10,285 results match your criteria Journal of neurology[Journal]


Exploring hyperhidrosis and related thermoregulatory symptoms as a possible clinical identifier for the dysautonomic subtype of Parkinson's disease.

J Neurol 2019 Apr 17. Epub 2019 Apr 17.

King's College London, Institute of Psychiatry, Psychology and Neuroscience, Department of Basic and Clinical Neurosciences, De Crespigny Park, London, SE5 8AF, UK.

Objective: To identify associated (non-)motor profiles of Parkinson's disease (PD) patients with hyperhidrosis as a dominant problem.

Methods: This is a cross-sectional, exploratory, analysis of participants enrolled in the Non-motor Longitudinal International Study (NILS; UKCRN No: 10084) at the Parkinson's Centre at King's College Hospital (London, UK). Hyperhidrosis scores (yes/no) on question 28 of the Non-Motor Symptom Questionnaire were used to classify patients with normal sweat function (n = 172) and excessive sweating (n = 56) (Analysis 1; n = 228). Read More

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http://dx.doi.org/10.1007/s00415-019-09325-wDOI Listing

Is Parkinson's disease a chronic low-grade inflammatory bowel disease?

J Neurol 2019 Apr 12. Epub 2019 Apr 12.

Inserm, U1235, 1 rue Gaston Veil, 44035, Nantes, France.

While the pathogenesis of Parkinson's disease is not fully understood, there is increasing evidence that inflammatory responses in the brain are implicated in both disease initiation and progression. The inflammatory process in Parkinson's disease is, however, not limited to the brain but also involves the gastrointestinal tract. High amounts of cytokines and inflammatory markers are found in the colon of Parkinson's disease patients and there is now strong epidemiological and genetical evidence linking Parkinson's disease to inflammatory bowel diseases. Read More

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http://dx.doi.org/10.1007/s00415-019-09321-0DOI Listing
April 2019
2 Reads

Hereditary neuropathy with liability to pressure palsies.

J Neurol 2019 Apr 15. Epub 2019 Apr 15.

Unit of Rare Neurodegenerative and Neurometabolic Diseases, Department of Clinical Neurosciences, Fondazione IRCCS Istituto Neurologico Carlo Besta, Milan, Italy.

Hereditary neuropathy with liability to pressure palsies (HNPP) is characterized by recurrent sensory and motor neuropathy in individual nerves starting in adolescence or young adulthood, focal conduction abnormalities at entrapment sites on nerve conduction studies, and sausage-like swellings (tomacula) of the myelin sheaths by nerve biopsy. It is characterized genetically by the deletion of the chromosome 17p11.2-p12 region including the peripheral myelin protein-22 gene in the overwhelming majority of cases. Read More

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http://dx.doi.org/10.1007/s00415-019-09319-8DOI Listing

Motor, cognitive and behavioral differences in MDS PSP phenotypes.

J Neurol 2019 Apr 15. Epub 2019 Apr 15.

Department of Medicine, Surgery and Dentistry, Neuroscience Section, Center for Neurodegenerative Diseases (CEMAND), University of Salerno, 84131, Baronissi (Salerno), Italy.

Introduction: Movement Disorder Society (MDS) new diagnostic criteria for Progressive Supranuclear palsy (PSP) identifying different disease phenotypes were recently released. The aim of the present study is to report on the cognitive and behavioral features of the different phenotypes diagnosed according to the MDS criteria.

Methods: Forty-nine PSP patients underwent an extensive battery of clinical assessments. Read More

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http://link.springer.com/10.1007/s00415-019-09324-x
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http://dx.doi.org/10.1007/s00415-019-09324-xDOI Listing
April 2019
1 Read

Prevalence and risk factors of stroke in the elderly in Northern China: data from the National Stroke Screening Survey.

J Neurol 2019 Apr 15. Epub 2019 Apr 15.

Department of Neurology, The Second Hospital of Tianjin Medical University, Tianjin, 300211, China.

Background: The overall global burden of stroke is considerable and increasing. In China, stroke is the leading cause of death and disability.

Methods: For this study, we used data from the National Stroke Screening Survey in 2012 and the 2010 Chinese population from sixth National Census of Populations to calculate a standardized (by age, gender, and education) stroke prevalence. Read More

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http://dx.doi.org/10.1007/s00415-019-09281-5DOI Listing

Vernon B. Mountcastle (1918-2015).

