PTG protein depletion rescues malin-deficient Lafora disease in mouse.

abstract：:Cytological evaluation of cerebrospinal fluid (CSF) is an important means of following response to intracavitary chemotherapy for leptomeningeal malignancy. We studied the feasibility of quantitative cytological evaluation by retrospective analysis of serial CSF specimens from 7 patients receiving phase I intracavitar...

journal_title：Annals of neurology

authors： Russack V,Kim S,Chamberlain MC

更新日期：1993-07-01 00:00:00

abstract：OBJECTIVE:The Epi4K Consortium recently identified 4 de novo mutations in the γ-aminobutyric acid type A (GABAA ) receptor β3 subunit gene GABRB3 and 1 in the β1 subunit gene GABRB1 in children with one of the epileptic encephalopathies (EEs) Lennox-Gastaut syndrome (LGS) and infantile spasms (IS). Because the etiology...

journal_title：Annals of neurology

authors： Janve VS,Hernandez CC,Verdier KM,Hu N,Macdonald RL

更新日期：2016-05-01 00:00:00

abstract：:Hereditary canine spinal muscular atrophy (HCSMA) features rapidly progressive muscle weakness that affects muscles in an apparent proximal-to-distal gradient. In the medial gastrocnemius (MG) muscle of homozygous HCSMA animals, motor unit tetanic failure is apparent before the appearance of muscle weakness and appear...

journal_title：Annals of neurology

authors： Balice-Gordon RJ,Smith DB,Goldman J,Cork LC,Shirley A,Cope TC,Pinter MJ

更新日期：2000-05-01 00:00:00

abstract：:Recently, transcranial Doppler sonography has been introduced into clinical practice for noninvasive investigation of the large intracranial arteries. To determine its accuracy for detection of stenosing or occluding lesions, 133 consecutive patients were studied by both transcranial Doppler sonography and selective c...

journal_title：Annals of neurology

authors： Ley-Pozo J,Ringelstein EB

更新日期：1990-11-01 00:00:00

abstract：:Calmodulin is a major Ca2+ -binding protein that may mediate many Ca2+ -regulated processes in neuronal function. Calmodulin is present in the presynaptic nerve terminal in association with synaptic vesicles and in postsynaptic density fractions. Several calmodulin-regulated synaptic biochemical processes have been id...

journal_title：Annals of neurology

authors： DeLorenzo RJ

更新日期：1984-01-01 00:00:00

abstract：:Creutzfeldt-Jakob disease (CJD) is a rare dementia that is generally found in older people and is caused by unusual infectious pathogens or prions. Using rabbit antisera raised against hamster scrapie prion proteins (HaPrPSc), we identified by immunoblotting human CJD prion proteins (HuPrPCJD) in the brains of 14 pati...

journal_title：Annals of neurology

authors： Bockman JM,Prusiner SB,Tateishi J,Kingsbury DT

更新日期：1987-06-01 00:00:00

abstract：:We measured serially the blood pressure of 7 normal controls and 11 Type I insulin-dependent and 6 Type II non-insulin-dependent diabetic patients during sleep. All subjects underwent 2 or 3 nights of monitoring during which blood pressure was measured with an arteriosonde, a Doppler-type system with a cuff that autom...

journal_title：Annals of neurology

authors： Guilleminault C,Mondini S,Hayes B

更新日期：1985-12-01 00:00:00

abstract：:One hundred fifty autopsy brains from patients with clinically diagnosed Alzheimer's disease (AD) were examined pathologically. The brains were received consecutively over a 3-year period from numerous sources as part of a research program in which one brain half was frozen for biochemical studies and the other half w...

journal_title：Annals of neurology

authors： Joachim CL,Morris JH,Selkoe DJ

更新日期：1988-07-01 00:00:00

abstract：:The prevalence and titer of serum antibodies to several Epstein-Barr virus (EBV) antigens were compared among patients with multiple sclerosis, healthy siblings of multiple sclerosis patients, patients with other neurological diseases, and healthy non-blood-related subjects. Serum-cerebrospinal fluid (serum-CSF) pairs...

journal_title：Annals of neurology

authors： Sumaya CV,Myers LW,Ellison GW,Ench Y

更新日期：1985-04-01 00:00:00

abstract：:Freezing of gait (FOG) is a common and debilitating, but largely mysterious, symptom of Parkinson disease. In this review, we will discuss the cerebral substrate of FOG focusing on brain physiology and animal models. Walking is a combination of automatic movement processes, afferent information processing, and intenti...

journal_title：Annals of neurology

authors： Snijders AH,Takakusaki K,Debu B,Lozano AM,Krishna V,Fasano A,Aziz TZ,Papa SM,Factor SA,Hallett M

