Caloric-induced nystagmus with isoelectric electroencephalogram.

abstract：:A total of 36 patients with Batten disease (juvenile-onset neuronal ceroid lipofuscinosis), homozygous or heterozygous for the major mutation, a 1.02-kb deletion, in the CLN3 gene, were studied to relate their genotype to their clinical phenotype. The onset of visual failure and epilepsy was highly concordant in both ...

journal_title：Annals of neurology

authors： Järvelä I,Autti T,Lamminranta S,Aberg L,Raininko R,Santavuori P

更新日期：1997-11-01 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

authors： Gorman GS,Schaefer AM,Ng Y,Gomez N,Blakely EL,Alston CL,Feeney C,Horvath R,Yu-Wai-Man P,Chinnery PF,Taylor RW,Turnbull DM,McFarland R

更新日期：2015-05-01 00:00:00

abstract：:Light and electron microscopic findings from two sural nerve biopsies obtained at a one-year interval from a patient with the clinical features of Seitelberger's disease are described. Ballooned axons with accumulations of membranous profiles, vesicles, mitochondria, and a homogeneous center were present, and there we...

journal_title：Annals of neurology

authors： Begeer JH,Houthoff HJ,van Weerden TW,de Groot CJ,Blaauw EH,le Coultre R

更新日期：1979-12-01 00:00:00

abstract：OBJECTIVE:Cerebellar dysfunction in multiple sclerosis (MS) contributes significantly to disability, is relatively refractory to symptomatic therapy, and often progresses despite treatment with disease-modifying agents. We previously observed that sodium channel Nav1.8, whose expression is normally restricted to the pe...

journal_title：Annals of neurology

authors： Shields SD,Cheng X,Gasser A,Saab CY,Tyrrell L,Eastman EM,Iwata M,Zwinger PJ,Black JA,Dib-Hajj SD,Waxman SG

更新日期：2012-02-01 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

authors： Lindvall O,Björklund A,Skagerberg G

更新日期：1983-09-01 00:00:00

abstract：OBJECTIVE:To evaluate the efficacy and safety of BHT-3009 in relapsing-remitting multiple sclerosis (MS) and to confirm that BHT-3009 causes immune tolerance. METHODS:BHT-3009 is a tolerizing DNA vaccine for MS, encoding full-length human myelin basic protein. Relapsing-remitting MS patients were randomized 1:1:1 into...

journal_title：Annals of neurology

authors： Garren H,Robinson WH,Krasulová E,Havrdová E,Nadj C,Selmaj K,Losy J,Nadj I,Radue EW,Kidd BA,Gianettoni J,Tersini K,Utz PJ,Valone F,Steinman L,BHT-3009 Study Group.

更新日期：2008-05-01 00:00:00

abstract：OBJECTIVE:Oral anticoagulation treatment (OAT) resumption is a therapeutic dilemma in intracerebral hemorrhage (ICH) care, particularly for lobar hemorrhages related to amyloid angiopathy. We sought to determine whether OAT resumption after ICH is associated with long-term outcome, accounting for ICH location (ie, loba...

journal_title：Annals of neurology

authors： Biffi A,Kuramatsu JB,Leasure A,Kamel H,Kourkoulis C,Schwab K,Ayres AM,Elm J,Gurol ME,Greenberg SM,Viswanathan A,Anderson CD,Schwab S,Rosand J,Testai FD,Woo D,Huttner HB,Sheth KN

更新日期：2017-11-01 00:00:00

abstract：:The fate of neurofilament (NF) proteins was examined in sections of nerves from rats that survived for 3 to 90 days following nerve transection, using anti-NF monoclonal antibodies and immunohistochemical techniques. The same methods were also applied to twenty-three human nerve biopsy specimens. Granular debris with ...

journal_title：Annals of neurology

authors： Trojanowski JQ,Lee VM,Schlaepfer WW

更新日期：1984-09-01 00:00:00

abstract：:If patients with left-sided hemispatial neglect bisect lines incorrectly because hemianopia or sensory hemiinattention prevents them from seeing how far the line extends to the left, a strategy that ensures their seeing the left side of the line in their normal field should improve performance. If patients have hemisp...

journal_title：Annals of neurology

authors： Heilman KM,Valenstein E

更新日期：1979-02-01 00:00:00

abstract：:Human prion diseases can occur as an idiopathic disorder (sporadic Creutzfeldt-Jakob disease) or can be acquired, as is the case for variant Creutzfeldt-Jakob disease. These disorders are characterized by the accumulation of a protease-resistant form of the host-encoded prion protein termed PrP(Sc) in the brains of af...

