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Recurrent acute necrotising encephalitis in a family: Case report
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P2-78 Recurrent acute necrotising encephalitis in a family: Case report lu, Selman Kesici, Yasemin Tas‚cı Yıldız, Gu¨lce C¸itil, Hu¨lya Kayılıog Deniz Yu¨ksel. Department of Pediatric Neurology, Dr. Sami Ulus Children's Research and Training Hospital, Turkey Introduction and aim: Acute necrotizing encephalopathy of childhood is a clinical entity presenting with progressive acute encephalopathy following a febril enfection. It’s rare but when it's related with RANBP2 mutation, it is expected to be recurrent patient and the other family members should be considered as under risk. Case Report 4 years old boy was referred to our hospital’s intensive care unit (ICU) because of refractory seizures, recurrent vomiting and loss of conciousness. His family history included a sister who had similar clinical history and findings when she was admitted to hospital 9 years ago, and died after her second encephalopathy attack.Our patient had fever and vomiting 3 days ago, and had a right hemiclonic seizure which lasts for 10 minutes. He was hospitalized elsewhere and he received intravenous ceftriaxone. For two days he didn’t have any symptoms. On the third day he vomited multiple times, signs of pyramidal and brain stem involvement were observed and he became encephalopathic. His glascow coma scale was 3 on arrival. He was entubated for mechanical ventilation. EEG showed slow-wave activity consistent with encephalopathy without any epileptiform discharges. Magnetic resonance imaging showed hyper-intensities in bilateral thalamic regions, external capsules, temporal lobes and cerebellar hemispheres. Susceptibility weighted imaging (SWI) series showed bilateral thalamic hemorrhage and necrosis dominantly in the right thalami. Based on this findings and family history of acute necrotizing encephalopathy of childhood we decided to give him 30 mg/kg per day pulse methylprednisolone. On the second day of methylprednisolone treatment he died. Genetic evaluation of RANBP2 mutation is being carried on. Conclusion: Acute necrotizing encephalopathy of childhood is a rare and fatal disorder. Bilateral thalamic hemorrhage and necrosis is characteristic for diagnosis. Mutation of the RANBP2 gene should be investigated, when it is seen recurrent in a patient or in other family members.
http://dx.doi.org/10.1016/j.ejpn.2017.04.977 P2-79 Polio-like phenotype in pediatric acute flaccid paralysis associated with enterovirus infection: The 2016 French experience M. Kossorotoff, M. Aubart, I. Schuffenecker, M. Leruez-Ville, F. Moulin, R. Carlier, C. Gitiaux, I. Desguerre. Department of Pediatric Neurology, APHP University Hospital Necker-Enfants malades, Paris, France Objective and Methods: In France in 2016, an excess of acute severe neurologic conditions associated with Enterovirus (EV) infection was observed and a nationwide case collection was performed. Within this case collection, we report our monocentric experience of 22 pediatric EV-associated severe neurologic conditions over 6 months and focus on the clinical, radiological, neurophysiological, and virological description of polio-like patients. Results: Several phenotypes were observed, related with outcome. Two thirds of patients had favorable outcome: they had either isolated cerebellitis (n¼4), rhombencephalitis (n¼4),
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encephalomyelitis (n¼3), isolated myelitis (n¼1), or acute inflammatory demyelinating polyneuropathy (n¼2). One third of patients had poor outcome, because of rhombencephalomyelitis associated with myocarditis (prolonged ventilatory support, n¼2), or acute flaccid paralysis with persistent polio-like motor deficit (n¼6). Polio-like patients associated initial monoplegia (n¼5) or suspended motor deficit (n¼1) with axonal motor neuropathy, and anterior spinal magnetic resonance imaging (MRI) abnormalities. Interestingly, muscle MRI showed segmental atrophia with denervation T2 hypersignal, without nerve root or nerve trunk associated topography, suggesting motoneuron lesion in anterior horn spine rather than radiculitis. These six children were positive for various non-polio enteroviruses, with only one patient positive for EV-68. Conclusion: These findings suggest that the mechanism of neurotoxicity towards the motoneuron in children with EV-associated polio-like syndromes may be related to still unknown environmental, immunological or virological cofactors rather than neurotropic virulence related to a single EV strain.
