Opsoclonus myoclonus syndrome oms is a rare neuroinflammatory disease of paraneoplastic, parainfectious or idiopathic origin, characterized by opsoclonus, myoclonus, ataxia, and behavioral and sleep disorders. Kinsbourne syndrome as the unique manifestation in children. Intravenous immunoglobulin with prednisone and risk. Opsoclonus is frequently associated with limb andor truncal jerking myoclonus, marked gait ataxia along with behavioral changes video 1. It is characterized by associated ocular, motor, behavioral, sleep, and language disturbances. Although rare, it raises important clinical and neurobio. Kinsbourne syndrome is a rare neurological disorder that primarily affects children previously healthy and aged. Opsoclonus myoclonus syndrome oms, also known as opsoclonusmyoclonusataxia oma, is a rare neurological disorder of unknown cause which appears to be the result of an autoimmune process involving the nervous system. Opsoclonusmyoclonus is a pervasive neurological syndrome of children and adults. While it occurs most when trying to move and worsens with agitation or stimulation, it can also be present at rest. Kinsbourne syndrome, known as opsoclonusmyoclonusataxia, described in 1962 by marcel kinsbourne, is a rare neurological disorder that primarily affects previously healthy children, aged between 6 and 36 months, and is characterized by the presence of opsoclonus rapid, irregular, horizontal and vertical eye movements and myoclonus that may affect trunk, limbs or face, and. Sometimes it is due to a selflimiting presumed parainfectious brainstem encephalitis but it may also represent a nonmetastatic.
By february, kates condition worsened to the point that she was not able to sit, stand or walk on. The opsoclonusmyoclonus ataxia syndrome omas also called kinsbourne syndrome or dancing eye syndrome is a rare but serious disorder characterized by opsoclonus, myoclonus, and ataxia, along with extreme irritability and behavioural changes. Myoclonic encephalopathy of infancy, an alternative. In other cases, it is associated with a viral infection such as influenza, epsteinbarr, hepatitis c, hiv,or coxsackie b or a bacterial infection such as as streptococcal, lyme disease, or. We report the case of a 19 month old girl with myoclonic encephalopathy of infants mei kinsbourne syndrome, on longterm therapy with acth for the occurrence of frequent relapses steroiddependent form. Opsoclonusmyoclonus syndrome american academy of ophthalmology. Developmental gerstmanns syndrome jama neurology jama. Bartter syndrome bs is a hereditary condition transmitted as an autosomal recessive bartter type 1 to 4 or dominant trait bartter type 5.
Sandifer syndrome or sandifers syndrome is an eponymous paediatric medical disorder, characterised by gastrointestinal symptoms and associated neurological features. The physio therapeutic diagnosis was neuromuscular changes leading. Kinsbourne syndrome, also known as opsoclonusmyoclonus syndrome or dancing eyes syndrome is a rare condition characterized by rapid, irregular, multidirectional eye movements opsoclonus, myoclonic movements in the trunk, face andor limbs and ataxia. Kinsbourne syndrome as complication of a mycoplasma penumoniae infection belgian journal of paediatrics 2019 volume 21 supplement 1. Kinsbourne syndrome as complication of a mycoplasma.
An association between neuroblastoma and opsoclonusmyoclonus syndrome oms was described as early as 1927 within the first report on the transformation of malignant neuroblastoma to a benign ganglioneuroma. Opsoclonusmyoclonus syndrome oms is a rare condition of unknown etiology that features opsoclonus, myoclonic jerks, behavioral disturbances, and ataxia. Toe agnosia in gerstmann syndrome journal of neurology. Kinsbourne, opsoclonus, mioclonus, ataxia, neuroblastoma. Opsoclonus myoclonus ataxia syndrome oma, also known as dancing eyes and dancing feet syndrome or kinsbourne syndrome,1 is a rare neurological disorder that affects 23% of over 650 children diagnosed with neuroblastoma every year in north america. The onset is usually abrupt, often severe, and it can become chronic. May 11, 2017 opsoclonusmyoclonus syndrome is often associated with the presence of a tumor such as neuroblastoma and referred to as a paraneoplastic syndrome. Opsoclonusmyoclonus syndrome genetic and rare diseases nih. Kinsbourne syndrome as complication of a mycoplasma pneumoniae infection. Abstract we report herein the case of a 14 monthold mexican female, without previous neurologic symptoms, who developed an opsoclonus myoclonus syndrome. Aug 15, 2017 we were told that her symptoms would resolve on their own but thats not what happened. Signs and symptoms of opsoclonusmyoclonus syndrome oms may include unsteady, trembling gait manner of walking sudden, brief, shocklike muscle spasms myoclonus.
Standardised neuropsychological tests of intelligence, memory. Kate unbalanced opsoclonus myoclonus syndrome oms youtube. Om dancing eye syndromekinsbourne syndrome is a rare neurological condition of subacute onset. Kinsbourne syndrome ks is a rare neurological disorder characterized by the. A 72 year old right handed woman had a focal lesion in the angular gyrus of the left hemisphere which was caused by a glioblastoma multiforme. It was not recognized at that time nor was it appreciated in the subsequent followup report on the same patient in 1959. Groupe international detude du syndrome opsoclonus myoclonus. Our results show treatment effect of prednisone and riskadapted chemotherapy both with and without intravenous immunoglobulin ivig. Kinsbourne syndrome, ataxia, neuroblas toma, paraneoplastic syndrome.