Authors:
Marco Cambiaghi

J Neurol 2019 Apr 15. Epub 2019 Apr 15.

The Italian Academy for Advanced Studies in America, Columbia University, New York, NY, USA.

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http://dx.doi.org/10.1007/s00415-019-09322-zDOI Listing

Occurrence of cerebral small vessel disease at diagnosis of MPO-ANCA-associated vasculitis.

J Neurol 2019 Apr 13. Epub 2019 Apr 13.

Division of Neurology, Department of Internal Medicine IV, Osaka Medical College, 2-7 Daigaku-machi, Takatsuki, Osaka, 569-8686, Japan.

Background: Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) often causes peripheral nervous system impairments. However, little is known about subclinical involvements of the central nervous system in AAV. We investigated the frequency and progression of cerebral small vessel disease (SVD) in patients with AAV. Read More

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http://dx.doi.org/10.1007/s00415-019-09318-9DOI Listing

Retraction Note to: Juvenile-onset myasthenia gravis: autoantibody status, clinical characteristics and genetic polymorphisms.

J Neurol 2019 Apr 11. Epub 2019 Apr 11.

Department of Clinical Medicine, University of Bergen, Bergen, Norway.

The Joint Editors-in-Chief have retracted this article [1] at the request of the University of Bergen and the Norwegian Board of Health Supervision. Read More

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http://dx.doi.org/10.1007/s00415-019-09292-2DOI Listing

Balanced sex distribution in patients with Menière's disease.

J Neurol 2019 Apr 10. Epub 2019 Apr 10.

Department of Neurology and DSGZ, Ludwig-Maximilians-Universität München, Campus Großhadern, Marchioninistr. 15, 81377, Munich, Germany.

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http://dx.doi.org/10.1007/s00415-019-09301-4DOI Listing
April 2019
1 Read

Musical hallucinations and their relation with epilepsy.

J Neurol 2019 Apr 10. Epub 2019 Apr 10.

Parnassia Psychiatric Institute, The Hague, The Netherlands.

Musical hallucinations are poorly understood phenomena. Their relation with epilepsy was first described over a century ago, but never systematically explored. We, therefore, reviewed the literature, and assessed all descriptions of musical hallucinations attributed to epileptic activity. Read More

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http://dx.doi.org/10.1007/s00415-019-09289-xDOI Listing

Association between sleep disturbance in Alzheimer's disease patients and burden on and health status of their caregivers.

J Neurol 2019 Apr 9. Epub 2019 Apr 9.

Medical Affairs, MSD K.K., Kitanomaru Square, 1-13-12 Kudan-kita, Chiyoda-ku, Tokyo, 102-8667, Japan.

Background: Sleep disturbance in Alzheimer's disease (AD) patients may have a negative impact not only on patients themselves but also on the physical and mental health of their caregivers. Detailed analysis of these issues is lacking.

Objective: This study investigated the association between sleep disturbance in AD patients and the burden on, and health status of, their caregivers in Japan. Read More

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http://dx.doi.org/10.1007/s00415-019-09286-0DOI Listing
April 2019
1 Read

Correction to: A cluster of disseminated small cortical lesions in MELAS: its distinctive clinical and neuroimaging features.

J Neurol 2019 Apr 9. Epub 2019 Apr 9.

Department of Neurology, Kitasato University School of Medicine, 1-15-1 Kitasato, Minami-ku, Sagamihara, Kanagawa, 252-0374, Japan.

The author would like to correct the errors in the publication of the original article. The corrected details are given below for your reading. Read More

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http://dx.doi.org/10.1007/s00415-019-09314-zDOI Listing

Risk of traffic accidents after onset of vestibular disease assessed with a surrogate marker.

J Neurol 2019 Apr 9. Epub 2019 Apr 9.

Division of Epidemiology, Institute of Social Paediatrics and Adolescents Medicine, Ludwig-Maximilians-Universität München, Haydnstr. 5, 81377, Munich, Germany.

Objectives: To determine if the risk of traffic accidents increases after disease onset in patients with acute vestibular disorders. That could provide a valid rationale for guidelines on driving restrictions.

Methods: 5,260,054 patient data (> 18 years of age) from a statutory health insurer were used to identify traffic injuries in incident cases of Menière's disease (MD) and vestibular neuritis (VN) in 2010-2013. Read More

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http://link.springer.com/10.1007/s00415-019-09300-5
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http://dx.doi.org/10.1007/s00415-019-09300-5DOI Listing
April 2019
1 Read

A novel COL6A2 mutation causing late-onset limb-girdle muscular dystrophy.

