patients with neonatal hyperbilirubinemia and bilirubin encephalopathy.

To understand the neurotoxicity of neonatal hyperbilirubinemia, auditory brainstem response (ABR) studies were performed in 75 jaundiced patients, who were divided into 4 groups in either a retrospective or prospective way. Retrospective ABR study in 10 known kernicteric patients (group I) showed elevation of the hearing threshold and delay of wave I, as well as prolongation of central brainstem transmission, in various degrees, in most cases. Six jaundiced infants (group II) with ABR testing before and after blood exchange transfusion (BET) showed shortening of wave latencies and increase in amplitude after BET. There were additional 20 infants with BET (group III) and 39 with phototherapy (group IV) receiving ABR testing after the therapeutic procedures. Prospective follow-up in groups II, III and IV showed normalization of the ABRs in all except one patient in the following months. These findings demonstrate the nature of bilirubin neurotoxicity and the prognostic value of ABRs in the monitoring of bilirubin toxicity. ABR testing is sensitive in reflecting the effect of hyperbilirubinemia, and provides a valuable guide for the early recognition and close follow-up of bilirubin neurotoxicity.

Multiphasic disseminated encephalomyelitis mimicking multiple sclerosis.

We report a case of multiphasic disseminated encephalomyelitis (MDEM) following viral illness presenting as multiple sclerosis (MS) in a 7-year-old boy. The patients had two episodes of alternating hemiparesis and other neurologic symptoms following viral infection, which were separated by 3 years. Neuroimaging studies demonstrated multiple, discrete, small nodules and large globular lesions in the cerebral white matter, basal ganglia, brainstem and cerebellar areas. Based on typical appearance of magnetic resonance imaging (MRI) and clinical manifestations including systemic symptoms such as fever, nausea, vomiting, headache and seizures followed by consciousness disturbance and other multifocal neurologic signs, the diagnosis of MDEM rather than that of MS was made. Because it is difficult to differentiate between MDEM and MS on the basis of the clinical history, the cerebrospinal fluid examination and evoked potential studies, this report emphasizes that the MRI study of the brain may provide an important clue for the diagnosis.

Topographic mapping and clinical analysis of benign childhood epilepsy with centrotemporal spikes.

We studied the topographic mapping of the electroencephalography (EEG) of 47 children whose clinical history and course were compatible with typical benign childhood epilepsy with centrotemporal spikes (BCECT). Twenty-nine (62%) patients showed typical dipole fields, with a negative potential field in the centrotemporal region and a positive field in the frontal region. Eighteen children did not demonstrate the typical dipole field. Their non-dipole rolandic discharges were localized in small fields of centrotemporal region. The patients with dipole fields in BCECT had significantly less frequent seizures than patients without dipole fields. Twelve of the 47 patients with BCECT (26%) had more than one EEG focus. The clinical courses of patients with multiple foci were not worse than those of patients with a single focus. We conclude that EEG topographic mapping is helpful in identifying typical or atypical EEG topographic patterns in patients with clinically diagnosed BCECT. We also conclude that the presence of dipole field usually indicates a better clinical course of epilepsy and multiple foci do not mean a poor clinical course.

The spectrum of postinfectious encephalomyelitis.

The medical records of 52 consecutive patients diagnosed with postinfectious encephalitis/encephalomyelitis during the period from 1980 to 1998, including 29 males and 23 females, were reviewed. These patients were divided into three groups according to their clinical and neurodiagnostic characteristics: (1) group I: postinfectious encephalitis, 38 patients; (2) group II: acute disseminated encephalomyelitis (ADEM), 13 patients; (3) group III: multiphasic disseminated encephalomyelitis (MDEM), one patient. Fever, headache/vomiting, seizure and disturbance of consciousness were common clinical features in all patients, while pictures of pyramidal, extrapyramidal, brainstem, and spinal cord lesions were more often found in the group II and group III patients than in the group I patients. Magnetic resonance imaging (MRI) revealed abnormal lesions in six (60%) of ten group I patients, but all group II (n=7) and group III (n=1) patients who received MRI study showed abnormal signals in various regions of the brain including the cerebral hemisphere, basal ganglia, brainstem and cerebellum. Patients with ADEM and MDEM had a longer clinical course and more neurological sequelae than group I patients. This study demonstrates the breadth of the clinical spectrum of postinfectious encephalomyelitis. Thorough clinical observations and appropriate neurodiagnostic studies such as MRI are crucial for the diagnosis.

