Background

Convulsion following an low voltage electrocution are transitory complications and rare to the child, due to the inhibition of the central nervous system or a hypoxia of a ventilatory or circulatory origin.

Objectives

The target is to describe a clinical form of seizure or convulsion due to electric shock

Methods

We report a case seizure or convulsion following an electric shock

Results

Infant of 22 months victim of an electrocution by an alternative current of 220 volt, found extended on his mother’s corpse. Admitted to the emergency in a condition of convulsive pain, she passed away after being given Midazolam and Phenobarbital. The heart rate was regular at ECG. A slight brain oedema was found. The rate of CPK and LDH were 2.5 times than normal. The neurological and biological evolution was favorable. An electro-encephalogram realized 72 hours after the accident showed an epileptogene temporal. The infant was put outgoing under Valporate of sodium.

Conclusion

This case is original because of the survival of the infant inspite of the violence of the electrical shock injury (dead mother) and the discovery of a secondary epilepsy. This stresses the importance of EEG in case of convulsion after a low voltage electrocution.

Background

Guillain-Barré syndrome is an autoimmune disease of the peripheral nervous system. It can affect all ages often with an obvious diagnosis. Clinical forms are diverse and include acute motor axonal neuropathy (AMAN).

Objectives

We report a pediatric case of AMAN discovered in a paraplegic child during a hospitalization in intensive care unit.

Methods

A 12 years old girl with antecedents of a complicated congenital spina bifida of a hydrocephalus and a paraplegia. Admitted through evacuation from a non pediatric hospital for the management of a respiratory infection evolving, in spite of the treatment in a pejorative way, towards a respiratory failure for which she had been intubated and ventilated.

Results

In intensive care, after the failure of the respiratory weaning and in the presence of a muscular weakness of the upper limbs, nerve conduction studies showed the presence of Guillain-Barré syndrome in its AMAN form. The child received intravenous immunoglobulins and a tracheotomy had been realized. The recovery of the driving function was very slow. The child is still under assisted ventilation.

Conclusion

This case is interesting, for the fact that the diagnosis of the Guillain-barré syndrome is easy with a normal infant whereas it could be very difficult with a ventilated and paraplegic infant.

Background

Measles Inclusion Body Encephalitis (MIBE) is a rare complication of measles infection or vaccination only described in immunodeficient patients. It occurs within a year of measles exposure. Diagnosis reached by clinical features, CSF anti measles antibody/measles RNA protein, MRI brain/EEG and brain biopsy alongwith underlying immunodeficiency.

Objectives

To describe 2 case reports of MIBE without evidence of immunodeficiency

Methods

We reviewed case notes of 2 patients with MIBE

Results

Case 1: 14 month boy presented with rapid neuroregression of motor-cognitive skillls / frequent seizures/ periodic asymmetric jerks.Clinical exome sequencing (CES) did not identify genetic immunodeficiency.

Case 2: 3 year boy/ consanguinity presented with neuroregression/periodic jerks/ dystonia/ partial seizures over 3 months. He was subsequently diagnosed with MIBE- brain biopsy not done. He required PICU care for aspiration pneumonia. CES awaited.

In both cases

No history of clinical measles

Measles vaccine at 10 months

EEG showed periodic jerks; CSF high titres of anti measles IgG

Extensive testing (immunonlogist consult) did not demonstrate immunodeficiency

Brain biopsy in case 1 did not show viral inclusion bodies.

Conclusion

MIBE has been described only in immunodeficient children- our cases had no evidence of immunodeficiency.

The diagnosis is suspected by typical clinical/ lab profile but also lesser time lag between exposure and presentation. SSPE- which presents 6-15 years post exposure - is a differential.

A limitation here is nonavailability of measles PCRs on brain biopsy.

We have 2 hypotheses:

Can MIBE occur in immunocompetent children OR Are MIBE and SSPE on the same clinical continuum with overlapping timelines?

Background

The impact of the type of congenital anomalies (CA) on the mortality and neonatal morbidities remains unknown.

Objectives

To investigate the epidemiology of CA and the association of the type of CA and mortality and major morbidities of very low birth weight infants (VLBWI).

