- Email: [email protected]
P112 – 2064 Acute necrotising encephalopathy in two children
S84
E U R O P E A N JO U R N A L O F PAEDIATRIC N E U R O L O G Y
and choreoathetoid movements. The anti-N-Methyl-D-Aspartate Receptor antibodies was positive in serum and CSF on 28th day. This case supported the presence of autoimmunity in the pathogenesis of herpes encephalitis. Therefore we believe that the cases with previous herpes ensefalitis who benefited from steroids should be re-evaluated in the light of this information.
P111 - 2090 VGKC antibodies: can become positive 4 weeks after presentation Singh J, Kashyape P, Kirkham F. Southampton General Hospital, UK – [email protected] Objective: To report a previously unreported characteristic of Voltage Gated Potassium Channel (VGKC) antibodies. Material and methods: Retrospective review of case notes. Results: 11 year old boy presented with acute onset focal seizures with occasional secondary generalisation. This was preceded by an upper respiratory infection approximately 2 weeks before seizure onset. He was also reported to have behavioural problems in the form of aggression which continued after seizure onset. His initial investigations all done within 1 week of seizure onset showed mild hyponatremia and mildly high signal in the left hippo campus which was felt to be secondary to the seizures rather than the cause for seizures. The EEG was encephalopathic and a left hemispheric electrographic seizure was noted during the recording with a left temporal origin. Urine organic acids, blood for lactate, ammonia, amino acid, acylcarnitines were negative. As the presentation was consistent with Autoimmune Encephalitis, extended antibody screen including against NMDAR, VGKC, GAD and others were negative at this time. Child was empirically treated for Autoimmune Encephalitis with high dose IV Methyl Prednisilone for 5 days followed by a tapering course. There was initial good response but seizures recurred within the week and so Levetiracetam was added. The repeat autoimmune screen, sent 4 weeks after seizure onset, confirmed the diagnosis of VGKC Encephalitis as the positive antibody result was reported. In view of this he was treated with intravenous immunoglobulin and responded favourably. It seems likely that he will require further immunomodulatory drugs. Conclusions: Our literature search did not bring up any reports of a similar presentation in which the antibodies became positive >4 weeks after initial presentation on repeat sampling in the paediatric population. This case adds an important and valuable vignette in the investigation and hence the treatment of this rare condition.
P112 - 2064 Acute necrotising encephalopathy in two children Diakogeorgiou A, Deconinck N, De Laet C, Dan B, Monier A. Department of Pediatric Neurology, HUDERF, ULB Brussels, Belgium – [email protected] Acute necrotising encephalopathy (ANEC) is a rare rapidly progressive paediatric encephalopathy. The MRI hallmark of the disease consists of symmetric, multifocal lesions in the thalami with variable involvement of the white matter, basal ganglia, brainstem and cerebellum. Pathogenesis is supposed cytokine mediated after viral infection. Mutations in RANBP2 have been recently associated with familial and recurrent cases of ANEC. Two children aged 3 and 10 years old respectively, presented with high fever and respiratory tract infection. Both developed altered consciousness rapidly progressing to coma. The youngest presented focal seizures. Routine laboratory tests were unremarkable except a slight elevation of serum aminotransferase for the second patient. CSF evaluation showed mild proteinorachia in the first patient and pleiocytosis in the second. Extensive infectious work-up was negative, except a positive Rhinovirus
17s (2013) S1 – S149
PCR on nasopharyngeal aspirate for to the first patient. Successive brain MRIs of the first patient demonstrated symmetric T2 hyperintensity in the thalami extending to the basal ganglia, infracortical white matter, cortex, and cerebellar peduncles. MRI of the second patient demonstrated asymmetric T2 hyperintensity in the insular cortex, the mesencephalon and the basal ganglia. Autoimmune, mitochondrial, organic acid and urea cycle disorders were excluded. NMDA receptor antibodies were absent. Genetic test for RANPB2 is pending. Brain biopsy in the second patient was not pathognomonic. Diagnosis of ANEC was evoked and corticosteroids were administred, followed by immunoglobulins and ultimately plasmapheresis. Clinical outcome was poor, with dystonic quadriplegia and dysautonomic disorder in both patients and additional parkinsonism in the second. Patients are currently severely disabled and necessitate gastrostomy feeding. Acute encephalopathy after viral illness is a severe condition requiring prompt management to improve neurological outcome. However, prognosis remains poor. The typical imaging features of ANEC will be developed in the poster.
