From: Septin-3 autoimmunity in patients with paraneoplastic cerebellar ataxia
Patient #, sex, age at neurological onset | Clinical summary | Cancer | Cancer therapy (start before/after onset of neurological symptoms) | Septin-3 IgG | Coexisting antibodies | Brain MRI | CSF findings | Immunotherapy (response) | Follow-up time (months) | Outcome |
---|---|---|---|---|---|---|---|---|---|---|
#1 male, 64a | Subacute progressive cerebellar syndrome with dysarthria, bilateral limb and gait ataxia | Malignant melanoma (Clark level IV) right groin with inguinal lymph node metastases | Adjuvant immunotherapy with interferon-alpha-2b (5 months before) | Serum 1:32.000 | Antinuclear Abs (1:320), GAD 65 Abs (28 units/ml, reference range 10 units/ml), GABAB receptor Abs 1:100 | Few small unspecific microangiopathic frontal lesions | 3 cells/µl, TPb 718 mg/l, QAlb mildly elevated, intrathecal IgG synthesis (56%)c | 3 × 500 mg IVMP (none), PE (transient), IVIG (transient), rituximab (transient) | 29 | Death |
#2 male, 69 | Subacute progressive cerebellar syndrome with dysarthria and marked gait ataxia | Malignant melanoma (Clark level IV) back with lung, liver and axillary lymph node metastases | Irradiation; adjuvant immunotherapy with nivolumab; dabrafenib and trametinib (19 months before) | Serum 1:10.000, CSF 1:1000 | None | Mild cerebellar atrophy, moderate microangiopathy | 13 cells/µl, TPb 611 mg/l, normal QAlb, CSF-specific OCB | 5 × 1000 mg IVMP (partial improvement) | 32 | Cerebellar syndrome stable, but worsening (epileptic seizures) due to cerebral metastases |
#3 male, 60 | Subacute progressive cerebellar syndrome with dysarthria, ataxia left leg, down beat nystagmus | Small-cell lung cancer with mediastinal lymph node metastases | Cisplatin/etoposide and atezolizumab (3 months after) | Serum 1:100.000 | Antinuclear Abs (1:160) | Normal | 20 cells/µl, TPb 889 mg/l, CSF-specific OCB | 5 × 250 mg IVMP, oral prednisolone (no further worsening) | 15 | Neurological symptoms stable |