Neurological rarity the opsoclonusmyoclonus syndrome. Myoclonus describes lighteninglike limb movements or limb jerks, that can also be more tremulous. In children oms is almost exclusively associated with neuroblastoma, whereas in adults small cell lung cancer sclc and breast cancer are the. However, myoclonus may be a symptom in more complex and disturbing sleep disorders, such as restless legs syndrome, and may require treatment by a doctor. Opsoclonusmyoclonus syndrome oms is an unusual entity of earlychildhood onset characterized by variable, irregular, abnormal movements in the trunk and limbs, myoclonus. Rituximab anticd20 adjunctive therapy for opsoclonus.
Opsoclonus myoclonus is a rare autoimmune condition characterized by cerebellar degeneration. Some persons with sleep myoclonus are rarely troubled by, or need treatment for, the condition. Opsoclonus myoclonus syndrome oms is characterized by the combination of opsoclonus and arrhythmic action myoclonus that predominantly involves trunk and limbs usually accompanied by axial ataxia and dysarthria. Other symptoms may include difficulty speaking, poorly articulated speech, or an inability to speak. Opsoclonusmyoclonus syndrome genetic and rare diseases. Background little is known about adultonset opsoclonusmyoclonus syndrome oms outside of individual case reports. Paraneoplastic opsoclonusmyoclonus syndrome oms is a rare complication of cancer characterised by chaotic, synchronous eye movements opsoclonus, spontaneous muscle jerks myoclonus, and ataxia. Positive gallium scan in the syndrome of opsoclonus. Opsoclonus which is the only universal component of the syndrome is the occurrence of involuntary, repetitive rapid conjugate ocular saccades that are irregular in. The opsoclonusmyoclonus syndrome is a rare and distinct neurological disorder characterised by rapid multidirectional conjugate eye movements opsoclonus, myoclonus and ataxia, along with behavioural changes in adults and irritability in children. Opsoclonus myoclonus syndrome in adults is a rare and heterogeneous disorder with the clinical features of opsoclonus, myoclonus, ataxia, and behavioral and sleep disturbances. The patient was treated with ganciclovir, steroids, and immunoglobulins with remarkable recovery.
Immunomodulatory therapies should be performed although the. The opsoclonusmyoclonus syndrome practical neurology. Opsoclonus myoclonus ataxia syndrome khadilkar s, benny r. Opsomyoclonus oms, also known as dancing eyesdancing feet syndrome and opsoclonus myoclonus syndrome, is a clinical syndrome which has a strong association with neuroblastoma. Clinical presentation the opso refers to the presence of inter. Review theneurobiology ofthe opsoclonusmyoclonus syndrome michael r. The most common primary malignant lesions associated with the adultonset opsoclonus myoclonus syndrome are carcinomas of the lung and breast. Objective opsoclonus myoclonus syndrome oms is a serious, often disabling neurological illness of early childhood which is frequently associated with occult neuroblastoma. Opsoclonus myoclonus syndrome is an autoimmune neurological disorder characterized by opsoclonus, myoclonus, ataxia, and behavioral changes. The etiology is most often paraneoplastic, especially neuroblastoma in children and oat cell.
The opsoclonus myoclonus syndrome jitendra kumar sahu1, kameshwar prasad2 the opsoclonus myoclonus syndrome is a rare and distinct neurological disorder characterised by rapid multidirectional conjugate eye movements opsoclonus, myoclonus and ataxia, along with behavioural changes in adults and irritability in children. The opsoclonus myoclonus syndrome in children is a rare entity which is characterized by irritability, chaotic ocular movements with vertical, horizontal, rotatory components opsoclonus along. It is characterized by associated ocular, motor, behavioral, sleep, and language disturbances. Myoclonus fact sheet national institute of neurological. Although rare, it raises important clinical and neurobio. In the pathogenesis, an autoimmune process with infectious or paraneoplastic trigger has been suggested. Opsoclonus myoclonus syndrome also known as dancing eyes dancing feet syndrome is a rare disease of inflammatory, parainfectious, paraneoplastic or idiopathic nature consisting of neurologic and behavioral disorders.
A patient presented with onset of myoclonus and ataxia by months, and associated with abnormal opsoclonus like eye movements at 16 months of age. Please click through our menu to help find answers to your questions. Theimmunopharmacology ofthe opsoclonusmyoclonus syndrome michaelr. Opsoclonus myoclonus syndrome oms is a rare neurologic disorder characterized by opsoclonus, myoclonus, ataxia and behavioral disturbance. Most common underlying causes are malignancy and paraneoplastic syndromes. Theopsoclonus myoclonus syndrome oms is apotentially dev.