Methods

A cohort study was performed using prospectively collected data from Korean Neonatal Network. A total of 8,156 VLBWI born between January 2013 and December 2016 were reviewed. Data from the patients with major CA (cases, n = 289) and the controls (n = 867), selected by 1:3 frequency matching for sex, multiplicity and gestational age, were compared.

Results

The overall prevalence of major CA in VLBWI was 3.5%, and CA involving multiple organs accounted for 17.3% (n = 50) of the cases. The most common type of CA was cardiac anomalies (n = 115, 31.0%), followed by digestive system (28.8%), other anomalies / syndromes 12.1% and urinary system 6.5%. The highest mortality was demonstrated in CA with respiratory system (78.6%), which accounts for only 3.8% of the total CA. In a multivariate logistic regression analysis revealed that CA confers greater hospital mortality (odds ratio, OR, 4.29 [95% confidence interval, CI, 3.31 to 7.32]) and higher incidence of moderate-to-severe bronchopulmonary dysplasia (3.07 [2.12 to 4.44]), grade 3/4 intraventricular hemorrhage (2.03 [1.19 to 3.48]).

Conclusion

Major CA was a risk factor of neonatal death or major morbidities in VLBWI. Further studies are needed to provide effective transport and management system for VLBWI with major CA.

Background

Children treated by ECMO present a high risk of neurological complications. As some animal studies have shown cerebral autoregulation (CA) impairment after exposure to ECMO, it may be a key factor in this context.

Objectives

The main objective was to investigate CA in children supported by ECMO. The second objective was to analyze the relationship between CA impairment and neurological outcome.

Methods

Study. Monocentric observational prospective study

Patients and setting. All children treated by ECMO in the PICU of the Universitary hospital of Nantes, France

Measurements. A correlation coefficient between the variations of regional cerebral oxygen saturation (rScO2) as a surrogate of cerebral blood flow and the variations of arterial blood pressure (ABP) was calculated as an index of autoregulation (cerebral oxygenation index (COx), ICM+ software®) during ECMO run. A COx > 0.3 was considered as critical.

Neurological outcome was assessed by the onset of an acute neurologic event (ANE) during the ECMO run.

Results

17 patients treated by veino-arterial (n=15) or veino-veinous ECMO (n=2) were included (median age 68 days, weight 4.1 kg). Mean COx, percentage of time with a COX> 0.3 and AUC for a COx>0.3 were respectively 0.11+/- 0.28, 11.1+/- 8.7 and 3.9+/-3.8. 8 patients (47%) presented an ANE and the time spent with a COx > 0.3 was 1.7 time higher in those patients versus others (14 vs 6.5%).

Conclusion

AR assessment was feasible in pediatric ECMO. The impact of the time in critical region needs to be explored in a larger cohort.

Background

Galen vein aneurysm can present with hydrocephalus. Here we present a complicated case necessitating multiple EVD placements after the embolization procedure.

Objectives

An 8 month old patient with history of ventriculoperitoneal (VP) shunt operation for hydrocephalus (Fig1) presented with a seizure and magnetic resonance imaging revealed 3 giant and 50 smaller size aneurysms and an arteriovenous malformation (Fig 2). Interventional radiology proceeded with an embolisation procedure for his arteriovenous fistula and he was admitted to our PICU. His VP shunt was externalized due to intraventricular bleeding after this procedure (Fig 3) His EVD was changed 15 times at the bedside due to clogging with bloody debris within 3 months’ period. All CSF screening cultures remained sterile and his shunt was internalized with clearance of blood in CSF. Unfortunately this lasted only a week, and his shunt was externalized again due to a dysfunction. Thereafter he developed ventriculitis caused by methicillin sensitive staphylococcus aureus and his EVD was changed for 7 more times. Bilateral interventions were performed aiming more effective CSF drainage without success. The child expired on 7 months after his admission due to progressive neurologic dysfunction.

Methods

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Results

Intraventricular hemorrhage after an embolization procedure may require many interventions for drainage of CSF. 23 EVD placements were performed, which was the highest number for a single patient in our unit.

