Neuropsychiatric changes precede classic motor symptoms in ALS and do not affect survival.

Abstract:

OBJECTIVES:To investigate patient susceptibility to neuropsychiatric symptoms in the context of progression of more classic motor symptoms in amyotrophic lateral sclerosis (ALS) and to examine the impact of neuropsychiatric symptoms on survival. METHODS:The study cohort consisted of 219 patients with ALS (limb onset = 159; bulbar onset = 60), with neuropsychiatric symptoms measured using the Motor Neuron Disease Behavioural Scale and more classic ALS symptoms assessed by the Amyotrophic Lateral Sclerosis Functional Rating Scale-Revised. For detection of symptom susceptibility (neuropsychiatric vs classic motor), a Rasch analysis was applied (n = 219). Cox proportional hazard regression models were used for the survival analysis (n = 115 patients), which incorporated neuropsychiatric and classic motor symptoms. RESULTS:Rasch analysis demonstrated that neuropsychiatric symptoms appeared earlier than classic motor features of ALS. However, differences in neuropsychiatric scores did not affect survival: patients with abnormalities in neuropsychiatric domains did not exhibit a different rate of survival than those without (χ(2), 3.447, p = 0.328, -2 log-likelihood 377.341). CONCLUSIONS:Neuropsychiatric symptoms appear before classic motor features in ALS, which corroborates the notion that ALS and frontotemporal dementia lie on a disease continuum. The early detection of neuropsychiatric symptoms will be critical to inform clinical decisions and alleviate carer burden. Importantly, subtle neuropsychiatric symptoms alone do not affect survival in ALS, which in turn confirms their pervasive nature in ALS.

journal_name

Neurology

journal_title

Neurology

authors

Mioshi E,Caga J,Lillo P,Hsieh S,Ramsey E,Devenney E,Hornberger M,Hodges JR,Kiernan MC

doi

10.1212/WNL.0000000000000023

subject

Has Abstract

pub_date

2014-01-14 00:00:00

pages

149-55

issue

2

eissn

0028-3878

issn

1526-632X

pii

WNL.0000000000000023

journal_volume

82

pub_type

杂志文章
  • Neuronal hyperexcitability in stroke-like episodes of MELAS syndrome.

    abstract:BACKGROUND:The pathogenesis of stroke-like episodes in patients with mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes (MELAS) remains unknown. METHODS:Fourteen stroke-like episodes in six patients with MELAS were studied using clinical, neuroradiologic, and electrophysiologic approaches...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/wnl.59.6.816

    authors: Iizuka T,Sakai F,Suzuki N,Hata T,Tsukahara S,Fukuda M,Takiyama Y

    更新日期:2002-09-24 00:00:00

  • Low-dose clozapine in the treatment of levodopa-induced mental disturbances in Parkinson's disease.

    abstract::Delusions and other manifestations of psychotic behavior are common side effects in Parkinson's disease (PD) patients chronically treated with dopaminergic drugs. Clozapine, a dibenzodiazepine derivative, is an antipsychotic drug largely devoid of extrapyramidal side effects. We evaluated the effects of low doses of c...

    journal_title:Neurology

    pub_type: 临床试验,杂志文章

    doi:10.1212/wnl.45.3.432

    authors: Rabey JM,Treves TA,Neufeld MY,Orlov E,Korczyn AD

    更新日期:1995-03-01 00:00:00

  • Repetitive transcranial magnetic stimulation of the motor cortex ameliorates spasticity in multiple sclerosis.

    abstract:OBJECTIVE:To investigate whether repetitive transcranial magnetic stimulation (rTMS) can modify spasticity. METHODS:We used high-frequency (5 Hz) and low-frequency (1 Hz) rTMS protocols in 19 remitting patients with relapsing-remitting multiple sclerosis and lower limb spasticity. RESULTS:A single session of 1 Hz rTM...

    journal_title:Neurology

    pub_type: 临床试验,杂志文章

    doi:10.1212/01.wnl.0000257818.16952.62

    authors: Centonze D,Koch G,Versace V,Mori F,Rossi S,Brusa L,Grossi K,Torelli F,Prosperetti C,Cervellino A,Marfia GA,Stanzione P,Marciani MG,Boffa L,Bernardi G