J Neurol 2019 Apr 8. Epub 2019 Apr 8.

Neuromuscular Research Center, Department of Neurology, Tampere University and University Hospital, Tampere, Finland.

Limb-girdle muscular dystrophies (LGMD) are genetic disorders characterized by weakness of predominantly proximal limb and trunk muscles due to progressive loss of muscle tissue. Collagen VI-related muscular dystrophies usually display more generalized muscle involvement combined with contractures and/or hyperlaxity of distal finger joints. LGMD-like phenotype of collagenopathy has only rarely been described and as reported is usually of childhood onset. Read More

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http://dx.doi.org/10.1007/s00415-019-09307-yDOI Listing
April 2019
1 Read
3.377 Impact Factor

Amyloid PETs are commonly negative in suspected Alzheimer's disease with an increase in CSF phosphorylated-tau protein concentration but an Aβ42 concentration in the very high range: a prospective study.

J Neurol 2019 Apr 8. Epub 2019 Apr 8.

Department of Nuclear Medicine and Nancyclotep Imaging Platform, Université de Lorraine, CHRU-Nancy, 54000, Nancy, France.

Background: Atypical cerebrospinal fluid (CSF) patterns, involving an increase in the concentration of phosphorylated-tau (P-tau) proteins but normal amyloid-β concentration, are not uncommon in patients with mild neurocognitive disorders and suspected Alzheimer's disease (AD). In these conditions, however, AD diagnosis may be ruled out in the absence of any amyloid deposition at positron-emission tomography (PET). This pilot cross-sectional study was aimed to determine whether this negativity of amyloid PET can be predicted by CSF profiles in such patients. Read More

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http://dx.doi.org/10.1007/s00415-019-09315-yDOI Listing
April 2019
4 Reads

Diagnosis strategy and Yacovino maneuver for anterior canal-benign paroxysmal positional vertigo.

J Neurol 2019 Apr 8. Epub 2019 Apr 8.

Department of Neurology, College of Medicine, Seoul National University, Seoul National University Bundang Hospital, 300 Gumi-dong, Bundang-gu, Seongnam, Gyeonggi-do, 463-707, South Korea.

Objective: To investigate the diagnosis strategy of anterior canal-benign paroxysmal positional vertigo (AC-BPPV) and the therapeutic effects of the Yacovino maneuver.

Methods: The clinical data of 40 AC-BPPV patients were collected. The nystagmus characteristics induced by the Dix-Hallpike (D-H) and straight head-hanging (SHH) tests, the diagnostic methods used, and the effectiveness of the Yacovino maneuver for the treatment of AC-BPPV were all retrospectively analyzed. Read More

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http://dx.doi.org/10.1007/s00415-019-09312-1DOI Listing

15-White Dots APP-Coo-Test: a reliable touch-screen application for assessing upper limb movement impairment in patients with cerebellar ataxias.

J Neurol 2019 Apr 6. Epub 2019 Apr 6.

Department of Medical and Surgical Sciences and Biotechnologies (DSBMC), Polo Pontino-University of Rome "Sapienza", Via Faggiana 34, 40100, Latina, Italy.

Background: The use of objective measurements is essential to assess disease progression and to evaluate the effectiveness of rehabilitation protocols and clinical treatments.

Aim: The purpose of this study was to develop a touch-screen application, that we named 15-White Dots APP-Coo-Test (15-WDACT), able to carry out quantitative and objective measurements of the rapid and coordinated upper limb movements, typically impaired in patients with cerebellar ataxias (CA).

Methods: A total of 87 CA patients and 170 healthy subjects participated in this study. Read More

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http://dx.doi.org/10.1007/s00415-019-09299-9DOI Listing
April 2019
2 Reads

Antecedent infections in Fisher syndrome: sources of variation in clinical characteristics.

J Neurol 2019 Apr 6. Epub 2019 Apr 6.

Department of Neurology and Clinical Neuroscience, Yamaguchi University Graduate School of Medicine, Minami-kogushi 1-1-1, Ube, Yamaguchi, 755-8505, Japan.