It is an extremely rare condition, affecting as few as 1 in 10,000,000 people per year. In the pediatric age group, opsoclonus myoclonus syndrome has two. The opsoclonusmyoclonus syndrome practical neurology. Opsoclonusmyoclonus syndrome oms is an inflammatory neurological disorder, often with paraneoplastic etiology. Opsoclonus myoclonus syndrome oms is a rare condition in children, that for expert consensus is defined to be met three of the four criteria. Data on its epidemiology, clinical features, and outcome are limited worldwide. Child, 1 year and 5 months, with a diagnosis of posterior mediastinal tumor and kinsbourne syndrome. Sandifers syndrome and gastro oesophageal reflux disease. Opsoclonusmyoclonus syndrome oms is well described in children also known as kinsbourne syndrome 1, usually occurring as a paraneoplastic neurologic accompaniment of neuroblastoma 1,2 with longterm neurologic, behavioral, and developmental sequelae.
The diagnosis of opsoclonus myoclonus is largely clinical and the condition is frequently mistaken for acute cerebellar ataxia. Sometimes it is due to a selflimiting presumed parainfectious brainstem encephalitis but it may also represent a nonmetastatic manifestation. Despite positivity for hu antigen in approximately 75% of neuroblastomas 6, serum antihu antibodies are present in only approximately 415% of children with neuroblastoma 6,7, and there has been no large series of the paraneoplastic subgroup. The administration of trazodone per os at low doses as an alternative to the previous treatment ensured complete remission, also on the occasion of a subsequent relapse.
Review theneurobiology ofthe opsoclonusmyoclonus syndrome. Opsoclonusmioclonus syndrome in acute cerebellar ataxia. Kinsbourne syndrome as the unique manifestation in. The tetrad of finger agnosia, dysgraphia, dyscalculia, and rightleft disorientation make up gerstmanns syndrome. Kinsbourne syndrome is a rare neurological disorder that primarily affects children previously healthy and. Opsoclonus myoclonus syndrome has variably been described as dancing eyes and dancing feet syndrome or kinsbourne syndrome. There was no previous history of infectious processes. Anatomopathological examination revealed ganglioneuroblastoma. Kinsbourne syndrome is a rare neurological disorder that primarily affects children previously healthy and aged between 6 and 36 months. Kinsbourne syndrome, known as opsoclonusmyoclonusataxia, described in 1962 by marcel kinsbourne, is a rare neurological disorder that primarily affects.
The association between neuroblastoma and opsoclonus. Screening for autoantibodies in children with opsoclonus. Kinsbourne syndrome is a rare neurological disorder. Adultonset opsoclonusmyoclonusataxia syndrome as a manifestation of brazilian lyme diseaselike syndrome. Neuroepidemiologic trends in 105 us cases of pediatric. Neurologo pediatra, hospital del valle, san pedro sula, cortes, honduras. Its etiology may be paraneoplasic neuroblastoma, nonparaneoplasic. There is a significant correlation between the syndrome and gastrooesophageal reflux disease gord. Opsoclonus myoclonus syndrome wikimili, the free encyclopedia. Yellow brick cinema relaxing music recommended for you. It is characterised by disturbed motor control of limbs, trunk and head with features of.
Sandifers syndrome is the association of gastrooesophageal reflux disease with spastic. Opsoclonus myoclonus syndrome oms is a rare neuroinflammatory disease of paraneoplastic, parainfectious or idiopathic origin, characterized by opsoclonus, myoclonus, ataxia, and. Pranzatelli departments ofneurology, pediatrics, andpharmacology, the george washington university, washington, dc, u. Opsoclonusmyoclonus syndrome nord national organization. Jul 31, 2018 a prospective study of the presentation and management of dancing eye syndrome opsoclonusmyoclonus syndrome in the united kingdom. Kinsbournes syndrome, opsoclonus mioclonus syndrome, infancy myoclonic encephalopathy, paraneoplastic syndrome, neuroblastoma. E uma condicao extremamente rara, afetando 1 a cada 10. It is an extremely rare condition, affecting as few as 1 in 10,000,000 people p. The disease associates hypokalemic alkalosis with varying degrees of. Myoclonic encephalopathy of infancy, an alternative name for oms.
The first symptom she had complained of was severe headache. Review theneurobiology ofthe opsoclonusmyoclonus syndrome michael r. The opsoclonusmyoclonusataxia syndrome omas also known as kinsbourne syndrome can occur as a single neurological event in children with paraneoplastic syndrome in a neuroblastoma low degree. Thimerosal exposure, neuroinflammation, and the symptoms of regressive autism autism omnibus proceedings marcel kinsbourne, m. Calming music, relaxing music, soothing music, calming music. If you have problems viewing pdf files, download the latest version of adobe. Opsoclonus myoclonus following allogeneic haematopoietic. This is the first randomised trial for patients with oma and neuroblastoma. By february, kates condition worsened to the point that. Opsoclonusmyoclonus syndrome genetic and rare diseases. Intravenous immunoglobulin with prednisone and riskadapted. Rare diseases information specialists for opsoclonusmyoclonus syndrome. Postintervention acute opsoclonus myoclonus syndrome. The leading hypothesis for the cause of oms is an autoimmune, inflammatory reaction targeting central nervous system tissues, triggered by either a paraneoplastic or an infectious event.
The following case report presents a patient exhibiting gerstmann syndrome accompanied by toe agnosia. It occurs in children aged between 6 and 36 months. Developmental gerstmanns syndrome may occur in braindamaged and apparently normal children. Physiotherapy in kinsbourne syndrome 29 physiotherapy quarterly formerly fizjoterapia 2018, 26 2 physiotherapyquarterly. Kinsbourne and warrington observed that the majority of their patients with gerstmann syndrome were not aphasic and concluded that the association of the four symptoms is more than coincidental. Neuroepidemiologic trends in 105 us cases of pediatric opsoclonusmyoclonus syndrome. The tetrad has been infrequently described in children with learning disability and has been called developmental gerstmanns syndrome dgs.
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