    更新日期:2007-03-27 00:00:00

  • Neurologic improvement after peripheral blood stem cell transplantation in POEMS syndrome.

    abstract:BACKGROUND:Polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes (POEMS) syndrome is a rare multisystem disorder associated with plasma cell dyscrasia. There is increasing evidence that high-dose chemotherapy with autologous peripheral blood stem cell transplantation (Auto-PBSCT) is an efficacious t...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/01.wnl.0000323811.42080.a4

    authors: Kuwabara S,Misawa S,Kanai K,Suzuki Y,Kikkawa Y,Sawai S,Hattori T,Nishimura M,Nakaseko C

    更新日期:2008-11-18 00:00:00

  • Sensitivity and specificity of transcranial Doppler ultrasonography in the diagnosis of vasospasm following subarachnoid hemorrhage.

    abstract::Vasospasm is the leading cause of death and disability in patients with aneurysmal subarachnoid hemorrhage (SAH). Transcranial Doppler ultrasonography (TCD) can detect the arterial narrowing noninvasively, but the sensitivity and specificity of this technique have not been reported in a population of patients with a h...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/wnl.39.11.1514

    authors: Sloan MA,Haley EC Jr,Kassell NF,Henry ML,Stewart SR,Beskin RR,Sevilla EA,Torner JC

    更新日期:1989-11-01 00:00:00

  • Insights into the natural history of spontaneous intracranial hypotension from infusion testing.

    abstract:OBJECTIVE:To assess the pathophysiologic changes in patients with spontaneous intracranial hypotension (SIH) based on measures of CSF dynamics, and on the duration of symptoms, in a retrospective case-controlled study. METHODS:We included consecutive patients investigated for SIH at our department from January 2012 to...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/WNL.0000000000009812

    authors: Häni L,Fung C,Jesse CM,Ulrich CT,Miesbach T,Cipriani DR,Dobrocky T,Z'Graggen WJ,Raabe A,Piechowiak EI,Beck J

    更新日期:2020-07-21 00:00:00

  • Midline hyperplasia with malformation of the fornical system.

    abstract::A physically and intellectually normally developed boy had a resection of a lumbar myelomeningocele shortly after birth and a shunt was inserted for hydrocephalus. He died at age 12 years after a traffic accident. Autopsy disclosed a hyperplasia of the cerebellar vermis. An abnormal median-sagittal triangular fiber tr...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/wnl.28.12.1302

    authors: Friede RL,Briner J

    更新日期:1978-12-01 00:00:00

  • Homonymous hemianopias: clinical-anatomic correlations in 904 cases.

    abstract:OBJECTIVE:To describe the clinical characteristics and clinical-anatomic correlations of homonymous hemianopia (HH). BACKGROUND:Homonymous hemianopia impairs visual function and frequently precludes driving. Most knowledge of HH is based on relatively few cases with clinical-anatomic correlations. METHODS:The authors...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/01.wnl.0000203913.12088.93

    authors: Zhang X,Kedar S,Lynn MJ,Newman NJ,Biousse V

    更新日期:2006-03-28 00:00:00

  • Intra-blood-brain barrier synthesis of IgG: comparison of IgG synthesis formulas in a computer model and in 1,629 consecutive specimens.

    abstract::We evaluated four formulas for estimating the intra-blood-brain barrier (IBBB) synthesis of IgG in a computer model of two types of BBB damage and in 1,629 consecutively received pairs of CSF and serum. These four formulas were the IgG synthesis rate (SR), IgG(loc) (ie, local), IgG index, and IgG extended index. Resul...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/wnl.42.3.510

    authors: Peter JB,Bowman RL

    更新日期:1992-03-01 00:00:00

  • Measurement of tissue oxygen consumption in patients with mitochondrial myopathy by noninvasive tissue oximetry.

