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Anti-Hu-associated brainstem encephalitis
  1. A Saiz1,
  2. J Bruna2,
  3. P Stourac3,
  4. M C Vigliani4,
  5. B Giometto5,
  6. W Grisold6,
  7. J Honnorat7,
  8. D Psimaras8,
  9. R Voltz9,
  10. F Graus1
  1. 1
    Service of Neurology, Institut d’Investigació Biomèdica August Pi i Sunyer (IDIBAPS), Hospital Clínic, Barcelona, Spain
  2. 2
    Service of Neurology, Hospital Universitari de Bellvitge, Hospitalet, Barcelona, Spain
  3. 3
    Department of Neurology, Masaryk University, Brno, Czech Republic
  4. 4
    Department of Neuroscienze, Torino, Italy
  5. 5
    Service of Neurology, “Ca’ Foncello” Hospital, Treviso, Italy
  6. 6
    Ludwig Boltzmann Institute für Neuroonkologie, Vienna, Austria
  7. 7
    Centre de Référence Maladies Rares “Syndromes Neurologiques Paranéoplasiques,” INSERM U842, Lyon, France
  8. 8
    Service of Neurology, Hôpital de la Salpêtrière, INSERM U495, Paris, France
  9. 9
    Department of Palliative Medicine, University Hospital, Cologne, Germany
  1. Dr F Graus, Servei de Neurologia, Hospital Clinic, Villarroel 170, Barcelona 08036, Spain; fgraus{at}clinic.ub.es

Abstract

Objective: A series of patients with anti-Hu-associated brainstem encephalitis is reviewed to better define the clinical presentation and to improve its recognition.

Methods: Data were collected from 14 patients diagnosed by members of the Paraneoplastic Neurological Syndromes Euronetwork, and eight patients from the literature who presented with isolated brainstem encephalitis and had anti-Hu antibodies.

Results: The median age of the 22 patients was 64 years (range 42–83), and 50% were men. All patients developed a subacute neurological syndrome, in days or weeks. Brain MRI was always normal. Mild cerebrospinal fluid pleocytosis was reported in only two patients. The following syndromes were identified on admission: A medullary syndrome was seen in 11 (50%) patients. Seven of them presented with dysphagia, dysarthria and central hypoventilation. The other four in addition of bulbar symptoms, without central hypoventilation, presented pontine manifestations. Six (27%) patients developed a pontine syndrome with paresis of the VI or VII cranial nerves, nystagmus, usually vertical, and gait ataxia. There was a rapid downward progression to the medulla in all patients. Five (23%) patients presented a ponto-mesencephalic syndrome with uni- or bilateral palsy of the III and VI cranial nerves and gait ataxia, but rapidly progressed to complete gaze paresis and medullary dysfunction.

Conclusions: The study confirms the predominant medullary involvement but also shows that half of the patients present with clinical features that indicate an upper, mainly pontine, dysfunction before downward progression.

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Footnotes

  • Competing interests: None.

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