Guillain-Barré syndrome in children: a cooperative study in Taiwan.

Seventy-two children with Guillain-Barré syndrome (GBS), diagnosed at 11 major teaching hospitals in Taiwan during the period 1986-1990, were studied retrospectively. There were 44 males and 28 females ranging in age from 7 months to 15 years. Preceding events could be traced in 61 patients (85%), including antecedent infection in 59 patients and previous vaccination in 2. As well as the consistent pictures of progressive weakness and generalized hyporeflexia, there were sensory complaints (26%), cranial nerve lesions (46%), respiratory failure (14%) and autonomic dysfunction (25%). Motor symptoms reached a maximum within 20 days in 88% of the patients, with the plateau lasting less than 2 weeks in 75%, and became stable within 3 months in 76%. Overall outcome showed complete recovery in 73% of the patients within 6 months after onset. Four (5.6%) had recurrence, and there was no mortality. The present study revealed that the annual incidence of GBS in Taiwan can be estimated roughly as 0.66 per 100,000 and that the course of childhood GBS is relatively benign.

Familial medulloblastoma in siblings: report in one family and review of the literature.

Two siblings in a family--a 5-year-old boy and an 8-year-old girl--suffered from progressive headache and gait disturbance in an interval of 1 year, consecutively. Neurologic manifestations were papilledema and truncal ataxia. Both of their computed tomography scans showed a large, well-enhanced tumor located in the cerebellar vermis with secondary hydrocephalus. Both had surgical resection followed by craniospinal irradiation and then chemotherapy. The pathologic findings confirmed the diagnosis of medulloblastomas. The family pedigree disclosed some other cancer in close relatives. These findings suggested a possible role of heredity in the oncogenesis of this tumor. To our knowledge, our cases are the seventh report of familial medulloblastoma occurring in nontwin siblings in the world.

Risk factors for subsequent epilepsy after febrile convulsions.

To evaluate the risk of subsequent epilepsy after febrile convulsions and the long-term prognosis of such patients, the risk factors for afebrile seizures following initial febrile convulsions were studied in 154 hospitalized children: 122 with simple febrile convulsions and 32 with complex features of febrile convulsions. The mean follow-up period was 7 years 2 months (range, 4 yr - 11 yr 2 mo). Nineteen patients (12.3%) developed subsequent epilepsy in the follow-up period. The seizure types in patients with subsequent epilepsy following febrile convulsions included generalized seizures in seven patients, complex partial seizures in five patients, partial seizures evolving to secondary generalized seizures in six patients and benign childhood epilepsy with centrotemporal spike in one patient. The occurrence of epilepsy was strongly associated with complex features of febrile convulsions, pre-existing neurodevelopmental abnormalities, family history of epilepsy, and abnormal electroencephalographic findings. However, the number of recurrences of febrile convulsions, sex, family history of febrile convulsions, age of onset and long-term prophylactic use of anticonvulsants for febrile convulsions were not significant factors for subsequent epilepsy. This study demonstrates the importance of identifying the risk factors for subsequent afebrile seizures after febrile convulsions.

Cerebral blood flow velocity in newborn infants with asphyxia.

