Hearing and dementia.

abstract：:Cardiac conduction and/or rhythm abnormalities (CCRA) are the most frequent and life-threatening complications in DM1. In order to determine prevalence, incidence, characteristics, age of onset and predictors of CCRA, CCRA progression and sudden cardiac death (SCD) in DM1, we collected ECG/24hECG-Holter data from a ye...

journal_title：Journal of neurology

authors： Bucci E,Testa M,Licchelli L,Frattari A,El Halabieh NA,Gabriele E,Pignatelli G,De Santis T,Fionda L,Vanoli F,Morino S,Garibaldi M,Di Pasquale A,Vanacore N,Botta A,Antonini G

更新日期：2018-04-01 00:00:00

abstract：:A patient is described who developed complex partial seizures with secondary generalization 3 years after a severe viral encephalitis with a CT and EEG identified lesion in the left insular cortex and its surrounding structures. When the seizures first occurred CT and MRI as well as repeated interictal conventional EE...

journal_title：Journal of neurology

authors： Dressler D,Voth E,Feldmann M,Henze T,Felgenhauer K

更新日期：1989-07-01 00:00:00

abstract：:Even though trigeminovestibular connections are well established in animals, mastication-induced dizziness has been described only as a vascular steal phenomenon in humans. We determined induction or modulation of nystagmus in two index patients with mastication-induced vertigo, 12 normal controls, and 52 additional p...

journal_title：Journal of neurology

authors： Park SH,Kim HJ,Kim JS,Koo JW,Oh SW,Kim DU,Kim JT,Welgampola M,Deriu F

更新日期：2014-03-01 00:00:00

abstract：OBJECTIVE:To determine the prevalence and characteristics of the cricopharyngeal bar (CPB), defined as marked protrusion with lacking relaxation and stricture of the upper esophageal sphincter on videofluoroscopy, in patients with inclusion body myositis (IBM). METHODS:We conducted a case-control study of comprehensiv...

journal_title：Journal of neurology

authors： Taira K,Yamamoto T,Mori-Yoshimura M,Sajima K,Takizawa H,Shinmi J,Oya Y,Nishino I,Takahashi Y

更新日期：2020-09-26 00:00:00

abstract：:Parvalbumin (a calcium-binding protein)-immunoreactive (PV-Ir) neurons in the cerebral cortex were examined in 20 postmortem brains obtained from elderly controls and patients with Pick's disease (PD). The type of PV-Ir neurons and their distribution in control and PD brains were similar. The number of PV-Ir neurons i...

journal_title：Journal of neurology

authors： Arai H,Noguchi I,Makino Y,Kosaka K,Heizmann CW,Iizuka R

更新日期：1991-07-01 00:00:00

abstract：OBJECTIVE:Patients with classic ataxia-telangiectasia (A-T) generally die in the second or third decade of life. Clinical descriptions of A-T tend to focus on the symptoms at presentation. However, during the course of the disease, other symptoms and complications emerge. As long-term survivors with classic A-T develop...

journal_title：Journal of neurology

authors： van Os NJH,van Deuren M,Weemaes CMR,van Gaalen J,Hijdra H,Taylor AMR,van de Warrenburg BPC,Willemsen MAAP

更新日期：2020-03-01 00:00:00

abstract：:Exercise therapy (ET) can be beneficial in disabled multiple sclerosis (MS) patients. Intermittent transcranial magnetic theta burst stimulation (iTBS) induces long-term excitability changes of the cerebral cortex and may ameliorate spasticity in MS. We investigated whether the combination of iTBS and a program of ET ...

journal_title：Journal of neurology

authors： Mori F,Ljoka C,Magni E,Codecà C,Kusayanagi H,Monteleone F,Sancesario A,Bernardi G,Koch G,Foti C,Centonze D

更新日期：2011-07-01 00:00:00

abstract：:We report on a 14-year-old boy with congenital isolated hemifacial hyperplasia. Hemifacial hypertrophy most likely represents a minor form of congenital hemihypertrophy. MRI of the soft tissue is particularly suitable to support the diagnosis and reveal associated bony asymmetries. ...

journal_title：Journal of neurology

authors： Urban PP,Bruening R

更新日期：2009-09-01 00:00:00

abstract：BACKGROUND:Persistent postural perceptual dizziness (PPPD) is a common chronic condition presenting in neurology and neuro-otology clinics. Symptoms lie on a spectrum in the general population. The cause is unknown and thought to involve interactions between visual and vestibular systems, but symptoms also correlate wi...

journal_title：Journal of neurology

authors： Powell G,Derry-Sumner H,Shelton K,Rushton S,Hedge C,Rajenderkumar D,Sumner P

更新日期：2020-08-01 00:00:00

abstract：:Specific mutations in COL6A3 have recently been reported as the cause of isolated recessive dystonia, which is a rare movement disorder. In all patients, at least one mutation was located in Exons 41 and 42. In an attempt to replicate these findings, we assessed by direct sequencing the frequency of rare variants in E...