The clinical features of Guillain-Barré syndrome (GBS) are highly variable, according to the type of antecedent infection. Although a major GBS phenotype, Fisher syndrome (FS), has been shown to be preceded by infections similar to those preceding GBS, whether or not the clinical features in FS also vary according to antecedent infection remains unclarified. Frequent antecedent infections among this study of 70 FS patients included Haemophilus influenzae [n = 15 (21%)], Campylobacter jejuni [n = 10 (14%)], and cytomegalovirus (CMV) [n = 6 (8. Read More

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http://dx.doi.org/10.1007/s00415-019-09308-xDOI Listing
April 2019
1 Read

Parkinsonian traits in amyotrophic lateral sclerosis (ALS): a prospective population-based study.

J Neurol 2019 Apr 4. Epub 2019 Apr 4.

Department of Neuroscience "Rita Levi Montalcini", Movement Disorders Center, University of Turin, Turin, Italy.

Background: Amyotrophic lateral sclerosis (ALS) is characterized by a spectrum of phenotypes, but only a few studies have addressed the presence of parkinsonian (PK) symptoms. The aim of our study was to investigate the occurrence of PK features in a prospective population-based cohort of ALS patients, determining their demographic, clinical, neuropsychological and genetic characteristics, and identifying their morphological and functional imaging correlates.

Methods: A consecutive series of ALS patients were enrolled and prospectively followed for 2 years. Read More

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http://link.springer.com/10.1007/s00415-019-09305-0
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http://dx.doi.org/10.1007/s00415-019-09305-0DOI Listing
April 2019
2 Reads

Livedo racemosa generalisata: an anthological vision through Vladímir Lébedev painting.

Authors:
José Berciano

J Neurol 2019 Apr 4. Epub 2019 Apr 4.

Service of Neurology, University Hospital "Marqués de Valdecilla (IDIVAL)", University of Cantabria, Santander, Spain.

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http://dx.doi.org/10.1007/s00415-019-09303-2DOI Listing

Mapping the neuroanatomy of functional decline in Alzheimer's disease from basic to advanced activities of daily living.

J Neurol 2019 Apr 4. Epub 2019 Apr 4.

School of Health Sciences, University of East Anglia, Norwich, UK.

Background: Impairments in activities of daily living (ADL) are a criterion for Alzheimer's disease (AD) dementia. However, ADL gradually decline in AD, impacting on advanced (a-ADL, complex interpersonal or social functioning), instrumental (IADL, maintaining life in community), and finally basic functions (BADL, activities related to physiological and self-maintenance needs). Information and communication technologies (ICT) have become an increasingly important aspect of daily functioning. Read More

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http://dx.doi.org/10.1007/s00415-019-09260-wDOI Listing
April 2019
2 Reads
3.377 Impact Factor

Serum and CSF neurofilament light chain levels in antibody-mediated encephalitis.

J Neurol 2019 Apr 3. Epub 2019 Apr 3.

Neurology Unit, Department of Neuroscience, Biomedicine and Movement Sciences, University of Verona, Policlinico GB Rossi, P.le LA Scuro 10, 37135, Verona, Italy.

Circulating and cerebrospinal fluid (CSF) neurofilament light chain (NfL) levels represent a reliable indicator of disease activity and axonal damage in different neuroinflammatory conditions. Recently, high CSF NfL levels have been detected in active autoimmune encephalitis, as opposed to significant lower levels after clinical improvement. The aim of the present study was to evaluate serum and CSF NfL concentration in patients with autoimmune encephalitis and to analyse the association between NfL levels and clinical, MRI, and CSF data. Read More

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http://dx.doi.org/10.1007/s00415-019-09306-zDOI Listing
April 2019
1 Read

Strength and muscle structure preserved during long-term therapy in a patient with hypokalemic periodic paralysis (Cav1.1-R1239G).

J Neurol 2019 Apr 1. Epub 2019 Apr 1.

Department of Neurophysiology, Ulm University, Albert Einstein-Allee 11, Ulm, Germany.

We report a young wheelchair-dependent patient with an unclear proximal myopathy and a heterozygous, de-novo Cav1.1-R1239G mutation suggesting hypokalemic periodic paralysis (HypoPP). Sonography showed a loss of the pennate pattern indicative of an edema, whereas fatty degeneration was excluded. Read More

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http://dx.doi.org/10.1007/s00415-019-09302-3DOI Listing

Crossed aphasia confirmed by fMRI in a case with nonfluent variant of primary progressive aphasia carrying a GRN mutation.

J Neurol 2019 May 29;266(5):1274-1279. Epub 2019 Mar 29.

