Anti-Hu-associated brainstem encephalitis
Objective: We review a series of patients with anti-Hu-associated brainstem encephalitis to better define the clinical presentation and to improve its recognition. Methods: We collected data from 14 patients diagnosed by members of the Paraneoplastic Neurological Syndromes Euronetwork, and eight pat...
| Authors: | ; ; ; ; ; ; ; ; ; |
|---|---|
| Format: | Article |
| Status: | Accepted version |
| Publication date: | 2008 |
| Country: | España |
| Institution: | Varias* (Consorci de Biblioteques Universitáries de Catalunya, Centre de Serveis Científics i Acadèmics de Catalunya) |
| Repository: | Recercat. Dipósit de la Recerca de Catalunya |
| OAI Identifier: | oai:recercat.cat:2445/222978 |
| Online Access: | https://hdl.handle.net/2445/222978 |
| Access Level: | Open access |
| Keyword: | Encefalitis Manifestacions neurològiques de les malalties Encephalitis Neurologic manifestations of general diseases |
| _version_ | 1878730196003061760 |
|---|---|
| author | Saiz Hinarejos, Albert |
| author2 | Bruna, Jordi Stourac, Pavel Vigliani, Maria Claudia Giometto, Bruno Grisold, Wolfgang Honnorat, Jérôme Psimaras, Dimitri Voltz, Raymond Graus Ribas, Francesc |
| author2_role | author author author author author author author author author |
| author_browse | Bruna, Jordi Giometto, Bruno Graus Ribas, Francesc Grisold, Wolfgang Honnorat, Jérôme Psimaras, Dimitri Saiz Hinarejos, Albert Stourac, Pavel Vigliani, Maria Claudia Voltz, Raymond |
| author_facet | Saiz Hinarejos, Albert Bruna, Jordi Stourac, Pavel Vigliani, Maria Claudia Giometto, Bruno Grisold, Wolfgang Honnorat, Jérôme Psimaras, Dimitri Voltz, Raymond Graus Ribas, Francesc |
| author_role | author |
| collection | Recercat. Dipósit de la Recerca de Catalunya |
| description | Objective: We review a series of patients with anti-Hu-associated brainstem encephalitis to better define the clinical presentation and to improve its recognition. Methods: We collected data 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 antiHu 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 CSF 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 linical features that indicate an upper, mainly pontine, dysfunction before downward progression. |
| eu_rights_str_mv | openAccess |
| format | article |
| id | ES_175e356a7b24dd8e357288e04deea63e |
| institution | Varias* (Consorci de Biblioteques Universitáries de Catalunya, Centre de Serveis Científics i Acadèmics de Catalunya) |
| instname_str | Varias* (Consorci de Biblioteques Universitáries de Catalunya, Centre de Serveis Científics i Acadèmics de Catalunya) |
| network_acronym_str | ES |
| network_name_str | España |
| oai_identifier_str | oai:recercat.cat:2445/222978 |
| publishDate | 2008 |
| publishDateSort | 2008 |
| publisher | BMJ |
| publisherStr | BMJ |
| reponame_str | Recercat. Dipósit de la Recerca de Catalunya |
| spelling | Anti-Hu-associated brainstem encephalitis Saiz Hinarejos, Albert Bruna, Jordi Stourac, Pavel Vigliani, Maria Claudia Giometto, Bruno Grisold, Wolfgang Honnorat, Jérôme Psimaras, Dimitri Voltz, Raymond Graus Ribas, Francesc Encefalitis Manifestacions neurològiques de les malalties Encephalitis Neurologic manifestations of general diseases Objective: We review a series of patients with anti-Hu-associated brainstem encephalitis to better define the clinical presentation and to improve its recognition. Methods: We collected data 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 antiHu 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 CSF 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 linical features that indicate an upper, mainly pontine, dysfunction before downward progression. BMJ https://hdl.handle.net/2445/222978 |
| spellingShingle | Anti-Hu-associated brainstem encephalitis Saiz Hinarejos, Albert Encefalitis Manifestacions neurològiques de les malalties Encephalitis Neurologic manifestations of general diseases |
| status_str | acceptedVersion |
| title | Anti-Hu-associated brainstem encephalitis |
| title_full | Anti-Hu-associated brainstem encephalitis |
| title_fullStr | Anti-Hu-associated brainstem encephalitis |
| title_full_unstemmed | Anti-Hu-associated brainstem encephalitis |
| title_short | Anti-Hu-associated brainstem encephalitis |
| title_sort | Anti-Hu-associated brainstem encephalitis |
| topic | Encefalitis Manifestacions neurològiques de les malalties Encephalitis Neurologic manifestations of general diseases |
| topic_facet | Encefalitis Manifestacions neurològiques de les malalties Encephalitis Neurologic manifestations of general diseases |
| url | https://hdl.handle.net/2445/222978 |