Anti-Yo positive dorsal root ganglionopathy

Abstract

A 62 year old Romanian lady presented with a 1 year history of pain in her hands and feet which radiated up her arms and legs. It progressed over the year to severe, sharp pain involving all four limbs, associated with a tingling sensation and numbness. Two months after onset of symptoms she required assistance to mobilise. On presentation she required bilateral assistance and used a wheelchair outside of her home. There was no sphincter disturbance. Past medical history included chronic hepatitis C infection (genotype 1b), ischaemic heart disease and hypertension. She was an ex-smoker. On examination, the gait was ataxic and she required the assistance of two people. She had a sensory ataxia which increased with her eyes closed. There was decreased soft touch and pinprick sensation to the elbows and knees bilaterally. Proprioception was decreased to the ankles. Vibration sensation was decreased to the elbows and knees. She was areflexic throughout. Plantars were downgoing bilaterally. Vitamin B12 and folate levels were normal. Serum electrophoresis was normal. Serum cryoglobulins were normal. HIV serology and syphilis serology were negative. ANA was positive at 1:80 with a nucleolar pattern. Cerebrospinal fluid examination was normal and oligoclonal bands were negative. On nerve conduction testing, the motor study was well preserved with uniformly absent sensory nerve action potentials consistent with a dorsal root ganglionopathy. CT thorax, abdomen and pelvis showed cirrhosis of the liver with splenomegaly but no evidence of any malignancy. She was treated with intravenous immunoglobulin therapy and started on pregabalin. There was minimal improvement in her condition. Western blot assay was negative for anti-HU, RI, MA1, MA2, anti-CV2/CRMP5 and anti-amphiphysin paraneoplastic antibody markers but positive for anti-Yo. GAD and MAG antibodies were also negative. This prompted a further search for a malignancy. Bilateral mammography was normal. PET scan showed intense uptake in bilateral ovarian masses and no other areas of abnormal uptake. Bilateral salpingo-oophorectomy was carried out and the histology showed stage III papillary serous carcinoma with omental metastases. She was commenced on carboplatin chemotherapy but only tolerated one cycle due to neutropenia, lymphopenia and thrombocytopenia. She had two further cycles of intravenous immunoglobulin therapy for worsening symptoms but with little improvement. We present this case as we believe it to be unusual that a paraneoplastic dorsal root ganglionopathy is associated with anti-Yo antibodies. Anti-Yo antibodies were first reported in 1983 by Greenlee et al. [1] when they reported antibodies to Purkinje cells in two patients with ovarian cancer and paraneoplastic cerebellar degeneration. Anti-Yo antibodies are usually associated with ovarian or breast cancer and typically present with a cerebellar syndrome [2]. Anti-Hu antibodies and CV2 antibodies are most commonly associated with dorsal root ganglionopathy or sensory neuronopathy as a paraneoplastic phenomenon [3]. Peripheral nerve manifestations associated with seropositivity for anti-Yo antibodies have been described previously by Tracy et al. They described six patients with peripheral neuropathies and positive anti-Yo antibodies from a cohort of 76 anti-Yo seropositive women who were P. McNamara (&) L. Costelloe J. Redmond Department of Neurology, St James’s Hospital, Dublin, Ireland e-mail: pmcnamara2004@hotmail.com