    abstract::We measured oxygen consumption in the exercising lower limb by using noninvasive tissue oximetry with the near-infrared spectra of hemoglobin in the quadriceps muscle during bicycle ergometer exercise in four normal controls and three patients with chronic progressive external ophthalmoplegia (CPEO) as well as one pat...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/wnl.49.3.837

    authors: Abe K,Matsuo Y,Kadekawa J,Inoue S,Yanagihara T

    更新日期:1997-09-01 00:00:00

  • Socioeconomic status and disability progression in multiple sclerosis: A multinational study.

    abstract:OBJECTIVE:To examine the association between socioeconomic status (SES) and disability outcomes and progression in multiple sclerosis (MS). METHODS:Health administrative and MS clinical data were linked for 2 cohorts of patients with MS in British Columbia (Canada) and South East Wales (UK). SES was measured at MS sym...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/WNL.0000000000007190

    authors: Harding KE,Wardle M,Carruthers R,Robertson N,Zhu F,Kingwell E,Tremlett H

    更新日期:2019-03-26 00:00:00

  • Connections between intrinsically photosensitive retinal ganglion cells and TBI symptoms.

    abstract::The majority of patients with traumatic brain injury (TBI) are classified as having a mild TBI. Despite being categorized as mild, these individuals report ongoing and complex symptoms, which negatively affect their ability to complete activities of daily living and overall quality of life. Some of the major symptoms ...

    journal_title:Neurology

    pub_type: 杂志文章,评审

    doi:10.1212/WNL.0000000000010830

    authors: Elenberger J,Kim B,de Castro-Abeger A,Rex TS

    更新日期:2020-11-03 00:00:00

  • Apolipoprotein E epsilon 4 and the pattern of regional brain atrophy in Alzheimer's disease.

    abstract:BACKGROUND:Although the APOE epsilon 4 allele increases the risk of developing AD, the effects of the epsilon 4 allele on brain atrophy in clinical AD patients are controversial. OBJECTIVE:To investigate a possible relationship between the genetic variants of APOE and brain atrophy in patients with AD. METHODS:Using ...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/wnl.57.8.1461

    authors: Hashimoto M,Yasuda M,Tanimukai S,Matsui M,Hirono N,Kazui H,Mori E

    更新日期:2001-10-23 00:00:00

  • A novel locus for hereditary spastic paraplegia with thin corpus callosum and epilepsy.

    abstract:BACKGROUND:Hereditary spastic paraplegia (HSP) are classified clinically as pure when progressive spasticity occurs in isolation or complicated when other neurologic abnormalities are present. At least 22 genetic loci have been linked to HSP, 8 of which are autosomal recessive (ARHSP). HSP complicated with the presence...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/01.wnl.0000208501.52849.dd

    authors: Al-Yahyaee S,Al-Gazali LI,De Jonghe P,Al-Barwany H,Al-Kindi M,De Vriendt E,Chand P,Koul R,Jacob PC,Gururaj A,Sztriha L,Parrado A,Van Broeckhoven C,Bayoumi RA

    更新日期:2006-04-25 00:00:00

  • Circadian sneezing.

    abstract::Prompted by the observation that a fellow medical student sneezed at about the same time in class every morning, we recorded the time of each of the 118 sneezes she made on 69 days over a 6.5-month period. Analysis of the sneeze times with the goodness-of-fit test revealed a highly nonuniform distribution (p < 0.0001)...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/wnl.44.3_part_1.369

    authors: Grant AC,Roter EP

    更新日期:1994-03-01 00:00:00

  • Detection of subtle gait disturbance and future fall risk in early multiple sclerosis.

    abstract:OBJECTIVE:To test the hypothesis that higher-challenge gait and balance tasks are more sensitive than traditional metrics to subtle patient-reported gait dysfunction and future fall risk in early multiple sclerosis (MS). METHODS:Persons with early MS (n = 185; ≤5 years diagnosed) reported gait function (MS Walking Sca...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/WNL.0000000000008938

    authors: Brandstadter R,Ayeni O,Krieger SC,Harel NY,Escalon MX,Katz Sand I,Leavitt VM,Fabian MT,Buyukturkoglu K,Klineova S,Riley CS,Lublin FD,Miller AE,Sumowski JF