journal_title：Journal of neurology

authors： Lohmann K,Schlicht F,Svetel M,Hinrichs F,Zittel S,Graf J,Lohnau T,Schmidt A,Mir P,Krause P,Lang AE,Jabusch HC,Wolters A,Kamm C,Zeuner KE,Altenmüller E,Naz S,Chung SJ,Kostic VS,Münchau A,Kühn AA,Brüggemann N,Kl

更新日期：2016-04-01 00:00:00

abstract：OBJECTIVE:Crossed aphasia (CA), usually referred to as an acquired language disturbance, is caused by a lesion in the cerebral hemisphere ipsilateral to the dominant hand, and the exact mechanism is not clear. The development of handedness is influenced by education and training and the impact of habitualization, while...

journal_title：Journal of neurology

authors： Tan X,Guo Y,Dun S,Sun H

更新日期：2018-07-01 00:00:00

abstract：:Additional autoimmune diseases in people with multiple sclerosis (MS) and their relatives have been studied many times. Studies have employed different designs, and yielded conflicting results. We performed a systematic review, and calculated overall risk of additional autoimmune diseases in people with MS and their f...

journal_title：Journal of neurology

authors： Dobson R,Giovannoni G

更新日期：2013-05-01 00:00:00

abstract：:Posterior leukoencephalopathy typically denotes neurotoxicity from immunosuppressive agents, malignant hypertension or eclampsia. It has not been documented in central nervous system angiitis. We present three cases associated with isolated cerebral angiitis after review of all cases of isolated CNS angiitis from 1998...

journal_title：Journal of neurology

authors： Wijdicks EF,Manno EM,Fulgham JR,Giannini C

更新日期：2003-04-01 00:00:00

abstract：:Regional cerebral glucose metabolism (rCMRGlc) and dopamine D2 receptor binding were measured in a 31-year-old, severely affected, untreated patient with Wilson's disease of 3 years' duration using positron emission tomography and 18F-deoxyglucose and 18F-methylspiperone ([18F]MSP), respectively. There was a severe re...

journal_title：Journal of neurology

authors： Schlaug G,Hefter H,Nebeling B,Engelbrecht V,Weiss P,Stöcklin G,Seitz RJ

更新日期：1994-10-01 00:00:00

abstract：:Hemichorea-hemiballism (HCHB) was infrequently related to cortical lesions such as tumor or infarction. Although functional derangement of the basal ganglia (BG) or the thalamus (Th) was suggested, pathomechanism of HCHB secondary to cortical lesions remains uncertain. We recruited the patients with HCHB secondary to ...

journal_title：Journal of neurology

authors： Hwang KJ,Hong IK,Ahn TB,Yi SH,Lee D,Kim DY

更新日期：2013-12-01 00:00:00

abstract：:A boy was suspected of having meningoencephalitis probably of viral origin, although all microbiological, including virological, tests were negative. Postmortem examination revealed a dissecting aortic aneurysm and disseminated intravascular coagulation involving the central nervous system exclusively. The clinical ev...

journal_title：Journal of neurology

authors： Hens L,Dom R,Goddeeris P,van den Bergh R

更新日期：1981-01-01 00:00:00

abstract：:Uric acid (UA) is reduced in multiple sclerosis (MS), and possibly relates to MS outcomes, with lower UA levels in subjects experiencing a relapse or presenting higher disability scores. The present retrospective longitudinal study evaluated UA variations in MS, in relation to clinical relapses, disability progression...

journal_title：Journal of neurology

authors： Moccia M,Lanzillo R,Costabile T,Russo C,Carotenuto A,Sasso G,Postiglione E,De Luca Picione C,Vastola M,Maniscalco GT,Palladino R,Brescia Morra V

更新日期：2015-01-01 00:00:00

abstract：:Neuromyelitis optica (NMO) is an inflammatory/demyelinating disorder predominantly affecting the optic nerves and spinal cord. Recent findings showed an underlying humoral abnormality in NMO, characterized by a serum antibody against aquaporin-4 (Aqp-4-Ab). In this study, we evaluated the Aqp-4-Ab status among Turkish...

journal_title：Journal of neurology

authors： Akman-Demir G,Tüzün E,Waters P,Içöz S,Kürtüncü M,Jarius S,Yapıcı Z,Mutlu M,Yeşilot N,Vincent A,Eraksoy M

更新日期：2011-03-01 00:00:00

abstract：:Levodopa therapy is essential for patients in the advanced stages of Parkinson's disease. However, at early stages, DA agonist therapy has similar efficacy in the treatment of parkinsonism and a lower incidence of motor complications compared to levodopa therapy several years after the initiation of the therapy. The m...

journal_title：Journal of neurology

authors： Kondo T

更新日期：2002-09-01 00:00:00

abstract：:Progressive multifocal leukoencephalopathy (PML) is a subacute demyelinating disease of the brain caused by the JC virus that occurs mainly in immunocompromised patients. The prognosis is very poor. As the lesion looks like non- specific leukoencephalopathy, making a diagnosis at the early stage is very difficult. We ...

