Central hypoventilation as the presenting symptom in Hu associated paraneoplastic encephalomyelitis

Gómez-Choco, Manuel J.; Zarranz, Juan J.; Saiz, Albert.; Forcadas, María I.; Graus, Francesc
October 2007
Journal of Neurology, Neurosurgery & Psychiatry;Oct2007, Vol. 78 Issue 10, p1143
Academic Journal
Central hypoventilation is usually caused by ischaemic or neoplastic lesions of the medulla and upper cervical spinal cord. An autoimmune disorder is not usually considered in the differential diagnosis of this syndrome. We retrospectively identified 14 patients from our database of 202 patients with Hu antibodies who presented with brainstem symptoms. Three were admitted to hospital because of central hypoventilation. All underwent intubation and mechanical ventilation. They could breathe properly while they were awake but suffered deep apnoeas during sleep. Two died, but one is still alive requiring ventilatory assistance during sleep. Autopsy was performed in one of the patients which showed severe inflammatory infiltrates and neuronal loss in the medulla. All patients had normal brain imaging studies and the cause of central hypoventilation was an unsolved problem until Hu antibodies were determined.


Related Articles

  • Antibodies and neuronal autoimmune disorders of the CNS. Graus, Francesc; Saiz, Albert; Dalmau, Josep // Journal of Neurology;Apr2010, Vol. 257 Issue 4, p509 

    We review the neuronal antibodies described in CNS disorders in order to clarify their diagnostic value, emphasize potentials pitfalls and limitations in the diagnosis of paraneoplastic neurological syndromes (PNS), and examine the current evidence for a possible pathogenic role. We propose to...

  • Autoimmune synaptische Enzephalopathien. Friese, M.A.; Magnus, T. // Der Nervenarzt;Oct2011, Vol. 82 Issue 10, p1239 

    Antibody-associated limbic encephalitis was usually seen as a paraneoplastic syndrome where the antibodies would target intracellular proteins. However, recent reports challenged this idea and described antibodies that target synaptic proteins expressed on the cell surface. These antibodies are...

  • Novel role for SLPI in MOG-induced EAE revealed by spinal cord expression analysis. Mueller, Andre M.; Pedré, Xiomara; Stempfl, Thomas; Kleiter, Ingo; Couillard-Despres, Sebastien; Aigner, Ludwig; Giegerich, Gerhard; Steinbrecher, Andreas // Journal of Neuroinflammation;2008, Vol. 5, Special section p1 

    Background: Experimental autoimmune encephalomyelitis (EAE) induced by myelin oligodendrocyte protein (MOG) in female Dark Agouti (DA) rats is a chronic demyelinating animal model of multiple sclerosis (MS). To identify new candidate molecules involved in the evolution or repair of EAE-lesions...

  • Spinal Cord Editor's Page August 2008. Wyndaele, J. J. // Spinal Cord;Aug2008, Vol. 46 Issue 8, p531 

    The article discusses various reports published within the issue, including the review of stem cell treatment for spinal cord lesion and the review on contemporary management of adult intramedullary spinal tumors, especially the pathology and neurological outcomes related to surgical resection.

  • Neuromuscular Paraneoplastic Syndromes: The Lambert-Eaton Myasthenic Syndrome. Prommer, Eric // Journal of Palliative Medicine;Sep2010, Vol. 13 Issue 9, p1159 

    Background: Disorders in which the effects of cancer occur at a site remote from the primary tumor are termed paraneoplastic disorders. The most well- known paraneoplastic disorder affecting the neuromuscular junction is the Lambert-Eaton myasthenic syndrome (LEMS). Discussion: We describe a...

  • Clinical analysis of paraneoplastic encephalitis associated with ovarian teratoma. Yasuyo Tonomura; Hiroshi Kataoka; Yasuo Hara; Miwa Takamure; Itirou Naba; Takanori Kitauti; Kozue Saito; Satoshi Ueno // Journal of Neuro-Oncology;Sep2007, Vol. 84 Issue 3, p287 

    Abstract Background  Recently, paraneoplastic encephalitis associated with ovarian teratoma has been described and related to an autoantibody. Methods: We describe four patients with ovarian teratoma-associated encephalitis (OTE) and compared their clinical pictures with those of...

  • Paraneoplastic limbic encephalitis associated with ovarian teratoma. LEE, KIAN-GUAN // Clinical Medicine;Feb2012, Vol. 12 Issue 1, p95 

    No abstract available.

  • Pyridoxal phosphatase is a novel cancer autoantigen in the central nervous system. BØe, A S; Bredholt, G; Knappskog, P M; Storstein, A; Vedeler, C A; Husebye, E S; Bøe, A S // British Journal of Cancer;10/18/2004, Vol. 91 Issue 8, p1508 

    Autoantibodies against many proteins are common in sera from patients with various types of cancer. These antibodies are sometimes involved in the development of conditions associated with cancer, such as paraneoplastic neurologic disorders. We used a human brain cDNA expression library and...

  • Autoimmune limbic encephalitis in 39 patients: immunophenotypes and outcomes. Bataller, L.; Kleopa, K. A.; Wu, G. F.; Rossi, J. E.; Rosenfeld, M. R.; Dalmau, J. // Journal of Neurology, Neurosurgery & Psychiatry;Apr2007, Vol. 78 Issue 4, p381 

    Background: About 40% of patients with limbic encephalitis do not have detectable CNS antibodies. Some of these patients have immune-mediated limbic encephalitis, but their frequency is unknown. Aims: (1) To determine the spectrum of limbic encephalitis identified on clinical grounds in a single...


Read the Article


Sign out of this library

Other Topics