    更新日期:2020-03-31 00:00:00

  • Cognitive decline in logopenic aphasia: more than losing words.

    abstract:OBJECTIVE:To track cognitive and language changes over time in patients with logopenic (lv-PPA) and semantic (sv-PPA) variants of primary progressive aphasia (PPA). METHODS:Thirteen consecutive patients with lv-PPA and 11 patients with sv-PPA underwent yearly evaluation for a mean of 3 years. Nineteen patients (11 lv-...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/WNL.0b013e318285c15b

    authors: Leyton CE,Hsieh S,Mioshi E,Hodges JR

    更新日期:2013-03-05 00:00:00

  • Cerebral dysfunction after chronic hypoxia in children.

    abstract::Although the long-term effects of acute anoxia have been studied, the effects of chronic hypoxia on the developing human brain have received little attention. We studied children with a cyanotic congenital heart defect to assess the impact of chronic hypoxia by eight measures: neurologic examination, visual evoked res...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/wnl.35.1.42

    authors: O'Dougherty M,Wright FS,Loewenson RB,Torres F

    更新日期:1985-01-01 00:00:00

  • Early clinicopathologic description of nodoparanodopathy in the 19th century.

    abstract::Nodoparanodopathy is a recent concept in the field of peripheral neuropathy, corresponding to peripheral nerve disorders stemming from an autoimmune attack directed and limited to the nodal region. This concept was identified using modern techniques of electrophysiology, immunology, and pathology (including electron m...

    journal_title:Neurology

    pub_type: 历史文章,杂志文章

    doi:10.1212/WNL.0000000000008399

    authors: Mathis S,Le Masson G,Vallat JM

    更新日期:2019-10-29 00:00:00

  • Familial band heterotopias simulating tuberous sclerosis.

    abstract::We report the clinical and neuroimaging findings of a mother and daughter with seizure disorders and band heterotopias seen on magnetic resonance imaging studies. These clinicoradiologic findings simulate those for a diagnosis of tuberous sclerosis complex. Clinicians should be aware of this migrational anomaly and it...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/wnl.43.7.1424

    authors: DiMario FJ Jr,Cobb RJ,Ramsby GR,Leicher C

    更新日期:1993-07-01 00:00:00

  • Striatonigral degeneration: clinical, MRI, and pathologic correlation.

    abstract::We report a patient with striatonigral degeneration in whom T2-weighted MRI imaging revealed low signal and atrophy in the putamen. Neuropathologic studies confirmed putaminal atrophy and iron deposition in the MRI low signal regions. ...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/wnl.40.4.710

    authors: O'Brien C,Sung JH,McGeachie RE,Lee MC

    更新日期:1990-04-01 00:00:00

  • Primary lateral sclerosis as a phenotypic manifestation of familial ALS.

    abstract::Primary lateral sclerosis (PLS) is a diagnosis of exclusion in patients with progressive spinobulbar spasticity and could be part of the clinical spectrum of ALS. Unlike ALS, which is familial in 5 to 10% of the cases, PLS has been described as a sporadic disorder in adults. The authors report two patients with PLS fr...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/01.WNL.0000162033.47893.F7

    authors: Brugman F,Wokke JH,Vianney de Jong JM,Franssen H,Faber CG,Van den Berg LH

    更新日期:2005-05-24 00:00:00

  • Dementia with Lewy bodies: basis of cingulate island sign.

    abstract:OBJECTIVES:To investigate clinical, imaging, and pathologic associations of the cingulate island sign (CIS) in dementia with Lewy bodies (DLB). METHODS:We retrospectively identified and compared patients with a clinical diagnosis of DLB (n=39); patients with Alzheimer disease (AD) matched by age, sex, and education (n...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/WNL.0000000000000734

    authors: Graff-Radford J,Murray ME,Lowe VJ,Boeve BF,Ferman TJ,Przybelski SA,Lesnick TG,Senjem ML,Gunter JL,Smith GE,Knopman DS,Jack CR Jr,Dickson DW,Petersen RC,Kantarci K

