Abstract:
OBJECTIVE:Clinical outcomes in multiple sclerosis (MS) are highly variable. We aim to determine the long-term clinical outcomes in MS, and to identify early prognostic features of these outcomes. METHODS:One hundred thirty-two people presenting with a clinically isolated syndrome were prospectively recruited between 1984 and 1987, and followed up clinically and radiologically 1, 5, 10, 14, 20, and now 30 years later. All available notes and magnetic resonance imaging scans were reviewed, and MS was defined according to the 2010 McDonald criteria. RESULTS:Clinical outcome data were obtained in 120 participants at 30 years. Eighty were known to have developed MS by 30 years. Expanded Disability Status Scale (EDSS) scores were available in 107 participants, of whom 77 had MS; 32 (42%) remained fully ambulatory (EDSS scores ≤3.5), all of whom had relapsing-remitting MS (RRMS), 3 (4%) had RRMS and EDSS scores >3.5, 26 (34%) had secondary progressive MS (all had EDSS scores >3.5), and MS contributed to death in 16 (20%). Of those with MS, 11 received disease-modifying therapy. The strongest early predictors (within 5 years of presentation) of secondary progressive MS at 30 years were presence of baseline infratentorial lesions and deep white matter lesions at 1 year. INTERPRETATION:Thirty years after onset, in a largely untreated cohort, there was a divergence of MS outcomes; some people accrued substantial disability early on, whereas others ran a more favorable long-term course. These outcomes could, in part, be predicted by radiological findings from within 1 year of first presentation. ANN NEUROL 2020;87:63-74.
journal_name
Ann Neuroljournal_title
Annals of neurologyauthors
Chung KK,Altmann D,Barkhof F,Miszkiel K,Brex PA,O'Riordan J,Ebner M,Prados F,Cardoso MJ,Vercauteren T,Ourselin S,Thompson A,Ciccarelli O,Chard DTdoi
10.1002/ana.25637subject
Has Abstractpub_date
2020-01-01 00:00:00pages
63-74issue
1eissn
0364-5134issn
1531-8249journal_volume
87pub_type
杂志文章abstract::A 9-year-old, blind boy with severe mental retardation with a chronic sleep/wake disturbance had a circadian rhythm of 24.75 hours and an internal desynchronization of the endogenous rhythms. Treatment with oral melatonin given at 6 PM induced a regular sleep/wake pattern. Melatonin, in this patient, convincingly entr...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410290318
更新日期:1991-03-01 00:00:00
abstract::Immunohistochemical examination of 20 Down's syndrome brains, using antibodies to alpha-, beta-, and gamma-synuclein, demonstrated many alpha-synuclein-positive Lewy bodies and dystrophic neurites in 50% of amygdala samples from Down's syndrome brains with Alzheimer's disease. Similar lesions were less common in other...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/1531-8249(199903)45:3<353::aid-ana11>3.0.c
更新日期:1999-03-01 00:00:00
abstract::A child with neurofibromatosis exhibited gigantism and acromegaly in association with a hypothalamic mass lesion. Bromocriptine, 5 mg daily, reduced somatic growth rate and restored biochemical homeostasis but had no effect on tumor growth. Radiation therapy arrested tumor enlargement and stabilized deteriorating visu...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410150318
更新日期:1984-03-01 00:00:00
abstract::The mechanism whereby dystrophin deficiency leads to excessive fibrosis and muscle degeneration is not known. The absence of dystrophin in skeletal muscle is associated with reduced plasma membrane stability as evidenced by elevated serum levels of the cytoplasmic enzyme creatine kinase. Basic fibroblast growth factor...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410350320
更新日期:1994-03-01 00:00:00
abstract:OBJECTIVE:Though effective anti-human immunodeficiency virus (HIV) therapies are now available, they have variable penetration into the brain. We therefore aimed to assess changes over calendar time in the risk for HIV-associated dementia (HIV-D), and factors associated with HIV-D risk. METHODS:Using Concerted Action ...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.21225
更新日期:2008-02-01 00:00:00
abstract::We describe the first non-Ashkenazi patient with adult polyglucosan body disease and decreased glycogen-branching enzyme (GBE) activity in leukocytes. Gene analysis revealed compound heterozygosity for two novel missense mutations Arg515His and Arg524Gln in the GBE gene. Both missense mutations are predicted to impair...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:
更新日期:2000-04-01 00:00:00
abstract:OBJECTIVE:Paroxysmal hemicrania (PH) is a severe, strictly unilateral headache that lasts 2 to 30 minutes, occurs more than five times daily, is associated with trigeminal autonomic symptoms, and is exquisitely responsive to indomethacin. The purpose of the study was to determine the brain structures active in PH. MET...