Pranzatelli departmentsofpediatrics, neurology, andpharmacology, the george washington university, and the nationalpediatricmyoclonus center, childrens research institute, washington, dc, u. Read more about symptoms, diagnosis, treatment, complications, causes and prognosis. It appears that in opsoclonus myoclonus syndrome an area of the brain called the cerebellum is attacked. We report the case of a 75yearold woman who was admitted in our department in early fall for altered mental status, opsoclonus, multifocal myoclonus, truncal titubation and generalized tremor, preceded by a 5. Opsoclonus myoclonus syndrome is a presumed autoimmunemediated syndrome characterized by acute or subacute onset of abnormal eye movements, myoclonic jerks, ataxia, dysarthria, and behavioral changes in the setting of bcell expansion within the cerebrospinal fluid.
Frontiers opsoclonusmyoclonus syndrome associated with. Opsoclonus myoclonus syndrome osmosis video library. Kate was losing her ability to stand, walk and talk. Opsoclonus myoclonus syndrome oms, also known as the dancing eye dancing feet syndrome voltz, 2002 is a rare clinical entity affecting children and adults.
Checking for direct pdf access through ovid abstract purpose to determine if rituximab, an anticd20 monoclonal antibody, reduces cerebrospinal fluid csf bcell expansion in opsoclonus myoclonus syndrome oms and results in clinical improvement. Opsoclonus myoclonus syndrome oms, also known as opsoclonus myoclonus ataxia oma, is a rare neurological disorder of unknown cause which appears to be the result of an autoimmune process involving the nervous system. She was diagnosed with opsoclonus myoclonus syndrome oms, a very rare neurological disorder. Neonatal opsoclonus eccles health sciences library j.
While it occurs most when trying to move and worsens with agitation or stimulation, it can also be present at rest. The opsoclonusmyoclonus syndrome is a rare and distinct neurological disorder characterised by rapid multidirectional conjugate eye movements opsoclonus, myoclonus and ataxia, along with. Naturally, the cohort is small, but patients have been followed up over time. Sometimes it is due to a selflimiting presumed parainfectious brainstem encephalitis but it may also represent a non. Adultonset opsoclonus myoclonus syndrome is also either paraneoplastic or parainfectious. Sometimes it is due to a selflimiting presumed parainfectious brainstem encephalitis but it may also represent a nonmetastatic manifestation. Response to rituximab in 3 children with opsoclonusmyoclonus syndrome resistant to conventional treatments. The term myoclonic encephalopathy or dancing eyes and dancing feet have been given to opsoclonus with myoclonus. The disorder is often controlled by treatment with adrenocorticotropic hormone acth. Opsoclonus myoclonus syndrome oms, also known as opsoclonusmyoclonusataxia oma, is a rare neurological disorder of unknown cause which appears to be the result of an autoimmune process involving the nervous system. Experience of a tertiary care university hospital is important as it gives information on a cohort of patients with this uncommon illness. Opsoclonus myoclonus syndrome oms is a rare neuroophthalmical disorder that affects children more often than adults.
Opsoclonusmyoclonus syndrome oms is an unusual entity of earlychildhood onset characterized by variable, irregular, abnormal movements in the trunk and limbs, myoclonus, and chaotic eye. The opsoclonus myoclonus syndrome or kinsbourne syndrome is a rare disorder characterized by the presence of opsoclonus, myoclonus, ataxia. It is an extremely rare condition, affecting as few as 1 in 10,000,000 people per year. Opsoclonus myoclonus syndrome oms is characterized by opsoclonus and arrhythmicaction myoclonus that predominantly involves the trunk, limbs, and head. Opsoclonus myoclonus is a pervasive neurological syndrome of children and adults. Symptoms include rapid, multidirectional eye movements opsoclonus, quick, involuntary muscle jerks myoclonus, uncoordinated movement ataxia, irritability, and sleep disturbance. The pathophysiology is thought to be immunological on the basis of paraneoplastic or infectious etiologies. Opsoclonus myoclonus syndrome oms is a very rare condition with different autoimmune, infectious and paraneoplastic aetiologies or in most cases idiopathic. Pranzatelli departments ofneurology, pediatrics, andpharmacology, the george washington university, washington, dc, u. Welcome you have probably arrived at this website because your child was recently diagnosed with opsoclonus myoclonus syndrome oms.
