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Hemiplegic Migraine

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https://www.readbyqxmd.com/read/28101483/intermittent-theta-slowings-in-contralateral-side-of-weakness-after-sleep-deprivation-on-spot-eeg-in-sporadic-hemiplegic-migraine
#1
Chan-Hyuk Lee, Man-Wook Seo, Byoung-Soo Shin, Tae-Ho Yang, Hyun-June Shin, Han Uk Ryu
Hemiplegic migraine (HM) is an uncommon type of migraine which is classified into sporadic and familial subtype. The noticed electroencephalogram (EEG) findings during HM attack are diffuse slowing contralateral to the weakened limb, but are usually normal in asymptomatic states. A 52-year-old woman who suffered from headache accompanying right arm weakness and aphasic symptoms admitted to our hospital. She underwent total five times of EEG including 2 times before admission. Only the last EEG exam after 24 hours of sleep deprivation (SD) showed intermittent slowing and higher amplitude of positive occipital sharp transients (POSTs) on the left parieto-occipital area...
December 2016: Journal of Epilepsy Research
https://www.readbyqxmd.com/read/28058944/epilepsy-in-hemiplegic-migraine-genetic-mutations-and-clinical-implications
#2
P Prontera, P Sarchielli, S Caproni, C Bedetti, L M Cupini, P Calabresi, C Costa
Objective We performed a systematic review on the comorbidities of familial/sporadic hemiplegic migraine (F/SHM) with seizure/epilepsy in patients with CACNA1A, ATP1A2 or SCN1A mutations, to identify the genotypes associated and investigate for the presence of mutational hot spots. Methods We performed a search in MEDLINE and in the Human Gene Mutation and Leiden Open Variation Databases for mutations in the CACNA1A, ATP1A2 and SCN1A genes. After having examined the clinical characteristics of the patients, we selected those having HM and seizures, febrile seizures or epilepsy...
January 1, 2017: Cephalalgia: An International Journal of Headache
https://www.readbyqxmd.com/read/28045726/postoperative-hemiplegic-migraine-after-a-laparoscopic-cholecystectomy-a-case-report
#3
Joseph Happel, Albin S Quiko, Huy Phun, Martin Collier, Amy Mortensen
We report the case of a 35-year-old woman who developed severe right-sided hemiplegia and hemisensory loss shortly after emergence from general anesthesia for a laparoscopic cholecystectomy. Her medical history was significant for migraine with aura and a family history of transient hemiparesis thought to be a result of a transient ischemic attack. The patient's deficits slowly resolved, and she was ultimately diagnosed with familial hemiplegic migraine after a negative workup for cerebrovascular accidents.
December 30, 2016: A & A Case Reports
https://www.readbyqxmd.com/read/28007337/missense-mutations-of-cacna1a-are-a-frequent-cause-of-autosomal-dominant-nonprogressive-congenital-ataxia
#4
Lorena Travaglini, Marta Nardella, Emanuele Bellacchio, Adele D'Amico, Alessandro Capuano, Roberto Frusciante, Matteo Di Capua, Raffaella Cusmai, Sabina Barresi, Silvia Morlino, José M Fernández-Fernández, Marina Trivisano, Nicola Specchio, Massimiliano Valeriani, Federico Vigevano, Enrico Bertini, Ginevra Zanni
BACKGROUND: Mutations in the CACNA1A gene, encoding the pore-forming CaV2.1 (P/Q-type) channel α1A subunit, localized at presynaptic terminals of brain and cerebellar neurons, result in clinically variable neurological disorders including hemiplegic migraine (HM) and episodic or progressive adult-onset ataxia (EA2, SCA6). Most recently, CACNA1A mutations have been identified in patients with nonprogressive congenital ataxia (NPCA). METHODS: We performed targeted resequencing of known genes involved in cerebellar dysfunction, in 48 patients with congenital or early onset ataxia associated with cerebellar and/or vermis atrophy...
November 30, 2016: European Journal of Paediatric Neurology: EJPN
https://www.readbyqxmd.com/read/27993427/reconsideration-of-the-diagnosis-and-treatment-of-childhood-migraine-a-practical-review-of-clinical-experiences
#5
Yoshiaki Saito, Gaku Yamanaka, Hideki Shimomura, Kazuhiro Shiraishi, Tomoyuki Nakazawa, Fumihide Kato, Yuko Shimizu-Motohashi, Masayuki Sasaki, Yoshihiro Maegaki
OBJECTIVE: To provide insight into the wide spectrum of migraine during childhood to establish practical and comprehensive treatment strategies. BACKGROUND: Although recent studies have confirmed the effect of anti-migraine agents in childhood headaches fulfilling the criteria of migraine without aura, there have been no studies regarding the efficacy of these drugs in childhood migraine without aura not filling the diagnostic criteria. METHODS: In total, 154 patients with a clinical diagnosis of migraine, with onset of repetitive headaches at the age of ⩽15years, were retrospectively included from clinics in seven tertiary medical centers...
