On the cause of hyporeflexia in the Holmes-Adie syndrome.


:Electrophysiologic studies were carried out on 11 patients with Holmes-Adie syndrome, 8 of whom had reduced or absent ankle jerks. Conduction velocities and evoked nerve and muscle compound action potentials in the peroneal, posterior tibial, and sural nerves were normal. The H reflex was absent (or virtually absent) in the patients with depressed reflexes. The amplitude of the composite Ia EPSP in single soleus motoneurons was estimated from changes in firing probability of voluntarily activated soleus motor units in response to stimulation of low threshold afferents in the tibial nerve. These amplitudes were used to test the afferent side of the reflex pathway. Composite group Ia EPSPs in Holmes-Aide patients with hyporeflexia were smaller than normal or absent, indicating that the areflexia in the Holmes-Aide syndrome is due to loss of large spindle afferents or reduced effectiveness of their monosynaptic connections to motoneurons.






Miyasaki JM,Ashby P,Sharpe JA,Fletcher WA




Has Abstract


1988-02-01 00:00:00












  • 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...


    pub_type: 杂志文章


    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

  • Multifocal central nervous system damage caused by toluene abuse.

    abstract::Four toluene abusers had evidence of severe multifocal central nervous system damage. Impairment of cognitive, cerebellar, brainstem, auditory, and pyramidal tract function, as well as CT evidence of cerebral cortical, cerebellar, and brainstem atrophy, have been noted. In addition, we found opsoclonus, ocular flutter...


    pub_type: 杂志文章


    authors: Lazar RB,Ho SU,Melen O,Daghestani AN

    更新日期:1983-10-01 00:00:00

  • Head trauma preceding PD: a case-control study.

    abstract:OBJECTIVE:To investigate the association of PD with preceding head trauma using a case-control study design. METHODS:The medical records-linkage system of the Rochester Epidemiology Project was used to identify 196 subjects who developed PD in Olmsted County, MN, from 1976 through 1995. Each incident case was matched ...


    pub_type: 杂志文章,评审


    authors: Bower JH,Maraganore DM,Peterson BJ,McDonnell SK,Ahlskog JE,Rocca WA

    更新日期:2003-05-27 00:00:00

  • Amyotrophic lateral sclerosis: thyrotropin-releasing hormone and histidyl proline diketopiperazine in the spinal cord and cerebrospinal fluid.

    abstract::In spinal cords from seven amyotrophic lateral sclerosis (ALS) patients and four controls, we found no difference in thyrotropin-releasing hormone (TRH) concentration relative to protein content, but there was a reduction per tissue wet weight in ALS. Immunohistochemical localization of TRH in ALS cord was unaltered. ...


    pub_type: 杂志文章


    authors: Jackson IM,Adelman LS,Munsat TL,Forte S,Lechan RM

    更新日期:1986-09-01 00:00:00

  • Sign language aphasia due to left occipital lesion in a deaf signer.

    abstract::Localization of sign language production and comprehension in deaf people has been described as similar to that of spoken language aphasia. However, sign language employs a visuospatial modality through visual information. We present the first report of a deaf signer who showed substantial sign language aphasia with s...


    pub_type: 杂志文章


    authors: Saito K,Otsuki M,Ueno S

    更新日期:2007-10-02 00:00:00

  • Psychometric evaluation of a new sensory scale in immune-mediated polyneuropathies. Inflammatory Neuropathy Cause and Treatment (INCAT) Group.

    abstract:OBJECTIVE:To perform a psychometric evaluation of the inflammatory neuropathy cause and treatment (INCAT) sensory sumscore (ISS) in sensory-motor immune-mediated polyneuropathies. This new sensory scale was evaluated to strive for uniformity in assessing sensory deficit in these disorders. METHODS:The ISS comprises vi...


    pub_type: 杂志文章


    authors: Merkies IS,Schmitz PI,van der Meché FG,van Doorn PA

    更新日期:2000-02-22 00:00:00

  • Healthy eating and reduced risk of cognitive decline: A cohort from 40 countries.