journal_title:Annals of neurology
pub_type: 临床试验,杂志文章
doi:10.1002/ana.20763
更新日期:2006-03-01 00:00:00
abstract::The oculocerebrorenal syndrome (OCRS), Lowe's syndrome, is an X-linked, recessive disease characterized by mental retardation, congenital corneal abnormalities and cataracts, growth failure, rickets, osseous abnormalities, renal dysfunction with periodic acidosis, hypotonia, and areflexia. Ultrastructural studies of s...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410160109
更新日期:1984-07-01 00:00:00
abstract:OBJECTIVE:The cortex of patients with cortical dysplasia contains several abnormal cell types. Among the dysplastic cells, cytomegalic neurons are known to be electrically hyperactive and may contribute to epileptic activity. In this study, we sought to identify molecular markers of cytomegalic neurons in focal or hemi...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.20949
更新日期:2006-10-01 00:00:00
abstract::We sought to determine whether the putative pontine micturition center in the human dorsal pons contains corticotrophin-releasing factor (CRF) neurons, and whether these neurons are depleted in patients with multiple system atrophy and bladder dysfunction. Brains were obtained at autopsy from 4 control subjects and 4 ...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.1258
更新日期:2001-11-01 00:00:00
abstract::The purpose of this study was to explore the feasibility of obtaining magnetic resonance-measured cerebral metabolic rate of oxygen utilization (MR-CMRO(2)) in acute ischemic stroke patients. Seven stroke patients were serially imaged: 4.5 +/- 0.9 hours (tp1), 3 to 5 days (tp2), and 1 to 3 months (tp3) after symptom o...
journal_title:Annals of neurology
pub_type: 临床试验,杂志文章
doi:10.1002/ana.10433
更新日期:2003-02-01 00:00:00
abstract::Twenty-five patients with recently diagnosed leptomeningeal metastases underwent a prospective clinical and electro-physiological study to assess the mode and extent of peripheral nerve and spinal root involvement. Motor conduction velocities and F wave latencies in the legs were found frequently to be disturbed compa...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410170611
更新日期:1985-06-01 00:00:00
abstract:OBJECTIVE:The prevalence of mitochondrial disease has proven difficult to establish, predominantly as a result of clinical and genetic heterogeneity. The phenotypic spectrum of mitochondrial disease has expanded significantly since the original reports that associated classic clinical syndromes with mitochondrial DNA (...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.24362
更新日期:2015-05-01 00:00:00
abstract:OBJECTIVE:Chronic, low-dose exposure to pesticides is suspected to increase the risk for Parkinson's disease (PD), but data are inconclusive. METHODS:We prospectively examined whether individuals exposed to pesticides have higher risk for PD than those not exposed. The study population comprised participants in the Ca...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.20904
更新日期:2006-08-01 00:00:00
abstract::We describe a kindred with 7 confirmed and 2 probable cases of subacute dementia accompanied by myoclonus. The inheritance pattern is consistent with autosomal dominance and shows anticipation. The pathological changes involve marked gliosis with neuronal loss of the dorsomedial and midline thalamic nuclei, with lesse...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410200209
更新日期:1986-08-01 00:00:00
abstract::To clarify the influence of gastric emptying on levodopa-related motor fluctuations in Parkinson's disease, we assessed mobility and plasma levodopa concentrations in 10 patients during five modes of levodopa administration: (1) standard intermittent oral (SIO), (2) intermittent duodenal (ID), (3) continuous duodenal ...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410230611
更新日期:1988-06-01 00:00:00
abstract::We report a large family with a temporal partial epilepsy syndrome inherited in an autosomal dominant mode, with a penetrance of about 80%. This epilepsy syndrome is benign, with age of onset in the second or third decade of life. It is characterized by rare partial seizures, usually secondarily generalized, arising m...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/1531-8249(199902)45:2<182::aid-ana8>3.0.co
更新日期:1999-02-01 00:00:00
abstract::Life table methods were used to determine the relative risk of Alzheimer's disease (AD) in relatives of index cases with AD. Risk of AD was assessed in 967 first-degree relatives of 128 probands with clinically diagnosed AD and 572 first-degree relatives of a control group consisting of 84 subjects with Parkinson's di...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410250511
更新日期:1989-05-01 00:00:00