Neuroimaging Research Unit, Institute of Experimental Neurology, Division of Neuroscience, San Raffaele Scientific Institute, Vita-Salute San Raffaele University, Via Olgettina, 60, 20132, Milan, Italy.

Objectives: To characterize patterns of language lateralization in a right-handed woman with nonfluent/agrammatic primary progressive aphasia (nfvPPA) clinical picture despite showing a prevalent right-sided brain damage.

Methods: We report a case of a 58-year-old woman with nfvPPA diagnosis (age at onset = 55) previously described as a crossed aphasia case with progranulin mutation. At 2 years from the first visit, patient underwent 3DT1-weighted and a task-based functional MRI (fMRI). Read More

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http://dx.doi.org/10.1007/s00415-019-09298-wDOI Listing
May 2019
1 Read

Prognostic factors in ALS: a comparison between Germany and China.

J Neurol 2019 Mar 28. Epub 2019 Mar 28.

Department of Neurology, Universitätsklinik Ulm, RKU, Oberer Eselsberg 45, 89081, Ulm, Germany.

Objective: Several independent prognostic factors, such as age of onset, type of onset, body mass index (BMI), and progression rate have been identified for amyotrophic lateral sclerosis (ALS) in Caucasians. The aim of this study was to identify such factors in Chinese patients and to compare their impact with German patients.

Methods: Comparison of prognostic factors was based on two hospital-based registries. Read More

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http://dx.doi.org/10.1007/s00415-019-09290-4DOI Listing
March 2019
1 Read

The etiology of Bell's palsy: a review.

J Neurol 2019 Mar 28. Epub 2019 Mar 28.

Shanxi Medical University School and Hospital of Stomatology, Taiyuan, 030001, China.

Bell's palsy is the most common condition involving a rapid and unilateral onset of peripheral paresis/paralysis of the seventh cranial nerve. It affects 11.5-53. Read More

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http://dx.doi.org/10.1007/s00415-019-09282-4DOI Listing
March 2019
1 Read

Cerebellar functional abnormalities in early stage drug-naïve and medicated Parkinson's disease.

J Neurol 2019 Mar 28. Epub 2019 Mar 28.

Shanghai Key Laboratory of Magnetic Resonance and Department of Physics, School of Physics and Materials Science, East China Normal University, 3663 North Zhong-Shan Road, Shanghai, 200062, People's Republic of China.

Parkinson's disease (PD) is a progressive neurological degenerative disorder characterized by impaired motor function and non-motor dysfunctions. While recent studies have highlighted the role of the cerebellum in PD, our understanding of its role in PD remains limited. In the present study, we used resting-state fMRI to evaluate dysfunctions within the cerebellum in PD patients treated with medication and drug-naïve PD patients. Read More

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http://dx.doi.org/10.1007/s00415-019-09294-0DOI Listing
March 2019
1 Read

Gaetano Rummo (1853-1917).

J Neurol 2019 Mar 28. Epub 2019 Mar 28.

School of Medicine and Surgery, University of Milano-Bicocca, via Cadore 48, 20900, Monza, Italy.

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http://dx.doi.org/10.1007/s00415-019-09291-3DOI Listing

Rituximab as induction therapy in refractory myasthenia gravis: 18 month follow-up study.

J Neurol 2019 Mar 27. Epub 2019 Mar 27.

Department of Neurology, Neurosciences Centre, All India Institute of Medical Sciences, New Delhi, 110029, India.

Background: Myasthenia gravis is an immune-mediated disorder characterized by easy fatigability and diurnal variation in skeletal muscle weakness. Aim of therapy is to prevent crisis and maintain remission. However, despite standard therapy, some remain refractory to treatment. Read More

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http://dx.doi.org/10.1007/s00415-019-09296-yDOI Listing
March 2019
9 Reads

Thrombocytopenia and declines in platelet counts: predictors of mortality and outcome after mechanical thrombectomy.

J Neurol 2019 Mar 27. Epub 2019 Mar 27.

Department of Diagnostic and Interventional Neuroradiology, Klinikum Rechts der Isar, Technical University Munich, Ismaninger Straße 22, 81675, Munich, Germany.

Background And Purpose: Acute ischemic stroke (AIS) has well-known risk factors. The role of platelets in patients treated using mechanical thrombectomy (MT) has not been studied. The aim of this study was to study if there is an association of initial thrombocytopenia (TP) and a decline of platelets counts (DPC) with the clinical outcomes, mortality and intracranial hemorrhage (ICH) rates in AIS patients treated with MT. Read More

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http://dx.doi.org/10.1007/s00415-019-09295-zDOI Listing
March 2019
1 Read

Risk factors for in-hospital and follow-up mortality after childhood arterial ischemic stroke.

