Abstract:
OBJECTIVE:A study was undertaken to describe the clinical spectrum, voltage-gated potassium channel (VGKC) complex antibody specificities, and central nervous system localization of antibody binding in 29 patients diagnosed with Morvan syndrome (MoS). METHODS:Clinical data were collected using questionnaires. Radioimmunoassay, cell-based assays, and mouse brain immunohistochemistry were used to characterize the serum antibodies. RESULTS:Neuromyotonia (100%), neuropsychiatric features (insomnia 89.7%, confusion 65.5%, amnesia 55.6%, hallucinations 51.9%), dysautonomia (hyperhidrosis 86.2%, cardiovascular 48.3%), and neuropathic pain (62.1%) were the most common manifestations. A total of 93.1% of MoS patients were male. VGKC-complex antibodies were present in 23 of 29 (79%) MoS patients at referral; 24 of 27 available sera had CASPR2, LGI1, or both CASPR2 and LGI1 antibodies (3 also with contactin-2 antibodies). CASPR2 antibodies were generally higher titer than LGI1 antibodies. Tumors (41.4%), mainly thymomas, were associated with CASPR2 antibodies and a poor prognosis, whereas LGI1 antibodies were associated with serum hyponatremia. In brain tissue regions including the hypothalamus, raphe, and locus coeruleus, commercial antibodies to LGI1 bound to neuronal cell bodies including the antidiuretic hormone-secreting and orexin-secreting hypothalamic neurons, whereas CASPR2 commercial antibodies bound more often to the neuropil. MoS antibodies bound similarly, but there was evidence of additional antibodies in some sera that were not adsorbed by LGI1- or CASPR2-expressing cells and bound to mouse Caspr2(-/-) tissue. INTERPRETATION:MoS is clinically distinct from other VGKC-complex antibody-associated conditions, and usually is associated with high-titer CASPR2 antibodies, often accompanied by lower-titer LGI1 antibodies. CASPR2 and LGI1 antibodies bind to multiple brain regions, which helps to explain the multifocal clinical features of this disease, but other antibodies are likely to play a role in some patients and need to be characterized in future studies.
journal_name
Ann Neuroljournal_title
Annals of neurologyauthors
Irani SR,Pettingill P,Kleopa KA,Schiza N,Waters P,Mazia C,Zuliani L,Watanabe O,Lang B,Buckley C,Vincent Adoi
10.1002/ana.23577subject
Has Abstractpub_date
2012-08-01 00:00:00pages
241-55issue
2eissn
0364-5134issn
1531-8249journal_volume
72pub_type
杂志文章,多中心研究abstract::Based on earlier findings that the presence of word comprehension impairment (a deficit in the meaning of words, or lexical semantics) in acute stroke was strongly associated with the presence of hypoperfusion or infarct in Wernicke's area, we tested the hypothesis that the severity of word comprehension impairment wa...
journal_title:Annals of neurology
pub_type: 临床试验,杂志文章
doi:10.1002/ana.1265
更新日期:2001-11-01 00:00:00
abstract::Plasma exchange (PE) is the standard treatment in Guillain-Barré syndrome (GBS) patients who have lost the ability to walk. The effect of exchanges before this stage and the optimal number of exchanges for the other patients are still unknown. We randomized 556 GBS patients according to severity and number of exchange...
journal_title:Annals of neurology
pub_type: 临床试验,杂志文章,多中心研究,随机对照试验
doi:10.1002/ana.410410304
更新日期:1997-03-01 00:00:00
abstract:OBJECTIVE:Both animal and human data suggest that noradrenergic stimulation may enhance motor performance after brain damage. We conducted a placebo-controlled, double-blind and crossover design study to investigate the effects of noradrenergic stimulation on the cortical motor system in hemiparetic stroke patients. M...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.22237
更新日期:2011-02-01 00:00:00
abstract::We studied patients with partial and primary generalized seizures using fluorine-18-labeled 2-fluorodeoxyglucose and positron emission tomography. Interictal studies of patients with partial seizures showed regions of focal or lateralized hypometabolism in 15 of 17 patients with unilateral electroencephalographic foci...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410150734
更新日期:1984-01-01 00:00:00
abstract::Striatal 18F-6-fluorodopa (FD) uptake constants were measured by positron emission tomography in (1) normal cynomolgus monkeys and (2) a series of cynomolgus and rhesus monkeys that had received intracarotid infusions of 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine (MPTP). After the animals were killed, the number and...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410340306
更新日期:1993-09-01 00:00:00
abstract::In 9 patients with progressive supranuclear palsy and in 27 controls, dopamine and homovanillic acid concentrations, choline acetyltransferase (CAT) activity, and the number of [3H]spiperone and [3H]quinuclidinyl benzilate binding sites were measured post mortem in the striatum (caudate nucleus, putamen, and nucleus a...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410180503
更新日期:1985-11-01 00:00:00
abstract::We demonstrated that an IgM M-protein from a patient with motor neuron syndrome had antibody activity against gangliosides GM1, GD1b, and asialo GM1. Studies with a sugar-binding lectin suggested that the epitope in the patient's M-IgM involved the Gal(beta 1-3) GalNAc moiety. Immunohistological techniques demonstrate...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410230517
更新日期:1988-05-01 00:00:00
abstract:OBJECTIVE:Migraine is among the most common and debilitating neurological conditions. Familial hemiplegic migraine type 1 (FHM1), a monogenic migraine subtype, is caused by gain-of-function of voltage-gated CaV 2.1 calcium channels. FHM1 mice carry human pathogenic mutations in the α1A subunit of CaV 2.1 channels and a...