http://dx.doi.org/10.1016/j.ejpn.2017.04.978 P2-80 Acute cerebellitis in children: A series of eight cases € c¸men, Mirac¸ Yıldırım, Rahs‚an Go Bahadır Konus‚kan, lu, Banu Anlar. Hacettepe University Faculty of Dilek Yalnızog Medicine, Departments of Pediatric Neurology, Ankara, Turkey Objective: Acute cerebellitis is a rare condition often characterized by severe headache, vomiting, fever, ataxia and loss of consciousness. Its course is usually self-limited but occasionally fulminant. Differentiation of acute infectious cerebellitis from postinfectious cerebellitis is usually based on the presence of fever and absence of a latent period following a non-specific infection. Methods: We present clinical and paraclinical findings, treatment and outcome data of eight children diagnosed with acute cerebellitis in our department between 2010-2016. Results: There were 5 girls and 3 boys aged 3-16.5 years, median 8 years. Main presenting symptoms were headache, fever, vomiting and ataxia. Most common MR imaging finding at presentation was bilateral, diffuse signal changes in cerebellar hemispheres. Two patients had hydrocephalus, one had herniation, and one, both hydrocephalus and herniation. Etiological work-up for infectious pathogens revealed mycoplasma pneumoniae and influenza virus in one case each. Two cases underwent lumbar puncture: one was normal, the other showed elevated protein and low glucose. Treatment included steroids in all cases, acyclovir in 7, antibiotics in 2, intravenous immunoglobulins in 1, oseltamivir in one case. No patients required surgery for hydrocephalus or herniation. Four patients had full recovery and four had residual neurologic deficits consisting in cerebellar tremor and ataxia (n¼3) and cognitive deficit (n¼2). Patients with neurological sequalae had cerebellar atrophic changes on follow-up MRI. Patients admitted >7 days after symptom onset recovered with sequalae while those admitted<3 days showed complete recovery. Conclusion: There were no fatalities in this series of acute cerebellitis, suggesting early diagnosis and treatment can increase the chance of uneventful recovery.
http://dx.doi.org/10.1016/j.ejpn.2017.04.979
P2-78 Recurrent acute necrotising encephalitis in a family: Case report lu, Selman Kesici, Yasemin Tas‚cı Yıldız, Gu¨lce C¸itil, Hu¨lya Kayılıog Deniz Yu¨ksel. Department of Pediatric Neurology, Dr. Sami Ulus Children's Research and Training Hospital, Turkey Introduction and aim: Acute necrotizing encephalopathy of childhood is a clinical entity presenting with progressive acute encephalopathy following a febril enfection. It’s rare but when it's related with RANBP2 mutation, it is expected to be recurrent patient and the other family members should be considered as under risk. Case Report 4 years old boy was referred to our hospital’s intensive care unit (ICU) because of refractory seizures, recurrent vomiting and loss of conciousness. His family history included a sister who had similar clinical history and findings when she was admitted to hospital 9 years ago, and died after her second encephalopathy attack.Our patient had fever and vomiting 3 days ago, and had a right hemiclonic seizure which lasts for 10 minutes. He was hospitalized elsewhere and he received intravenous ceftriaxone. For two days he didn’t have any symptoms. On the third day he vomited multiple times, signs of pyramidal and brain stem involvement were observed and he became encephalopathic. His glascow coma scale was 3 on arrival. He was entubated for mechanical ventilation. EEG showed slow-wave activity consistent with encephalopathy without any epileptiform discharges. Magnetic resonance imaging showed hyper-intensities in bilateral thalamic regions, external capsules, temporal lobes and cerebellar hemispheres. Susceptibility weighted imaging (SWI) series showed bilateral thalamic hemorrhage and necrosis dominantly in the right thalami. Based on this findings and family history of acute necrotizing encephalopathy of childhood we decided to give him 30 mg/kg per day pulse methylprednisolone. On the second day of methylprednisolone treatment he died. Genetic evaluation of RANBP2 mutation is being carried on. Conclusion: Acute necrotizing encephalopathy of childhood is a rare and fatal disorder. Bilateral thalamic hemorrhage and necrosis is characteristic for diagnosis. Mutation of the RANBP2 gene should be investigated, when it is seen recurrent in a patient or in other family members.
