Summary about Disease
Opsoclonus Myoclonus Syndrome (OMS), also known as "dancing eyes-dancing feet" syndrome, is a rare neurological disorder characterized by rapid, involuntary, multi-directional eye movements (opsoclonus) and brief, shock-like muscle contractions (myoclonus). It often affects children, but can occur in adults. In children, it's frequently associated with neuroblastoma (a type of childhood cancer), while in adults, it can be linked to other cancers or have an unknown cause. The syndrome is believed to be autoimmune in nature, where the body's immune system mistakenly attacks the nervous system.
Symptoms
Opsoclonus: Rapid, involuntary, chaotic eye movements occurring in all directions.
Myoclonus: Brief, involuntary muscle jerks or spasms affecting limbs, trunk, and sometimes face.
Ataxia: Difficulty with coordination and balance, leading to unsteady gait.
Irritability: Increased fussiness, agitation, and emotional lability.
Sleep disturbances: Difficulty falling or staying asleep.
Speech problems: Slurred or hesitant speech.
Developmental regression: Loss of previously acquired skills (in children).
Behavioral changes: Personality changes, anxiety, or social withdrawal.
Causes
Autoimmune response: The primary suspected cause is an autoimmune reaction, where the body's immune system attacks the nervous system.
Neuroblastoma: In children, OMS is often associated with neuroblastoma, a tumor of the sympathetic nervous system. The tumor antigens trigger the autoimmune response.
Other cancers: In adults, OMS can be associated with other cancers, such as lung cancer, breast cancer, or lymphoma, triggering paraneoplastic syndrome.
Viral infections: In some cases, OMS has been linked to viral infections.
Idiopathic: In some instances, the cause of OMS remains unknown (idiopathic).
Medicine Used
Treatment for OMS typically involves a combination of therapies aimed at suppressing the immune system and addressing any underlying cancer, if present. Common medications include:
Corticosteroids: Prednisone or other corticosteroids to reduce inflammation and suppress the immune system.
Intravenous Immunoglobulin (IVIG): Infusion of antibodies to modulate the immune system.
Adrenocorticotropic Hormone (ACTH): Stimulates the adrenal glands to produce corticosteroids.
Rituximab: A monoclonal antibody that targets B cells (a type of immune cell).
Chemotherapy: If associated with neuroblastoma or other cancer.
Immunosuppressants: Other immunosuppressant drugs like cyclophosphamide may be used in severe cases.
Symptomatic Treatment: Medications to manage specific symptoms such as myoclonus (e.g., clonazepam).
Is Communicable
No, Opsoclonus Myoclonus Syndrome is not communicable. It is not caused by an infectious agent and cannot be spread from person to person. It is an autoimmune or paraneoplastic disorder.
Precautions
Since OMS is not communicable, standard precautions for preventing the spread of infectious diseases do not apply. Precautions are focused on managing the symptoms and potential complications of the syndrome itself and the side effects of treatment:
Protect from falls: Due to ataxia and balance problems, measures should be taken to prevent falls, such as modifying the home environment.
Manage medication side effects: Monitor for and manage potential side effects of immunosuppressant medications.
Prevent infections: Immunosuppressant medications increase the risk of infection, so avoid exposure to sick individuals.
Supportive care: Provide supportive care to address developmental delays, behavioral changes, and sleep disturbances.
How long does an outbreak last?
OMS is not an "outbreak" in the traditional sense of an infectious disease. It is a chronic condition that can persist for months to years. The duration varies depending on the underlying cause, the severity of the symptoms, and the effectiveness of the treatment. Some individuals may experience relapses and remissions, while others may have persistent symptoms. Early diagnosis and treatment can improve the long-term outcome.
How is it diagnosed?
Diagnosis of OMS typically involves:
Clinical evaluation: Assessing the characteristic symptoms of opsoclonus, myoclonus, ataxia, and behavioral changes.
Neurological examination: Evaluating reflexes, coordination, and other neurological functions.
Eye movement recordings: Electrooculography (EOG) can objectively document the presence of opsoclonus.
Imaging studies: MRI of the brain to rule out other neurological conditions.
Tumor screening: In children, screening for neuroblastoma with urine catecholamine levels, MIBG scan, and/or biopsy. In adults, screening for underlying cancer based on risk factors.
Lumbar puncture: Cerebrospinal fluid (CSF) analysis may show elevated antibodies or inflammatory markers.
Blood tests: To evaluate for autoimmune markers and rule out other conditions.
Timeline of Symptoms
The onset of OMS can be rapid or gradual.
Initial symptoms: Often include irritability, sleep disturbances, and ataxia.
Progression: Opsoclonus and myoclonus typically develop within days or weeks.
Developmental regression: In children, loss of previously acquired skills may occur.
Behavioral changes: Personality changes and emotional lability may emerge over time.
Chronic phase: Without treatment, symptoms can persist for months or years, with potential for relapses and remissions.
Important Considerations
Early diagnosis is crucial: Prompt diagnosis and treatment are essential to improve outcomes and minimize long-term neurological sequelae.
Underlying cause: Identifying and treating any underlying cause, such as neuroblastoma or other cancers, is critical.
Multidisciplinary approach: Management of OMS requires a multidisciplinary approach involving neurologists, oncologists, immunologists, and other specialists.
Long-term follow-up: Regular monitoring is necessary to assess treatment response, manage side effects, and address any long-term complications.
Impact on development: OMS can have significant impact on a child's development, requiring ongoing support and therapy.