A pulsed-Doppler real-time technique was used to assess the anterior cerebral artery (ACA) flow velocity in 30 asphyxiated infants during the first 5 days after birth. Thirty healthy term infants were also studied as controls. Peak systolic flow velocity (PSFV), end diastolic flow velocity (EDFV), mean flow velocity (MFV), and pulsatility index (PI) were measured. Mean PI values of the ACA of the asphyxiated and control groups were 0.63 +/- 0.05 vs 0.71 +/- 0.04 (p less than 0.001) on the first day; 0.64 +/- 0.03 vs 0.70 +/- 0.05 (p less than 0.001) on the third day and 0.69 +/- 0.04 vs 0.70 +/- 0.04 (p greater than 0.05) on the fifth day of life. The asphyxiated group also had significantly higher EDFV and MFV than those of control group until the 5th day of life. The low PI values of asphyxiated infants were mainly due to an increase in EDFV. These results suggest that the cerebrovascular resistance of asphyxiated infants, as reflected by the PI, is low during the first 3 days of life, indicating an increase in cerebral blood flow. With this technique, we can repeatedly and safely evaluate the cerebral hemodynamic changes in asphyxiated newborn infants.

Subacute necrotizing encephalomyelopathy (Leigh's disease): report of a case.

A 5-month-old male infant was referred to us for evaluation of progressive hypotonia. He had growth retardation, feeding difficulty and general weakness. Brain sonography and computed tomography demonstrated symmetrical lesions in the caudate, lenticular nuclei, thalamus and hypothalamus, suggesting bilateral necrosis. Lactate and pyruvate levels in the blood and cerebral spinal fluid were persistently elevated. A biopsy of the quadriceps muscle showed increased subsarcolemmal mitochondrial enzyme activity on light microscopy. Electron microscopy of the muscle showed deformed and bizarre mitochondria. The patient eventually died at the age of 8 months. Autopsy showed bilateral necrotic foci in the caudate, lenticular nuclei, thalamus, hypothalamus, midbrain, and pons. Histopathologic findings included spongiform degeneration of the affected brain tissue. The characteristic clinical and pathological findings confirmed this case as subacute necrotizing encephalo-myelopathy of Leigh's type. To our knowledge, this is the first autopsy-proven case of Leigh's disease in Taiwan.

Diagnostic value of cerebrospinal fluid immunoglobulin G (IgG) in pediatric neurological diseases.

A total of 71 cerebrospinal fluid (CSF) specimens with paired serum samples from 16 controls and 55 patients with various neurologic diseases ranging in age from 1 to 15 years old were studied using a rate nephelometer to measure their immunoglobulin G (IgG) and albumin levels in both their CSF and serum. The patients included 15 cases of encephalitis, 6 cases of purulent meningitis, 20 cases of aseptic meningitis, 2 cases of multiple sclerosis and 12 cases of other noninflammatory neurologic diseases. The values of the CSF IgG quotient, IgG index, and de novo central nervous system (CNS) IgG synthesis were then calculated for each subject. In the control group, the mean values were as follows: CSF IgG, 1.45 mg/dl; IgG quotient, 6.2%; IgG index, 0.55; and de novo IgG synthesis, -1.17 mg/day. Those with CNS inflammatory diseases had significantly higher CSF IgG levels and IgG quotients compared with controls (p less than 0.05). The IgG index was selectively elevated in cases of encephalitis (p less than 0.005). The values for de novo IgG synthesis showed significant increase in all types of CNS inflammatory diseases except for the group with aseptic meningitis. In the noninflammatory diseases, none of the CSF parameters revealed any significant differences from those of the control group. Six cases (40%) of encephalitis showed normal CSF protein value, but an abnormal IgG component, IgG index and de novo IgG synthesis. Quantitation of IgG and other derivatives in CSF gives a sensitive supplementary reference for the detection of CNS infection.

Blood-brain barrier damage in children with central nervous system infections.

