Opsoclonus-Myoclonus-Ataxia Syndrome (Dancing Eyes–Dancing Feet)
Description
● Introduction:
• An uncommon neurological condition brought on by autoimmune dysfunction.
• About 1 in 10,000,000 people are affected annually; 2–3% of youngsters are affected.
➣ Causes/Associations:
• Streptococcal, West Nile virus, HIV, post-viral, and rickettsial post-infectious encephalopathy.
• Paraneoplastic syndrome, including small cell lung cancer in adults and neuroblastoma in children.
• Episodes following a stroke.
● Case 1 (a woman, age 38)
Ataxia (axial + appendicular), tremor, opsoclonus, myoclonus, dysarthria, hypotonia, tiredness, irritability, and fever are some of the symptoms.
➣ Examining:
• Normal CSF.
• MRI: Not abnormal.
• Serology: Negative, meaning no hidden cancer.
Kinsbourne encephalitis (post-infectious OMS) is the diagnosis.
Treatment: IV methylprednisolone followed by supportive treatment and oral prednisone.
➣ Result:
• At first, the myoclonus diminished.
• Ocular flutter → ocular dysmetria → Opsoclonus improved.
• Complete recovery in six months, with little ataxia at one month.
● Highlights of the discussion include:
• Synonyms for OMS include Kinsbourne Syndrome, Dancing Eyes Syndrome, Opsoclonus–Myoclonus–Ataxia (OMA), and Dancing Feet Syndrome.
➣ Cause of paraneoplastic:
• Neuroblastoma in children (higher survival, good histology, but long-term neurological aftereffects).
• Lung and breast malignancies in adults (anti-Ri antibodies).
➣ Post-infectious cause:
• Usually occurs after a viral disease, such as a fever, respiratory infection, or gastrointestinal ailment.
• Most people get well in around two months.
➣ Pathophysiology:
• Involvement of the brainstem and cerebellum.
• Aberrant eye movements are caused by abnormalities in the burst, tonic, and pause cells of the pontine reticular formation.
• Opsoclonus and myoclonus are caused by lesions in the dentato-rubral pathway.
• Autopsy: dentate demyelination, loss of Purkinje cells.
● Options for Treatment:
➣ Immunotherapy:
• High-dose corticosteroids (oral taper → IV methylprednisolone).
• ACTH (effective but partial recovery in certain youngsters).
• Variable outcomes, or IVIg.
• Immunosuppressants (rituximab, azathioprine, and cyclophosphamide).
➣ Treatment for symptoms:
• Trazodone (for sleep and irritability), propranolol, baclofen, valproic acid, and benzodiazepines (clonazepam).
• Plasmapheresis (for relapses that are not responsive to steroids).
• Oncological treatment: surgery and chemotherapy (with varying outcomes) if neuroblastoma is present.
Protocol
● Medical/therapeutic Management:
• To lessen autoimmune CNS inflammation, use high-dose corticosteroids (methylprednisolone pulses → oral prednisone taper).
• Use imaging and laboratory testing to rule out underlying cancer (paraneoplastic cause).
• Symptomatic assistance with balance, coordination, and movement.
• Keep an eye out for relapses and continue treatment if symptoms continue.
● Nursing interventions (issues encountered + solutions):
• Ataxia/impaired mobility → Use walkers/rails, range-of-motion exercises, and assistance with ambulation.
• Fall/injury risk Use bed alarms, keep the space free, supervise transfers and provide adequate illumination.
• Aspiration risk and swallowing trouble => Evaluate upright feeding, thicker fluids, and NG tube if necessary.
• Modified coordination and communication → Make use of gestures, clear words, other forms of communication, and regular reorientation.
• Pain/muscle stiffness → Use positioning, physiotherapy, and analgesics/muscle relaxants.
•Emotional anguish or anxiety => Assure, explain, include family, and keep the surroundings low-stimulus.
• Poor self-care → Use adapted equipment, promote involvement, and help with ADLs.
• Malnutrition risk and nutritional imbalance → Track consumption, work with a dietician, and provide small, frequent meals.
• Infection risk → Preserve asepsis, keep an eye on labs and vital signs, and encourage cleanliness.
• Steroid side effects: Keep an eye on blood sugar, blood pressure, electrolytes, skin care, and bone health.
● Pancreatic Cancer with Local Advancement (TS-1 + Anlotinib Therapy):
➣ Medical/Therapeutic Management:
• For chemo-resistant illness, switch to TS-1 (oral chemotherapy) plus anlotinib (targeted treatment).
• Chemotherapy and radiotherapy together for improved tumour management.
• Keep an eye out for adverse effects and preserve your quality of life.
➣ Nursing Interventions (Implications and Solutions):
• Side effects of chemotherapy (gastrointestinal distress, mucositis, myelosuppression) → Track CBC, LFTs, and hydration; give antiemetics; take care of your mouth; and support your diet.
• Side effects of targeted therapy (hypertension, skin problems) → Track blood pressure, skin, and liver and kidney function; inform patients about warning symptoms.
• Side effects of radiation (fatigue, skin responses) → Skin care, balance between rest and exercise, and keep an eye on gastrointestinal problems.
• Nutrition and weight reduction → Dietary cooperation, high-protein/calorie meals, weight tracking, and supplementation.
• Pain management: Evaluate pain, give analgesics, and employ non-pharmacologic techniques.
• activities intolerance and fatigue → Prioritise important work, take breaks, and engage in graded activities.
• Risk of infection/neutropenia → Vigilant monitoring, strict aseptic treatment, and hygiene education for patients.
• Psychological and emotional distress → Counsel, include family and support groups, and educate the patient.
• Adherence to treatment → Provide information on oral medication schedules, remind patients, and track adherence.
• Symptom distress (nausea, vomiting, mucositis) → Frequent evaluations, hydration, and dietary changes.
Notes
For more information visit
10.7860/JCDR/2014/7184.4339