Quantitative muscle MRI and ultrasound for facioscapulohumeral muscular dystrophy: complementary imaging biomarkers.

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：OBJECTIVE:To quantify spinal cord atrophy and its impact on clinical disability in spinocerebellar ataxia (SCA) type 3 and 6. METHODS:Atrophy of the upper spinal cord was assessed by high resolution T1-weighted MRI of patients with SCA3 (n = 14) and SCA6 (n = 10). Furthermore, two groups of age- and sex-matched health...

journal_title：Journal of neurology

authors： Lukas C,Hahn HK,Bellenberg B,Hellwig K,Globas C,Schimrigk SK,Köster O,Schöls L

更新日期：2008-08-01 00:00:00

abstract：:Although silent infarcts (SIs) are frequent in stroke patients, their clinical significance remains controversial, and their effect on stroke outcome remains unclear. This study evaluated the prevalence of SI on computed tomography, associated factors, and the effect on outcome in stroke patients. We studied 202 conse...

journal_title：Journal of neurology

authors： Corea F,Hénon H,Pasquier F,Leys D,Lille Stroke\/Dementia Study Group.

更新日期：2001-04-01 00:00:00

abstract：:We aimed to assess the intrasubject reproducibility of a technology-based levodopa (LD) therapeutic monitoring protocol administered in supervised versus unsupervised conditions in patients with Parkinson's disease (PD). The study design was pilot, intrasubject, single center, open and prospective. Twenty patients wer...

journal_title：Journal of neurology

authors： Lopane G,Mellone S,Corzani M,Chiari L,Cortelli P,Calandra-Buonaura G,Contin M

更新日期：2018-06-01 00:00:00

abstract：:Brain metastases occur in 20-40% of patients with cancer and their frequency has increased over time. Lung, breast and skin (melanoma) are the commonest sources of brain metastases, and in up to 15% of patients the primary site remains unknown. After the introduction of MRI, multiple lesions have outnumbered single le...

journal_title：Journal of neurology

authors： Soffietti R,Rudā R,Mutani R

更新日期：2002-10-01 00:00:00

abstract：:A total of 15 patients affected by idiopathic dystonia (7 with generalized and 8 with focal or segmental dystonia) were subjected to therapy with bromocriptine at low doses, pimozide and trihexyphenidyl. The symptoms were evaluated by giving a progressive score in relation to the intensity of the dystonic symptom to e...

journal_title：Journal of neurology

authors： Girotti F,Scigliano G,Nardocci N,Angelini L,Broggi G,Giovannini P,Caraceni T

更新日期：1982-01-01 00:00:00

abstract：BACKGROUND:Classifying and diagnosing peripheral vestibular disorders based on their symptoms is challenging due to possible symptom overlap or atypical clinical presentation. To improve the diagnostic trajectory, gadolinium-based contrast-enhanced magnetic resonance imaging of the inner ear is nowadays frequently used...

journal_title：Journal of neurology

authors： van der Lubbe MFJA,Vaidyanathan A,Van Rompaey V,Postma AA,Bruintjes TD,Kimenai DM,Lambin P,van Hoof M,van de Berg R

更新日期：2020-12-01 00:00:00

abstract：:Relapses are a characteristic clinical feature of multiple sclerosis (MS), but an appreciation of factors that cause them remains elusive. In this study, we have examined seasonal variation of relapse in a large population-based MS cohort and correlated observed patterns with age, sex, disease course, and climatic fac...

journal_title：Journal of neurology

authors： Harding K,Tilling K,MacIver C,Willis M,Joseph F,Ingram G,Hirst C,Wardle M,Pickersgill T,Ben-Shlomo Y,Robertson N

更新日期：2017-06-01 00:00:00

abstract：:This study of frontotemporal dementia (FTD) was carried out to determine whether MR spectroscopy can provide an in vivo marker for the neuronal loss and gliosis that occur in this condition. We compared spectra in frontal and temporal regions known to be affected early in the course of the disease with spectra in the ...

journal_title：Journal of neurology

authors： Coulthard E,Firbank M,English P,Welch J,Birchall D,O'Brien J,Griffiths TD

更新日期：2006-07-01 00:00:00

abstract：BACKGROUND:Since the detection of autoantibodies against neuronal surface antigens, autoimmune encephalitis (AE) has been more frequently diagnosed, especially in patients with symptoms typical of limbic encephalitis, such as seizures, short-term memory deficits, or psychosis. However, the clinical spectrum of AE may b...

journal_title：Journal of neurology

authors： Baumgartner A,Rauer S,Hottenrott T,Leypoldt F,Ufer F,Hegen H,Prüss H,Lewerenz J,Deisenhammer F,Stich O