The blood-brain barrier (BBB) function in childhood central nervous system (CNS) infections was evaluated, using cerebrospinal fluid (CSF) and paired serum samples from 30 controls and 74 patients aged from 1 to 15 years with various CNS infections. The 74 patients included 34 cases of aseptic meningitis, 24 of encephalitis and 16 of purulent meningitis. The degree of BBB damage was graded by CSF/serum albumin ratio, and IgG production by IgG index (IgG ratio/albumin ratio of CSF to serum). In the acute stage, patients with purulent meningitis had greater elevation of the albumin ratio than other study groups. The patients with encephalitis, especially chronic encephalitis, had a selectively elevated IgG index compared to other groups. Most patients with aseptic meningitis showed little or no elevation of albumin ratio and IgG index. This study demonstrated the nature of various degrees of BBB damage and intra-BBB IgG production in different infectious CNS diseases in children. BBB damage is most severe in cases of purulent meningitis, while the intrathecal IgG production is greatest in chronic encephalitis. These abnormalities disappear as the inflammatory processes subside.

Spontaneous closure of ventricular septal defects in the first year of life.

BACKGROUND AND PURPOSE: The recent increase in the prevalence of ventricular septal defect (VSD) has been ascribed to the improved detection of small defects with echocardiography and the wider use of screening. The aim of this study was to determine the prevalence and timing of spontaneous closure of specific types of VSD in neonates using echocardiographic screening and follow-up. METHODS: Two-dimensional color Doppler echocardiography was performed in 3,472 clinically normal full-term neonates born at Cathay General Hospital to detect isolated VSD. The relative prevalence of muscular versus perimembranous defects and their outcome in the first year of life were evaluated. RESULTS: VSD was found in 74 neonates (34 male, 40 female), resulting in a prevalence of 21.3/1,000 live births. There were 48 muscular, 25 perimembranous, and one subpulmonic defects. Of the 74 patients, 11 were lost to follow-up. Within the observation period of 12 months, spontaneous closure occurred in 40 patients in the muscular group and in six patients in the perimembranous group. The overall rate of spontaneous closure was 73% by the end of the first year. Only five patients with perimembranous defects received digoxin therapy. CONCLUSIONS: The prevalence of VSD in this series of neonates was 21.3/1,000 live births. The most common location of VSD in the neonatal period was in the region of the muscular septum. Muscular defects were more likely to close spontaneously than perimembranous defects. Most muscular defects underwent spontaneous closure during the 12-month follow-up period.

[Unilateral thalamic tumor with atrophy of ipsilateral cortical cortex: report of a case].

Ipsilateral brain atrophy is rare in neoplastic lesions of the brain, but it has been reported in patients with a thalamic tumor. We report a Chinese boy who presented with a right focal motor seizure and right side weakness at the age of six and half years when an electroencephalogram (EEG) showed focal epileptic discharges over the left hemisphere, but computed tomography (CT) of the brain failed to reveal a definite mass lesion. The weakness became gradually worse. On admission at age 8, follow-up CT scan revealed a huge tumor (5 x 5 x 7 cm) compressing the third and lateral ventricles with mixed densities in the left thalamus and centrum semiovale. The scan after contrast infusion showed a marked enhancement of the tumor. Instead of peri-mass edema surrounding the tumor, the overlying cerebral tissue showed atrophy of the ipsilateral cortical layer. He received subtotal resection of the tumor. The pathology proved to be germinoma. A test of tumor markers revealed a high human chorionic gonadotrophin level in the blood and cerebrospinal fluid. A short course of radiotherapy and chemotherapy was given after surgery. He has been well for the past two years.

[Clinical and cellular biologic diagnosis of Cockayne syndrome: a case report].