更新日期：2016-11-01 00:00:00

abstract：OBJECTIVE:The discovery of a posture-dependent effect on the difference between intraocular pressure (IOP) and intracranial pressure (ICP) at the level of lamina cribrosa could have important implications for understanding glaucoma and idiopathic intracranial hypertension and could help explain visual impairments in as...

journal_title：Annals of neurology

authors： Eklund A,Jóhannesson G,Johansson E,Holmlund P,Qvarlander S,Ambarki K,Wåhlin A,Koskinen LO,Malm J

更新日期：2016-08-01 00:00:00

abstract：:We studied rhesus monkeys with hemiparkinsonism or bilateral parkinsonism produced by unilateral or bilateral intracarotid administration of 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine. Using a standardized clinical rating scale, 4 hemiparkinsonian monkeys showed a 13 to 56% (mean, 36%) spontaneous improvement during...

journal_title：Annals of neurology

authors： Kurlan R,Kim MH,Gash DM

更新日期：1991-06-01 00:00:00

abstract：OBJECTIVE:To examine age-related changes in the neural systems for reading in nonimpaired and dyslexic children and adolescents. METHODS:Functional magnetic resonance imaging was used to study age-related changes in the neural systems for reading in a cross-sectional sample of 232 right-handed children 7 to 18 years o...

journal_title：Annals of neurology

authors： Shaywitz BA,Skudlarski P,Holahan JM,Marchione KE,Constable RT,Fulbright RK,Zelterman D,Lacadie C,Shaywitz SE

更新日期：2007-04-01 00:00:00

abstract：:Hyperekplexia is a rare, autosomal dominant neurological disorder characterized by hypertonia, especially in infancy, and by an exaggerated startle response. This disorder is caused by mutations in the alpha 1 subunit of the inhibitory glycine receptor (GLRA1). We previously reported two GLRA1 point mutations detected...

journal_title：Annals of neurology

authors： Shiang R,Ryan SG,Zhu YZ,Fielder TJ,Allen RJ,Fryer A,Yamashita S,O'Connell P,Wasmuth JJ

更新日期：1995-07-01 00:00:00

abstract：:Apolipoprotein E has been implicated in modifying neurological outcome after traumatic brain injury, although the mechanisms by which this occurs remain poorly defined. To investigate the role of endogenous apolipoprotein E following acute brain injury, noninvasive magnetic resonance imaging was performed on anestheti...

journal_title：Annals of neurology

authors： Lynch JR,Pineda JA,Morgan D,Zhang L,Warner DS,Benveniste H,Laskowitz DT

更新日期：2002-01-01 00:00:00

abstract：:Striopallidodentate calcinosis (Fahr's disease) is characterized clinically by seizures, rigidity, and dementia and pathologically by mineral deposition in the basal ganglia, dentate nucleus, and cerebral cortex. Disorders of iron and calcium-phosphate metabolism are thought to play a role in its pathogenesis. We pres...

journal_title：Annals of neurology

authors： Beall SS,Patten BM,Mallette L,Jankovic J

更新日期：1989-10-01 00:00:00

abstract：OBJECTIVE:Neonatal white matter injury (NWMI) is a lesion found in preterm infants that can lead to cerebral palsy. Although antagonists of bone morphogenetic protein (BMP) signaling, such as Noggin, promote oligodendrocyte precursor cell (OPC) production after hypoxic-ischemic (HI) injury, the downstream functional ta...

journal_title：Annals of neurology

authors： Sabo JK,Heine V,Silbereis JC,Schirmer L,Levison SW,Rowitch DH

更新日期：2017-04-01 00:00:00

abstract：:We report on a 12-year-old girl with a severe subacute to chronic bifrontal leukoencephalopathy. By clinical, biochemical, radiological, and neuropathological criteria, a diagnosis of inflammatory myelinoclastic diffuse sclerosis was reached. This is the third fully documented case. ...

journal_title：Annals of neurology

authors： Mehler MF,Rabinowich L

更新日期：1988-04-01 00:00:00

abstract：:Hemophilus influenzae is the most common cause of bacterial meningitis in children, and a high percentage of survivors are at risk for long-term sequelae. To explore the mechanisms responsible for these sequelae, a neonatal rat model was used to define the behavioral, electrophysiological, and biochemical changes foll...

journal_title：Annals of neurology

authors： Konkol RJ,Chapman L,Breese GR,Collier AM,Kilts C,Finley C,Vogel RR,Mailman RB,Bendeich EG

更新日期：1987-04-01 00:00:00

abstract：:Four infants had bilateral hippocampal sclerosis by magnetic resonance scans, including oblique coronal fast spin echo images of the temporal lobes; [18F]fluorodeoxyglucose-positron emission tomographic scans, done in 2 infants, showed isolated bilateral anterior temporal lobe hypometabolism. All had epilepsy with epi...

