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Antineuronal antibody-associated paraneoplastic neurological syndromes (PaNSs) result from tumour-stimulated autoimmune attacks against components of the nervous system. The rare antineuronal antibody anti-Ri (ANNA-2) was initially thought to be closely associated with paraneoplastic opsoclonus–myoclonus syndrome. Recently, however, it has been found in several other PaNSs.1 First-line treatment in PaNSs is removal of the underlying tumour. Second-line treatment is immunosuppression, which, although extensively used, especially when no tumour is detected, is often ineffective. Often, patients die from relentlessly progressive PaNS rather than the underlying neoplasm.2 Here, we report the 2-year follow-up of an anti-Ri-positive steroid-responsive myeloneuropathy. No tumour was detected. Immunosuppressive treatment was tailored on the basis of clinical relapses, inflammatory changes in cerebrospinal fluid (CSF) and somatosensory evoked potentials (SEPs).
A 65-year-old woman, a retired administrator, was referred to our department with a 10-month history of progressive gait difficulties and ascending sensory loss in her legs, eventually having become wheelchair bound. Her medical history was unremarkable apart from enlarged axillary lymph nodes that were excised 9 months earlier. Histological examination showed only inflammatory changes. She had received hormone replacement therapy for 11 years. She never smoked.
On neurological examination, deep tendon reflexes were absent. She had spastic paraparesis with bilateral extensor plantar responses. Bilateral hypaesthesia for light touch up to the knees and diminished vibration sense were observed distally in her legs. Magnetic resonance imaging (MRI) showed symmetrical T2-hyperintense multisegmental (C6–TH3/TH8–TH12) cervicothoracal lesions of the spinal cord, with gadolinium enhancement restricted to the lateral parts (fig 1A). Cerebral …