Neurologic outcome after fetal inflammatory response syndrome: Trimester-specific considerations

Clinical signs and neuroimaging patterns associated with the fetal inflammatory response syndrome (FIRS) worsen or mimic the clinical repertoire after intrapartum hypoxic-ischemic encephalopathy (HIE) during labor and/or parturition. Diagnostic considerations expressed as neonatal encephalopathy (NE) must consider chronic as well as acute factors associated with FIRS. Trimester-specific factors adversely alter the interactions of the maternal/placental/fetal (MPF) triad and influence the postnatal phenotype of FIRS. Anticipatory guidance for families by clinicians caring for survivors with FIRS, as well as researchers, must consider acute and chronic effects that influence neurologic outcome. Novel neurotherapeutic interventions must include prenatal preventive as well as peripartum/postnatal rescue and repair strategies to effectively reduce the presence and severity of sequelae from FIRS.     

Acute Campylobacter jejuni gastroenteritis in children. Study of 23 cases

Twenty-three cases of acute Campylobacter jejuni (CJ) enteritis were studied over à 8 month-period in hospitalized children. 906 fecal cultures were collected during the same period from children aged 1 month to 14 years. CJ was isolated in 33 (3.6%). The authors analyse the epidemiologic, pathogenic, clinical and therapeutic aspects of the disease in the 23 hospitalized children. After Salmonella, CJ is one of the most frequent etiologic agents for bacterial acute enteritis; its pathogenic mechanism is that of an invasion, mainly enteric. The clinical picture consists of diarrhea, fever and abdominal pain, difficult to assess accurately in young children. The disease is usually limited to the digestive tract and recovery occurs without antibiotic therapy.     

Unusual encephalopathy after acute chest syndrome in sickle cell disease: acute necrotizing encephalitis

Stroke is the most common neurologic complication of sickle cell disease. Acute chest syndrome (ACS) is a known risk factor for stroke in this population. Two patients (a 12-year-old boy and a 6-year-old girl) developed acute change of mental status and focal neurologic signs during episodes of ACS. The clinical and radiologic findings were compatible with acute necrotizing encephalitis, a variant of acute demyelinating encephalomyelitis. Patients with acute neurologic deterioration in conjunction with ACS should be evaluated thoroughly for other causes of central nervous system disease including infectious/parainfectious processes as well as stroke.     

Glutaric acidemia, type I, missed by newborn screening in an infant with dystonia following promethazine administration

We report a child initially diagnosed with promethazine-induced dystonia despite a lack of response to diphenhydramine therapy. On further evaluation, the child was diagnosed with glutaric acidemia, type I (GA-I), an autosomal recessive inborn error of metabolism caused by the deficiency of glutaryl-CoA dehydrogenase. The characteristic clinical feature of GA-I is an acute encephalopathic and neurologic crisis typically occurring during a catabolic state. Despite slow improvement, many patients do not fully recover from a neurologic crisis, and residual neurologic morbidity can be significant. Although newborn screening using tandem mass spectrometry is expected to enable presymptomatic diagnosis of GA-I, this patient was not detected by newborn screening with tandem mass spectrometry. Therefore, a high suspicion of GA-I must be maintained in the evaluation of childhood dystonia, even when newborn screening results are reportedly normal.     

Electrophysiologic studies, computed tomography, and neurologic outcome in acute bacterial meningitis

To determine the value of computed tomography and electrophysiologic studies in predicting neurologic outcome, we prospectively studied 41 children with acute bacterial meningitis, using clinical examination, computed tomography of the head, electroencephalography, brain-stem auditory evoked response, and visual evoked potential mapping during the acute illness. Two children died; 32 of the remaining 39 children were reviewed clinically, electrophysiologically, and with computed tomography between 5 and 38 months after the illness. The electrophysiologic data obtained during the illness were not found to alter the acute-stage management. Focal or generalized suppression, demonstrated on the electroencephalogram, was associated with a poor outcome. Cerebral infarction and edema, demonstrated by computed tomography of the head, were predictive of a poor outcome, but enlarged ventricular and subarachnoid spaces and increased subdural effusions were of no predictive value. Neither computed tomographic scans nor electrophysiologic data were better indicators of neurologic prognosis than the clinical examination.     

Monosomy 7, Diabetes Insipidus and Acute Myeloid Leukemia in Childhood

The triad of diabetes insipidus (DI), monosomy 7, and acute myeloid leukemia in a 7—year old boy is described. This triad has been described in adults but not in children. The DI ran a transient, self-limiting course and required no specific therapy. The pathogenesis of DI remains unknown, and its transient nature may result in this component of the triad going unnoticed.     