http://dx.doi.org/10.1016/j.ejpn.2017.04.977 P2-79 Polio-like phenotype in pediatric acute flaccid paralysis associated with enterovirus infection: The 2016 French experience M. Kossorotoff, M. Aubart, I. Schuffenecker, M. Leruez-Ville, F. Moulin, R. Carlier, C. Gitiaux, I. Desguerre. Department of Pediatric Neurology, APHP University Hospital Necker-Enfants malades, Paris, France Objective and Methods: In France in 2016, an excess of acute severe neurologic conditions associated with Enterovirus (EV) infection was observed and a nationwide case collection was performed. Within this case collection, we report our monocentric experience of 22 pediatric EV-associated severe neurologic conditions over 6 months and focus on the clinical, radiological, neurophysiological, and virological description of polio-like patients. Results: Several phenotypes were observed, related with outcome. Two thirds of patients had favorable outcome: they had either isolated cerebellitis (n¼4), rhombencephalitis (n¼4),
e121
encephalomyelitis (n¼3), isolated myelitis (n¼1), or acute inflammatory demyelinating polyneuropathy (n¼2). One third of patients had poor outcome, because of rhombencephalomyelitis associated with myocarditis (prolonged ventilatory support, n¼2), or acute flaccid paralysis with persistent polio-like motor deficit (n¼6). Polio-like patients associated initial monoplegia (n¼5) or suspended motor deficit (n¼1) with axonal motor neuropathy, and anterior spinal magnetic resonance imaging (MRI) abnormalities. Interestingly, muscle MRI showed segmental atrophia with denervation T2 hypersignal, without nerve root or nerve trunk associated topography, suggesting motoneuron lesion in anterior horn spine rather than radiculitis. These six children were positive for various non-polio enteroviruses, with only one patient positive for EV-68. Conclusion: These findings suggest that the mechanism of neurotoxicity towards the motoneuron in children with EV-associated polio-like syndromes may be related to still unknown environmental, immunological or virological cofactors rather than neurotropic virulence related to a single EV strain.
http://dx.doi.org/10.1016/j.ejpn.2017.04.978 P2-80 Acute cerebellitis in children: A series of eight cases € c¸men, Mirac¸ Yıldırım, Rahs‚an Go Bahadır Konus‚kan, lu, Banu Anlar. Hacettepe University Faculty of Dilek Yalnızog Medicine, Departments of Pediatric Neurology, Ankara, Turkey Objective: Acute cerebellitis is a rare condition often characterized by severe headache, vomiting, fever, ataxia and loss of consciousness. Its course is usually self-limited but occasionally fulminant. Differentiation of acute infectious cerebellitis from postinfectious cerebellitis is usually based on the presence of fever and absence of a latent period following a non-specific infection. Methods: We present clinical and paraclinical findings, treatment and outcome data of eight children diagnosed with acute cerebellitis in our department between 2010-2016. Results: There were 5 girls and 3 boys aged 3-16.5 years, median 8 years. Main presenting symptoms were headache, fever, vomiting and ataxia. Most common MR imaging finding at presentation was bilateral, diffuse signal changes in cerebellar hemispheres. Two patients had hydrocephalus, one had herniation, and one, both hydrocephalus and herniation. Etiological work-up for infectious pathogens revealed mycoplasma pneumoniae and influenza virus in one case each. Two cases underwent lumbar puncture: one was normal, the other showed elevated protein and low glucose. Treatment included steroids in all cases, acyclovir in 7, antibiotics in 2, intravenous immunoglobulins in 1, oseltamivir in one case. No patients required surgery for hydrocephalus or herniation. Four patients had full recovery and four had residual neurologic deficits consisting in cerebellar tremor and ataxia (n¼3) and cognitive deficit (n¼2). Patients with neurological sequalae had cerebellar atrophic changes on follow-up MRI. Patients admitted >7 days after symptom onset recovered with sequalae while those admitted<3 days showed complete recovery. Conclusion: There were no fatalities in this series of acute cerebellitis, suggesting early diagnosis and treatment can increase the chance of uneventful recovery.
http://dx.doi.org/10.1016/j.ejpn.2017.04.979