نتایج جستجو برای: choreoathetosis

تعداد نتایج: 1147  

Journal: :Human mutation 2012
Julian Schubert Roberta Paravidino Felicitas Becker Andrea Berger Nerses Bebek Amedeo Bianchi Knut Brockmann Giuseppe Capovilla Bernardo Dalla Bernardina Yukio Fukuyama Georg F Hoffmann Karin Jurkat-Rott Anna-Kaisa Anttonen Gerhard Kurlemann Anna-Elina Lehesjoki Frank Lehmann-Horn Massimo Mastrangelo Ulrike Mause Stephan Müller Bernd Neubauer Burkhard Püst Dietz Rating Angela Robbiano Susanne Ruf Christopher Schroeder Andreas Seidel Nicola Specchio Ulrich Stephani Pasquale Striano Jens Teichler Dilsad Turkdogan Federico Vigevano Maurizio Viri Peter Bauer Federico Zara Holger Lerche Yvonne G Weber

Mutations in PRRT2 have been described in paroxysmal kinesigenic dyskinesia (PKD) and infantile convulsions with choreoathetosis (PKD with infantile seizures), and recently also in some families with benign familial infantile seizures (BFIS) alone. We analyzed PRRT2 in 49 families and three sporadic cases with BFIS only of Italian, German, Turkish, and Japanese origin and identified the previou...

2013
Hee Jae Huh Kyoo-ho Cho Ji Eun Lee Min-Jung Kwon Chang-Seok Ki Phil Hyu Lee

Ataxia-telangiectasia (A-T) is a rare autosomal recessive neurodegenerative disorder. It is characterized by early-onset, progressive cerebellar ataxia, oculomotor apraxia, choreoathetosis, conjunctival telangiectasias, immunodeficiency, and an increased risk of malignancy. Although A-T is known to be the most common cause of progressive cerebellar ataxia in childhood, there have been no confir...

2013
Romana Höftberger Thaís Armangue Frank Leypoldt Francesc Graus Josep Dalmau

Classic herpes simplex virus encephalitis (HSVE) is an acute viral infection that usually follows a monophasic disease course; however some patients, mainly children, experience a relapse within weeks or months after the initial event. In a subset of these patients a viral reactivation is unlikely because the CSF PCR for HSV is negative, repeated MRI does not show new necrotic lesions, and the ...

Journal: :Developmental medicine and child neurology 2012
Elegast Monbaliu Els Ortibus Jos De Cat Bernard Dan Lieve Heyrman Peter Prinzie Paul De Cock Hilde Feys

AIM The aim of this study was to examine the reliability and validity of the Dyskinesia Impairment Scale (DIS). The DIS consists of two subscales: dystonia and choreoathetosis. It measures both phenomena in dyskinetic cerebral palsy (CP). METHOD Twenty-five participants with dyskinetic CP (17 males; eight females; age range 5–22y; mean age 13y 6mo; SD 5y 4mo), recruited from special schools f...

Journal: :International Journal of Medical Reviews and Case Reports 2017

Journal: :The Journal of clinical investigation 2002
Heiko Krude Barbara Schütz Heike Biebermann Arpad von Moers Dirk Schnabel Heidi Neitzel Holger Tönnies Dagmar Weise Antony Lafferty Siegfried Schwarz Mario DeFelice Andreas von Deimling Frank van Landeghem Roberto DiLauro Annette Grüters

The occurrence of neurological symptoms and developmental delay in patients affected by congenital hypothyroidism (CH) has been attributed to the lack of thyroid hormone in the developing CNS. Accordingly, after the introduction of neonatal screening programs for CH, which allowed early and adequate treatment, an almost normal outcome for most CH patients could be achieved. However, a few patie...

2016
Dogu Aydin Finn Somnier Lisbeth H. Lassen

INTRODUCTION The occurrence of more or less monosymptomatic paraneoplastic choreoathetosis associated with anti-CRMP5/CV2 antibodies is rare. Typically, such autoantibodies are associated with a more classical syndrome - paraneoplastic encephalomyelitis. Frequently, small cell lung carcinoma (SCLC) is the related neoplastic finding. CASE REPORT We present a 71-year-old woman who developed vis...

2016
Bi-Chun Duan Wen-Chin Weng Kuang-Lin Lin Lee Chin Wong Sung-Tse Li Mei-Hsin Hsu Jainn-Jim Lin Pi-Chaun Fan Ming-I. Lin Nan-Chang Chiu Yu-Ching Lin Huei-Shyong Wang Kun-Long Hung Wang-Tso Lee

Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is one of the most common autoimmune encephalitis that presents with a wide variety of movement disorders. The purpose of our study is to review the manifestations and duration of movement disorders in different ages with NMDAR encephalitis.A retrospective cohort of 28 patients (20 females and 8 males) with positive cerebrospinal fluid (CS...

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