journal_title：Annals of neurology

authors： Head MW,Bunn TJ,Bishop MT,McLoughlin V,Lowrie S,McKimmie CS,Williams MC,McCardle L,MacKenzie J,Knight R,Will RG,Ironside JW

更新日期：2004-06-01 00:00:00

abstract：:We studied local cerebral blood flow, as measured by autoradiography with digital image processing and by tissue morphology, in six rats 4 hours after occlusion of the proximal middle cerebral artery. A consistent, three-dimensional pattern of graded reductions in local cerebral blood flow involved the affected hemisp...

journal_title：Annals of neurology

authors： Tyson GW,Teasdale GM,Graham DI,McCulloch J

更新日期：1984-06-01 00:00:00

abstract：:A 13-year-old boy with imipramine overdose developed seizures, respiratory arrest, and coma. Abnormalities of oculovestibular reflexes, electroencephalograms, and brainstem auditory evoked potentials were monitored in relation to measurements of drug levels. An alpha-coma electroencephalographic pattern evolved into o...

journal_title：Annals of neurology

authors： Pulst SM,Lombroso CT

更新日期：1983-11-01 00:00:00

abstract：OBJECTIVE:We compared outcomes after treatment with direct oral anticoagulants (DOACs) and vitamin K antagonists (VKAs) in patients with atrial fibrillation (AF) and a recent cerebral ischemia. METHODS:We conducted an individual patient data analysis of seven prospective cohort studies. We included patients with AF an...

journal_title：Annals of neurology

authors： Seiffge DJ,Paciaroni M,Wilson D,Koga M,Macha K,Cappellari M,Schaedelin S,Shakeshaft C,Takagi M,Tsivgoulis G,Bonetti B,Kallmünzer B,Arihiro S,Alberti A,Polymeris AA,Ambler G,Yoshimura S,Venti M,Bonati LH,Muir KW,Ya

更新日期：2019-06-01 00:00:00

abstract：:Cylindrical spirals are unique membranous structures that were detected in skeletal muscle of a mother and one of her two children; all three have percussion myotonia but no evidence of weak or wasted skeletal muscles. Muscle cramps, stiffness, posteffort muscle tightness, myotonic lid lag, and the cylinders appear or...

journal_title：Annals of neurology

authors： Bove KE,Iannaccone ST,Hilton PK,Samaha F

更新日期：1980-06-01 00:00:00

abstract：:To examine the antiparkinsonian effects of blocking glycineB receptors, we designed a pilot study testing the potent and selective antagonist, PAMQX, in 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine-treated primates. PAMQX had no intrinsic effects but markedly potentiated the antiparkinsonian action of levodopa. In a d...

journal_title：Annals of neurology

authors： Papa SM,Auberson YP,Greenamyre JT

更新日期：2004-11-01 00:00:00

abstract：:Residual tumor cells remain beyond the margins of every glioblastoma (GBM) resection. Their resistance to postsurgical therapy is considered a major driving force of mortality, but their biology remains largely uncharacterized. In this study, residual tumor cells were derived via experimental biopsy of the resection m...

journal_title：Annals of neurology

authors： Glas M,Rath BH,Simon M,Reinartz R,Schramme A,Trageser D,Eisenreich R,Leinhaas A,Keller M,Schildhaus HU,Garbe S,Steinfarz B,Pietsch T,Steindler DA,Schramm J,Herrlinger U,Brüstle O,Scheffler B

更新日期：2010-08-01 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

authors： Little BW,Brown PW,Rodgers-Johnson P,Perl DP,Gajdusek DC

更新日期：1986-08-01 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

authors： Eikermann-Haerter K,Arbel-Ornath M,Yalcin N,Yu ES,Kuchibhotla KV,Yuzawa I,Hudry E,Willard CR,Climov M,Keles F,Belcher AM,Sengul B,Negro A,Rosen IA,Arreguin A,Ferrari MD,van den Maagdenberg AM,Bacskai BJ,Ayata C

更新日期：2015-08-01 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

authors： Zadra A,Pilon M,Montplaisir J

更新日期：2008-04-01 00:00:00

abstract：:A 42-year-old homosexual man without evidence of immune deficiency developed cerebral granulomatous angiitis in association with the isolation of human T-lymphotropic virus type III (HTLV-III) from brain tissue and cerebrospinal fluid. This syndrome may be an additional neurological sequela of HTLV-III infection. ...