journal_title:Annals of neurology
pub_type: 杂志文章
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journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.21827
更新日期:2010-01-01 00:00:00
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journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.21776
更新日期:2009-11-01 00:00:00
abstract::The anatomy of the recently discovered diencephalospinal dopaminergic system is summarized and its possible role in physiological and pathological processes suggested. The cell bodies of origin of this system are localized periventricularly in the dorsal hypothalamus and caudal thalamus, and the terminal innervations ...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410140302
更新日期:1983-09-01 00:00:00
abstract::The genetic architecture of common epilepsies is largely unknown. HCNs are excellent epilepsy candidate genes because of their fundamental neurophysiological roles. Screening in subjects with febrile seizures and genetic epilepsy with febrile seizures plus revealed that 2.4% carried a common triple proline deletion (d...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.21909
更新日期:2010-04-01 00:00:00
abstract:OBJECTIVE:Friedreich ataxia (FRDA), an autosomal recessive neurodegenerative disease caused by mutations in the gene encoding for the mitochondrial protein frataxin, is characterized by ataxia and gait instability, immobility, and eventual death. We evaluated corneal confocal microscopy (CCM) quantification of corneal ...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.25355
更新日期:2018-12-01 00:00:00
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pub_type: 杂志文章
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journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410330411
更新日期:1993-04-01 00:00:00
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journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410320613
更新日期:1992-12-01 00:00:00
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journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410240303
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pub_type: 杂志文章
doi:10.1002/ana.410390614
更新日期:1996-06-01 00:00:00
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journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410170509
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pub_type: 杂志文章
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pub_type: 杂志文章
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journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.24241
更新日期:2014-10-01 00:00:00
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journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.21093
更新日期:2007-04-01 00:00:00
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journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410140414
更新日期:1983-10-01 00:00:00
abstract::The in vivo demyelinating capacity of sera from 27 patients with Guillain-Barré syndrome (GBS) and 47 other individuals was studied by intraneural injection into rat sciatic nerves. The morphological features of the nerves in cross section taken just proximal to the site of needle insertion was assessed 48 hours after...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410110112
更新日期:1982-01-01 00:00:00
abstract::We prepared RNA probes from cloned segments of human and murine enteroviruses (EVs) for in situ hybridization of skeletal muscle biopsies from patients with dermatomyositis (DM), polymyositis, other inflammatory myopathies, and noninflammatory muscle diseases, and from normal control subjects. A probe derived from The...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410260204
更新日期:1989-08-01 00:00:00
abstract::In a double-blinded, placebo-controlled, crossover study in seven mitochondrial myopathy patients (MM), we investigated whether lowering of lactate with dichloroacetate (DCA) can improve exercise tolerance and oxidative capacity in MM. DCA lowered plasma lactate at rest and during exercise (from 10.5 +/- 2.0 to 5.0 +/...
journal_title:Annals of neurology
pub_type: 临床试验,杂志文章,随机对照试验
doi:
更新日期:2001-05-01 00:00:00
abstract::This paper attempts to place into perspective the present state of clinical investigation with PET in neurology. To this end we briefly review the work of the neurology group of the MRC Cyclotron Unit at Hammersmith Hospital, by reference to studies performed in normal subjects and in patients with cerebrovascular dis...
journal_title:Annals of neurology
pub_type: 杂志文章
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更新日期:1984-01-01 00:00:00
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journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410040614
更新日期:1978-12-01 00:00:00
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journal_title:Annals of neurology
pub_type: 杂志文章
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更新日期:2015-04-01 00:00:00