P113 - 2059 Recurrent acute disseminated encephalomyelitis in a child or multiple sclerosis? Bouchaala W, Chaari D, Jemaa R, Sakka S, Kammoun F, Triki C. Child neurology department, Hedi Chaker Hospital, Sfax, Tunisia – [email protected] Introduction: A number of clinical and para-clinical data are similar between multiple sclerosis (MS) and acute dessiminated encephalomyelitis (ADEM), making difficult the distinguishing between these two diseases. The differentiation of these two conditions is still important both in diagnostic and therapeutic decision. Aim: Through the observation of a patient with recurrent neurological symptoms, we will try to find criteria to distinguish between these 2 entities. Case report: A 9 years old boy without any previous medical history presented at the age of 3 years an acutepost infectious cerebellar syndrome spontaneously regressive. MRI showed T2 signal hyperitensity lesion at the brain stem, basal ganglia, temporal lobes and external capsule. Four months later, he had presented an episod of hemiplegia and brain MRI showed new hypersignal in spinal cord extented to more two vertebers and in the cerebral white matter with regression of the lesions described above. The child received corticostroids in bolus. Actually, the patient had left hemiparesis and MRI showed disappearence of brain and spinal lesion with spinal atrophy at three months and 6 years later. The visual evoked potentials showed bilateral retrobulbar optic neuritis. Discussion: The differential diagnosis between MS and ADEM is more difficult in child then in adult. Although, some clinical and radiological criteria are predictive of ADEM: recent post infectious motor disorder, lesions with same age and some specific location as the brain stem, cortex, subcortical white matter and with favorable radiological evolution. In case of recurrent ADEM, clinical and radiological symptoms are located in the same territory then the first episode. The diagnosis of MS is suggested by the appearance of other lesion in different territory and the presence of infraclinical retrobulbar optic neuritis.
P114 - 2049 Autoimmune limbic encephalitis and DRESS syndrome: is there an association? Maras H, Ipekci B, Yologlu N, Aydogan M, Kara B. Kocaeli University Medical Faculty, Department of Pediatrics, Division of Child Neurology, Kocaeli, Turkey – [email protected] Autoimmune encephalitis (AE) is characterised by intractable seizures, cognitive impairment and psychiatric symptoms. The
E U R O P E A N JO U R N A L O F PAEDIATRIC N E U R O L O G Y
and choreoathetoid movements. The anti-N-Methyl-D-Aspartate Receptor antibodies was positive in serum and CSF on 28th day. This case supported the presence of autoimmunity in the pathogenesis of herpes encephalitis. Therefore we believe that the cases with previous herpes ensefalitis who benefited from steroids should be re-evaluated in the light of this information.
P111 - 2090 VGKC antibodies: can become positive 4 weeks after presentation Singh J, Kashyape P, Kirkham F. Southampton General Hospital, UK – [email protected] Objective: To report a previously unreported characteristic of Voltage Gated Potassium Channel (VGKC) antibodies. Material and methods: Retrospective review of case notes. Results: 11 year old boy presented with acute onset focal seizures with occasional secondary generalisation. This was preceded by an upper respiratory infection approximately 2 weeks before seizure onset. He was also reported to have behavioural problems in the form of aggression which continued after seizure onset. His initial investigations all done within 1 week of seizure onset showed mild hyponatremia and mildly high signal in the left hippo campus which was felt to be secondary to the seizures rather than the cause for seizures. The EEG was encephalopathic and a left hemispheric electrographic seizure was noted during the recording with a left temporal origin. Urine organic acids, blood for lactate, ammonia, amino acid, acylcarnitines were negative. As the presentation was consistent with Autoimmune Encephalitis, extended antibody screen including against NMDAR, VGKC, GAD and others were negative at this time. Child was empirically treated for Autoimmune Encephalitis with high dose IV Methyl Prednisilone for 5 days followed by a tapering course. There was initial good response but seizures recurred within the week and so Levetiracetam was added. The repeat autoimmune screen, sent 4 weeks after seizure onset, confirmed the diagnosis of VGKC Encephalitis as the positive antibody result was reported. In view of this he was treated with intravenous immunoglobulin and responded favourably. It seems likely that he will require further immunomodulatory drugs. Conclusions: Our literature search did not bring up any reports of a similar presentation in which the antibodies became positive >4 weeks after initial presentation on repeat sampling in the paediatric population. This case adds an important and valuable vignette in the investigation and hence the treatment of this rare condition.