J Neurol 2019 Mar 26. Epub 2019 Mar 26.

Department of Radiology, School of Medicine, Pontificia Universidad Católica de Chile, Santiago, Chile.

Objectives: To explore risk factors contributing to 30-day and long-term survival in children with a first episode of arterial ischemic stroke (AIS).

Study Design: Single center prospective observational study including 119 children aged between 30 days and 18 years, with a first episode of AIS between 2003 and 2015. Diagnosis was confirmed with magnetic resonance images. Read More

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http://dx.doi.org/10.1007/s00415-019-09293-1DOI Listing

Brain white matter changes in asymptomatic carriers of Leber's hereditary optic neuropathy.

J Neurol 2019 Mar 25. Epub 2019 Mar 25.

Department of Radiology and Tianjin Key Laboratory of Functional Imaging, Tianjin Medical University General Hospital, No. 154, Anshan Road, Heping District, Tianjin, 300052, China.

Objective: Subclinical abnormalities, including microangiopathy, swelling of nerve fibers, visual field abnormalities and visual functional impairments had been reported in Leber's hereditary optic neuropathy (LHON) carriers. The purpose of this study was to investigate microstructural changes of brain white matter in asymptomatic LHON carriers using DTI and tract-based spatial statistics (TBSS).

Methods: DTI and neuro-ophthalmologic measurements were acquired in 14 LHON carriers and 15 gender- and age-matched healthy controls, and diffusion metrics, including fractional anisotropy (FA), axial (AD), radial diffusion (RD) and mean diffusion (MD) were calculated. Read More

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http://dx.doi.org/10.1007/s00415-019-09284-2DOI Listing
March 2019
7 Reads

Chronic headache with vasospasm treated with nimodipine.

J Neurol 2019 Mar 25. Epub 2019 Mar 25.

Department of Neurology, Serviço de Neurologia, Hospital de Santa Maria, Avenida Professor Egas Moniz, 1649-035, Lisbon, Portugal.

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http://dx.doi.org/10.1007/s00415-019-09287-zDOI Listing
March 2019
3.377 Impact Factor

Triple and quadruple cervical artery dissections: a systematic review of individual patient data.

J Neurol 2019 Mar 23. Epub 2019 Mar 23.

Department of Neurology, Amsterdam UMC, Location AMC, Meibergdreef 9, 1105 AZ, Amsterdam, The Netherlands.

Background And Purpose: Simultaneous dissection of three or four cervical arteries rarely occurs. As a result, limited information is available on clinical characteristics, underlying causes, treatment, and outcome of these patients.

Methods: We performed a systematic review of individual patient data on triple and quadruple cervical artery dissection (CeAD). Read More

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http://link.springer.com/10.1007/s00415-019-09269-1
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http://dx.doi.org/10.1007/s00415-019-09269-1DOI Listing
March 2019
6 Reads

Relapse rates and long-term outcome in primary angiitis of the central nervous system.

J Neurol 2019 Mar 23. Epub 2019 Mar 23.

Department of Neurology, University Medical Center Hamburg-Eppendorf, Hamburg, Germany.

Objective: To analyze the treatment response in patients with primary angiitis of the central nervous system (PACNS).

Methods: In a single-center retrospective observational study, we assessed relapses, remission, and long-term outcome by use of the modified Rankin Scale (mRS) under different immunotherapies. Eligible patients had CNS biopsy in favor of PACNS or neuroimaging compatible with PACNS after exclusion of an alternative diagnosis. Read More

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http://dx.doi.org/10.1007/s00415-019-09285-1DOI Listing

Acute posterior multifocal placoid pigment epitheliopathy after alemtuzumab treatment for relapsing-remitting multiple sclerosis.

J Neurol 2019 Mar 20. Epub 2019 Mar 20.

Department of Clinical Neurosciences, University of Cambridge, Cambridge, UK.

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http://dx.doi.org/10.1007/s00415-019-09288-yDOI Listing
March 2019
1 Read

Screening of SLC2A1 in a large cohort of patients suspected for Glut1 deficiency syndrome: identification of novel variants and associated phenotypes.