December 16, 2016: Brain & Development
https://www.readbyqxmd.com/read/27980114/sodium-pumps-mediate-activity-dependent-changes-in-mammalian-motor-networks
#6
Laurence D Picton, Filipe Nascimento, Matthew J Broadhead, Keith T Sillar, Gareth B Miles
: Ubiquitously expressed sodium pumps are best known for maintaining the ionic gradients and resting membrane potential required for generating action potentials. However, activity- and state-dependent changes in pump activity can also influence neuronal firing and regulate rhythmic network output. Here we demonstrate that changes in sodium pump activity regulate locomotor networks in the spinal cord of neonatal mice. The sodium pump inhibitor, ouabain, increased the frequency and decreased the amplitude of drug-induced locomotor bursting, effects which were dependent on the presence of the neuromodulator dopamine...
December 15, 2016: Journal of Neuroscience: the Official Journal of the Society for Neuroscience
https://www.readbyqxmd.com/read/27919769/transcriptomic-changes-in-rat-cortex-and-brainstem-after-cortical-spreading-depression-with-or-without-pre-treatment-with-migraine-prophylactic-drugs
#7
Cèlia Sintas, Noèlia Fernàndez-Castillo, Marta Vila-Pueyo, Patricia Pozo-Rosich, Alfons Macaya, Bru Cormand
: Migraine with aura is a subtype of migraine consisting of neurological disturbances that usually precede headache. Cortical spreading depression (CSD) is the likely pathophysiological correlate of the aura phase of migraine, found in common and rare forms of migraine, such as familial hemiplegic migraine (FHM). CSD is a depolarization wave that propagates across the cerebral gray matter transiently suppressing neuronal activity. Prophylactic treatments for migraine, such as topiramate (TPM) or valproate (VPA), reduce the number of CSD events...
December 2, 2016: Journal of Pain: Official Journal of the American Pain Society
https://www.readbyqxmd.com/read/27919014/a-novel-scn1a-mutation-identified-in-a-chinese-family-with-familial-hemiplegic-migraine-a-case-report
#8
Yang Zhang, Ning Chen, Muke Zhou, Jian Guo, Jiang Guo, Li He
BACKGROUND: Familial hemiplegic migraine (FHM) is a rare type of migraine with aura that is characterized by transient hemiparesis. Mutations in three genes (CACNA1A, ATP1A2, and SCN1A) have been found to cause FHM. Among these, nine SCN1A gene mutations were reported to cause familial hemiplegic migraine type 3 (FHM3). However, none of them was reported in China. METHOD: The clinical manifestations of a Chinese FHM family were recorded and all coding exons and flanking intronic regions of the CACNA1A, ATP1A2, and SCN1A genes were tested for mutations...
November 8, 2016: Cephalalgia: An International Journal of Headache
https://www.readbyqxmd.com/read/27881154/targeted-next-generation-sequencing-identifies-novel-notch3-gene-mutations-in-cadasil-diagnostics-patients
#9
Neven Maksemous, Robert A Smith, Larisa M Haupt, Lyn R Griffiths
BACKGROUND: Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is a monogenic, hereditary, small vessel disease of the brain causing stroke and vascular dementia in adults. CADASIL has previously been shown to be caused by varying mutations in the NOTCH3 gene. The disorder is often misdiagnosed due to its significant clinical heterogeneic manifestation with familial hemiplegic migraine and several ataxia disorders as well as the location of the currently identified causative mutations...
November 24, 2016: Human Genomics
https://www.readbyqxmd.com/read/27871455/episodic-ataxia-and-sca6-within-the-same-family-due-to-the-d302n-cacna1a-gene-mutation
#10
Luca Pradotto, Monica Mencarelli, Matteo Bigoni, Alessandra Milesi, Anna Di Blasio, Alessandro Mauro
Several dominant mutations of CACNA1A gene were associated with at least three different allelic disorders: spino-cerebellar ataxia type 6 (SCA6), episodic ataxia type 2 (EA2), and familial hemiplegic migraine-1 (FHM1). It is generally thought that loss-of-function mutations are associated with EA2, gain-of-function missense mutations with FHM1, and abnormal CAG expansions with SCA6. But, overlapping features, atypical symptoms and co-occurrence of distinct phenotypes within the same family were reported. We describe a four generation family showing different phenotypes ranging from EA2 to SCA6 and carrying the p...
December 15, 2016: Journal of the Neurological Sciences
https://www.readbyqxmd.com/read/27818813/familial-hemiplegic-migraine-with-severe-attacks-a-new-report-with-atp1a2-mutation
#11
E Martínez, R Moreno, L López-Mesonero, I Vidriales, M Ruiz, A L Guerrero, J J Tellería
Introduction. Familial hemiplegic migraine (FHM) is a rare disorder characterized by migraine attacks with motor weakness during the aura phase. Mutations in CACNA1A, ATP1A2, SCN1A, and PRRT2 genes have been described. Methods. To describe a mutation in ATP1A2 gene in a FHM case with especially severe and prolonged symptomatology. Results. 22-year-old woman was admitted due to migraine-type headache and sudden onset of right-sided weakness and aphasia; she had similar episodes in her childhood. Her mother was diagnosed with hemiplegic migraine without genetic confirmation...
