Opsoclonus myoclonus syndrome (NORD)
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Opsoclonus myoclonus syndrome (NORD)
Pathology
Adrenal gland disorders
Congenital adrenal hyperplasia
Primary adrenal insufficiency
Waterhouse-Friderichsen syndrome
Hyperaldosteronism
Adrenal cortical carcinoma
Cushing syndrome
Conn syndrome
Thyroid gland disorders
Thyroglossal duct cyst
Hyperthyroidism
Graves disease
Thyroid eye disease (NORD)
Toxic multinodular goiter
Thyroid storm
Hypothyroidism
Euthyroid sick syndrome
Hashimoto thyroiditis
Subacute granulomatous thyroiditis
Riedel thyroiditis
Postpartum thyroiditis
Thyroid cancer
Parathyroid gland disorders
Hyperparathyroidism
Hypoparathyroidism
Hypercalcemia
Hypocalcemia
Pancreatic disorders
Diabetes mellitus
Diabetic retinopathy
Diabetic nephropathy
Pituitary gland disorders
Hyperpituitarism
Pituitary adenoma
Hyperprolactinemia
Prolactinoma
Gigantism
Acromegaly
Hypopituitarism
Growth hormone deficiency
Pituitary apoplexy
Sheehan syndrome
Hypoprolactinemia
Constitutional growth delay
Diabetes insipidus
Syndrome of inappropriate antidiuretic hormone secretion (SIADH)
Gonadal dysfunction
Precocious puberty
Delayed puberty
Premature ovarian failure
Polycystic ovary syndrome
Androgen insensitivity syndrome
Kallmann syndrome
5-alpha-reductase deficiency
Polyglandular syndromes
Autoimmune polyglandular syndrome type 1 (NORD)
Endocrine tumors
Multiple endocrine neoplasia
Pancreatic neuroendocrine neoplasms
Zollinger-Ellison syndrome
Carcinoid syndrome
Pheochromocytoma
Neuroblastoma
Opsoclonus myoclonus syndrome (NORD)
Endocrine system pathology review
Adrenal insufficiency: Pathology review
Adrenal masses: Pathology review
Hyperthyroidism: Pathology review
Hypothyroidism: Pathology review
Thyroid nodules and thyroid cancer: Pathology review
Parathyroid disorders and calcium imbalance: Pathology review
Diabetes mellitus: Pathology review
Cushing syndrome and Cushing disease: Pathology review
Pituitary tumors: Pathology review
Hypopituitarism: Pathology review
Diabetes insipidus and SIADH: Pathology review
Multiple endocrine neoplasia: Pathology review
Neuroendocrine tumors of the gastrointestinal system: Pathology review
Assessments
Opsoclonus myoclonus syndrome (NORD)
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Content Reviewers
Rishi Desai, MD, MPHOpsoclonus-myoclonus syndrome is also called OMS or dancing eyes-dancing feet syndrome.
Opsoclonus describes seemingly random, involuntary, and rapid eye movements in the horizontal, vertical, and diagonal directions.
Myoclonus describes lightening-like limb movements or limb jerks, that can also be more tremulous.
The syndrome also causes ataxia, which often results in the inability to walk, sit or crawl, and the loss of previously attained abilities, like speech.
Generally speaking, OMS is thought to be an autoimmune condition in which the body attacks the nervous system, resulting in the characteristic signs and symptoms.
The precise cause of OMS is unknown, but in children, it’s often due to a paraneoplastic syndrome.
The idea is that a hidden or occult tumor - like a neuroblastoma or ganglioneuroblastoma - triggers the body’s immune system, and the resulting antibodies unintentionally target healthy cells of the nervous system.
CD20+ B-cells that get into the CSF fluid seem to play an important role.
One brain area that gets affected is the cerebellum, which plays an important role in coordinating body movements.
In older children and adolescents OMS is thought to be triggered by an infection, and in adults, it’s often due to a paraneoplastic process due to lung or breast cancer, for example.
In children with OMS, there’s almost always ataxia, so it’s sometimes referred to as OMAS - with the A representing ataxia.
In fact, most toddlers with OMS are initially misdiagnosed with acute cerebellar ataxia of childhood.
The diagnosis of OMS is also often based on symptoms like opsoclonus and myoclonus which can appear abruptly, progressing over days to weeks.
Summary
Opsoclonus-myoclonus syndrome (OMS) is a rare condition, characterized by rapid, uncontrolled eye movements (opsoclonus) and sudden twitching or jerking of muscles (myoclonus). It can also present with ataxia, which often results in the inability to walk, sit or crawl, and the loss of previously attained abilities, like speech.
The cause of OMS is not well understood, but it is thought to be due to an autoimmune condition in which the body attacks the nervous system, resulting in characteristic signs and symptoms. Treatment involves immunosuppressive therapy like corticosteroids, intravenous immunoglobulin or IVIG, drugs like cyclophosphamide, and rituximab.
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