Paraneoplastic neurological disorders in children with benign ovarian tumors.

AIM: Paraneoplastic neurological diseases (PND) are rare, but potentially treatable disorders. Paraneoplastic encephalitis is rapidly emerging as an important but likely under-recognized condition in children. The aim of this study was to assess the prevalence and spectrum of PND in children with benign ovary tumor and the long-term outcome. PATIENTS AND METHODS: We retrospectively reviewed the charts of all female patients below 18years of age diagnosed with a benign ovarian tumor proven by pathology between January 1993 and December 2010. All the clinical symptoms developed within 5years of tumor diagnosis and the related investigations were recorded. RESULTS: There were total 133 children and adolescents with benign ovarian tumors, mostly mature teratoma. Six patients (4.5%) had neuropsychiatric manifestations and all but one were beyond age 10years. The most common neuropsychiatric presentations were depression or low mood (84%), headache (50%), mutism (50%), hypoventilation (50%), seizures (30%), hallucination (30%), vomiting and hypersalivation (30%). Three patients (2.2%) had serious PND including acute disseminated encephalomyelitis in 1 and anti-N-methyl-d-aspartate receptor (NMDAR) encephalitis in 2. Although all of three improved after tumor removal, one without immunotherapy had neurological sequelae and prolonged ICU stay. CONCLUSION: The prevalence of PND in benign ovary tumor is not so uncommon in children. It is important to survey ovary tumors in female adolescents with subacute presentation of multiple-level involvement of neuraxis where no clear alternate diagnosis is possible. Treatment of serious PND associated with ovary tumors should include immunotherapy in addition to tumor removal.

Tc-99m HMPAO brain SPECT imaging in children with acute cerebellar ataxia.

PURPOSE: We prospectively evaluated consecutive cases of acute cerebellar ataxia in children to identify brain perfusion SPECT features associated with acute cerebellar ataxia and to correlate the brain SPECT findings to clinical severity and prognosis. RESULTS: Among the 10 consecutive children with acute cerebellar ataxia, 7 had abnormal Tc-99m HMPAO brain SPECT findings (4 cerebellar hypoperfusion, 5 unilateral cortical and/or subcortical hypoperfusion, and 1 unilateral cortical hyperperfusion), in contrast to no abnormalities disclosed by CT or MR imagings. Furthermore, the extent of brain perfusion SPECT abnormalities correlated significantly with the clinical severity and the recovery time of cerebellar ataxia. Sixty percent of children with acute cerebellar ataxia had cortical and/or subcortical involvement, which implied the wider involvement of acute cerebellar ataxia than what has been thought. CONCLUSION: The present data suggest that Tc-99m HMPAO brain SPECT is a sensitive modality for providing prognostic information in childhood acute cerebellar ataxia.

Long-term neuroplasticity effects of febrile seizures in the developing brain.

Febrile seizures (FS) are the most common seizure disorder in childhood, occurring in 2%-5% of children. Regarding the large number of children with FS, it is important to delineate whether early-life FS alters long-term neuroplasticity, especially the neurocognitive function and subsequent temporal lobe epilepsy (TLE). Recent epidemiological studies reassure that most FS do not adversely affect global intelligence and hippocampal function, such as memory. However, there are concerns regarding those children who experience FS during the first postnatal year, having prior developmental delay and pre- or peri-natal events. The epidemiological data do not support a causal relationship between FS and TLE. However, magnetic resonance imaging studies confirmed that prolonged and focal FS can occasionally produce acute hippocampal injury that evolves into atrophy. Moreover, the common coexistence of hippocampal sclerosis and asymmetric cortical dysgenesis in TLE patients argues for a 'double-hit' theory for TLE. Animal studies have revealed that the exposure of hippocampal neurons to FS early in life, particularly prolonged or frequently repetitive FS, or together with brain malformation, may lead to sustained dysfunction of these cells including long-term memory impairment or epileptogenesis, in spite of the absence of neuronal damage. Recent clinical and molecular genetic studies suggest that the relationship between FS and later epilepsy is frequently genetic, and there are a number of syndrome-specific genes for FS. However, these channelopathies account for a small proportion of FS cases. The clinical management, therefore, is based mainly on the phenotypic features of FS and the subsequent seizures.

Neonatal adrenoleukodystrophy presenting with seizure at birth: a case report and review of the literature.

Neonatal seizures are critical conditions because they are usually related to significant illnesses that require a specific therapy. Antepartum and peripartum seizures are very rare, and represent signs of prenatal-onset neurologic dysfunction. A review of the literature revealed that the main etiologies include severe brain malformations, multiple anomalies, and metabolic encephalopathy. A high incidence of early fatality and serious neurologic sequelae were noted. To our knowledge, this is the first case report of neonatal adrenoleukodystrophy presenting with seizure at birth. These very-early-onset seizures may require unique diagnostic and therapeutic considerations, in contrast with the later onset of seizures in neonates.