Un cas de volvulus aigu de l''estomac chez l''enfant

Gastric volvulus is an abnormal rotation of the stomach around its axis. The acute form is a surgical emergency, which doesn't tolerate any therapeutic delay. We report on a case in a young girl, revealed by painful epigastric mass and distended gastric bubble on abdominal X-ray. This case was been successfully treated by detorsion and gastropexy. The clinical picture of gastric volvulus is characterised by the Borchardt triad of symptoms: upper abdominal pain, vomiting and difficulty to pass a nasogastric tube. The gastrointestinal barium series and CT-scan help to confirm the diagnosis. As was demonstrated in this case, the treatment is surgical by reducing the volvulus and fixing the stomach to prevent recurrence.     

Acute,unintentional pediatric brodifacoum ingestions

BACKGROUND: Brodifacoum is the major rodenticide used in the United States today. It is similar to warfarin but has more potent and prolonged effects. Large overdoses and chronic intoxication have been associated with significant coagulopathies and death. Currently, the management of acute unintentional ingestions by young children is controversial. METHODS: American Association of Poison Control Centers (AAPCC) data from 1993 to 1996 were retrospectively searched for acute, unintentional brodifacoum exposures without coingestions followed-up to a known outcome in children aged 6 years and younger. The cases were analyzed by management site, symptoms, therapy, and outcome. Cases coded as having clinical or laboratory evidence of coagulopathy were further evaluated for severity. RESULTS: Our study reviewed 10,762 cases that involved single, acute, unintentional ingestions of brodifacoum. All of the patients were followed-up to a known outcome as defined by AAPCC data collection standards. In this cohort, there were no deaths or major effects reported. Although 67 patients reported evidence of coagulopathy, no major effects or deaths were reported. Minor and moderate effects were reported in 38 and 54 children, respectively. Management occurred outside of a healthcare facility in 5404 (50.2 %) cases. Approximately half of all the children received some form of gastrointestinal decontamination. Decontamination had no effect on the distribution of outcomes. Adverse effects from decontamination therapy were reported in 42 patients. CONCLUSION: Acute pediatric ingestions of brodifacoum rarely caused clinical effects and were not associated with life-threatening symptoms or death in young children. It seems reasonable that acute unintentional ingestions of small quantities of brodifacoum by young children can be adequately managed with home observation and parent education.     

Vitamin E deficiency during chronic childhood cholestasis: presence of sural nerve lesion prior to 2 1/2 years of age

Vitamin E malabsorption and deficiency during chronic childhood cholestasis has been associated with a progressive ataxic neurologic syndrome. Hyporeflexia, the first sign of neurologic dysfunction, may begin prior to age 2 years, but severe symptoms do not develop until age 5 to 10 years. To establish the age of onset of neuropathologic lesions, we prospectively evaluated four young children with severe cholestasis. Malabsorption and deficiency of vitamin E were documented by low serum vitamin E concentrations, low serum vitamin E to total serum lipids ratios, elevated hydrogen peroxide hemolysis, and impaired absorption of a pharmacologic dose of alpha-tocopherol. Abnormal neurologic findings in two patients were limited to areflexia, ptosis, mild truncal ataxia, and hypotonia; two patients had minimal signs of neurologic dysfunction. Sural nerve histology at age 6 to 25 months revealed a degenerative axonopathy involving large-caliber myelinated fibers, but without quantitative axonal loss. Muscle histology and histochemistry tests yielded normal results. Our study suggests that neurologic injury may occur during the first two years of life in vitamin E-deficient children with cholestatic hepatobiliary disease, obligating aggressive attempts at correcting this deficiency state at a very young age.     

Optic neuropathy in McCune-Albright syndrome: an indication for aggressive treatment

McCune-Albright syndrome consists of the triad polyostotic fibrous dysplasia, café-au-lait spots and autonomous hyperfunction of one or more endocrine systems. The most frequent neurological complication of craniofacial fibrous dysplasia is visual loss. We describe a 17-y-old boy with McCune-Albright syndrome and acute loss of vision in the left eye caused by encroachment of the left optic nerve by fibrous dysplastic lesions. Neurosurgical intervention improved left eye vision. Aggressive intervention is indicated in cases of acute visual loss in patients with craniofacial fibrous dysplasia. This is supported by a review of other reported cases.     

