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P21.3 Peripheral facial palsy associated with viral infections in children
Poster sessions P21.3 Peripheral facial palsy associated with viral infections in children E. Diana1 , M. Sandu1 *, R.I. Teleanu1 , D. Vasile1 , D. Plesca1 . 1 Pediatric Neurology Clinic “Dr. V. Gomoiu” Hospital, Bucharest, Romania Purpose: A prospective clinical and virological study on 42 patients with acute, peripheral facial paralysis was carried out in consecutive cases during one year. Methods: Children hospitalized at the “Dr. V. Gomoiu” Children Hospital, Bucharest, Romania, from 2009 to 2010 with clinical signs of peripheral facial palsy were studied prospectively; we evaluated each patient with cerebral IRM, cerebral spinal fluid and blood analysis, EEG. Results: 20 cases with no detectable cause (idiopathic), 19 cases with infectious causes, 3 cases with demyelization diseases (multiple sclerosis); we found 3 cases with Epstein Barr virus, 8 cases with Lyme disease, 5 cases with herpes simplex virus and 3 cases with cytomegalovirus; we are describing the case of a 3 years old girl with facial diplegia that was induced by the infection with herpes simplex virus type 1 and the case of 5 years old boy with left peripheral facial palsy that was induced by the infection with Epstein Barr virus. Conclusions: It’s important to diagnosticate promptly and correctly a facial palsy to obtain a quick and total recovery. P21.4 Pontine tegmental cap dysplasia: further definition in a patient with a mild presentation B.T. Poll-The1 *, C. Majoie1 , M. Caan1 , J. Niermeijer2 , M. Weterman2 , F. Baas2 , P. Barth2 . 1Academic Medical Center Amsterdam, Pediatric Neurology, Amsterdam, The Netherlands, 2 Academic Medical Center, University of Amsterdam, The Netherlands, Netherlands The descriptive name pontine tegmental cap dysplasia (PTCD: Barth et al 2007) specifies a distinct hindbrain malformation with abnormal axonal guidance in the brainstem, causing ventral pontine hypoplasia, “capping” of the pontine tegmentum, caused by an ectopic transverse nerve bundle and cerebellar hypoplasia. To date, ten patients have been reported with PTCD. Most patients have severe motor and cognitive impairments, variable cranial nerve involvement and vertebral anomalies. We now report an 111/2 year old boy with PTCD and a very mild clinical presentation. Clinical findings include hearing impairment, nystagmus, impaired swallowing, ataxic gait, thermolability during the night and normal cognition. Examination on a 3 Tesla MRI scanner for diffusion tensor imaging with colour coding tractography revealed additional insights on the abnormal axonal pathfinding. The disorder may be more common than realized and should also be suspected in relatively mild patients. The etiology is unknown. P21.5 A central nervous system tuberculosis mimicking ADEM 1 1 ¨ *, Z.I.Hasıloglu1 , S. Lacinel ¸ , S. Albayram1 , S. Uysal1 . O. Unver 1 Istanbul University Cerrahpa¸sa Medical School, Turkey Tuberculosis is still an important cause of morbidity and mortality in developing countries. Although uncommon, central nervous system (CNS) tuberculosis causes neurologic sequela and high rates of mortality if the treatment is delayed. Modern imaging is a cornerstone in the early diagnosis of CNS tuberculosis and may prevent unnecessary morbidity and mortality. Contrast enhanced magnetic resonance imaging (MR) is generally considered as the modality of choice in the detection and assesment of CNS tuberculosis. Here we present a nine year old girl who was brought with the complaint of seizures and diagnosed as ADEM radiologically
S117 and treated with pulse steroid. After the cessationof steroid therapy her complaints reversed and she was referred to our clinic. On her cranial MRI tuberculomas were detected and she was diagnosed as CNS tuberculosis. P21.6 Mild encephalitis/encephalopathy with a reversible splenial lesion (MERS): two new caucasian patients with uncommon clinical features Z. Liptai1 *, B. Ivady1 , G. Varallyay2 , P. Barsi2 . 1 Szent Laszlo Hospital, Budapest, Hungary, 2 Semmelweis University, Budapest, Hungary MERS has been increasingly reported recently, mostly by Japanese authors. We report two new cases of Caucasian patients and discuss literature data and own observations concerning possible pathomechanism. 1. 15-years-old girl developed headache, fever, dizziness, vomiting and stiff neck in 4 days. CSF showed elevated protein and cell count, se-Na was 131 mmol/L. MRI (day 7) was normal, but she remained febrile, had cerebral oedema and had episodes of confusion. MRI on day 11 showed a T2hyperintense lesion with restricted diffusion in the callosal splenium. Adenoviral infection was proved, and the girl had a protracted course of recovery. MRI signal alterations decreased in 6 days and disappeared in 3 months. 2. 12.5-years-old girl manifested with headache, apathy, drowsiness and vomiting. On day 5 she experienced inability to speak and right-sided weakness lasting 12 hours. She was confused and disoriented. MRI disclosed a tiny area of increased T2-signal and restricted diffusion in the splenium. Se-Na was 133 mmol/L, CSF cell count and protein was markedly elevated, and enteroviral infection was detected. Echocardiography showed minimal mitral and tricuspid valve prolapse. Her complaints resolved in a week and MRI was normal 7 weeks later. These two non-epileptic children increase the small number of Caucasian patients reported so far. Both had hyponatraemia, encephalitis with significant CSF changes and patient 2 had transient ischemic attack (not yet reported in MERS) possibly at least partly due to the cerebral oedema resulting in MERS P21.7 Biotin non-responsive familial infantile bilateral striatal necrosis associated with primary influenzae A infection T.H. Yeo1 *, C. De Goede2 , Y.J. Crow3 . 