J Neurol 2019 Mar 20. Epub 2019 Mar 20.

Department of Pediatric Neuroscience, Fondazione IRCCS Istituto Neurologico Carlo Besta, Milan, Italy.

Glucose transporter type 1 deficiency syndrome (Glut1 DS) is a rare neurological disorder caused by impaired glucose delivery to the brain. The clinical spectrum of Glut1 DS mainly includes epilepsy, paroxysmal dyskinesia (PD), developmental delay and microcephaly. Glut1 DS diagnosis is based on the identification of hypoglycorrhachia and pathogenic mutations of the SLC2A1 gene. Read More

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http://dx.doi.org/10.1007/s00415-019-09280-6DOI Listing
March 2019
1 Read
3.377 Impact Factor

A cluster of disseminated small cortical lesions in MELAS: its distinctive clinical and neuroimaging features.

J Neurol 2019 Mar 19. Epub 2019 Mar 19.

Department of Neurology, Kitasato University School of Medicine, 1-15-1 Kitasato, Minami-ku, Sagamihara, Kanagawa, 252-0374, Japan.

Objectives: To investigate a diversity of stroke-like episodes (SLEs) in mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS), and report a disseminated form of SLEs (D-SLEs) attributed to a cluster of disseminated small cortical lesions.

Methods: We retrospectively reviewed the clinical information of 27 MELAS patients seen at Kitasato University Hospital between January 1990 and April 2018. Among those, we selected 13 patients with m. Read More

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http://dx.doi.org/10.1007/s00415-019-09283-3DOI Listing
March 2019
1 Read

Thrombus hallmarks reveal atherothrombotic stroke aetiology.

J Neurol 2019 Mar 19. Epub 2019 Mar 19.

Neuroimmunology Unit, Institute of Experimental Neurology, San Raffaele Scientific Institute, Via Olgettina 60, 20132, Milan, Italy.

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http://dx.doi.org/10.1007/s00415-019-09279-zDOI Listing

Heterogeneity in costs and prognosis for acute ischemic stroke treatment by comorbidities.

J Neurol 2019 Mar 16. Epub 2019 Mar 16.

Department of Neurology, Yonsei University College of Medicine, 50-1 Yonsei-ro, Seodaemun-gu, Seoul, 03722, South Korea.

Objective: Comorbidities are prevalent among stroke patients. The current study assesses the variations in cost and stroke prognosis by concurrent comorbidities in patients with acute ischemic stroke.

Methods: The Charlson comorbidity index was used as the composite comorbidity level (0 none, 1 mild, 2 moderate, and ≥ 3 severe). Read More

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http://dx.doi.org/10.1007/s00415-019-09278-0DOI Listing
March 2019
4 Reads

Effect of levodopa on handwriting tasks of different complexity in Parkinson's disease: a kinematic study.

J Neurol 2019 Mar 15. Epub 2019 Mar 15.

Monash Medical Centre, Melbourne, Australia.

Levodopa treatment does improve Parkinson's disease (PD) dysgraphia, but previous research is not in agreement about which aspects are most responsive. This study investigated the effect of levodopa on the kinematics of writing. Twenty-four patients with PD of less than 10 years duration and 25 age-matched controls were recruited. Read More

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http://dx.doi.org/10.1007/s00415-019-09268-2DOI Listing
March 2019
1 Read
3.377 Impact Factor

Autoimmune encephalitis: frequency and prognosis.

J Neurol 2019 May;266(5):1287-1289

Division of Psychological Medicine and Clinical Neuroscience, Cardiff University, University Hospital of Wales, Heath Park, Cardiff, CF14 4XN, UK.

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http://dx.doi.org/10.1007/s00415-019-09273-5DOI Listing

Congenital myopathies are mainly associated with a mild cardiac phenotype.

J Neurol 2019 Mar 14. Epub 2019 Mar 14.

Department of Neurology, Copenhagen University Hospital, Rigshospitalet, Copenhagen, Denmark.

Background: To evaluate the prevalence of cardiac involvement in patients with congenital myopathies and the association to specific genotypes.

Methods: We evaluated patients with physical examination, electrocardiogram, echocardiography, and 48-h Holter monitoring. Follow-up was performed for major events. Read More

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http://dx.doi.org/10.1007/s00415-019-09267-3DOI Listing
March 2019
5 Reads

Prognostic significance of body weight variation after diagnosis in ALS: a single-centre prospective cohort study.