Sigmoid sinus thrombosis following mastoiditis: early diagnosis enhances good prognosis

Sigmoid sinus thrombosis following mastoiditis is a rare, but potentially life-threatening, condition. Its treatment usually consists of systemic antibiotics and mastoidectomy. In this report, we describe a pediatric case of sigmoid sinus thrombosis following mastoiditis, presenting with nonspecific symptoms such as fever, otalgia, and headache. Diagnosis was based on magnetic resonance imaging. The patient responded very well to intravenous antibiotics with a rapid clinical improvement and complete recanalization of the thrombosed sigmoid sinus. In conclusion, mastoiditis may present few clinical symptoms. In case of treatment failure or new-onset neurologic deficit in children with acute otitis media, life-threatening complications associated with mastoiditis should be considered. Early diagnosis is important, as favorable prognosis can be achieved with conservative management without performing any surgical intervention.     

Post-infectious acute cerebellar ataxia in children

Acute cerebellar ataxia is a relatively common neurologic disorder among children. Our aim was to characterize the clinical picture, etiology, and prognosis of acute cerebellar ataxia. The medical records of all children with a diagnosis of acute cerebellar ataxia hospitalized in our center and Hasharon Medical Center from 1990 to 2001 were reviewed. The diagnosis of acute cerebellar ataxia was based on the following criteria: acute onset of ataxia with or without nystagmus; absence of known genetic predisposing factors, such as familial degenerative disorders; and absence of drug intoxication, bacterial meningitis, and metabolic disorders. Thirty-nine children were identified; 54% were male; mean age at presentation was 4.8 +/- 3.8 years. All patients were observed for at least 1 year. A prodromal febrile illness was noted in 74.4%: varicella, 31%; mumps, 20%; nonspecific viral infection, 15.4%; mycoplasma, 5%; Epstein Barr virus, 3%. Latency from the prodromal illness to the onset of ataxia was 8.8 +/- 7.4 days. The most common associated neurologic findings were nystagmus and dysmetria. Full gait recovery took less than 2 weeks on average, and the longest duration of neurologic signs was 24 days (mumps-related). Acute cerebellar ataxia in childhood is a self-limited disease. The recovery was faster than that reported in previous publications and was complete in all children without any neurologic sequelae. Imaging studies are needed only in atypical presentation or if there is no spontaneous improvement after 1 to 2 weeks.     

Low morbidity and mortality of status epilepticus in children

In an ongoing study of status epilepticus, 193 children with status epilepticus of varying causes have been followed up for a mean period of 13.2 months. Of these, 97 patients were recruited prospectively. The patients' ages ranged from 1 month to 18 years (mean, 5.0 years). The cause of the status epilepticus was classified as idiopathic in 46 cases, remote symptomatic in 45, febrile in 46, acute symptomatic in 45, and progressive neurologic in 11. The mortality and incidence of sequelae following status epilepticus was low and primarily a function of etiology. Seven children died within 3 months of having the seizure. New neurologic deficits were found in 17 (9.1%) of the 186 survivors. All of the deaths and 15 of the 17 sequelae occurred in the 56 children with acute or progressive neurologic insults. Only two of the 137 children with other causes sustained any new deficits (P less than .001). Duration of the status epilepticus affected outcome only within the acute symptomatic group (P less than .05). Neurologic sequelae occurred in 29% of infants younger than 1 year of age, 11% of children 1 to 3 years of age, and 6% of children older than 3 years of age. However, this was a reflection of the greater incidence of acute neurologic disease in the younger age groups. Within each cause, age did not affect outcome. Of the 193 children, 61 (32%) had a history of prior unprovoked seizures. Of the 125 surviving children with no history of prior unprovoked seizures, 37 (30%) had subsequent unprovoked seizures.(ABSTRACT TRUNCATED AT 250 WORDS)     

Neurologic events after partial exchange transfusion for priapism in sickle cell disease

We describe six boys with homozygous sickle cell disease, aged 7 to 13 years, in whom acute, severe neurologic abnormalities developed 1 to 11 days after partial exchange transfusion was performed to treat priapism that was unresponsive to more conservative therapy. Hemoglobin levels were 10.5 to 13.4 gm/dl (mean 12.1 gm/dl), and hemoglobin S levels were 18% to 33% (mean 27%) before the onset of neurologic complications. Severe headache was the initial finding in five patients, four of whom had increased intracranial pressure and three of whom required tracheal intubation and hyperventilation. Four patients had seizures; three had focal neurologic deficits for more than 24 hours. Cerebral arteriography demonstrated vascular abnormalities, including irregularity, stenosis, and complete occlusion of vessels. Patients treated with regular erythrocyte transfusions had no recurrence of neurologic signs or symptoms when hemoglobin S levels were kept at 30% to 50%. The occurrence of serious neurologic complications after partial exchange transfusion in patients with homozygous sickle cell disease from three centers indicates the possibility of a causal relationship between the events. Early and thorough investigation of neurologic symptoms, especially severe headache, is warranted in this clinical setting.     

