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P19.13 A childhood multiple sclerosis case presenting with syringomyelia and posterior cord findings
Poster sessions of the disease activity. Imunosupressants or monoclonal antibodies are another treatment options in a failure of imunomodulatory treatment. Aim: Presentation of a successful treatment of relapsing remitting pediatric MS case with monoclonal antibodies after the treatment failure of interferons b and imunosupressive therapy. A case report of a pediatric onset relapsing remitting MS is presented. He started pediatric with a polisymptomatic first episode in the age of 13 years, treated with acute methylprednisolon i.v. pulse therapy, followed by oral tappering due to steroid dependancy. Before starting imunomodulatory interferon b treatment, methotrexate was intoduced due to further disease progression in next months. He received dual treatment with interferon b and methotrexate for almost 2 years being free of relapses and with stable EDSS during that time. There were no signs of disease activity in MRI. Methotrexate was stopped after that period due to evolution of hepatopathy but he continued to receive interferons b. Relapses repeated shortly after stopping methotrexate, MRI disclosed new lesions and EDSS worsened. There were no neutralizing antibodies to interferons. He started treatment with natalizumab in January 2010 (age 16.5 years) without any side effects or relapses so far and his EDSS improved as well. Conclusion: Natalizumab is used for a treatment of relapsing remitting MS cases in adults after the treatment failure of imunomodulatory or imunosupressive therapy. There is an evidence of a rising number of pediatric MS cases who were successully treated with monoclonal antibodies as well. P19.12 Acute hemorrhagic leukoencephalitis in infant with rapid neurological deterioration 1 ˇ R. Kuzmanic*, K. Kolic2 , J. Meˇstrovic´ 3 , B. Polic3 , ´ Samija D. Fradelic4 , J. Markic3 , B. Reˇsic´ 1 , M. Tomasovic´ 1 . 1 Department of Child Neurology, Clinical Hospital Split, Medical School of University of Split, Split, Croatia, 2 Department of Radiology, Clinical Hospital Split, Medical School of University of Split, Split, Croatia, 3 Intensive Care Units, Clinical Hospital Split, Medical School of University of Split, Split, Croatia, 4 Clinical Hospital Split, Medical School of University of Split, Split, Croatia
Background: Acute hemorrhagic leukoencephalitis, also known as acute necrotizing encephalopathy is a hyperacute and frequently fatal form of acute disseminated encephalomyelitis. Acute hemorrhagic encephalomyelitis is relatively rare (less than 100 cases have been reported in the medical literature as of 2010), it is seen in about 2% of acute disseminated encephalomyelitis cases. Aim of the study: We report a case of eight month female infant who developed a picture of acute hemorrhagic encephalomyelitis, which progressed from acute disseminated encephalomyelitis in period less of one week. Results: She was referred to the hospital because of high fever and severe convulsions following a 2-day prodromal illness with cough and diarrhea. The initial MR imaging findings showed high intensity lesions of the thalamus, pons and medulla oblongata on the T2-weighted, FLAIR and low signal intensity on the diffusion weighted images. After eight days of admission, follow-up MR imaging findings showed high intensity signal in basal ganglia on T1-weighted that indicate the presence of hemorrhage without the restriction of diffusion. MR imaging findings was supported by progressive symptoms of the extrapyramidal system with uncoordinated and inappropriate movement of limb and head, muscular hypotonic without eye contact. Conclusion: Case represents an example of an atypical occurrence of age for acute hemorrhagic leukoencephalitis with extremely rapid progression from initial disseminated
