Anti-Hu-associated brainstem encephalitis.
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.
Saiz
A
A
Servei de Neurologia, Hospital Clinic, Barcelona, Spain.
Bruna
J
J
Stourac
P
P
Vigliani
M C
MC
Giometto
B
B
Grisold
W
W
Honnorat
J
J
Psimaras
D
D
Voltz
R
R
Graus
F
F
D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000970:Antineoplastic Agents / Q000627:therapeutic use; D020295:Brain Stem Neoplasms / Q000473:pathology*; D051959:ELAV Proteins / Q000276:immunology*; D005260:Female; D006801:Humans; D007167:Immunotherapy; D008297:Male; D008526:Medulla Oblongata / Q000473:pathology; D008636:Mesencephalon / Q000473:pathology; D008875:Middle Aged; D019635:Neurosurgical Procedures; D020361:Paraneoplastic Syndromes, Nervous System / Q000473:pathology* / Q000601:surgery / Q000628:therapy*; D011149:Pons / Q000473:pathology; D016896:Treatment Outcome
D000970:Antineoplastic Agents; D051959:ELAV Proteins