Pediatric West Nile virus infection: neurologic disease presentations during the 2002 epidemic in Cuyahoga County, Ohio

Knowledge is currently limited about West Nile virus (WNV) infection and its sequelae among children. Available evidence suggests that when compared with adults, children less than 18 years old can be at high risk for WNV exposure and infection yet manifest a lower risk for WNV-related morbidity and mortality. We detail clinical features of pediatric West Nile-associated neurologic disease (WNND) epidemic cases in Cuyahoga County during 2002. We present a structured review of pediatric and adult WNND cases hospitalized in Cuyahoga County, Ohio. During the epidemic, 5 children were hospitalized with confirmed WNND (estimated incidence = 1.4/100,000 children 5-17 years old at risk). Compared with adults, children had shorter hospitalization (mean, 4.6 versus 12.3 days), fewer neurologic symptoms, better neurologic outcomes, and lower mortality (0% versus 5.3%). Cerebrospinal fluid results were similar. When compared with adults, children had significantly lower rates of WNND. Children are at a decreased risk for severe WNV and less likely to present with neurologic signs or suffer neurologic sequelae.     

The pivotal role of deep vein thrombophlebitis in the development of acute disseminated staphylococcal disease in children

Deep vein thrombophlebitis (DVT) and septic pulmonary emboli (PE) are rare in children. The association of DVT and acute disseminated staphylococcal disease (DSD) during childhood has not been previously reported. We report 3 children who developed a triad of DVT, septic PE, and acute osteomyelitis with Staphylococcus aureus cultured from blood and bone. One child succumbed, while 2 survived following prolonged, morbid hospitalizations. The rapid clinical deterioration observed in these patients might be caused by the aggressiveness of staphylococcal infection combined with an ongoing showering of septic emboli from the ileo-femoral DVT. We suggest that infected DVT with septic PE had a pivotal role in the development of DSD in these children. The presence of this triad should prompt aggressive treatment with the appropriate antibiotics, anticoagulation, surgical drainage, and assisted ventilation when indicated.     

Pediatric liver transplant following near catastrophic head bleed: Lessons learned

Evaluation for liver transplant candidacy is a multidisciplinary effort that involves all aspects of clinical care including social work, nutrition, and a multitude of medical specialties. The prognosis of a pretransplant clinical condition is integrated into the decision to list a patient. Herein, we report a successful liver transplant and recovery of a 3‐month‐old male following a large right hemispheric subdural hematoma related to acute coagulopathy secondary to undiagnosed end‐stage liver disease. On presentation with jaundice, lethargy, and unequal pupils, a CT scan was obtained which demonstrated a large right subdural hematoma with herniation. Once his coagulopathy was corrected, he went for decompressive craniectomy. He survived with medically controlled seizures and improving L‐sided neglect and extremity weakness. Six weeks later, given his continued neurologic recovery and worsening liver function, the decision was made to list him for liver transplantation. One month later, he underwent orthotopic liver transplant. His post‐operative hospital course was complicated by DVTs and heparin‐induced thrombocytopenia, but no neurologic decline, and he was eventually discharged from the hospital on post‐op day 26. Three years later, he has a well‐functioning allograft and no clinically evident neurologic deficits. The prognosis following pediatric neurologic trauma remains somewhat unclear as recovery and neurologic examinations can be influenced by numerous extrinsic factors. This is one of the first reports of near full neurologic recovery of a pediatric liver transplant recipient following a large subdural hematoma with herniation.     

Patients with acute, fulminant form of SSPE

Subacute sclerosing panencephalitis (SSPE) usually begins insidiously and follows a subacute course with relentless but slow progression to death. In recent years, however, patients with acute or fulminant course were reported. In this article, we report on three patients (2 girls, 1 boy) with SSPE who developed an acute and fulminant course. Subacute sclerosing panencephalitis may be seen with more atypical symptoms and more acute and fulminant courses due to various undetermined reasons. Early diagnosis is very important for the effectiveness of treatment. Children presenting with acute or subacute neurologic symptoms should be examined for SSPE, especially if they have no risk factors for hereditary neurodegenerative/ neurometabolic diseases, and it is more important if those children were not vaccinated or were infected with measles.     

Respiratory symptoms in a boy revealing Carney triad

The association of gastrointestinal stromal cell tumor (GIST), paraganglioma, and pulmonary chondroma is known as the Carney triad, occurring predominantly in young adult females. We present the case of a 14-year-old male with respiratory symptoms resulting in the diagnosis Carney triad.