Contributions
Abstract: EP1332
Type: Poster Sessions
Abstract Category: Clinical aspects of MS - Paediatric MS
Classical Devic Neuromyelis optica (NMO) is a rare severe demyelinating disease presenting with the clinical triada of optic neuritis, longitudinally extensive myelitis and positive serum anti-AQP4 antibodies and has a female predisposition (F:M 6.5:1) in Asians, Indians and Africans. However the disease is poorly studied in Kazakhstan.
The aim of the study was to describe 3 cases of Devic NMO hospitalized to our clinic within the last 5 years.
Materials and methods: 3 cases of child NMO, 2 girls and 1 boy of Kazakh (Asian) origin. The diagnosis was proven by brain and spinal cord MRI, optical coherent tomography and presence of serum anti-AQP4 antibodies in dynamics.
Results: One boy developed NMO at 9 years, one girl at 6 years and another girl at 14 years old. The hallmark of the disease in all patients was severe bilateral optic neuritis. All patients showed spastic paresis as a disease debut: a 14-years old girl had tetraparesis, the 6 years old girl - right side hemiparesis and the 8-years old boy - spastic paraparesis and ataxia. Further they had exacerbations every 4-6 months. The MRI of brain showed T-2 active periventricular lesions in all patients, and classical long. Longitudinally extensive myelitis was seen in 2 patients - a boy (at cervical level) and a 14-years old girl (at cervical and thoracic level). Optical coherent tomography showed significant retinal nerve fiber layer (RNFL) thickness decrease (up to 50% of normal) in all children. Anti-AQP4 antibodies performed in all children dynamically varied 1:80-1:160 (minimal positive titer < 1:10). The basic treatment with high dose methylprednisone (10-30 mg/kg 5-7 days) had mild effect on spasticity, but vision progressively worsened. Vision improvement was achieved by of Intravenous immunoglobulin IgG (IVIG) - 10% Octagam dosed 2 gram/kg for 3-5 days, each course was repeated every 2 months, totally 3-7 courses were done. A 14 years old girl developed severe encephalitic symptoms with necrotic lesion in left hemisphere, deep perifocal and optic disk edema which was stabilized on high dose IVIG (4 g/kg for 5 days) and 0,4 mg/kg of dexamethasone i.v, for 5 days followed by methylprednisone 20 mg/kg i.v. for 5 days, yet, plasma exchange had no effect.
Conclusion: Child Devic NMO seen in Kazakh Asian patients had severe clinical course with rapid development of partly irreversible vision loss and spastic syndrome with good clinical recovery when high dose IVG was repeatedly applied.
Disclosure: Name: nothing to disclose
Abstract: EP1332
Type: Poster Sessions
Abstract Category: Clinical aspects of MS - Paediatric MS
Classical Devic Neuromyelis optica (NMO) is a rare severe demyelinating disease presenting with the clinical triada of optic neuritis, longitudinally extensive myelitis and positive serum anti-AQP4 antibodies and has a female predisposition (F:M 6.5:1) in Asians, Indians and Africans. However the disease is poorly studied in Kazakhstan.
The aim of the study was to describe 3 cases of Devic NMO hospitalized to our clinic within the last 5 years.
Materials and methods: 3 cases of child NMO, 2 girls and 1 boy of Kazakh (Asian) origin. The diagnosis was proven by brain and spinal cord MRI, optical coherent tomography and presence of serum anti-AQP4 antibodies in dynamics.
Results: One boy developed NMO at 9 years, one girl at 6 years and another girl at 14 years old. The hallmark of the disease in all patients was severe bilateral optic neuritis. All patients showed spastic paresis as a disease debut: a 14-years old girl had tetraparesis, the 6 years old girl - right side hemiparesis and the 8-years old boy - spastic paraparesis and ataxia. Further they had exacerbations every 4-6 months. The MRI of brain showed T-2 active periventricular lesions in all patients, and classical long. Longitudinally extensive myelitis was seen in 2 patients - a boy (at cervical level) and a 14-years old girl (at cervical and thoracic level). Optical coherent tomography showed significant retinal nerve fiber layer (RNFL) thickness decrease (up to 50% of normal) in all children. Anti-AQP4 antibodies performed in all children dynamically varied 1:80-1:160 (minimal positive titer < 1:10). The basic treatment with high dose methylprednisone (10-30 mg/kg 5-7 days) had mild effect on spasticity, but vision progressively worsened. Vision improvement was achieved by of Intravenous immunoglobulin IgG (IVIG) - 10% Octagam dosed 2 gram/kg for 3-5 days, each course was repeated every 2 months, totally 3-7 courses were done. A 14 years old girl developed severe encephalitic symptoms with necrotic lesion in left hemisphere, deep perifocal and optic disk edema which was stabilized on high dose IVIG (4 g/kg for 5 days) and 0,4 mg/kg of dexamethasone i.v, for 5 days followed by methylprednisone 20 mg/kg i.v. for 5 days, yet, plasma exchange had no effect.
Conclusion: Child Devic NMO seen in Kazakh Asian patients had severe clinical course with rapid development of partly irreversible vision loss and spastic syndrome with good clinical recovery when high dose IVG was repeatedly applied.
Disclosure: Name: nothing to disclose