Abstracts: Poster Presentations, the Seventh European Paediatric Neurology Society (EPNS) Congress 97

condution studies (NCS). All the investigations were carriedout by Medelec Mystro type MS 20 electromyograph. None ofpatients showed overt clinical signs of polyneuropathy.Results: While unilateral facial motor NCS showed markedlyreduced nerve conduction velocities (NCVs) and prolongeddistal latencies, motor NCS of above nerves of upper andlower limbs revealed considerably reduced NCVs. Compoundmotor action potential amplitudes were near normal. In3 patients we observed mildly elevated protein content incerebrospinal fluid.Conclusions: In children with idiopathic Bell’s palsy whoshow preferably demyelination of facial motor nerves,upper and lower motor NCS may also reveal subclinicalelectrophysiologic abnormalities which are suggestive ofgeneralized demyelinating polyneuropathy.

IAP040 Ramsay Hunt syndrome: a rare cause of facialpalsy in childhood

E. Demir*, E. Arhan, A. Akturk, T. Hirfanoglu, A. Serdaroglu,K. Gucuyener. Gazi Hospital/Department of Pediatric Neurology,Gazi University, Ankara, Turkey

Background: Ramsay Hunt syndrome is rare disorder inchildhood, characterised by facial palsy, herpetic vesicles inthe auricle and vestibulocohlear dysfunction. It is caused byreactivation of latent Varicella zoster virus in the geniculateganglia. Facial palsy improves with acyclovir and steroidtherapy. Complete recovery depends on early treatment atthe onset facial palsy.Case report: We report a 12-year-old girl who presented withright peripheral facial palsy, ear pain, and simultaneouslydeveloped vesicles in the auricle. She was diagnosed asRamsay Hunt syndrome.We started treatment two days afterthe onset of facial palsy and intravenous acyclovir was givenfor 7 days, followed by oral acyclovir for a week. Facial palsyimproved completely within 20 days.Conclusion: Our case shows that Ramsay Hunt syndromecan be treated with acyclovir alone and also indicatesthe importance of early diagnosis and treatment on goodoutcome.

IAP041 Mycoplasma pneumoniae-associatedopsoclonus-myoclonus syndrome in threeadolescents

S. Strozzi1 *, S. Flury2, A. Duppenthaler2, M. Steinlin1.1University Children’s Hospital Bern, Division of PediatricNeurology, 2University Children’s Hospital Bern, Department ofPediatrics, Switzerland

Background: Opsoclonus-myoclonus syndrome (OMS) is arare movement disorder characterized by involuntary, chaoticocular saccades, myoclonus and/or ataxia. OMS appearstypically in young children, commonly associated with a viralinfection or a neuroblastoma. OMS is also described in adults,usually in more than 40 years old patients, as a idiopathic,parainfectious or paraneoplastic condition. There are onlyfew reports of OMS in teens.Case reports:We report three adolescents with a acute onsetof OMS associated with mycoplasma pneumoniae-infection.The patients, aged between 11 and 15 years, presented withopsoclonus, myoclonus and/or ataxia, following a febrilerespiratory tract illness. Cerebrospinal fluid examinationrevealed mild pleocytosis and intrathecal immunglobulinproduction. Brain MRI was normal and diagnostic work-upfor underlying tumor (urinary catecholamine metabolites,abdominal ultrasound and thoracic radiography) negative.Serology for mycoplasma pneumoniae was highly reactive inall three patients. The patients were treated with high dosemethylprednisolon intravenous, followed by prednisolonperorally for 1−6 months. The recovery was complete in allthree patients within 3 months whitout relapse.

Conclusion: OMS can occur also in adolescents, especially asa parainfectious condition. As described in few prior reports,the clinical course of parainfectious OMS in elder childrenseems to be monophasic and more benign.

IAP042 Opsoclonus-Myoclonus-Ataxia: neuropsychologicaland neuroradiological sequelae

E. De Grandis1 *, F. Battaglia1, A. Pessagno1, P. Liberati1,P. Angelini2, M. Conte2, C. Gandolfo3, G. Morana3,M. Mancardi1, E. Veneselli1. 1O.U. Child Neuropsychiatry,Gaslini Institute, University of Genoa, Italy, 2Department ofHematology-Oncology, Gaslini Institute, Genoa, Italy, 3Division ofNeuroradiology, Gaslini Institute, Genoa, Italy

Background: Opsoclonus-Myoclonus-Ataxia (OMA) is a se-rious neurologic condition that is often a paraneoplasticmanifestation of occult neuroblastoma (NB) in early child-hood. Despite different treatments, the outcome includesdevelopmental and neuropsychological sequelae.Aims: Evaluation of neurodevelopmental sequelae in OMAwith/without NB.Methods: We studied 17 pts observed from 1983 to 2006 atGaslini Institute; in 14 patients NB was demonstrated. Meanfollow-up was of 7 years (7mo to 23yrs). At follow-up recordswere reviewed and all children underwent neurological ex-amination, 13 children underwent comprehensive cognitiveand neuropsychological evaluations; MRI was performed in14/17 pts.Results: Two pts had complete recovery after NB resection;14 pts received treatment with steroids and 8 of themcombined treatment with steroids and IVIg, prolonged for anaverage of 16 mo (3−67 mo). Four unresponsive pts receivedACTH and/or immunosuppressive agents.At follow-up 5/14 pts (36%) presented mental retardationand 13/13 pts showed various degree of neuropsychologicalsequelae. Five out of 14 pts (36%) had cerebellar atrophy onMRI.Conclusions: OMA causes developmental sequelae that arenot adequately prevented by current treatment. As statedby the other few neuropsychological studies, OMA may be aprogressive encephalopathy rather than a time-limited singleinsult.

IAP043 Limbic encephalitis in a 9-year-old child dueto voltage-gated potassium channel antibody(VGKC-Ab)

H.F. Cakmakli1 *, K.K. Oguz2, G. Haliloglu3, M. Topcu3.1Hacettepe University, Department of Pediatrics, 2HacettepeUniversity, Department of Radiology, 3Hacettepe University,Departmentof Pediatric Neurology, Ankara, Turkey

Background: Limbic encephalitis is characterized by subacuteonset of episodic memory impairment, disorientation andagitation commonly associated with hallucinations, sleepdisturbances and histological evidence of medial temporallobe inflammation. Limbic encephalitis is considered tobe paraneoplastic in origin with poor prognosis and non-paraneoplastic, autoimmune due to voltage-gated potassiumchannel antibodies (VGKC-ab) with a potentially treatablenature.Case presentation: A previously healthy 9-year-old girl wasreferred to our hospital with memory impairment, decreasein academic performance and social relations. Her pasthistory and family history were unremarkable. She had aprogressive loss of speech within two months and markedslowing in daily motor activity. On physical examinationshe had poor orientation, no spontaneous speech, apathy,dysarthria and wide-based gait. Electroencephalographyshowed mild encephalopathy with bilateral backgroundslowing in frontal regions. CSF viral serology, metabolic