1 Department of Paediatric Neurology, Royal Manchester Children’s Hospital, Manchester, United Kingdom, Department of Paediatric Neurology, Royal Preston Hospital, Preston, United Kingdom, 2 Department of Paediatric Neurology, Royal Preston Hospital, Preston, United Kingdom, 3 University of Manchester, Manchester Academic Health Science Centre, Central Manchester University Hospitals NHS Foundation Trust, St Mary’s Hospital, Manchester, United Kingdom Introduction: Infantile bilateral striatal necrosis (IBSN) is a rarely described basal ganglia disorder characterised by symmetrical degeneration of striatum. Familial cases have been previously reported, and related genetic abnormalities included mitochondrial inheritance and nup62 mutations. Biotin-responsive basal ganglia disease (BBGD) is a recessive disorder and characterised by subacute encephalopathy, developmental arrest, movement disorder and quadriparesis. We report on two brothers presented with IBSN associated with recent primary infection of Influenza A, and their clinical presentations phenotypically resemble those with BBGD. Case Study: The two brothers presented during the first year of life with subacute encephalopathy following upper respiratory tract infection, developmental arrest, choreathetosis, evolving generalised dystonia, spasticity and rigidity. Extensive neurometabolic investigations were all unremarkable. Sibling 2 had serological evidence of
S117 and treated with pulse steroid. After the cessationof steroid therapy her complaints reversed and she was referred to our clinic. On her cranial MRI tuberculomas were detected and she was diagnosed as CNS tuberculosis. P21.6 Mild encephalitis/encephalopathy with a reversible splenial lesion (MERS): two new caucasian patients with uncommon clinical features Z. Liptai1 *, B. Ivady1 , G. Varallyay2 , P. Barsi2 . 1 Szent Laszlo Hospital, Budapest, Hungary, 2 Semmelweis University, Budapest, Hungary MERS has been increasingly reported recently, mostly by Japanese authors. We report two new cases of Caucasian patients and discuss literature data and own observations concerning possible pathomechanism. 1. 15-years-old girl developed headache, fever, dizziness, vomiting and stiff neck in 4 days. CSF showed elevated protein and cell count, se-Na was 131 mmol/L. MRI (day 7) was normal, but she remained febrile, had cerebral oedema and had episodes of confusion. MRI on day 11 showed a T2hyperintense lesion with restricted diffusion in the callosal splenium. Adenoviral infection was proved, and the girl had a protracted course of recovery. MRI signal alterations decreased in 6 days and disappeared in 3 months. 2. 12.5-years-old girl manifested with headache, apathy, drowsiness and vomiting. On day 5 she experienced inability to speak and right-sided weakness lasting 12 hours. She was confused and disoriented. MRI disclosed a tiny area of increased T2-signal and restricted diffusion in the splenium. Se-Na was 133 mmol/L, CSF cell count and protein was markedly elevated, and enteroviral infection was detected. Echocardiography showed minimal mitral and tricuspid valve prolapse. Her complaints resolved in a week and MRI was normal 7 weeks later. These two non-epileptic children increase the small number of Caucasian patients reported so far. Both had hyponatraemia, encephalitis with significant CSF changes and patient 2 had transient ischemic attack (not yet reported in MERS) possibly at least partly due to the cerebral oedema resulting in MERS P21.7 Biotin non-responsive familial infantile bilateral striatal necrosis associated with primary influenzae A infection T.H. Yeo1 *, C. De Goede2 , Y.J. Crow3 . 1 Department of Paediatric Neurology, Royal Manchester Children’s Hospital, Manchester, United Kingdom, Department of Paediatric Neurology, Royal Preston Hospital, Preston, United Kingdom, 2 Department of Paediatric Neurology, Royal Preston Hospital, Preston, United Kingdom, 3 University of Manchester, Manchester Academic Health Science Centre, Central Manchester University Hospitals NHS Foundation Trust, St Mary’s Hospital, Manchester, United Kingdom Introduction: Infantile bilateral striatal necrosis (IBSN) is a rarely described basal ganglia disorder characterised by symmetrical degeneration of striatum. Familial cases have been previously reported, and related genetic abnormalities included mitochondrial inheritance and nup62 mutations. Biotin-responsive basal ganglia disease (BBGD) is a recessive disorder and characterised by subacute encephalopathy, developmental arrest, movement disorder and quadriparesis. We report on two brothers presented with IBSN associated with recent primary infection of Influenza A, and their clinical presentations phenotypically resemble those with BBGD. Case Study: The two brothers presented during the first year of life with subacute encephalopathy following upper respiratory tract infection, developmental arrest, choreathetosis, evolving generalised dystonia, spasticity and rigidity. Extensive neurometabolic investigations were all unremarkable. Sibling 2 had serological evidence of