J Neurol 2019 Mar 13. Epub 2019 Mar 13.

Department of Neurology, Tokyo Metropolitan Neurological Hospital, 2-6-1 Musashidai, Fuchu, Tokyo, 183-0042, Japan.

Background: Body weight reduction after disease onset is an independent predictor of survival in amyotrophic lateral sclerosis (ALS), but significance of weight variation after diagnosis remains to be established.

Objective: To investigate weight variation after diagnosis and its prognostic significance in patients with ALS as a prospective cohort study.

Methods: Seventy-nine patients with ALS were enrolled in this study. Read More

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http://link.springer.com/10.1007/s00415-019-09276-2
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http://dx.doi.org/10.1007/s00415-019-09276-2DOI Listing
March 2019
3 Reads

Clinical presentation of Moyamoya angiopathy in Europeans: experiences from Germany with 200 patients.

J Neurol 2019 Mar 13. Epub 2019 Mar 13.

Department of Neurology, Alfried Krupp Hospital, Alfried-Krupp-Strasse 21, 45130, Essen, Germany.

Introduction: Moyamoya angiopathy (MMA) is a rare vasopathy, especially among European Caucasians. Data about demographics, clinical presentation, comorbid conditions, radiological findings as well as laboratory and cerebral spinal fluid (CSF) data are sparse.

Methods: Patients with MMA treated in the Alfried Krupp Hospital, Essen, Germany, between 2010 and 2017 with focus on demographic, clinical, radiological and laboratory as well as CSF data were evaluated retrospectively. Read More

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http://link.springer.com/10.1007/s00415-019-09277-1
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http://dx.doi.org/10.1007/s00415-019-09277-1DOI Listing
March 2019
4 Reads

Defining metronidazole-induced encephalopathy.

J Neurol 2019 May 12;266(5):1272-1273. Epub 2019 Mar 12.

Department of Medicine, GIM Section, Medical College of Wisconsin, Milwaukee, WI, USA.

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http://dx.doi.org/10.1007/s00415-019-09275-3DOI Listing
May 2019
3.377 Impact Factor

Efficacy and safety of alemtuzumab in a real-life cohort of patients with multiple sclerosis.

J Neurol 2019 Mar 12. Epub 2019 Mar 12.

Multiple Sclerosis Center, University of Cagliari/ATS Sardegna, Via Is Guadazzonis, 2, 09126, Cagliari, Italy.

Background: No postmarketing randomised clinical trials are available about alemtuzumab, and real-world data are limited. We aimed to analyse the efficacy and safety of alemtuzumab in a single-centre cohort of patients with relapsing-remitting MS.

Methods: Patients who took alemtuzumab were enrolled. Read More

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http://dx.doi.org/10.1007/s00415-019-09272-6DOI Listing
March 2019
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Distinctive speech signature in cerebellar and parkinsonian subtypes of multiple system atrophy.

J Neurol 2019 Mar 11. Epub 2019 Mar 11.

Center for Neurodegenerative Diseases (CEMAND), Department of Medicine, Surgery and Dentistry "Scuola Medica Salernitana", University of Salerno, Salerno, Italy.

Although motor speech disorders represent an early and prominent clinical feature of multiple system atrophy (MSA), the potential usefulness of speech assessment as a diagnostic tool has not yet been explored. This cross-sectional study aimed to provide a comprehensive, objective description of motor speech function in the parkinsonian (MSA-P) and cerebellar (MSA-C) variants of MSA. Speech samples were acquired from 80 participants including 18 MSA-P, 22 MSA-C, 20 Parkinson's disease (PD), and 20 healthy controls. Read More

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http://dx.doi.org/10.1007/s00415-019-09271-7DOI Listing
March 2019
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Granulomatous CNS inflammation associated with seminoma.

J Neurol 2019 Mar 11. Epub 2019 Mar 11.

Centre for Neurosarcoidosis, Neuroimmunology Unit, Institute of Immunity and Transplantation, University College London, London, NW3 2PF, UK.

Two cases in which a neurological disorder was identified pathologically to be due to a granulomatous infiltration were found after diagnosis to have an associated testicular seminoma with pathologically proven lymphatic metastasis. We present the clinical and imaging features, and pathological appearances of the lymphatic tissue and the brain. We summarise the literature to date and discuss the pathogenesis of the disorder and its treatment. Read More

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http://dx.doi.org/10.1007/s00415-019-09270-8DOI Listing
March 2019
1 Read