Anti‐Ri antibody‐associated paraneoplastic brainstem encephalitis successfully treated after treating the underlying malignancy with letrozole

Anti-Ri autoimmune encephalitis associated with breast cancer

“…Involvement of the brainstem can lead to ophthalmoplegia, truncal ataxia, opsoclonus-myoclonus syndrome, and decreased central respiratory drive. Anti-Ri encephalitis requires extensive and urgent investigation of the underlying tumor whose treatment is imperative 1,2,3,4 .…”

Paraneoplastic Syndromes

“…Symptoms improve with therapy, especially if started early; dramatic improvement uncommon if associated with anti-Hu PCD Methylprednisolone, cyclophosphamide, rituximab, plasmapheresis, IVIG 30 Treatment does not generally alter course of the syndrome 4 OM Glucocorticoids, plasmapheresis, IVIG 5 Successful treatment of malignancy often leads to partial or complete remission of symptoms 5Chronic GI pseudo-obstruction Octreotide, prednisone, azathioprine, budesonide, rituximab, cyclophosphamide; bowel resection is not an initial treatment but may become necessary57 Successful treatment of the malignancy generally leads to resolution of skin changes, but one-third of patients have residual motor impairment. Serum creatine kinase can be monitored to gauge response to therapy30 Successful treatment of the malignancy can lead to improvement of symptoms; immunosuppression can also be beneficial48,58 Uniquely, treatment of the malignancy does not affect the course of the syndrome; steroids may improve symptoms, but most cases progress to blindness 4Benzodiazepines and antispasmodic agents effective for symptomatic relief; immunosuppression has been tried, but too few cases for drug trials50 Necrotizing myelopathy High-dose glucocorticoids or IVIG (for initial therapy); once clinically stabilized, consider steroid-sparing therapy such as cyclophosphamide, mycophenolate mofetil, azathioprine, plasmapheresis; avoid treatments that only deplete B cells (eg, rituximab) unless the patient has onconeural antibodies to cell surface antigens (eg, aquaporin, VGKC)51 Maximal therapy rarely leads to clinical improvement, although it may stabilize symptoms for a while and delay time to wheelchair. Young patients are more responsive to therapy51 Motor neuron disease Plasmapheresis, IVIG[59][60][61] Successful treatment of malignancy may result in improved symptoms[59][60][61] Treatment can lead to improvement of the symptoms 45.…”