    abstract:OBJECTIVE:We sought to determine the association of dietary factors and risk of cognitive decline in a population at high risk of cardiovascular disease. METHODS:Baseline dietary intake and measures of the Mini-Mental State Examination were recorded in 27,860 men and women who were enrolled in 2 international parallel...


    pub_type: 杂志文章,多中心研究,随机对照试验


    authors: Smyth A,Dehghan M,O'Donnell M,Anderson C,Teo K,Gao P,Sleight P,Dagenais G,Probstfield JL,Mente A,Yusuf S,ONTARGET and TRANSCEND Investigators.

    更新日期:2015-06-02 00:00:00

  • Prevalence of MRI abnormalities in people with epilepsy in rural China.

    abstract:OBJECTIVE:To assess the prevalence of brain MRI abnormalities in people with epilepsy in rural China and to compare it with that of individuals in the United Kingdom. METHODS:Brain MRI scans were obtained in people with epilepsy who participated in a rural community-based program in China between July 2010 and Decembe...


    pub_type: 杂志文章


    authors: Davagnanam I,Chen Z,Hoskote C,Ding D,Yang B,Wang Y,Wang T,Li W,Duncan JS,Wang W,Sander JW,Kwan P

    更新日期:2020-09-01 00:00:00

  • Nearly ubiquitous tissue distribution of the scrapie agent precursor protein.

    abstract::The "modified host protein" model of scrapie proposes that the transmissible agent is composed of the degradation-resistant protein, Sp33-37, and that clinical and pathologic signs result from neurotoxic accumulations of this protein. Sp33-37 is an abnormal, amyloidogenic isoform of the normally occurring cellular pro...


    pub_type: 杂志文章


    authors: Bendheim PE,Brown HR,Rudelli RD,Scala LJ,Goller NL,Wen GY,Kascsak RJ,Cashman NR,Bolton DC

    更新日期:1992-01-01 00:00:00

  • Optic neuritis in the elderly: prognosis for visual recovery and long-term follow-up.

    abstract::We evaluated 14 patients with idiopathic optic neuritis, 50 years of age or older, within 1 month of symptomatic onset from 1980 through 1985. We obtained follow-up information from case records an average of 13 months after initial examination and from telephone interviews an average of 57 months after initial evalua...


    pub_type: 杂志文章


    authors: Jacobson DM,Thompson HS,Corbett JJ

    更新日期:1988-12-01 00:00:00

  • Laryngeal botulinum toxin injections for disabling stuttering in adults.

    abstract::Stuttering is an action-induced speech disorder with involuntary, audible, or silent repetitions or prolongations in the utterance of short speech elements (sounds, syllables) and words. Symptomatic treatment programs frequently have initial success; persistent benefit is variable and many patients remain disabled. St...


    pub_type: 临床试验,杂志文章


    authors: Brin MF,Stewart C,Blitzer A,Diamond B

    更新日期:1994-12-01 00:00:00

  • Modeling disease trajectory in Duchenne muscular dystrophy.

    abstract:OBJECTIVE:To quantify disease progression in individuals with Duchenne muscular dystrophy (DMD) using magnetic resonance biomarkers of leg muscles. METHODS:MRI and magnetic resonance spectroscopy (MRS) biomarkers were acquired from 104 participants with DMD and 51 healthy controls using a prospective observational stu...


    pub_type: 杂志文章


    authors: Rooney WD,Berlow YA,Triplett WT,Forbes SC,Willcocks RJ,Wang DJ,Arpan I,Arora H,Senesac C,Lott DJ,Tennekoon G,Finkel R,Russman BS,Finanger EL,Chakraborty S,O'Brien E,Moloney B,Barnard A,Sweeney HL,Daniels MJ,Walter

    更新日期:2020-04-14 00:00:00

  • Detection of cortical neuron loss in motor neuron disease by proton magnetic resonance spectroscopic imaging in vivo.