Conclusion

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Background

Vomiting, poor weight gain and respiratory problems are generally attributed to gastrointestinal reflux disease in infants. Here, we present a case who underwent further examination because of vomiting, failure to thrive and respiratory distress.

Objectives

A 3 month old male as transferred to our pediatric intensive care unit (PICU) due a seizure followed by cardiorespiratory arrest while being hospitalized due to gastrointestinal reflux disease and respiratory distress attributed to pneumonia. His symptoms were of 6 weeks duration, for which he was admitted twice for work up and therapy. Trial of antireflux treatment was not successful. His further workup included bronchoscopy to rule out laryngomalacia and reflux and an esophago-gastro-duodenal screening . Despite preemptive antibiotic treatment and antireflux medications symptoms did not regress Following a generalized seizure, he arrested on the ward. Cranial tomography showed hydrocephalus due to a posterior fossa tumor. After intubation and supportive management, he was transferred to our PICU for surgical management. He underwent an emergent ventriculoperitoneal shunt, followed by subtotal resection of the massive tumor. Pathology showed atypic rhabdoid teratoid tumor.

Methods

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Results

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Conclusion

Central nervous system tumors are the most common solid tumors seen in childhood and children may present with reflux symptoms. This should be kept in mind when infants have persistent symptoms despite antireflux therapy.

Background

Intoxication with drugs is one of the major causes of preventable morbidity and mortality in childhood.

Objectives

The aim of this study was to evaluate the incidence of intoxication and suicide in childhood and to investigate the preventability of intoxication.

Methods

The study was done in Elazig Education and Research Hospital and Elazig City Hospital Pediatric Intensive Care Units. Children between the ages of 0-18 years old who were brought to our 3rd stage child intensive care unit between 01.05.2018-01.12.2018 were evaluated prospectively.

Results

Of the 174 cases ages were 13 months old to 17 years and mean age was 8.25 ± 0.27 years. Of the 48 cases, 27 (56%) were accidental intoxications, and 21 were suicidal intoxications (44%). 54% of cases were male; 62% were female. Central nervous system drugs (32.9%) were the most common causes of drug-related ones. 42.9% of the cases in the drug intake for suicidal purposes had taken high dose of the drug. We had no mortality.

Conclusion

Intoxication with drugs is one of the most important causes of preventable morbidity and mortality in the world and in our country. It is observed that the number of children with depression and suicidal intoxications are extremely high in children and its age has fallen to 11 years old. Considering the increase in the number of intoxication cases in the last decade, the number of children with suicidal behaviour is found increasing.

Background

Triple A (Allgrove) syndrome was first described by Allgrove in 1978 . About 100 cases have been reported. Clinical picture is characterized by achalasia, alacrimia and ACTH-resistant adrenal failure.

A progressive neurological syndrome including central, peripheral and autonomic nervous system impairment, and mild mental retardation is often associated.

We report a 4 years-old boy presenting with Addison’s disease, dysphagia, alacrmia, and recent onset seizure and acute stroke.

Objectives

The aim is to diagnose the cause of seizure and stroke in this case of Allgrove syndrome

Methods

At admission to PICU, plasma ACTH level, serum electrolytes,basal cortisol level were ordered

Stress dose glucocorticoid therapy started

Brain MRI, and Functional spectroscopy study were ordered

Brain CT angiography is recommended after MRI

pANCA, cANCA antibodies are ordered later

Results

At admission plasma ACTH level was elevated, serum electrolytes were normal, basal cortisol level was normal, and stress dose glucocorticoid therapy continued

Brain MRI showed multiple bilateral asymmetrical relatively large cortical and subcortical T2 and FLAIR hyperintense patches with marked DWI restrictions may be representing recent ischemic insult. recommended to do brain CT angiography

Functional spectroscopy study showed normal levels of the main metabolites

Brain CT angiography showed definite cerebral vasculitis with beaded pattern

ANCA antibodies : negative

The patient recieved prednisolone at dose of 2 mg/ kg/day. All neurological motor deficits improved after 3 weeks daily therapy

Conclusion

To our knowledge, this is the first report describing cerebral vasulitis in a patient with Allgrove syndrome

(Written informed consent was obtained from the parents for publication of this Case report)