更新日期：2019-01-01 00:00:00

abstract：:Headache attributed to ingestion of a cold stimulus (ICHD-3 beta 4.5.1) is also known as ice cream headache (ICH). This cross-sectional epidemiological study included 283 students (10-14-year-olds) attending a grammar school in Germany, their parents (n = 401), and 41 teachers. A self-administered questionnaire was us...

journal_title：Journal of neurology

authors： Zierz AM,Mehl T,Kraya T,Wienke A,Zierz S

更新日期：2016-06-01 00:00:00

abstract：OBJECTIVE:Widespread metabolic changes are seen in neurodegenerative disease and could be used as biomarkers for diagnosis and disease monitoring. They may also reveal disease mechanisms that could be a target for therapy. In this study we looked for blood-based biomarkers in syndromes associated with frontotemporal lo...

journal_title：Journal of neurology

authors： Murley AG,Jones PS,Coyle Gilchrist I,Bowns L,Wiggins J,Tsvetanov KA,Rowe JB

更新日期：2020-08-01 00:00:00

abstract：:The three main immunoglobulin classes obey the basic principles of passive protein transfer at the blood-CSF barrier and the serum-derived portions could therefore be quantified with the help of the permeability marker albumin. The Ig fractions secreted into the CSF by sessile plasma cell clones have been determined i...

journal_title：Journal of neurology

authors： Felgenhauer K

更新日期：1982-01-01 00:00:00

abstract：:Three cases of Fahr's syndrome are described. All patients had disturbances of calcium metabolism and had had a meningoencephalitis in childhood. It is suggested that gliovascular changes, induced by cerebral inflammation, can later facilitate the occurrence of calcification of the striopallidodentate system when abno...

journal_title：Journal of neurology

authors： Morgante L,Vita G,Meduri M,Di Rosa AE,Galatioto S,Coraci MA,Di Perri R

更新日期：1986-02-01 00:00:00

abstract：OBJECTIVE:Amyotrophic lateral sclerosis type 8 (ALS8) is a familial form of motor neuron disease, with predominance of lower motor neuron degeneration, and is caused by mutation of the vesicle-associated membrane protein-associated protein B (VAPB). We aimed to compare the cognitive profile of patients with ALS8 and he...

journal_title：Journal of neurology

authors： de Alcântara C,Cruzeiro MM,França MC Jr,Camargos ST,de Souza LC

更新日期：2019-08-01 00:00:00

abstract：:Post-concussion symptoms (PCS) (such as headaches, irritability, anxiety, dizziness, fatigue and impaired concentration) are frequently experienced by patients who have sustained a minor head injury (MHI). The post-concussion syndrome has been defined as a clinical state where 3 or more symptoms persist for more than ...

journal_title：Journal of neurology

authors： Ingebrigtsen T,Waterloo K,Marup-Jensen S,Attner E,Romner B

更新日期：1998-09-01 00:00:00

abstract：:In Huntington's disease (HD) atrophy of the caudate nucleus and putamen has been described many years before clinical manifestation. Volume changes of the pallidum, thalamus, brainstem, accumbens nucleus, hippocampus, and amygdala are less well investigated, or reported with contradicting results. The aim of our study...

journal_title：Journal of neurology

authors： van den Bogaard SJ,Dumas EM,Acharya TP,Johnson H,Langbehn DR,Scahill RI,Tabrizi SJ,van Buchem MA,van der Grond J,Roos RA,TRACK-HD Investigator Group.

更新日期：2011-03-01 00:00:00

abstract：BACKGROUND:The symptomatic treatment of myotonia and myalgia in patients with dystrophic and non-dystrophic myotonias is often not satisfactory. Some patients anecdotally report symptoms' relief through consumption of cannabis. METHODS:A combination of cannabidiol and tetrahydrocannabinol (CBD/THC) was prescribed as c...

journal_title：Journal of neurology

authors： Montagnese F,Stahl K,Wenninger S,Schoser B

更新日期：2020-02-01 00:00:00

abstract：:Moderate unilateral cerebral ischemia was produced by microembolism in 24 adult cats. Two million plastic microspheres with a diameter of 15 +/- 5 microns were injected into the left common carotid artery via the lingual artery. The physiological and metabolic responses to embolism were accessed by electrocorticograp...

journal_title：Journal of neurology

authors： Sugi T,Schuier FJ,Hossmann KA,Zülch KJ

更新日期：1980-01-01 00:00:00

abstract：:The MS-ID (Multiple Sclerosis Information Dividend) project was initiated by the European Multiple Sclerosis Platform (EMSP) in 2007 in order to identify and address major inequalities of MS treatment and care and thus eliminate disparities across the EU. One major approach to reach these goals in the longer term is t...