Opsoclonus myoclonus syndrome is a very rare disorder with onset usually in the second year of life, and the clinical features of opsoclonus, myoclonus, ataxia, irritability, sleep disturbance, and, often but by no means invariably, an associated neuroblastoma. Opsoclonusmyoclonus syndrome oms, also known as opsoclonus myoclonus ataxia, is a syndrome that includes opsoclonus along with diffuse or focal body myoclonus and truncal titubation with or without ataxia and other cerebellar signs. Opsoclonus myoclonus is a rare neurological disorder characterized by an unsteady, trembling gait, myoclonus brief, shocklike muscle spasms, and opsoclonus irregular, rapid eye movements. Half of all cases occur in children with a neuroblastoma. Opsoclonus myoclonus syndrome oms, also known as dancing eye syndrome des, is a rare neurological condition which develops over days or weeks in early childhood. It occurs most often as a paraneoplastic syndrome when a cancer remote to the brain induces cerebellar dysfunction that is unrelated to metastases. The onset of oms is usually abrupt and often severe. In 50% of oms patients, the syndrome is the result of the body creating antibodies to fight a cancerous tumor called neuroblastoma. Opsoclonus myoclonus ataxia oma is a rare neurological syndrome characterized with with autoimmune background.
Investigating neuroblastoma in childhood opsoclonus. Although longterm outcomes have historically been poor, including motor and cognitive disabilities, the advent of new and more aggressive immunotherapy regimens may be improving prognosis in opsoclonus myoclonus syndrome. Opsoclonusmyoclonus syndrome oms is a rare disorder that affects the nervous system. Opsoclonus is characterised by involuntary, irregular, but conjugate saccadic eye movements either multidirectional or horizontal ocular flutter precipitated by change of fixation. The opsoclonus myoclonus syndrome oms is caused by infections and neoplasms. Ofatumumab for refractory opsoclonusmyoclonus syndrome following neuroblastoma.
Around half of children diagnosed with opsoclonus myoclonus syndrome are found to have a neuroblastoma tumour. Opsoclonus myoclonus with multiple paraneoplastic syndromes and vgcc antibodies volume 36 issue 4 colin b. Opsoclonus myoclonus syndrome is also called oms or dancing eyesdancing feet syndrome. Opsoclonus describes seemingly random, involuntary, and rapid eye movements in the horizontal, vertical, and diagonal directions. Since it is more common in children with neuroblastoma, in particular, and can be rarely seen in adults, we. The syndrome of opsoclonus and myoclonus may be the first presenting symptom of neuroblastoma. Opsoclonus myoclonus information page national institute. This page from great ormond street hospital gosh explains the causes, symptoms and treatment of opsoclonus myoclonus syndrome dancing eye syndrome omsdes also known as kinsbourne syndrome. The cerebellum is responsible for coordinating eye movements, muscle coordination and speech. Opsoclonus myoclonus syndromedancing eye syndrome oms. One in one million opsoclonus myoclonus syndrome lurie.
As investigation methods vary significantly, the authors assessed the usefulness of imaging and metabolic studies in tumour detection. We still dont know and may never know why kate got oms. Paraneoplastic opsoclonus myoclonus syndrome is seen in older adults and has a higher frequency of encephalopathy. Josephson, ian grant, timothy benstead skip to main content accessibility help we use cookies to distinguish you from other users and to provide you with a better experience on our websites. Signs and symptoms of opsoclonus myoclonus syndrome oms may include unsteady, trembling gait manner of walking sudden, brief, shocklike muscle spasms myoclonus. Myoclonic encephalitis in childhood usually recovers over a period of weeks to months, but the clinical course may be protracted and recovery incomplete. Symptoms include rapid, multidirectional eye movements opsoclonus, quick, involuntary muscle jerks, uncoordinated movement ataxia, irritability, and sleep disturbance. Opsoclonusmyoclonus is another cns syndrome that comprises myoclonic jerks of the limbs and trunk, with opsoclonus, involuntary, arrhythmic, highamplitude, multidirectional saccades. The onset is oftentimes abrupt and can be relatively severe. Topiramate responsive adultonset opsoclonus myoclonus. Epileptic activity was seen on eeg 2 years later as well as two episodes of myoclonic seizures. Opsoclonus myoclonus syndrome oms is an inflammatory neurological disorder, often occurring as a paraneoplastic syndrome with neurological symptoms being the first sign of an occult tumor. Opsoclonus myoclonus ataxia syndrome often referred to as omas or opsoclonus myoclonus syndrome is an autoimmune disorder of the nervous system characterized by new movements of the limbs and eyes, abnormal behaviors, sleep dysregulation, and difficulty talking. A child with this disorder and treated with acth gel had abnormal uptake of 67 ga in both adrenal glands during studies to attempt to detect an occult neuroblastoma.
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