    更新日期:2014-08-26 00:00:00

  • Facial frequency manipulation normalizes face discrimination in AD.

    abstract::People with AD have deficient contrast sensitivity and impaired face discrimination. The authors presented photographs of unfamiliar faces of three different sizes to enhance the low, middle, or high facial frequency information (cycles per face). Patients with AD demonstrated normal discrimination of small faces only...

    journal_title:Neurology

    pub_type: 临床试验,杂志文章

    doi:10.1212/wnl.54.12.2316

    authors: Cronin-Golomb A,Cronin-Golomb M,Dunne TE,Brown AC,Jain K,Cipolloni PB,Auerbach SH

    更新日期:2000-06-27 00:00:00

  • Cyproheptadine reduces or prevents ischemic central nervous system damage.

    abstract::Several drugs that inhibit the effects of serotonin may reduce or prevent experimental CNS ischemic damage, but these drugs are not approved for human use in the United States. Administration of cyproheptadine (which is available for clinical use) 15 minutes before or 5 minutes after the onset increased the duration o...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/wnl.35.4.584

    authors: Zivin JA

    更新日期:1985-04-01 00:00:00

  • Proximal myotonic myopathy with MRI white matter abnormalities of the brain.

    abstract::Proximal myotonic myopathy (PROMM) is an autosomal dominantly inherited multisystemic disorder characterized by myotonia, proximal muscle weakness, and cataracts. This disorder is not linked to the gene locus of myotonic dystrophy (DM). We describe three new families with PROMM. In all patients, CTG repeats of the DM ...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/wnl.48.1.33

    authors: Hund E,Jansen O,Koch MC,Ricker K,Fogel W,Niedermaier N,Otto M,Kuhn E,Meinck HM

    更新日期:1997-01-01 00:00:00

  • Establishment of epilepsy surgery in Peru.

    abstract::Epilepsy surgery is a well-established treatment for certain types of intractable epilepsy. While there is a relatively high number of epilepsy surgery centers in Canada and the United States, the same cannot be said about many other parts of the world, such as South America. Although there are notable exceptions, suc...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/WNL.0000000000006029

    authors: Steven DA,Vasquez CM,Delgado JC,Zapata-Luyo W,Becerra A,Barreto E,Arango MF,Burneo JG

    更新日期:2018-08-21 00:00:00

  • Antigen processing gene polymorphisms in HLA-DR2 multiple sclerosis.

    abstract::The association between multiple sclerosis (MS) and alleles of the HLA class II genes indicates that at least one MS susceptibility gene is linked to the HLA class II region. However, the actual locus responsible has not been precisely identified. The recent cloning of new genes involved in antigen processing that map...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/wnl.43.6.1192

    authors: Liblau R,van Endert PM,Sandberg-Wollheim M,Patel SD,Lopez MT,Land S,Fugger L,McDevitt HO

    更新日期:1993-06-01 00:00:00

  • Mild cognitive impairment: incidence and vascular risk factors in a population-based cohort.

    abstract:OBJECTIVE:We examined the incidence of mild cognitive impairment (MCI) and its potential vascular risk factors in a prospective population-based study. METHODS:An age-stratified random population-based cohort (baseline n = 1,982), followed for up to 4 years, was annually assessed for cognitive and everyday functioning...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/WNL.0b013e318295d776

    authors: Ganguli M,Fu B,Snitz BE,Hughes TF,Chang CC

    更新日期:2013-06-04 00:00:00

  • Long-lasting conduction block in hereditary neuropathy with liability to pressure palsies.

    abstract::We detected 29 conduction blocks in 12 patients with hereditary neuropathy and liability to pressure palsies. The blocks occurred at entrapment sites, most often the ulnar nerve at the elbow. Some had lasted for several years. This kind of block seems to be characteristic of the neuropathy, and may be related to the l...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/wnl.35.11.1639

    authors: Magistris MR,Roth G

    更新日期:1985-11-01 00:00:00