journal_title：Journal of neurology

authors： Flachenecker P,Khil L,Bergmann S,Kowalewski M,Pascu I,Pérez-Miralles F,Sastre-Garriga J,Zwingers T

更新日期：2010-10-01 00:00:00

abstract：:Presymptomatic studies in ALS have consistently captured considerable disease burden long before symptom manifestation and contributed important academic insights. With the emergence of genotype-specific therapies, however, there is a pressing need to address practical objectives such as the estimation of age of sympt...

journal_title：Journal of neurology

authors： Chipika RH,Siah WF,McKenna MC,Li Hi Shing S,Hardiman O,Bede P

更新日期：2020-10-31 00:00:00

abstract：:Electrophysiological findings in 40 cases of non-progressive myopathies are reported, and compared with a group of 20 cases of Duchenne progressive muscular dystrophy and a control group. In all cases the electrophysiological changes were of the mild s. c. myogenic type. The involvement of proximal and distal muscles ...

journal_title：Journal of neurology

authors： Hausmanowa-Petrusewicz I,Ryniewicz B

更新日期：1976-02-13 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：:A myasthenia gravis (MG) patient who seems to have recovered can later have recurrence of myasthenic signs. Clearly clinical remission does not always correspond to the normalization of all the factors involved in the pathogenesis of the disease. In ten patients who had apparently recovered from MG, electromyographic ...

journal_title：Journal of neurology

authors： Scoppetta C,Bartoccioni E,David P,Flamini G,Lo Monaco M,Scuderi F,Tonali P

更新日期：1982-01-01 00:00:00

abstract：:An autopsy case of Creutzfeld-Jacob disease with widespread amyloid plaques is reported. A 45-year-old Japanese man, whose father had died of a similar disease, had a 5-year illness characterized by progressive cerebellar signs. Mental changes and brain-stem signs developed in the late stage. Myoclonus frequently occu...

journal_title：Journal of neurology

authors： Yagishita S,Iwabuchi K,Amano N,Yokoi S

更新日期：1989-03-01 00:00:00

abstract：:Cortical tubers are very common in tuberous sclerosis complex (TSC) and widely vary in size, appearance and location. The relationship between tuber features and clinical phenotype is unclear. The aim of the study is to propose a classification of tuber types along a spectrum of severity, using magnetic resonance imag...

journal_title：Journal of neurology

authors： Gallagher A,Grant EP,Madan N,Jarrett DY,Lyczkowski DA,Thiele EA

更新日期：2010-08-01 00:00:00

abstract：:Oculomotor dysfunction in Parkinson's disease (PD) is mainly characterized by a fragmentation of memory-guided gaze shifts (target is reached by several hypometric saccades). Since this phenomenon can also be observed in normal subjects, we scrutinized its pathophysiological significance in PD patients. We recorded ho...

journal_title：Journal of neurology

authors： Kimmig H,Haussmann K,Mergner T,Lücking CH

更新日期：2002-06-01 00:00:00

abstract：:Cerebellar ataxias (CAs) represent a heterogeneous group of sporadic or inherited disorders. The clinical spectrum of CAs is continuously expanding. Our understanding of the mechanisms leading to the clinical deficits has improved over these last decades, in particular thanks to progress in genetics, neuroimaging and ...

journal_title：Journal of neurology

authors： Gandini J,Manto M,Bremova-Ertl T,Feil K,Strupp M

更新日期：2020-04-01 00:00:00

abstract：:Multiple system atrophy (MSA) manifests as a combination of dysautonomia and motor symptoms/signs. However, rare cases presenting with autonomic failures in absence of motor symptoms/signs until their deaths have been reported and are referred to as non-motor MSA. To clarify pathological findings underlying non-motor ...

journal_title：Journal of neurology

authors： Riku Y,Watanabe H,Mimuro M,Iwasaki Y,Ito M,Katsuno M,Sobue G,Yoshida M

更新日期：2017-11-01 00:00:00

abstract：:Two siblings with multiple intracranial haemangiomatosis are reported, each having psychomotor retardation, epilepsy and gyriform type calcification in the occipital and frontal lobes. There were haemangiomas in one autopsy case with calcification in the deep layers of the cortex and subcortical white matter. ...

journal_title：Journal of neurology

authors： Tateno A,Matsui A,Sakuragawa N,Nonaka I,Arima M

更新日期：1985-01-01 00:00:00