What is Opsoclonus-myoclonus syndrome (OMS)?

Opsoclonus-myoclonus syndrome (OMS), also known as dancing eye syndrome, is a rare neurological disorder characterized by abnormal eye movements (opsoclonus) and sudden, brief muscle contractions (myoclonus). OMS predominantly affects children but can occur in adults as well. This blog aims to shed light on the symptoms, causes, and available treatments for this complex syndrome.

Symptoms of Opsoclonus-Myoclonus Syndrome

Opsoclonus and myoclonus are the primary symptoms of OMS, but the syndrome may also involve other neurological manifestations. Here are some common symptoms experienced by individuals with OMS:

  • Opsoclonus: Opsoclonus refers to involuntary, rapid, and irregular eye movements that occur in all directions. These uncontrolled eye movements may be accompanied by nystagmus (rhythmic, repetitive eye movements). The eyes' erratic motion can make it challenging for affected individuals to focus and maintain visual stability.
  • Myoclonus: Myoclonus refers to sudden, brief muscle contractions or jerks that can affect various body parts. It commonly affects the limbs, trunk, and face. Myoclonus episodes may be spontaneous or triggered by external stimuli.
  • Ataxia: Many individuals with OMS experience ataxia, which is characterized by uncoordinated movements, lack of balance, and difficulties with motor control. This can lead to unsteady gait and problems with fine motor skills.
  • Behavioral and cognitive changes: OMS can cause behavioral and cognitive abnormalities, including irritability, mood swings, language difficulties, attention deficits, and memory problems. Some children may also exhibit regression in developmental milestones.
  • Sleep disturbances: Sleep disturbances such as insomnia, excessive daytime sleepiness, and nightmares are common in individuals with OMS.

Causes and Pathophysiology

  • The exact cause of OMS is still unknown, although research suggests that it may be an autoimmune disorder triggered by an underlying infection or a neuroblastoma (a type of tumor that affects nerve tissue). In some cases, OMS may occur as a paraneoplastic syndrome, where the immune system mistakenly attacks normal cells in response to cancer.
  • The immune system's role in OMS is supported by the presence of antibodies in the blood and cerebrospinal fluid of affected individuals. Antibodies against certain proteins, including anti-Ri and anti-Hu, have been identified in some OMS cases. However, not all individuals with OMS have detectable antibodies, indicating that other factors may also contribute to the development of the syndrome.

Diagnosis

Diagnosing OMS can be challenging due to its rarity and overlapping symptoms with other neurological conditions. A comprehensive evaluation is necessary to rule out other potential causes of opsoclonus, myoclonus, and ataxia. Diagnostic tests may include blood tests, imaging studies (such as MRI), and lumbar puncture to examine the cerebrospinal fluid.

OMS treatment aims to control symptoms and manage the underlying cause, if identified. Therapeutic interventions may involve a multidisciplinary approach, including neurologists, pediatricians, oncologists (in case of associated tumors), and other healthcare professionals.


Treatment Options

  • Immunotherapy: Corticosteroids, intravenous immunoglobulin (IVIG), and plasmapheresis are commonly used immunotherapies to modulate the immune response and reduce symptom severity. These treatments aim to suppress the immune system's abnormal activity.
  • Tumor management: In cases where OMS is associated with an underlying neuroblastoma or other tumor, appropriate tumor management.

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