journal_title：Journal of neurology

authors： Shirai S,Yabe I,Kano T,Shimizu Y,Sasamori T,Sato K,Hirotani M,Nonaka T,Takahashi I,Matsushima M,Minami N,Nakamichi K,Saijo M,Hatanaka KC,Shiga T,Tanaka S,Sasaki H

更新日期：2014-12-01 00:00:00

abstract：:A 37-year-old man presented with an acute amnestic syndrome of Korsakoff's type and an upper brain-stem oculomotor syndrome. After a moderate improvement with steroid therapy, he developed progressive behavioural changes due to a frontal lobe syndrome, in addition to motor and visual impairment. Memory performance was...

journal_title：Journal of neurology

authors： Vighetto A,Charles N,Salzmann M,Confavreux C,Aimard G

更新日期：1991-09-01 00:00:00

abstract：:In contrast to other neurodegenerative diseases, sporadic Creutzfeldt-Jakob disease (sCJD) is rarely diagnosed in patients older than 75 years. Data describing the characteristics of sCJD in the very old are rare and inconclusive. Therefore, a historical cohort study was designed to evaluate clinical, cerebrospinal fl...

journal_title：Journal of neurology

authors： Karch A,Raddatz LM,Ponto C,Hermann P,Summers D,Zerr I

更新日期：2014-05-01 00:00:00

abstract：:Movement disorder emergencies include any movement disorder which evolves over hours to days, in which failure to appropriately diagnose and manage can result in patient morbidity or mortality. It is crucial that doctors recognize these emergencies with accuracy and speed by obtaining the proper history and by being f...

journal_title：Journal of neurology

authors： Poston KL,Frucht SJ

更新日期：2008-08-01 00:00:00

abstract：:In this review, we describe the wide clinical spectrum of features that can be seen in multiple system atrophy (MSA) with a focus on the premotor phase and the non-motor symptoms providing an up-to-date overview of the current understanding in this fast-growing field. First, we highlight the non-motor features at dise...

journal_title：Journal of neurology

authors： Chelban V,Catereniuc D,Aftene D,Gasnas A,Vichayanrat E,Iodice V,Groppa S,Houlden H

更新日期：2020-09-01 00:00:00

abstract：:Optic neuritis usually presents with rapid and gradual loss of vision that is either complete or incomplete, and typically associated with retro-orbital pain. To our knowledge there have been no documented reports of optic neuritis presenting with multiple episodes of amaurosis fugax, the sudden and transient loss of ...

journal_title：Journal of neurology

authors： Awad AM,Estephan B,Warnack W,Stüve O

更新日期：2009-12-01 00:00:00

abstract：:Silver syndrome/SPG17 is a motor manifestation of mutations in the BSCL2 gene and usually presents as a complicated form of hereditary spastic paraplegia (HSP). We present clinical data, follow-up, and genetic results of seven patients with Silver syndrome/SPG17 including a family with a variable intrafamilial phenoty...

journal_title：Journal of neurology

authors： Musacchio T,Zaum AK,Üçeyler N,Sommer C,Pfeifroth N,Reiners K,Kunstmann E,Volkmann J,Rost S,Klebe S

更新日期：2017-01-01 00:00:00

abstract：:Two siblings with Lafora disease (LD) are described: one with epilepsy, myoclonus, EEG abnormalities, severe dementia and many Lafora bodies (LBs) in muscle and skin tissue; the other with myoclonus, epilepsy, EEG abnormalities and LBs in muscle and in skin tissue, without dementia. The findings suggest that the diagn...

journal_title：Journal of neurology

authors： Iannaccone S,Zucconi M,Quattrini A,Nemni R,Comola M,Taccagni L,Smirne S

更新日期：1991-07-01 00:00:00

abstract：:Global aphasia is described as a unitary syndrome which is characterized by a severe impairment of all linguistic capabilities. Speech production is extremely limited and consists of stereotyped phrases, recurring utterances or a few isolated words which are usually neologistically distorted. The patients are unable t...

journal_title：Journal of neurology

authors： Stachowiak FJ,Huber W,Kerschensteiner M,Poeck K,Weniger D

更新日期：1977-01-13 00:00:00

abstract：:The etiology of spontaneous cervical artery dissection (sCAD) is unknown. An underlying connective tissue disorder has been suggested. As a collagen disease is conceivable several genes encoding fibrillar collagens have been condsidered as candidate genes for sCAD. We analysed the COL3A1 gene in patients with spontane...

journal_title：Journal of neurology

authors： von Pein F,Välkkilä M,Schwarz R,Morcher M,Klima B,Grau A,Ala-Kokko L,Hausser I,Brandt T,Grond-Ginsbach C

更新日期：2002-07-01 00:00:00

abstract：:The pharmacokinetics of free and total valproic acid (VPA) in plasma and whole blood after oral administration during steady state was investigated in seven infants (mean age 10.7 months) receiving monotherapy. The VPA concentrations in whole blood closely followed those in plasma but at a reduced level. A positive co...

journal_title：Journal of neurology

authors： Herngren L,Lundberg B,Nergårdh A

更新日期：1991-09-01 00:00:00