Neuroblastoma presenting as opsoclonus-myoclonus: A series of six cases and review of literature

Abstract

The opsoclonus-myoclonus 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. Data on its epidemiology, clinical features, and outcome are limited worldwide. The aim of the study was to evaluate the clinical profile and outcome of children with OMAS. A retrospective data of all children presented to Pediatric oncology clinic with a diagnosis of opsoclonus-myoclonus from 2013 to 2016 were collected. 6 patients with a diagnosis of OMAS were presented over a 4-year period. All 6 cases had paraneoplastic etiology. All Children had good outcome without any relapse. Paraneoplastic opsoclonus myoclonus had a good outcome in our experience.

Neuroblastoma-associated Opsoclonous Myoclonous Ataxia Syndrome: Profile and Outcome Report on 15 Egyptian Patients

“…The primary tumor site was the adrenal gland in 5 patients (33%); in concordance to other OMAS studies, there was predominance of tumors that occurred in the paraspinal region: 8 patients (53%) either mediastinal or retroperitoneal 2…”

“…17 The primary tumor site was the adrenal gland in 5 patients (33%); in concordance to other OMAS studies, there was predominance of tumors that occurred in the paraspinal region: 8 patients (53%) either mediastinal or retroperitoneal. 2 Localized low disease stages found in most of study patients altogether with LR disease (53%) was in agreement with both Children's Oncology Group (COG) and Children's Cancer Group (CCG) studies. However, IR proportion was much higher in COG results that formed 83% of their cases.…”

“…Only 2 patients were younger than 1 year at diagnosis with median age of 2 years for the whole study group, similar to the reported average in the literature 17. Some investigators reported slightly lower median age of 18 months,13 and uncommonly diagnosed before 1 year in some reports 2. The decreased incidence in infancy might be associated with lesser ability to manufacture specific antibodies in younger infants.…”

“…3 Approximately, 70% to 80% of children with OMAS will have long-term neurological sequelae. 2,3 Although all study patients remained alive, their neurological outcome was poor, as most patients had neurological sequelae as expected with OMAS 5-year PFS of 33.3 ± 12.2%. Whereas 10/15 patients (67%) eventually reached a complete resolution of their symptoms with treatment, 5 had persistence of OMAS symptoms such as opsoclonus, myoclonus, ataxia and dysarthria.…”

Neuroblastoma-associated Opsoclonous Myoclonous Ataxia Syndrome: Profile and Outcome Report on 15 Egyptian Patients

“…The primary tumor site was the adrenal gland in 5 patients (33%); in concordance to other OMAS studies, there was predominance of tumors that occurred in the paraspinal region: 8 patients (53%) either mediastinal or retroperitoneal 2…”

“…17 The primary tumor site was the adrenal gland in 5 patients (33%); in concordance to other OMAS studies, there was predominance of tumors that occurred in the paraspinal region: 8 patients (53%) either mediastinal or retroperitoneal. 2 Localized low disease stages found in most of study patients altogether with LR disease (53%) was in agreement with both Children's Oncology Group (COG) and Children's Cancer Group (CCG) studies. However, IR proportion was much higher in COG results that formed 83% of their cases.…”

“…Only 2 patients were younger than 1 year at diagnosis with median age of 2 years for the whole study group, similar to the reported average in the literature 17. Some investigators reported slightly lower median age of 18 months,13 and uncommonly diagnosed before 1 year in some reports 2. The decreased incidence in infancy might be associated with lesser ability to manufacture specific antibodies in younger infants.…”

“…3 Approximately, 70% to 80% of children with OMAS will have long-term neurological sequelae. 2,3 Although all study patients remained alive, their neurological outcome was poor, as most patients had neurological sequelae as expected with OMAS 5-year PFS of 33.3 ± 12.2%. Whereas 10/15 patients (67%) eventually reached a complete resolution of their symptoms with treatment, 5 had persistence of OMAS symptoms such as opsoclonus, myoclonus, ataxia and dysarthria.…”

Clinical profile, prognostic indicators, and therapeutic outcomes of pediatric opsoclonus-myoclonus-ataxia syndrome: A single-center experience from South India

“…There are several Indian studies with variable outcomes in the tumor versus nontumor group. [3031] It can be seen in our study that there is a tendency for paraneoplastic OMS for monophasic course after tumor removal though the duration to attain initial remission was longer when compared to the nontumor group. Nontumor group tends to have multiphasic course, usually with relapses precipitated by intercurrent infections and medication tapering.…”