journal_title：Annals of neurology

authors： DeLong GR,Heinz ER

更新日期：1997-07-01 00:00:00

abstract：:Using two-color flow cytometric analysis, we studied peripheral blood lymphocyte subsets in 15 patients with human T-cell lymphotropic virus type I-associated myelopathy. The percentage of CD4+ 4B4+ cells (helper inducer T cell) was significantly increased in the patients with the myelopathy, compared with 16 healthy ...

journal_title：Annals of neurology

authors： Itoyama Y,Kira J,Fujii N,Goto I,Yamamoto N

更新日期：1989-08-01 00:00:00

abstract：OBJECTIVE:We aimed to determine the risk of short- and long-term stroke, as well as accidental injury, in patients discharged from an emergency department who were given a diagnosis of a peripheral vestibular disorder. METHODS:In this population-based, retrospective, cohort study, we identified all adult patients who ...

journal_title：Annals of neurology

authors： Atzema CL,Grewal K,Lu H,Kapral MK,Kulkarni G,Austin PC

更新日期：2016-01-01 00:00:00

abstract：:We report early-onset parkinsonism and dementia of 18 years' duration in a 52-year-old man whose grandfather and father had suffered from a similar neurological disease. In this patient, we found neuronal loss in various brain regions including the substantia nigra and cerebral cortex, Lewy bodies, cotton wool plaques...

journal_title：Annals of neurology

authors： Ishikawa A,Piao YS,Miyashita A,Kuwano R,Onodera O,Ohtake H,Suzuki M,Nishizawa M,Takahashi H

更新日期：2005-03-01 00:00:00

abstract：:Mitochondria are cellular organelles crucial for energy supply and calcium homeostasis in neuronal cells, and their dysfunction causes seizure activity in some rare human epilepsies. To directly test whether mitochondrial respiratory chain enzymes are abnormal in the most common form of chronic epilepsy, temporal lobe...

journal_title：Annals of neurology

authors： Kunz WS,Kudin AP,Vielhaber S,Blümcke I,Zuschratter W,Schramm J,Beck H,Elger CE

更新日期：2000-11-01 00:00:00

abstract：:A young man developed pathological thirst and hyperdipsia, hyperphagia, disordered temperature regulation, a lowered threshold for aggressive behavior, apathy, impaired memory, and seizures following encephalitis. He had marked hyponatremia. Bouts of water drinking produced water intoxication and precipitated status e...

journal_title：Annals of neurology

authors： Howe JG,Penney MD,Currie S,Morgan D

更新日期：1983-02-01 00:00:00

abstract：:Demonstration of intrathecal IgG production is employed in the diagnosis of various neurological disorders. This pathological IgG fraction in cerebrospinal fluid (CSF) can be visualized directly as oligoclonal bands by electrophoresis or isoelectric focusing or can be calculated as "excess" or "synthesized" IgG accord...

journal_title：Annals of neurology

authors： Lefvert AK,Link H

更新日期：1985-01-01 00:00:00

abstract：:Neurosurgical procedures such as the dorsal root entry zone operation, ganglionectomy, and spinal-cord stimulation have been offered to patients with intractable post-herpetic neuralgia (PHN). Poor efficacy or high morbidity have limited the overall usefulness of these procedures. We recently conducted a preliminary o...

journal_title：Annals of neurology

authors： Pappagallo M,Campbell JN

更新日期：1994-01-01 00:00:00

abstract：:Microelectrode recording methods for stereotactic localization of the subthalamic nucleus (STN) and surrounding structures are described. These methods accurately define targets for chronic deep brain stimulation in the treatment of Parkinson's disease. Mean firing rates and a burst index were determined for all recor...

journal_title：Annals of neurology

authors： Hutchison WD,Allan RJ,Opitz H,Levy R,Dostrovsky JO,Lang AE,Lozano AM

更新日期：1998-10-01 00:00:00

abstract：:Eighty-three patients with epidural spinal cord compression, from metastatic cancer were treated with high-dose adrenocorticosteroids and a new radiation fractionation protocol. Only those patients were included who had complete or almost complete block on myelography and who had not received prior radiation therapy t...

journal_title：Annals of neurology

authors： Greenberg HS,Kim JH,Posner JB

更新日期：1980-10-01 00:00:00

abstract：:Astrocytic necrosis is a prominent pathological feature of neuromyelitis optica (NMO) lesions and is clinically relevant. We report 5 NMO-related cases, all with longitudinally extensive lesions in the upper cervical cord, who underwent cervical cord (1) H-magnetic resonance spectroscopy. Lower myo-inositol/creatine v...

journal_title：Annals of neurology

authors： Ciccarelli O,Thomas DL,De Vita E,Wheeler-Kingshott CA,Kachramanoglou C,Kapoor R,Leary S,Matthews L,Palace J,Chard D,Miller DH,Toosy AT,Thompson AJ

更新日期：2013-08-01 00:00:00