Diagnostic approach to recurrent bacterial meningitis in children.

Recurrence of bacterial meningitis in children is not only potentially life-threatening, but also involves or induces psychological trauma to the patients through repeated hospitalization and multiple invasive investigations if the underlying cause remains undetected. Bacteria migration, along congenital or acquired pathways from the skull or spinal dural defects, gains entrance into the central nervous system (CNS) and should be taken into consideration when children face recurrent bacterial meningitis, however, symptoms and signs of cerebrospinal fluid (CSF) rhinorrhea or otorrhea are rare in such patients. Without evidence of CSF leakage, a cranial symptom/sign or coccygeal cutaneous stigmata may suggest the approximate lesion site, diagnosis and detection remains difficult. To detect an occult dural lesion along the craniospinal axis, such as basal encephalocele, dermal sinus tract, or neurenteric cyst, a detailed clinical evaluation and the use of the modern diagnostic imaging methods is necessary. Because of the possibility of concomitant occurrence of more than one malformation, both the frontal and the lateral skull base should be carefully evaluated. Precise localization of the dural lesion is a prerequisite for successful surgical repair. In addition, the bacteria specificity could leave significant clues: Pneumoccocus or Hemophilus suggests cranial dural defects, E. coli or other gram negative bacilli suggests spinal dural defects, and meningococci suggest immunologic deficiency. Asplenia or immunodeficiency such as complement or immunoglobulin deficiency rarely causes recurrent meningitis without a history of frequent infection of non-CNS areas. Salmonella meningitis or brain abscess should not be treated incompletely or inadequately and could lead to recrudescence, relapse or recurrence of bacterial meningitis. Antibiotic (penicillin or trimethoprim-sulfamethoxazole) induced meningitis may repetitively occur on occasion.

Seizures complicating infantile and childhood bacterial meningitis.

In this study, 116 patients, at least 1 month of age but younger than 5 years, were identified with culture-proven bacterial meningitis. A comparison was made between the clinical data of the patients with and without seizures during hospitalization. Seizures during acute bacterial meningitis accounted for 47% (55/116) of the episodes. Time interval between the onset of bacterial meningitis and that of seizures was 1 to 20 days (mean, 4 days). Twelve of these 55 patients had one or more afebrile seizures after completing the treatment. At follow-up of at least 1 year after completing treatment, 26 patients had good outcomes, whereas the other 29 patients had poor outcomes. A strong correlation between the findings of abnormalities through neuroimaging and the occurrence of seizures during hospitalization was observed. The long-term outcomes of patients with infantile and childhood bacterial meningitis, who had seizures during the acute phase of bacterial meningitis, were worse than the outcomes of those who did not have such seizures. No child developed late seizures unless there were acute seizures. Factors associated with seizures during acute bacterial meningitis include disturbed consciousness on admission, abnormal neuroimaging findings, and low glucose and high concentration of total proteins in cerebrospinal fluid.

Bacterial meningitis in infants: the epidemiology, clinical features, and prognostic factors.

This 16-year (1986-2001) retrospective study enrolled 80 infantile patients (aged, 30-365 days old) with culture-proven bacterial meningitis. The most prevalent pathogens were Salmonellaspecies, Streptococcus (S.) agalactiae, Escherichia (E.) coli, and Haemophilus (H.) influenzae, accounting for about 59% of the episodes. Meningitis caused by Salmonella species, E. coli and H. influenzae occurs more often in the older infants, while that caused by S. agalactiae occurs more often in young infants. Our study revealed a decrease in the proportion of Salmonella meningitis from 27% in the first 8 years to 9% in the second 8 years with E. coli replacing Salmonella species as the leading pathogen of this disease during the second period. Overall mortality rate for both periods of time was 11%. However, if we take those with undesirable poor outcomes into account, 43% of patients could be considered treatment failures. The study also reveals a high prevalence of neurological complications when this disease is caused by H. influenzae, S. pneumoniae, and Salmonella species. Stepwise logistic regression analysis revealed that only initial changing levels of consciousness (P = 0.006) were independently associated with treatment failure. The most frequent neurological complications associated with this disease included subdural empyema, hydrocephalus, cerebral infarctions, and seizures. Because therapeutic regimens may require attention to the eradication of bacterial pathogen but also the neurological complications, early diagnosis and choice of appropriate antibiotics are essential to increasing the possibility of survival.

A clinical and electrophysiologic survey of childhood Guillain-Barré syndrome.

