Anti-Hu-associated brainstem encephalitis
- A Saiz1,
- J Bruna2,
- P Stourac3,
- M C Vigliani4,
- B Giometto5,
- W Grisold6,
- J Honnorat7,
- D Psimaras8,
- R Voltz9,
- F Graus1
- 1Service of Neurology, Institut d’Investigació Biomèdica August Pi i Sunyer (IDIBAPS), Hospital Clínic, Barcelona, Spain
- 2Service of Neurology, Hospital Universitari de Bellvitge, Hospitalet, Barcelona, Spain
- 3Department of Neurology, Masaryk University, Brno, Czech Republic
- 4Department of Neuroscienze, Torino, Italy
- 5Service of Neurology, “Ca’ Foncello” Hospital, Treviso, Italy
- 6Ludwig Boltzmann Institute für Neuroonkologie, Vienna, Austria
- 7Centre de Référence Maladies Rares “Syndromes Neurologiques Paranéoplasiques,” INSERM U842, Lyon, France
- 8Service of Neurology, Hôpital de la Salpêtrière, INSERM U495, Paris, France
- 9Department of Palliative Medicine, University Hospital, Cologne, Germany
- Dr F Graus, Servei de Neurologia, Hospital Clinic, Villarroel 170, Barcelona 08036, Spain; fgraus{at}clinic.ub.es
- Received 14 July 2008
- Revised 30 September 2008
- Accepted 6 October 2008
- Published Online First 17 November 2008
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.
Footnotes
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Competing interests: None.