    abstract::We performed proton magnetic resonance spectroscopic imaging (1H-MRSI) in patients with motor neuron disease (MND) to evaluate the distribution and extent of cortical neuron damage or loss as reflected by decreased N-acetyl (NA) to creatine (Cr) resonance intensity ratios. We examined premotor (superior frontal gyrus)...


    pub_type: 临床试验,杂志文章


    authors: Pioro EP,Antel JP,Cashman NR,Arnold DL

    更新日期:1994-10-01 00:00:00

  • What is the risk of developing parkinsonism following neuroleptic use?

    abstract::Use of neuroleptic medications is common among elderly patients. Using multivariate models, the authors found that use of antipsychotic medications, age, and disability were strong predictors of developing self-reported Parkinson disease (PD) in the subsequent year among 1992 to 2000 Medicare Current Beneficiary Surve...


    pub_type: 杂志文章


    authors: Noyes K,Liu H,Holloway RG

    更新日期:2006-03-28 00:00:00

  • Plasma exchange for acute attacks of CNS demyelination: Predictors of improvement at 6 months.

    abstract:BACKGROUND:Plasma exchange (PE) is used to treat severe episodes of CNS demyelination unresponsive to corticosteroids. Predictors of long-term response are not well known. METHODS:We retrospectively reviewed the medical records of 41 patients consecutively treated by PE between January 1995 and July 2007. The primary ...


    pub_type: 杂志文章


    authors: Llufriu S,Castillo J,Blanco Y,Ramió-Torrentà L,Río J,Vallès M,Lozano M,Castellà MD,Calabia J,Horga A,Graus F,Montalban X,Saiz A

    更新日期:2009-09-22 00:00:00

  • Primary writing tremor and myoclonic writer's cramp.

    abstract::Four patients with primary writing tremor had a focal, task-specific tremor that responded to anticholinergic drugs. Physiologic features included EMG activity alternating in antagonist muscles, 5- to 20-microV cerebral potentials evoked by stretch of pronator teres, and no C-reflexes. Another patient had myoclonic je...


    pub_type: 杂志文章


    authors: Ravits J,Hallett M,Baker M,Wilkins D

    更新日期:1985-09-01 00:00:00

  • Mesencephalic cholinergic nuclei in progressive supranuclear palsy.

    abstract::Using an antibody against choline acetyltransferase (ChAT), mesencephalic cholinergic cell nuclei were studied in autopsy material from 3 cases of progressive supranuclear palsy (PSP) and 4 controls. ChAT-immunoreactive neurons were quantified in sections that spanned the rostrocaudal extent of each nucleus. In PSP, t...


    pub_type: 杂志文章


    authors: Juncos JL,Hirsch EC,Malessa S,Duyckaerts C,Hersh LB,Agid Y

    更新日期:1991-01-01 00:00:00

  • Haploinsufficiency of CSF-1R and clinicopathologic characterization in patients with HDLS.

    abstract:OBJECTIVE:To clarify the genetic, clinicopathologic, and neuroimaging characteristics of patients with hereditary diffuse leukoencephalopathy with spheroids (HDLS) with the colony stimulating factor 1 receptor (CSF-1R) mutation. METHODS:We performed molecular genetic analysis of CSF-1R in patients with HDLS. Detailed ...


    pub_type: 杂志文章


    authors: Konno T,Tada M,Tada M,Koyama A,Nozaki H,Harigaya Y,Nishimiya J,Matsunaga A,Yoshikura N,Ishihara K,Arakawa M,Isami A,Okazaki K,Yokoo H,Itoh K,Yoneda M,Kawamura M,Inuzuka T,Takahashi H,Nishizawa M,Onodera O,Kakita

    更新日期:2014-01-14 00:00:00

  • Calcification of the basal ganglia as a cause of levodopa-resistant parkinsonism.