abstract::A family with Gerstmann-Sträussler-Scheinker disease had coincidental clinical onset in three members of two generations, a phenomenon suggesting a common source of a transmissible agent. A regular dietary supplement in this family was home-bred rabbit. The clinical picture, although generally similar to that in previ...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410140611
更新日期:1983-12-01 00:00:00
abstract::Solitary neoplastic proliferation of plasma cells (plasmacytoma) rarely occurs in the central nervous system. The longest follow-Up in nine previously reported patients was 5 years. We have followed two patients with solitary intracranial plasmacytoma for 8 and 13 years, respectively. At the time of biopsy diagnosis, ...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410110514
更新日期:1982-05-01 00:00:00
abstract::Chronic measles virus infection of the brain causes subacute sclerosing panencephalitis (SSPE), a progressive, relentless fatal disorder. We report a 52-year-old male who developed focal, chronic persistent measles virus infection of the brain following interferon and ribavirin therapy for hepatitis C, and who respond...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.24183
更新日期:2014-06-01 00:00:00
abstract::We report the use of a new stable isotope-labeled form of levodopa (LD) to examine in vivo central LD metabolism in Parkinson's disease (PD). Eight patients representing a wide spectrum of disease severity were administered 50 mg of carbidopa orally followed in 1 hour by an intravenous bolus of 150 mg of stable isotop...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410420305
更新日期:1997-09-01 00:00:00
abstract:OBJECTIVE:Somnambulism affects up to 4% of adults and constitutes one of the leading causes of sleep-related violence and self-injury. Diagnosing somnambulism with objective instruments is often difficult because episodes rarely occur in the laboratory. Because sleep deprivation can precipitate sleepwalking, we aimed t...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.21339
更新日期:2008-04-01 00:00:00
abstract::beta-Dystroglycan, a 43-kd transmembrane dystrophin-associated glycoprotein, plays an important role in linking dystrophin to the laminin-binding alpha-dystroglycan. alpha-/beta-Dystroglycan is encoded by a single gene on chromosome 3p21 and ubiquitously expressed in muscle and nonmuscle tissues. No known human diseas...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410400617
更新日期:1996-12-01 00:00:00
abstract::We report four children with idiopathic stroke syndromes who were assayed for human leukocyte antigen (HLA) class I markers and found to have HLA-B51 in common. This finding suggests that there may be a genetic predisposition for "idiopathic" childhood stroke, and host factors, possibly in concert with environmental f...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410310618
更新日期:1992-06-01 00:00:00
abstract:OBJECTIVE:Infantile spasms are seizures associated with a severe epileptic encephalopathy presenting in the first 2 years of life, and optimal treatment continues to be debated. This study evaluates early and sustained response to initial treatments and addresses both clinical remission and electrographic resolution of...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.24594
更新日期:2016-03-01 00:00:00
abstract::The objective of this study was to assess which features of temporal lobe proton magnetic resonance spectroscopic imaging (1H-MRSI) are associated with satisfactory surgical outcome in patients with intractable temporal lobe epilepsy and bilateral hippocampal atrophy. We studied 21 patients with intractable temporal l...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:
更新日期:2000-02-01 00:00:00
abstract::Identifying asymptomatic older individuals at elevated risk for developing Alzheimer disease (AD) is of clinical importance. Among 1,081 asymptomatic older adults, a recently validated polygenic hazard score (PHS) significantly predicted time to AD dementia and steeper longitudinal cognitive decline, even after contro...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.25029
更新日期:2017-09-01 00:00:00
abstract:OBJECTIVE:Patients with temporal lobe epilepsy often display cognitive comorbidity with recurrent seizures. However, the cellular mechanisms underlying the impairment of neuronal information processing remain poorly understood in temporal lobe epilepsy. Within the hippocampal formation neuronal networks undergo major r...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.24348
更新日期:2015-04-01 00:00:00
abstract::Results of intensive prevalence surveys on multiple sclerosis carried out in different small regions of Italy have suggested that this country falls into the high-frequency zone for the disease. To verify this hypothesis by studying a large population, we conducted intensive incidence and prevalence survey in the prov...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410170117
更新日期:1985-01-01 00:00:00