In this 16-year (1986-2001) retrospective study, 23 childhood patients were identified with Guillain-Barré syndrome. According to clinical and electrophysiologic findings, 18 patients manifested acute inflammatory demyelinating polyradiculoneuropathy, 2 had Miller Fisher syndrome, 1 had axonal forms, and 2 were unclassified. Seasonal preponderance was evident in 39% of patients with Guillain-Barré syndrome, developing the disease in the winter (November to January) with upper respiratory infection the most frequent preceding event. The most common manifestation was limb weakness, with various degrees of motor weakness in 22 patients. Bulbar involvement was the most common cranial palsy, and it was evident in 30% of the episodes. Only one of these progressed to mechanical ventilation during hospitalization. Altogether, approximately 61% of the episodes exhibited sensory symptoms. At a follow-up of 1 year or more, 20 patients recovered and 3 had residua. Furthermore, no fatality occurred in our study. Our study also demonstrates that the clinical course of childhood Guillain-Barré syndrome has a shorter recovery time as compared with an adult patient group. Therapeutic outcome is favorable for patients who receive prompt treatment.

Neonatal bacterial meningitis in southern Taiwan.

To determine the epidemiologic trends, prognostic factors, and therapeutic results of neonatal bacterial meningitis, 60 neonatal patients with culture-proven neonatal bacterial meningitis were enrolled in this study. To compare changes over time, the appearance of disease among the patients was divided into two equal periods (1986-1993 and 1994-2001). Group B streptococci were the most common causative pathogens, accounting for approximately 32% of the episodes. Escherichia coli, the next most common pathogen, was more frequently observed in the second period. Seventy-seven percent of gram-negative bacilli isolates were resistant to ampicillin. Moreover, oxacillin-resistant Staphylococcus and ampicillin-resistant group B streptococci strains occurred in the second periods as late-onset neonatal bacterial meningitis. The overall mortality rates for the first and second study period were 17% and 8%, respectively. However, if individuals with poor outcomes were taken into account, 38% of patients were considered treatment failures. Significant prognostic factors included the presence of seizures, thrombocytopenia, and high cerebrospinal fluid protein and low cerebrospinal fluid glucose concentration. Although the mortality rate was significantly reduced in the second period, there has been increasing incidence of the emergence of resistant strains presenting a therapeutic challenge. The presentation in neonatal bacterial meningitis might be nonspecific, and blood culture results were negative in 45% of the episodes. Early diagnosis, choice of appropriate antibiotics, and correction of metabolic derangement are essential to improving outcomes.

Vocal cord paralysis after transcatheter coil embolization of patent ductus arteriosus.

BACKGROUND: In patients with patent ductus arteriosus (PDA) after Gianturco coil embolization, vocal cord paralysis (VCP) had not been previously described. This study investigates the risk factors of coil embolization associated with VCP. METHODS: We reviewed the medical records of all patients who had undergone transcatheter closure of PDA with a Gianturco coil between March 1998 and May 2001, and 75 patients (age range, 6 months to 55 years; mean age, 5.5 years) were identified. The procedure was performed with local anesthesia and with sedation in some young children. No patient required general anesthesia or endotracheal intubation. RESULTS: Three of the 75 patients had hoarseness caused by VCP after coil embolization. Patients with VCP had a longer ductus length (P <.01) and a smaller ductus diameter (P <.01) than patients without VCP. The pathogenesis of VCP may be caused by tense stretching and angulation of the ductus arteriosus induced by the implanted coil, which leads to compression injury of the adjacent left recurrent laryngeal nerve. CONCLUSIONS: A long ductus length (> or =12 mm) and a small ductus diameter (<1 mm) are significant risk factors of VCP after coil embolization in children <1 year of age. We recommend that coil embolization be performed very carefully in patients with PDA.

Echocardiographic guidance for transcatheter coil occlusion of patent ductus arteriosus in the catheterization laboratory.

The objective of this study was to investigate the feasibility of transthoracic echocardiography as a guide to monitor the completeness of patent ductus arteriosus (PDA) occlusion immediately after coil implantation. In all, 52 patients who underwent Gianturco coil implantation for PDA occlusion were evaluated by transthoracic echocardiography within 15 minutes after the first coil implantation in the catheterization laboratory. According to Doppler echocardiographic findings, these patients were classified into 3 groups: group 1, complete occlusion without residual shunt; group 2, a residual PDA diameter < 1 mm and no continuous waveforms detected; and group 3, a residual PDA diameter >or= 1 mm or continuous waveforms. There were 34 patients in group 1, 10 patients in group 2, and 8 patients in group 3. In a 12-month follow-up the complete occlusion rate was 100%, 90%, and 87.5% in groups 1, 2, and 3, respectively. We conclude that immediate Doppler echocardiography is useful in assessing the status of residual PDA just after coil implantation. Residual shunt < 1 mm and lack of continuous waveform on Doppler scan indicate sufficient ductal closure of PDA.