    abstract::We present a patient with levodopa-resistant parkinsonism due to basal ganglia calcification related to surgically-induced hypoparathyroidism. The calcification was seen on computerized axial tomography but not on skull x-rays. Such calcification should be looked for in all cases of levodopa-resistant parkinsonism, si...


    pub_type: 杂志文章


    authors: Klawans HL,Lupton M,Simon L

    更新日期:1976-03-01 00:00:00

  • New NBIA subtype: genetic, clinical, pathologic, and radiographic features of MPAN.

    abstract:OBJECTIVE:To assess the frequency of mutations in C19orf12 in the greater neurodegeneration with brain iron accumulation (NBIA) population and further characterize the associated phenotype. METHODS:Samples from 161 individuals with idiopathic NBIA were screened, and C19orf12 mutations were identified in 23 subjects. D...


    pub_type: 杂志文章


    authors: Hogarth P,Gregory A,Kruer MC,Sanford L,Wagoner W,Natowicz MR,Egel RT,Subramony SH,Goldman JG,Berry-Kravis E,Foulds NC,Hammans SR,Desguerre I,Rodriguez D,Wilson C,Diedrich A,Green S,Tran H,Reese L,Woltjer RL,Hayfli

    更新日期:2013-01-15 00:00:00

  • Brain atrophy and lesion load in a large population of patients with multiple sclerosis.

    abstract:OBJECTIVE:To measure white matter (WM) and gray matter (GM) atrophy and lesion load in a large population of patients with multiple sclerosis (MS) using a fully automated, operator-independent, multiparametric segmentation method. METHODS:The study population consisted of 597 patients with MS and 104 control subjects....


    pub_type: 杂志文章


    authors: Tedeschi G,Lavorgna L,Russo P,Prinster A,Dinacci D,Savettieri G,Quattrone A,Livrea P,Messina C,Reggio A,Bresciamorra V,Orefice G,Paciello M,Brunetti A,Coniglio G,Bonavita S,Di Costanzo A,Bellacosa A,Valentino P,Quar

    更新日期:2005-07-26 00:00:00

  • Circadian fluctuations of contrast sensitivity in Parkinson's disease.

    abstract::Spontaneous circadian fluctuations of motor symptoms in Parkinson's disease (PD) often occur, with dysfunction typically less severe in the early morning than in the afternoon. In 23 PD patients with or without a history of circadian motor fluctuations, we studied contrast sensitivity (CS), a non-motor function, consi...


    pub_type: 杂志文章


    authors: Struck LK,Rodnitzky RL,Dobson JK

    更新日期:1990-03-01 00:00:00

  • Efficacy of treatment of MS with IFNbeta-1b or glatiramer acetate by monthly brain MRI in the BECOME study.

    abstract:BACKGROUND:There are no published MRI studies comparing interferon beta 1b (IFNbeta-1b) and glatiramer acetate (GA) for treatment of relapsing multiple sclerosis (MS). OBJECTIVE:To compare the efficacy of IFNbeta-1b and GA for suppression of MS disease activity as evidenced on frequent brain MRI. METHODS:A total of 7...


    pub_type: 杂志文章,随机对照试验


    authors: Cadavid D,Wolansky LJ,Skurnick J,Lincoln J,Cheriyan J,Szczepanowski K,Kamin SS,Pachner AR,Halper J,Cook SD

    更新日期:2009-06-09 00:00:00

  • Depression of motor cortex excitability by low-frequency transcranial magnetic stimulation.

    abstract::We studied the effects of low-frequency transcranial magnetic stimulation (TMS) on motor cortex excitability in humans. TMS at 0.1 Hz for 1 hour did not change cortical excitability. Stimulation at 0.9 Hz for 15 minutes (810 pulses), similar to the parameters used to induce long-term depression (LTD) in cortical slice...


    pub_type: 杂志文章


    authors: Chen R,Classen J,Gerloff C,Celnik P,Wassermann EM,Hallett M,Cohen LG

    更新日期:1997-05-01 00:00:00

  • A phase 3 randomized study evaluating sialic acid extended-release for GNE myopathy.

