{
    "abstract": "Cerebellar ataxia with sensory ganglionopathy is a rare neurological combination that can occur in some hereditary ataxias including mitochondrial diseases and in gluten sensitivity. Individually each condition can be a classic paraneoplastic neurological syndrome. We report a patient with this combination who was diagnosed with light-chain myeloma ten years after initial presentation.\nA 65-year-old Caucasian lady was referred to our Ataxia Clinic because of a 6-year history of progressive unsteadiness and a 2-year history of slurred speech. Past medical history included arterial hypertension. The patient was a non-smoker was not consuming alcohol excessively. There was no family history of ataxia. Neurological examination revealed prominent gaze-evoked nystagmus, heel to shin ataxia, gait ataxia, reduced reflexes and loss of vibration sensation in the legs. Cerebellar ataxia was confirmed using magnetic resonance spectroscopy of the cerebellum and sensory ganglionopathy using neurophysiological assessments including blink reflex study. A muscle biopsy that was arranged to explore the possibility of mitochondrial disease revealed amyloidosis. Urinalysis confirmed the presence of light chains. A bone marrow biopsy confirmed the diagnosis of light chain multiple myeloma.\nWhilst it could be argued that this could simply be a coincidence, the rarity of these conditions and the absence of an alternative aetiology for the neurological dysfunction argue in favour of a paraneoplastic phenomenon.",
    "authors": [
        {
            "affiliation": "Academic Department of Neurosciences, Sheffield Teaching Hospitals NHS Foundation Trust, Sheffield, UK ; University of Sheffield, Royal Hallamshire Hospital, Royal Hallamshire Hospital, Glossop Rd, Sheffield, South Yorkshire S10 2JF UK.",
            "firstname": "Panagiotis",
            "initials": "P",
            "lastname": "Zis"
        },
        {
            "affiliation": "Academic Department of Neurosciences, Sheffield Teaching Hospitals NHS Foundation Trust, Sheffield, UK.",
            "firstname": "Dasappaiah Ganesh",
            "initials": "DG",
            "lastname": "Rao"
        },
        {
            "affiliation": "Histopathology Department, Sheffield Teaching Hospitals NHS Foundation Trust, Sheffield, UK.",
            "firstname": "Bart E",
            "initials": "BE",
            "lastname": "Wagner"
        },
        {
            "affiliation": "Histopathology Department, Sheffield Teaching Hospitals NHS Foundation Trust, Sheffield, UK.",
            "firstname": "Lucinda",
            "initials": "L",
            "lastname": "Nicholson-Goult"
        },
        {
            "affiliation": "Department of Neuroradiology, Sheffield Teaching Hospitals NHS Foundaiton Trust, Sheffield, UK.",
            "firstname": "Nigel",
            "initials": "N",
            "lastname": "Hoggard"
        },
        {
            "affiliation": "Academic Department of Neurosciences, Sheffield Teaching Hospitals NHS Foundation Trust, Sheffield, UK ; University of Sheffield, Royal Hallamshire Hospital, Royal Hallamshire Hospital, Glossop Rd, Sheffield, South Yorkshire S10 2JF UK.",
            "firstname": "Marios",
            "initials": "M",
            "lastname": "Hadjivassiliou"
        }
    ],
    "conclusions": null,
    "copyrights": null,
    "doi": "10.1186/s40673-016-0060-4\n10.1002/mds.26158\n10.1016/S1474-4422(05)70096-X\n10.1007/s00415-016-8082-7\n10.1038/ajg.2015.434\n10.1111/j.1750-3639.1999.tb00224.x\n10.1016/S0140-6736(03)14027-5\n10.1136/postgradmedj-2011-130471\n10.1186/s40673-016-0055-1\n10.1017/cjn.2015.361\n10.1111/j.1600-0404.2011.01620.x\n10.4103/0971-4065.65304\n10.1215/15228517-2007-008\n10.4103/0976-3147.158794\n10.1212/01.wnl.0000203345.29020.db\n10.1097/00005792-199203000-00001\n10.1002/mus.24943\n10.1016/S1474-4422(08)70060-7\n10.1136/jnnp.2003.034447",
    "journal": "Cerebellum & ataxias",
    "keywords": [
        "Amyloid",
        "Ataxia",
        "Cerebellar",
        "Myeloma",
        "Paraneoplastic",
        "Sensory ganglionopathy"
    ],
    "methods": null,
    "publication_date": "2017-01-12",
    "pubmed_id": "28074147\n25757427\n15907739\n26961897\n26832652\n10219743\n9855346\n12907013\n23014671\n27729985\n26795713\n22070551\n20835325\n17522337\n26167030\n16567713\n1312211\n26467754\n18339348\n15258215",
    "results": null,
    "title": "Cerebellar ataxia and sensory ganglionopathy associated with light-chain myeloma.",
    "xml": "<Element 'PubmedArticle' at 0x7885047ba480>"
}