journal_title：Annals of neurology

authors： Yankner BA,Skolnik PR,Shoukimas GM,Gabuzda DH,Sobel RA,Ho DD

更新日期：1986-09-01 00:00:00

abstract：:Existing and emerging viral central nervous system (CNS) infections are major sources of human morbidity and mortality. Treatments of proven efficacy are currently limited predominantly to herpesviruses and human immunodeficiency virus (HIV). Development of new therapies has been hampered by the lack of appropriate an...

journal_title：Annals of neurology

authors： Nath A,Tyler KL

更新日期：2013-09-01 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

authors： Argov Z,Siegal T

更新日期：1985-06-01 00:00:00

abstract：:To understand the pathogenesis of human immunodeficiency virus-induced neuropathology, it is critical to know the dynamics of viral replication in the central nervous system. Viral decay kinetics were mathematically analyzed from multiple serial specimens of ventricular cerebrospinal fluid and plasma during antiretrov...

journal_title：Annals of neurology

authors： Eggers C,Stuerenburg HJ,Schafft T,Zöllner B,Feucht HH,Stellbrink HJ,van Lunzen J

更新日期：2000-06-01 00:00:00

abstract：:Detrusor hyperreflexia is a relevant clinical symptom for patients suffering from Parkinson's disease. In a series of 16 patients, we demonstrated that subthalamic deep brain stimulation has a significant and urodynamically recordable effect leading to a normalization of pathologically increased bladder sensibility. ...

journal_title：Annals of neurology

authors： Seif C,Herzog J,van der Horst C,Schrader B,Volkmann J,Deuschl G,Juenemann KP,Braun PM

更新日期：2004-01-01 00:00:00

abstract：:We compared epilepsy phenotypes with genotypes of Angelman syndrome (AS), including chromosome 15q11-13 deletions (class I), uniparental disomy (class II), methylation imprinting abnormalities (class III), and mutation in the UBE3A gene (class IV). Twenty patients were prospectively selected based on clinical cytogene...

journal_title：Annals of neurology

authors： Minassian BA,DeLorey TM,Olsen RW,Philippart M,Bronstein Y,Zhang Q,Guerrini R,Van Ness P,Livet MO,Delgado-Escueta AV

更新日期：1998-04-01 00:00:00

abstract：:Auditory evoked magnetic fields to noise/square-wave sequences, presented once every 2.2 seconds, were recorded in 8 patients who had ischemic lesions in the auditory cortex or in its vicinity. In 2 patients with large temporoparietal lesions, the magnetic 100-msec deflection (N100m) was absent over the damaged side. ...

journal_title：Annals of neurology

authors： Mäkelä JP,Hari R,Valanne L,Ahonen A

更新日期：1991-07-01 00:00:00

abstract：:For unknown reasons, placebos reduce seizures in clinical trials in many patients. It is also unclear why some drugs showing statistical superiority to placebo in one trial may fail to do so in another. Using Seizuretracker.com, a patient-centered database of 684,825 seizures, we simulated "placebo" and "drug" trials....

journal_title：Annals of neurology

authors： Goldenholz DM,Moss R,Scott J,Auh S,Theodore WH

更新日期：2015-09-01 00:00:00

abstract：OBJECTIVE:Determine the probability of attaining complete remission in children with nonsyndromic epilepsy (NSE) over the course of ≥10 years from initial diagnosis; identify early predictors of complete remission; and assess the risk of relapse after achieving complete remission. METHODS:In a prospective community-ba...

journal_title：Annals of neurology

authors： Berg AT,Testa FM,Levy SR

更新日期：2011-10-01 00:00:00

abstract：OBJECTIVE:Episodic memory retrieval is reliant upon cognitive control systems, of which 2 have been identified with functional neuroimaging: a cingulo-opercular salience network (SN) and a frontoparietal executive network (EN). In Alzheimer's disease (AD), pathology is distributed throughout higher-order cortices. The ...

journal_title：Annals of neurology

authors： Dhanjal NS,Wise RJ

更新日期：2014-08-01 00:00:00

abstract：:Effective surgical treatment of patients with intractable complex partial seizures depends on accurate preoperative seizure focus localization. We evaluated seizure localization with interictal and immediate postictal single photon emission computed tomographic images of cerebral perfusion using technetium-99m-hexamet...

journal_title：Annals of neurology

authors： Rowe CC,Berkovic SF,Sia ST,Austin M,McKay WJ,Kalnins RM,Bladin PF

更新日期：1989-11-01 00:00:00