Neuroblastoma-associated Opsoclonous Myoclonous Ataxia Syndrome: Profile and Outcome Report on 15 Egyptian Patients

“…The primary tumor site was the adrenal gland in 5 patients (33%); in concordance to other OMAS studies, there was predominance of tumors that occurred in the paraspinal region: 8 patients (53%) either mediastinal or retroperitoneal 2…”

“…17 The primary tumor site was the adrenal gland in 5 patients (33%); in concordance to other OMAS studies, there was predominance of tumors that occurred in the paraspinal region: 8 patients (53%) either mediastinal or retroperitoneal. 2 Localized low disease stages found in most of study patients altogether with LR disease (53%) was in agreement with both Children's Oncology Group (COG) and Children's Cancer Group (CCG) studies. However, IR proportion was much higher in COG results that formed 83% of their cases.…”

“…Only 2 patients were younger than 1 year at diagnosis with median age of 2 years for the whole study group, similar to the reported average in the literature 17. Some investigators reported slightly lower median age of 18 months,13 and uncommonly diagnosed before 1 year in some reports 2. The decreased incidence in infancy might be associated with lesser ability to manufacture specific antibodies in younger infants.…”

“…3 Approximately, 70% to 80% of children with OMAS will have long-term neurological sequelae. 2,3 Although all study patients remained alive, their neurological outcome was poor, as most patients had neurological sequelae as expected with OMAS 5-year PFS of 33.3 ± 12.2%. Whereas 10/15 patients (67%) eventually reached a complete resolution of their symptoms with treatment, 5 had persistence of OMAS symptoms such as opsoclonus, myoclonus, ataxia and dysarthria.…”

Clinical profile, prognostic indicators, and therapeutic outcomes of pediatric opsoclonus-myoclonus-ataxia syndrome: A single-center experience from South India

“…There are several Indian studies with variable outcomes in the tumor versus nontumor group. [3031] It can be seen in our study that there is a tendency for paraneoplastic OMS for monophasic course after tumor removal though the duration to attain initial remission was longer when compared to the nontumor group. Nontumor group tends to have multiphasic course, usually with relapses precipitated by intercurrent infections and medication tapering.…”

Neuroblastoma-associated Opsoclonous Myoclonous Ataxia Syndrome: Profile and Outcome Report on 15 Egyptian Patients

“…The primary tumor site was the adrenal gland in 5 patients (33%); in concordance to other OMAS studies, there was predominance of tumors that occurred in the paraspinal region: 8 patients (53%) either mediastinal or retroperitoneal 2…”

“…17 The primary tumor site was the adrenal gland in 5 patients (33%); in concordance to other OMAS studies, there was predominance of tumors that occurred in the paraspinal region: 8 patients (53%) either mediastinal or retroperitoneal. 2 Localized low disease stages found in most of study patients altogether with LR disease (53%) was in agreement with both Children's Oncology Group (COG) and Children's Cancer Group (CCG) studies. However, IR proportion was much higher in COG results that formed 83% of their cases.…”

“…Only 2 patients were younger than 1 year at diagnosis with median age of 2 years for the whole study group, similar to the reported average in the literature 17. Some investigators reported slightly lower median age of 18 months,13 and uncommonly diagnosed before 1 year in some reports 2. The decreased incidence in infancy might be associated with lesser ability to manufacture specific antibodies in younger infants.…”

“…3 Approximately, 70% to 80% of children with OMAS will have long-term neurological sequelae. 2,3 Although all study patients remained alive, their neurological outcome was poor, as most patients had neurological sequelae as expected with OMAS 5-year PFS of 33.3 ± 12.2%. Whereas 10/15 patients (67%) eventually reached a complete resolution of their symptoms with treatment, 5 had persistence of OMAS symptoms such as opsoclonus, myoclonus, ataxia and dysarthria.…”

Clinical profile, prognostic indicators, and therapeutic outcomes of pediatric opsoclonus-myoclonus-ataxia syndrome: A single-center experience from South India

“…There are several Indian studies with variable outcomes in the tumor versus nontumor group. [3031] It can be seen in our study that there is a tendency for paraneoplastic OMS for monophasic course after tumor removal though the duration to attain initial remission was longer when compared to the nontumor group. Nontumor group tends to have multiphasic course, usually with relapses precipitated by intercurrent infections and medication tapering.…”