Anti-Hu-associated brainstem encephalitis

Sáiz, Albert; Bruna, Jordi; Šťourač, Pavel; Vigliani, Maria Claudia; Giometto, Bruno; Grisold, Wolfgang; Honnorat, Jérôme; Psimaras, Dimitri; Voltz, Raymond; Graus, Francesc

doi:10.1136/jnnp.2008.158246

Cited by 106 publications

(49 citation statements)

References 30 publications

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“…Consequently, the absence of CSF abnormalities in PNS with movement disorders does not allow to exclude the diagnosis. Some classical PNS are characterized by peculiar MRI features whereas others are not [35]. To define whether patients with paraneoplastic chorea/dystonia have distinctive MRI findings, we carried out a systematic review of the available data.…”

Section: Discussionmentioning

confidence: 99%

Chorea and related movement disorders of paraneoplastic origin: the PNS EuroNetwork experience

et al. 2011

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Chorea and other movement disorders are rarely described as paraneoplastic. The aim of this study was to describe 13 patients with paraneoplastic chorea and dystonia collected by the members of the paraneoplastic neurological syndrome (PNS) EuroNetwork and to review 29 cases from the literature. We analyzed neurological symptoms, severity of the neurological syndrome, delay in neurological diagnosis, associated cancer, oncological and neurological treatments received, and outcome. Eleven (1.2%) out of 913 patients with PNS were identified in the EuroNetwork register. Two more patients not included in the register were added. The overall population consisted of 13 patients with a median age of 75 years (range 49-82 years). In most patients, the movement disorder was classical choreoathetosis with symmetric involvement of the trunk, neck, and limbs. A minority of patients presented unilateral chorea, dystonia, and orobuccal dyskinesia. Associated symptoms, as polyneuropathy, encephalitis, psychiatric disturbances, or visual defects, were often present. The movement disorder usually had a subacute course. The most frequently associated cancer was small cell lung cancer (SCLC). Lymphoma, bowel, or kidney cancers were also reported. CV2/CRMP5 was the most frequently associated antibody, followed by Hu. Hyperintense lesions of the basal ganglia on T2-weighted images were seldom observed. Response to cancer therapy was observed in a minority of patients, but survival was short (17 months). As in other neurological diseases, movement disorders should also be suspected as paraneoplastic when they develop subacutely in older patients (usually over 50) and often in the presence of other ancillary neurological symptoms.

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Section: Discussionmentioning

confidence: 99%

Chorea and related movement disorders of paraneoplastic origin: the PNS EuroNetwork experience

et al. 2011

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“…Acquired central hypoventilation has been associated with brainstem tumors and ischemic lesions and Chiari malformations. Central hypoventilation has been reported in anti-Hu paraneoplastic brainstem encephalomyelitis 1 and in patients later identified as having anti-NMDA encephalitis. 2 To our knowledge, there are no reports of central hypoventilation after prophylactic cranial irradiation and its effects on the brainstem and cerebellum are unknown.…”

Section: Discussionmentioning

confidence: 98%

Paraneoplastic cerebellar ataxia with central hypoventilation

Kunchok

Barnes

Boyer

et al. 2017

Neurol Neuroimmunol Neuroinflamm

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“…Hu abs may occur together with other antineuronal abs such as voltage gated calcium channels (VGCC), anti-amphiphysin, anti-CV2, anti-Ri, but they may exceptionally occur in systemic autoimmune disorders with neurological complications in the absence of an underlying neoplastic disease. Hu abs positive patients may present with cerebellar ataxia, encephalomyelitis, limbic encephalitis, brainstem encephalitis, epilepsia partialis continua, sensorimotor neuropathies with predominant motor involvement, sensory neuronopathy, neuromyotonia, and autonomic dysfunction including gastrointestinal motility disorders (7)(8)(9). Complete response of the tumour seems to have a favourable influence on the clinical course whereas concomitant immunotherapy does not seem to adversely affect tumour outcome by impairing the immune response to the tumour.…”

Section: Hu Antibodiesmentioning

confidence: 93%

Antineuronal autoantibodies in neurological disorders

Bürk

2011

BioMolecular Concepts

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Autoantibodies (abs) related to neurological disease are currently classified into two large groups depending on the site of the respective target antigen: Group I encompasses abs that recognise intracellular antigens (Hu, Yo, Ri, CV2/CRMP5, amphiphysin, Ma2, SOX, ZIC, GAD, adenylate kinase 5, homer 3), whereas group II abs are targeted against neuronal cell membrane antigens (VGKC, AMPA-R, GABAB-R, NMDA-R, Glycine-R, VGCC, metabotropic GluR1). Both abs groups can be further subdivided according to their diagnostic impact for paraneoplastic or non-paraneoplastic neurological disease. The review gives an overview of the common characteristics of each group and provides more detailed information on single abs and the associated clinical syndromes.

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Anti-Hu-associated brainstem encephalitis

Abstract: 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.

Cited by 106 publications

References 30 publications

Chorea and related movement disorders of paraneoplastic origin: the PNS EuroNetwork experience

Chorea and related movement disorders of paraneoplastic origin: the PNS EuroNetwork experience

Paraneoplastic cerebellar ataxia with central hypoventilation

Antineuronal autoantibodies in neurological disorders

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