S111 encephalomyelitis and dramatic neurological symptoms that have developed within one week of admission. P19.13 A childhood multiple sclerosis case presenting with syringomyelia and posterior cord findings P. Topaloglu Tekturk1 *, T. Aydin2 , Z. Yapici1 , M. Eraksoy1 . 1 Division of Child Neurology, Department of Neurology, Istanbul University Faculty of Medicine, Capa-Istanbul, Turkey, 2 Goztepe Education and Research Hospital, Department of Neurology, Kadikoy − Istanbul, Turkey Syringomyelia is described as a cavitary enlargement of the spinal cord. Among patients with multiple sclerosis (MS), non-communicating syringomyelia (NCS) has been described in various reports on small case series or case studies including post-mortem analysis. It can be a subtle finding without clinical correlates but it has been considered that syrinx formations are more likely to be a consequence of MS cord pathology than a coincidental finding. We want to present a 15 years old boy presenting with paresthesia of his legs and walking difficulty. His neurological examination revealed long tract and posterior cord involvement and bilateral sensory deficit below L1 level. His routine blood tests including hemogram, serum electrolytes, vitamin B12, vasculitic markers, routine biochemical, liver and thyroid function tests were within normal limits. His electromyography revealed chronic L4-S1 anterior horn involvement. His cranial MRI showed multiple supra and infra tentorial demyelinating lesions without contrast enhancement and his cervical MRI showed syringomyelic cavitation starting from the craniocervical junction to the beginning of dorsal level, and milimetric contrast enhancement at the level of C1-C2 vertebra. Among children with MS, presenting with posterior cord findings due to syringomyelia is unusual, and it can be suggested that syringomyelic cavities may develop during the early course of MS even through childhood and they may be the presenting symptom of pediatric multiple sclerosis. P19.14 Acute demyelinating motor neuropathy 1 T.R.I. Lonnqvist *, M. Overmyer1 , L. Valanne2 , L. Lauronen3 . ¨ 1 Division of Child Neurology, Department of Gynecology and Pediatrics, Helsinki University Central Hospital, Helsinki, Finland; 2 Helsinki Medical Imaging Center, University of Helsinki, Helsinki, Finland; 3 Department of Children’s Clinical Neurophysiology, Helsinki University Central Hospital, Helsinki, Finland
Background: Pure acute demyelinating predominantly motor neuropathy is rare among acute inflammatory neuropathies. Aims of the study: To report a 13-year-old boy with an infection triggered acute demyelinating motor neuropathy with brisk tendon reflexes. Methods: Repetitive neurological examinations and i.e. following laboratory test were performed: serum and CSF antibodies and PCR against different viruses and Borrelia, bacteria and virus culture from CSF, nasopharyngeal secretion and feces, ganglioside antibodies, brain and spinal MRI, and ENMG. Results: The patient had a sinusitis with fever and elevated CRP (87 mg/l) treated with amoxicillin. Two weeks after the acute phase he became tired with distal muscle weakness and numbness in his hands and feet. He was tired, but oriented, had mildly decreased sharp touch distally, but no other sign of dysesthesia. He had facial and distal muscle weakness, brisk deep tendon reflexes, but negative Babinski sign. His speech was slurred, and he had difficulties in swallowing. CRP and other infection parameters were normalized. Muscle enzymes and transaminase were normal, but CSF protein was mildly elevated (464 mg/l). Hemophilus influenzae was detected in the nasal secretion. MRI revealed
Background: Acute hemorrhagic leukoencephalitis, also known as acute necrotizing encephalopathy is a hyperacute and frequently fatal form of acute disseminated encephalomyelitis. Acute hemorrhagic encephalomyelitis is relatively rare (less than 100 cases have been reported in the medical literature as of 2010), it is seen in about 2% of acute disseminated encephalomyelitis cases. Aim of the study: We report a case of eight month female infant who developed a picture of acute hemorrhagic encephalomyelitis, which progressed from acute disseminated encephalomyelitis in period less of one week. Results: She was referred to the hospital because of high fever and severe convulsions following a 2-day prodromal illness with cough and diarrhea. The initial MR imaging findings showed high intensity lesions of the thalamus, pons and medulla oblongata on the T2-weighted, FLAIR and low signal intensity on the diffusion weighted images. After eight days of admission, follow-up MR imaging findings showed high intensity signal in basal ganglia on T1-weighted that indicate the presence of hemorrhage without the restriction of diffusion. MR imaging findings was supported by progressive symptoms of the extrapyramidal system with uncoordinated and inappropriate movement of limb and head, muscular hypotonic without eye contact. Conclusion: Case represents an example of an atypical occurrence of age for acute hemorrhagic leukoencephalitis with extremely rapid progression from initial disseminated
