Myoclonic encephalopathy of infants

Kinsbourne, Marcel

doi:10.1136/jnnp.25.3.271

Cited by 253 publications

(121 citation statements)

References 10 publications

Supporting

Mentioning

106

Contrasting

Unclassified

Order By: Relevance

“…A wellknown disorder encountered in children and adults is OMS. 22,23 Patients with OMS present with vertigo, dizziness, nausea and vomiting, tremulousness, and imbalance. Neurological examination reveals generalized myoclonic jerks, and lightning-like haphazard eye movements, consistent with myoclonus of the extraocular muscles (opsoclonus).…”

Section: A Common and Stereotyped Cns Disorder: Nmda Receptor Antibodmentioning

confidence: 99%

Paraneoplastic and Other Autoimmune Disorders of the Central Nervous System

McKeon

2013

The Neurohospitalist

View full text Add to dashboard Cite

As a result of the burgeoning growth of disease-specific neural autoantibody markers available for diagnostic patient evaluation, there has been increasing awareness of autoimmune central nervous system (CNS) disorders in hospital practice. Hospital-based neurologists have also taken great interest in these disorders since many occur in the setting of an occult systemic cancer which can be detected and treated at an early stage, and many affected patients are responsive to immunotherapy. Associated neurological disorders are typically subacute in onset, some are common or classic (eg, limbic encephalitis, cerebellar degeneration), but others have atypical or multifocal presentations. For patients with a suspected paraneoplastic disorder, many and costly oncological evaluations may be required for diagnosis. Comprehensive serological and cerebrospinal fluid (CSF) evaluation for neural autoantibodies may permit a focused cancer evaluation (eg, antineuronal nuclear antibody type 1 [ANNA-1] is associated with small cell lung carcinoma), and in some circumstances may indicate the likelihood of a good response to therapy (eg, voltagegated potassium channel complex antibody) or poor neurological prognosis (eg, purkinje cell cytoplasmic antibody type 1 [antiYo]). Positron-emission tomography-computed tomography (PET-CT) imaging of trunk may increase the diagnostic yield for certain cancers where other modalities have been negative. For some patients, rapid treatment with immunotherapy may facilitate marked improvement, or full recovery; multiple sequential trials of one or more of steroids, intravenous immunoglobulin or plasma exchange, or combination therapy are often required. For patients with N-methyl-D-aspartate receptor antibody encephalitis, early treatment with immunosuppressants and weeks or months of supportive intensive care may additionally be required. One or more of clinical examination, electroencephalogram (including video telemetry), and imaging provide objective parameters to which posttreatment outcomes can be compared.

show abstract

Section: A Common and Stereotyped Cns Disorder: Nmda Receptor Antibodmentioning

confidence: 99%

Paraneoplastic and Other Autoimmune Disorders of the Central Nervous System

McKeon

2013

The Neurohospitalist

View full text Add to dashboard Cite

show abstract

“…8 Rituximab seems ideal for the study and treatment of childhood opsoclonus-myoclonus syndrome (OMS), a serious neurologic disorder in need of more effective therapy. 9 OMS is purported to be humorally mediated, and antibodies to diverse autoantigens have been described. 10,11 Removal of autoantibodies through plasmapheresis at the toddler stage, when OMS usually develops, is not feasible technically.…”

mentioning

confidence: 99%

Immunologic and Clinical Responses to Rituximab in a Child With Opsoclonus-Myoclonus Syndrome

et al. 2005

View full text Add to dashboard Cite

show abstract

“…As seqüelas mais graves foram observadas em um paciente que não fez uso de ACTH 2 . No caso 5 de Kinsbourne 7 , o de menina que apresentou a síndrome com 10 meses de idade e na vigência de um processo infeccioso, e recidiva do quadro com idade cte 2 anos e 9 meses e que veio a falecer, foi feito estudo anátomo-patológteo Este mostrou marcada diminuição das células de Puríkinje, depleção das células da camada interna granular com proliferação de astrócitos na camada de Bergmann, em algumas áreas do cerebelo. Não havia qualquer alteração no núcleo denteado.…”

Section: Comentáriosunclassified

Polimioclonia-opsoclono: síndrome de Kinsbourne. Relato de um caso

Vieira¹,

Rosa²

1985

Arq. Neuro-Psiquiatr.

View full text Add to dashboard Cite

Na literatura, a síndrome de Kinsbourne 12 é apresentada com grande sinonímia, entre as quais as de encefalopatia mioclônica do recém-nato 7 , síndrome da doença dos olhos M» 6 , polimioclonia infantil 10 , encefalopatia mioclônica da infância 1,2,10,12, encefalopatia cerebelar aguda 1 , síndrome dos movimentos irregulares e rápidos dos olhos e das pálpebras na infância *, encefalopatia mioclô-nica com opsoclono 1 » 3 , síndrome da ataxia-opsoclono mioclonia 12 , polimioclonia com opsoclono 9 e encefalopatia polimioclônica infantil 5 . Kinsbourne 7 a descreveu como entidade que afeta crianças de até dois anos de idade, caracterizada por ataxia e estado de mal mioclônico que envolve, especialmente, a musculatura extrínseca dos olhos.No presente trabalho relatamos a ocorrência de polimioclonia-opsoclono em menino de nove anos de idade, após infecção por herpes zoster, e com boa evolução.

show abstract

Myoclonic encephalopathy of infants

Cited by 253 publications

References 10 publications

Paraneoplastic and Other Autoimmune Disorders of the Central Nervous System

Paraneoplastic and Other Autoimmune Disorders of the Central Nervous System

Immunologic and Clinical Responses to Rituximab in a Child With Opsoclonus-Myoclonus Syndrome

Polimioclonia-opsoclono: síndrome de Kinsbourne. Relato de um caso

Contact Info

Product

Resources

About