We report a case of Cockayne syndrome. A 6-year-old boy presented with a progeroid face, dwarfism, psychomotor retardation, skin photosensitivity and retinal pigmented degeneration. Neurological study disclosed slowed nerve conduction velocities and a brain CT showed calcification in the basal ganglia. Auditory brain stem evoked potential showed prolonged interpeak latency of wave I to wave V. Laboratory evaluation revealed mild liver dysfunction and peripheral eosinophilia. Fibroblast cultures from the patient and his family were exposed to ultraviolet (UV) light of 254 nm, ranging from 1 to 10 J/m2. Under 1 J/m2 irradiation, the surviving fraction of the fibroblasts from the patient, his mother, and a control subject were 40%, 50%, 90% respectively. If the fibroblasts of these subjects were exposed to 2 J/m2 and 3 J/m2 irradiation, the surviving fraction changed to 10%, 22%, 80% and 1.5%, 9%, 68%, respectively. However, fibroblasts from his sister and father showed the same surviving fraction as the control. The study showed that fibroblasts from the patient and his mother were extremely sensitive to UV light irradiation. We also study the concentration of the pyrimidine dimer of DNA in the patient and the control subject. Pyrimidine dimer showed no difference between the patient and the normal subject before and after 24-hour UV irradiation. These results suggest that the sensitivity to UV of Cockayne fibroblasts may be due to a ligase deficiency or to a replicon initiation disturbance in Cockayne cells.

Neurologic involvement in an outbreak of enterovirus 71 infection: a hospital-based study.

Enterovirus (EV) can cause varied clinical manifestations. Involvement of the central nervous system (CNS) with the nonpolio EVs are common and important causes of morbidity in children. To investigate the manifestations of nonpolio enteroviral infections with CNS involvement during the EV outbreak, from February 1998 to January 1999, we collected 153 hospitalized patients in our pediatric ward caused by nonpolio EV infections which were diagnosed by history, clinical features, or detected from viral cultures. Fourteen patients (9.2%) had CNS presentations, 13 males and one female. The ages ranged from one month to 10.3 years. The spectrum of CNS presentations included aseptic meningitis (4 cases, 28.6%), encephalitis (5 case, 35.7%), encephalomyelitis (3 cases, 21.4%), and poliomyelitis-like syndrome (2 cases, 14.3%). Among these patients, 8 cases (57.1%) were isolated with EV71 from at least one site of rectal or throat swab sampling. Two fatal cases were presented as encephalitis and complicated with pulmonary edema. Generally, enteroviral infections are considered as a benign infectious disease in children. However, pediatricians should keep in mind that EV71 has caused several endemic outbreaks and continues to be an occasional cause of severe CNS disease. Early evaluation and appropriate treatment of CNS enteroviral infections may minimize the neurologic sequelae.

Epstein-Barr virus encephalitis in children.

Fourteen children with Epstein-Barr virus (EBV) encephalitis admitted to our pediatric department during the period 1988 to 1998 were collected and reviewed to characterize the clinical, laboratory and neuroradiological findings. There were 7 boys and 7 girls. The age of onset ranged from 10 months to 14 years. Among them, 5 patients belonged to Alice in Wonderland syndrome, 5 were diagnosed as acute viral encephalitis, 1 presented with acute meningoencephalitis followed by cerebellitis, the remaining 3 cases attributed to acute disseminated encephalomyelitis. The main symptoms were fever (43%), seizure (36%), bizarre behavior (31%), headache (21%) and metamorphopsia (36%). The presenting signs included altered consciousness (50%), meningeal sign (14%), bulbar sign (14%), cerebellar sign (7%), and cranial nerve palsy (7%). Classic findings of infectious mononucleosis were obscure. The laboratory data showed the existence of atypical lymphocyte in only one case but positive serology for EBV infection in all patients. Pleocytosis was found in 3 (30%) of 10 patients examined. Eight (67%) of 12 patients had nonspecific electroencephalographic changes in the acute stage. Computed tomography (CT) scans were abnormal in 2 (40%) of 5 patients tested; while magnetic resonance image (MRI) disclosed lesions in 5 (56%) of 9 patients, with abnormal signals in various parts of the brain. Single photon emission computed tomography (SPECT) brain scan showed abnormal perfusion lesions in 3 (75%) of 4 patients studied. The results demonstrate the diversity of neurological manifestations of EBV encephalitis. EBV should be considered in any acute neurological illness of uncertain etiology in the pediatric population. While MRI remains the image of choice in EBV encephalitis, SPECT detects the abnormal perfusion more precisely in a substantial number of patients.