We discuss the assessment and differential diagnoses of a middle-aged man who presented with trismus, double vision and behavioural problems. MRI scan of the brain was initially normal, but a month later showed high signal in the hippocampal region on fluid attenuated inversion recovery sequence (FLAIR) imaging. We suspected a paraneoplastic brainstem encephalitis because of his smoking history, rapidly progressive symptoms and abnormal brainstem signs. A positron emission tomography-CT scan identified abnormal subcarinal nodes, shown on biopsy to be metastatic small cell lung cancer. He is currently undergoing treatment with chemotherapy and radiotherapy.
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http://dx.doi.org/10.1136/practneurol-2017-001819 | DOI Listing |
Int J Mol Sci
December 2024
Cerebro, Emoción y Conducta, School of Medicine, Universidad de las Américas (UDLA), Quito 170124, Ecuador.
Paraneoplastic cerebellar degeneration (PCD) is a rapidly progressive, immune-mediated syndrome characterized by the degeneration of Purkinje cells, often associated with the presence of antibodies targeting intracellular antigens within these cells. These autoantibodies are implicated in the induction of cytotoxicity, leading to Purkinje cell death, as demonstrated in in vitro models. However, the precise roles of antibodies and T lymphocytes in mediating neuronal injury remain a subject of ongoing research, with T cells appearing to be the main effectors of cerebellar injury.
View Article and Find Full Text PDFNeurol Neuroimmunol Neuroinflamm
January 2025
From the Department of Neurology and Center for Multiple Sclerosis and Autoimmune Neurology (A.D., L.C., J.J.-W.C., B.G.W., S.A.B., S.J.P., E.P.F.), Mayo Clinic College of Medicine, Rochester, MN; Department of Neurosciences (A.D.), Biomedicine, and Movement Sciences, University of Verona, Italy; Department of Radiology (K.N.K.), Mayo Clinic; Department of Ophthalmology (J.J.-W.C.), Mayo Clinic College of Medicine, Rochester, MN; Department of Neurology (D.M.W., C.V.-S.), Mayo Clinic, Scottsdale, AZ; Department of Neurology (B.G.W.), University of Virginia, Charlottesville; Department of Neurology (A.S.L.-C.), Mayo Clinic College of Medicine, Jacksonville, FL; Neurology Unit (E.S.), University Hospital of Sassari, Italy; and Department of Laboratory Medicine and Pathology (S.J.P., E.P.F.), Mayo Clinic College of Medicine, Rochester, MN.
Respirol Case Rep
December 2024
Division of Critical Care Medicine, Department of Internal Medicine National Cheng Kung University Hospital Tainan Taiwan.
Anti-Hu antibody-related paraneoplastic neurologic syndrome (PNS), a rare disease primarily associated with small cell lung carcinoma, is characterized by diverse neurologic manifestations. Central sleep apnea, although rare, is specific to anti-Hu antibody-related PNS. Herein, we present a case of out-of-hospital cardiac arrest attributed to hypercapnic central sleep apnea and detail the subsequent workup that revealed anti-Hu brainstem encephalitis.
View Article and Find Full Text PDFFront Immunol
November 2024
Department of Neurology, Nepean Hospital, Kingswood, NSW, Australia.
Progressive encephalomyelitis with rigidity and myoclonus (PERM) is a rare but debilitating disease within the stiff person syndrome (SPS) spectrum characterised by muscle rigidity, spasms, myoclonus, dysautonomia, and brainstem dysfunction. The exact pathogenetic mechanism is unclear, although there is an association with the presence of glycine receptor antibodies in serum and cerebrospinal fluid, and some cases are paraneoplastic. Here, we report a case of paraneoplastic, glycine receptor antibody-positive PERM associated with an otherwise subclinical monoclonal B-cell lymphocytosis (MBL) of the non-CLL phenotype, which may be, in turn, likely secondary to long-term methotrexate use [i.
View Article and Find Full Text PDFAntibodies (Basel)
September 2024
First Neurology Department, Eginition University Hospital, National and Kapodistrian University of Athens, 11528 Athens, Greece.
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