Periodic lateralized epileptiform discharges of pediatric patients in Taiwan.

Periodic lateralized epileptiform discharges are special electroencephalographic abnormalities present in adults with stroke, brain tumor, intracranial hemorrhage, or other rare etiologies. Few reports focused on the etiologies in pediatric patients. We retrospectively reviewed 8002 of our pediatric electroencephalographic records for the past 12 years and listed all associated illness and their outcomes. Forty-four children with periodic lateralized epileptiform discharges were collected. We found that there was an obvious difference in etiologies of our pediatric patients from those reported in the literature. Nearly two thirds of our patients (28 children) were associated with central nervous system infections. The other etiologies included head injury, encephalopathy, epilepsy, and others. Herpes simplex virus was responsible for two thirds (12) of the 18 children with identified pathogens causing a central nervous system infection. Ten patients failed to have a defined pathogen. Periodic lateralized epileptiform discharges have a different clinical significance in pediatric patients than in adults. In Taiwan, central nervous system infection is the most common etiology of periodic lateralized epileptiform discharges in pediatric patients. Herpes simplex virus, although the most common pathogen, should not be considered to be the only cause of encephalitis in children with periodic lateralized epileptiform discharges.

Tuberculous meningitis in infancy.

The lack of specific symptoms and signs in patients with tuberculous meningitis makes early diagnosis difficult. To our knowledge, there has been no report in the literature focusing on tuberculous meningitis patients younger than 1 year of age. In this report, we reviewed the clinical features and laboratory findings of seven infants with tuberculous meningitis encountered during a 15-year period. All patients had fever, cough, and alternation of consciousness at presentation. Five patients had bulging anterior fontanel, and five had generalized tonic-clonic seizures. The purified protein derivative skin test was positive in six patients. Six patients had hyponatremia. All seven patients had abnormal cerebrospinal fluid findings, and six of them demonstrated cell counts less than 500 cells/mm(3) with lymphocytic predominance. Brain sonography examination revealed hydrocephalus in all seven patients. Therefore we conclude that antituberculosis therapy should be promptly initiated in any young infant with a clinical impression of meningitis in the context of cerebrospinal fluid white cell count of less than 500 cells/mm(3) and lymphocytic predominance, hyponatremia, and hydrocephalus.

Oral wooden stick injury complicated by meningitis and brain abscess.

Meningitis is rarely seen following oral injury. We describe a 3-year-old boy developing meningitis and brain abscess following a penetrating oral wooden stick injury. There was no cerebrospinal fluid rhinorrhea noted. A cerebrospinal fluid culture yielded viridans streptococcus. Brain magnetic resonance imaging and computed tomography revealed a multiloculated ring-enhancing mass. This patient underwent surgical drainage and completed 8-week antibiotic therapy. The patient demonstrated a late and dismal complication of a penetrating oral injury. At 2-year follow-up the patient was in good condition. A penetrating oral wooden stick inury should be regarded as potentially serious.

Transcranial ultrasound diagnosis of intracranial lesions in children with headaches.

The diagnostic value of transcranial ultrasound for intracranial lesions in children with headaches was evaluated. From January 1995 to December 1998, 444 children with headaches for more than 2 weeks visited our Pediatric Neurologic and Neurosurgical Outpatient Clinics. A 2-MHz-sector sonographic transducer was used to perform transcranial ultrasound. Nineteen patients were lost to follow-up. Two patients, both 15 years of age, failed to have a successful examination. Thirteen of the enrolled 423 patients presented with headaches and other symptoms and positive neurologic signs and had positive magnetic resonance imaging findings of intracranial lesions correlating with their transcranial ultrasound findings. Twelve of the remaining 410 children complaining of isolated headache had abnormal transcranial ultrasound findings. Nine of the 12 patients were confirmed to have intracranial lesions by magnetic resonance imaging studies. Among the nine patients, five had brain tumors, two patients had hydrocephalus, one patient had intracerebral hematoma, and one patient had temporal arachnoid cyst. The other three patients were misinterpreted to have a suprasellar lesion by transcranial ultrasound. These three patients and the remaining 398 children were regularly followed for at least 12 months. All revealed no evidence of an intracranial lesion. The sensitivity and specificity of transcranial ultrasound to detect intracranial lesions for children with isolated headaches were 75% and 99.7%, respectively. The average saving from utilizing this technique was $222 U.S. dollars per person. We suggest that transcranial ultrasound study is a reliable, convenient, time-saving, and economic diagnostic tool in detecting intracranial lesions in children with headaches.