P112 - 2064 Acute necrotising encephalopathy in two children Diakogeorgiou A, Deconinck N, De Laet C, Dan B, Monier A. Department of Pediatric Neurology, HUDERF, ULB Brussels, Belgium – [email protected] Acute necrotising encephalopathy (ANEC) is a rare rapidly progressive paediatric encephalopathy. The MRI hallmark of the disease consists of symmetric, multifocal lesions in the thalami with variable involvement of the white matter, basal ganglia, brainstem and cerebellum. Pathogenesis is supposed cytokine mediated after viral infection. Mutations in RANBP2 have been recently associated with familial and recurrent cases of ANEC. Two children aged 3 and 10 years old respectively, presented with high fever and respiratory tract infection. Both developed altered consciousness rapidly progressing to coma. The youngest presented focal seizures. Routine laboratory tests were unremarkable except a slight elevation of serum aminotransferase for the second patient. CSF evaluation showed mild proteinorachia in the first patient and pleiocytosis in the second. Extensive infectious work-up was negative, except a positive Rhinovirus
17s (2013) S1 – S149
PCR on nasopharyngeal aspirate for to the first patient. Successive brain MRIs of the first patient demonstrated symmetric T2 hyperintensity in the thalami extending to the basal ganglia, infracortical white matter, cortex, and cerebellar peduncles. MRI of the second patient demonstrated asymmetric T2 hyperintensity in the insular cortex, the mesencephalon and the basal ganglia. Autoimmune, mitochondrial, organic acid and urea cycle disorders were excluded. NMDA receptor antibodies were absent. Genetic test for RANPB2 is pending. Brain biopsy in the second patient was not pathognomonic. Diagnosis of ANEC was evoked and corticosteroids were administred, followed by immunoglobulins and ultimately plasmapheresis. Clinical outcome was poor, with dystonic quadriplegia and dysautonomic disorder in both patients and additional parkinsonism in the second. Patients are currently severely disabled and necessitate gastrostomy feeding. Acute encephalopathy after viral illness is a severe condition requiring prompt management to improve neurological outcome. However, prognosis remains poor. The typical imaging features of ANEC will be developed in the poster.
P113 - 2059 Recurrent acute disseminated encephalomyelitis in a child or multiple sclerosis? Bouchaala W, Chaari D, Jemaa R, Sakka S, Kammoun F, Triki C. Child neurology department, Hedi Chaker Hospital, Sfax, Tunisia – [email protected] Introduction: A number of clinical and para-clinical data are similar between multiple sclerosis (MS) and acute dessiminated encephalomyelitis (ADEM), making difficult the distinguishing between these two diseases. The differentiation of these two conditions is still important both in diagnostic and therapeutic decision. Aim: Through the observation of a patient with recurrent neurological symptoms, we will try to find criteria to distinguish between these 2 entities. Case report: A 9 years old boy without any previous medical history presented at the age of 3 years an acutepost infectious cerebellar syndrome spontaneously regressive. MRI showed T2 signal hyperitensity lesion at the brain stem, basal ganglia, temporal lobes and external capsule. Four months later, he had presented an episod of hemiplegia and brain MRI showed new hypersignal in spinal cord extented to more two vertebers and in the cerebral white matter with regression of the lesions described above. The child received corticostroids in bolus. Actually, the patient had left hemiparesis and MRI showed disappearence of brain and spinal lesion with spinal atrophy at three months and 6 years later. The visual evoked potentials showed bilateral retrobulbar optic neuritis. Discussion: The differential diagnosis between MS and ADEM is more difficult in child then in adult. Although, some clinical and radiological criteria are predictive of ADEM: recent post infectious motor disorder, lesions with same age and some specific location as the brain stem, cortex, subcortical white matter and with favorable radiological evolution. In case of recurrent ADEM, clinical and radiological symptoms are located in the same territory then the first episode. The diagnosis of MS is suggested by the appearance of other lesion in different territory and the presence of infraclinical retrobulbar optic neuritis.
P114 - 2049 Autoimmune limbic encephalitis and DRESS syndrome: is there an association? Maras H, Ipekci B, Yologlu N, Aydogan M, Kara B. Kocaeli University Medical Faculty, Department of Pediatrics, Division of Child Neurology, Kocaeli, Turkey – [email protected] Autoimmune encephalitis (AE) is characterised by intractable seizures, cognitive impairment and psychiatric symptoms. The