    abstract:OBJECTIVE:To investigate the efficacy and safety of aceneuramic acid extended-release (Ace-ER), a treatment intended to replace deficient sialic acid, in patients with GNE myopathy. METHODS:UX001-CL301 was a phase 3, double-blind, placebo-controlled, randomized, international study evaluating the efficacy and safety o...


    pub_type: 杂志文章,多中心研究,随机对照试验


    authors: Lochmüller H,Behin A,Caraco Y,Lau H,Mirabella M,Tournev I,Tarnopolsky M,Pogoryelova O,Woods C,Lai A,Shah J,Koutsoukos T,Skrinar A,Mansbach H,Kakkis E,Mozaffar T

    更新日期:2019-04-30 00:00:00

  • Discrepancy between stimulus response and tolerance of pain in Alzheimer disease.

    abstract:BACKGROUND:Affective-motivational and sensory-discriminative aspects of pain were investigated in patients with mild to moderate Alzheimer disease (AD) and healthy elderly controls using the cold pressor test tolerance and repetitive stimuli of warmth and heat stimuli, evaluating the stimulus-response function. METHOD...


    pub_type: 杂志文章


    authors: Jensen-Dahm C,Werner MU,Jensen TS,Ballegaard M,Andersen BB,Høgh P,Waldemar G

    更新日期:2015-04-14 00:00:00

  • Delineation of the movement disorders associated with FOXG1 mutations.

    abstract:OBJECTIVE:The primary objective of this research was to characterize the movement disorders associated with FOXG1 mutations. METHODS:We identified patients with FOXG1 mutations who were referred to either a tertiary movement disorder clinic or tertiary epilepsy service and retrospectively reviewed medical records, cli...


    pub_type: 杂志文章


    authors: Papandreou A,Schneider RB,Augustine EF,Ng J,Mankad K,Meyer E,McTague A,Ngoh A,Hemingway C,Robinson R,Varadkar SM,Kinali M,Salpietro V,O'Driscoll MC,Basheer SN,Webster RI,Mohammad SS,Pula S,McGowan M,Trump N,Jenkin

    更新日期:2016-05-10 00:00:00

  • Treatment of CNS sarcoidosis with infliximab and mycophenolate mofetil.

    abstract:OBJECTIVE:To describe the effects of the anti-tumor necrosis factor neutralizing antibody, infliximab, and the antiproliferative immunosuppressant, mycophenolate mofetil, in refractory neurosarcoidosis. METHODS:We treated patients with biopsy-proven sarcoidosis and CNS involvement, who had failed treatment with steroi...


    pub_type: 杂志文章


    authors: Moravan M,Segal BM

    更新日期:2009-01-27 00:00:00

  • Multifocal visual evoked potentials are influenced by variable contrast stimulation in MS.

    abstract:OBJECTIVE:To test the hypothesis that patients with multiple sclerosis (MS) with intereye asymmetry on low contrast letter acuity, and thickness of the retinal nerve fiber layer (RNFL), would exhibit corresponding changes in cortical timing and amplitude responses on pattern reversal multifocal visual evoked potentials...


    pub_type: 杂志文章


    authors: Frohman AR,Schnurman Z,Conger A,Conger D,Beh S,Greenberg B,Sutter E,Calabresi PA,Balcer LJ,Frohman TC,Frohman EM

    更新日期:2012-08-21 00:00:00

  • Methyl bromide intoxication: neurologic features, including simulation of Reye syndrome.

    abstract::Three family members intoxicated with methyl bromide presented with a variety of neuropsychiatric manifestations including coma, severe status epilepticus, hyporeflexia, and acute psychosis. The simulation of Reye syndrome in the child emphasizes the need for careful toxicologic screening of all children presenting wi...


    pub_type: 杂志文章


    authors: Shield LK,Coleman TL,Markesbery WR

    更新日期:1977-10-01 00:00:00