S111 encephalomyelitis and dramatic neurological symptoms that have developed within one week of admission. P19.13 A childhood multiple sclerosis case presenting with syringomyelia and posterior cord findings P. Topaloglu Tekturk1 *, T. Aydin2 , Z. Yapici1 , M. Eraksoy1 . 1 Division of Child Neurology, Department of Neurology, Istanbul University Faculty of Medicine, Capa-Istanbul, Turkey, 2 Goztepe Education and Research Hospital, Department of Neurology, Kadikoy − Istanbul, Turkey Syringomyelia is described as a cavitary enlargement of the spinal cord. Among patients with multiple sclerosis (MS), non-communicating syringomyelia (NCS) has been described in various reports on small case series or case studies including post-mortem analysis. It can be a subtle finding without clinical correlates but it has been considered that syrinx formations are more likely to be a consequence of MS cord pathology than a coincidental finding. We want to present a 15 years old boy presenting with paresthesia of his legs and walking difficulty. His neurological examination revealed long tract and posterior cord involvement and bilateral sensory deficit below L1 level. His routine blood tests including hemogram, serum electrolytes, vitamin B12, vasculitic markers, routine biochemical, liver and thyroid function tests were within normal limits. His electromyography revealed chronic L4-S1 anterior horn involvement. His cranial MRI showed multiple supra and infra tentorial demyelinating lesions without contrast enhancement and his cervical MRI showed syringomyelic cavitation starting from the craniocervical junction to the beginning of dorsal level, and milimetric contrast enhancement at the level of C1-C2 vertebra. Among children with MS, presenting with posterior cord findings due to syringomyelia is unusual, and it can be suggested that syringomyelic cavities may develop during the early course of MS even through childhood and they may be the presenting symptom of pediatric multiple sclerosis. P19.14 Acute demyelinating motor neuropathy 1 T.R.I. Lonnqvist *, M. Overmyer1 , L. Valanne2 , L. Lauronen3 . ¨ 1 Division of Child Neurology, Department of Gynecology and Pediatrics, Helsinki University Central Hospital, Helsinki, Finland; 2 Helsinki Medical Imaging Center, University of Helsinki, Helsinki, Finland; 3 Department of Children’s Clinical Neurophysiology, Helsinki University Central Hospital, Helsinki, Finland
Background: Pure acute demyelinating predominantly motor neuropathy is rare among acute inflammatory neuropathies. Aims of the study: To report a 13-year-old boy with an infection triggered acute demyelinating motor neuropathy with brisk tendon reflexes. Methods: Repetitive neurological examinations and i.e. following laboratory test were performed: serum and CSF antibodies and PCR against different viruses and Borrelia, bacteria and virus culture from CSF, nasopharyngeal secretion and feces, ganglioside antibodies, brain and spinal MRI, and ENMG. Results: The patient had a sinusitis with fever and elevated CRP (87 mg/l) treated with amoxicillin. Two weeks after the acute phase he became tired with distal muscle weakness and numbness in his hands and feet. He was tired, but oriented, had mildly decreased sharp touch distally, but no other sign of dysesthesia. He had facial and distal muscle weakness, brisk deep tendon reflexes, but negative Babinski sign. His speech was slurred, and he had difficulties in swallowing. CRP and other infection parameters were normalized. Muscle enzymes and transaminase were normal, but CSF protein was mildly elevated (464 mg/l). Hemophilus influenzae was detected in the nasal secretion. MRI revealed