2016: Case Reports in Neurological Medicine
https://www.readbyqxmd.com/read/27807184/prevalence-of-lifetime-depression-in-a-large-hemiplegic-migraine-cohort
#12
Mark A Louter, Nadine Pelzer, Irene de Boer, Babette E C Kuijvenhoven, Willebrordus P J van Oosterhout, Erik W van Zwet, Michel D Ferrari, Gisela M Terwindt
OBJECTIVE: To determine the prevalence of depression and determinants associated with depression in a large population of hemiplegic migraine (HM) patients. METHODS: We conducted a cross-sectional, validated questionnaire study among 89 well-defined HM patients and 235 headache-free controls. The prevalence of lifetime depression and its relation to migraine characteristics was assessed. RESULTS: HM patients had increased odds for lifetime depression (odds ratio 3...
November 2, 2016: Neurology
https://www.readbyqxmd.com/read/27790126/hemiplegic-migraine-presenting-with-prolonged-somnolence-a-case-report
#13
Christian Saleh, Geneviève Pierquin, Stefan Beyenburg
Hemiplegic migraine is a rare and complex disease, characterized by migraine with a reversible motor aura. Hemiplegic migraine can be easily misdiagnosed at its first presentation with an atypical severe form of migraine, a stroke, multiple sclerosis, metabolic disorders, conversion disorder or an epilepsy. We present the case of a young 24-year-old male patient, who since the age of 4 years had been having multiple episodes of migraine associated with hemiparesis, paraesthesia, prolonged somnolence, aphasia and confusion...
September 2016: Case Reports in Neurology
https://www.readbyqxmd.com/read/27770403/characterising-the-premonitory-stage-of-migraine-in-children-a-clinic-based-study-of-100-patients-in-a-specialist-headache-service
#14
N Karsan, P Prabhakar, P J Goadsby
BACKGROUND: The premonitory stage of migraine attacks, when symptomatology outside of pain can manifest hours to days before the onset of the headache, is well recognised. Such symptoms have been reported in adults in a number of studies, and have value in predicting an impending headache. These symptoms have not been extensively studied in children. We aimed to characterise which, if any, of these symptoms are reported in children seen within a Specialist Headache Service. METHODS: We reviewed clinic letters from the initial consultation of children and adolescents seen within the Specialist Headache Service at Great Ormond Street Hospital between 1999 and 2015 with migraine in whom we had prospectively assessed clinical phenotype data...
December 2016: Journal of Headache and Pain
https://www.readbyqxmd.com/read/27734045/metabolomic-changes-in-csf-of-migraine-patients-measured-with-1-h-nmr-spectroscopy
#15
Ronald Zielman, Rudmer Postma, Aswin Verhoeven, Floor Bakels, Willebrordus P J van Oosterhout, Axel Meissner, Arn M J M van den Maagdenberg, Gisela M Terwindt, Oleg A Mayboroda, Michel D Ferrari
BACKGROUND: Migraine is a common episodic brain disorder. Treatment options and diagnosis are hampered by an incomplete understanding of disease pathophysiology and the lack of objective diagnostic markers. The aim of this study was to identify biochemical differences characteristic for different subtypes of migraine in cerebrospinal fluid (CSF) of migraine patients using an exploratory (1)H-NMR-based metabolomics approach. METHODS: CSF was obtained, in between migraine attacks, via lumbar puncture from patients with hemiplegic migraine, migraine with aura, migraine without aura, and healthy controls...
October 13, 2016: Molecular BioSystems
https://www.readbyqxmd.com/read/27651281/combined-early-treatment-in-hemiplegic-attacks-related-to-cacna1a-encephalopathy-with-brain-oedema-blocking-the-cascade
#16
Francesca Camia, Livia Pisciotta, Giovanni Morana, Maria Cristina Schiaffino, Salvatore Renna, Paola Carrera, Maurizio Ferrari, Maria Giuseppina Baglietto, Edvige Veneselli, Laura Siri, Maria Margherita Mancardi
INTRODUCTION: Variants in the CACNA1A gene on chromosome 19p13 result in a spectrum of neurological phenotypes ranging from familial or sporadic hemiplegic migraine to congenital or progressive encephalopathies. Patients with CACNA1A variants often show acute attacks with ataxia or hemiplegia till coma, sometimes related to unilateral brain oedema. No guidelines for the medical management of these attacks are available since treatment is empiric, and many cases do not respond to common antimigraine drugs...
September 19, 2016: Cephalalgia: An International Journal of Headache
https://www.readbyqxmd.com/read/27445835/atp1a2-mutations-in-migraine-seeing-through-the-facets-of-an-ion-pump-onto-the-neurobiology-of-disease
#17
REVIEW
Thomas Friedrich, Neslihan N Tavraz, Cornelia Junghans
Mutations in four genes have been identified in familial hemiplegic migraine (FHM), from which CACNA1A (FHM type 1) and SCN1A (FHM type 3) code for neuronal voltage-gated calcium or sodium channels, respectively, while ATP1A2 (FHM type 2) encodes the α2 isoform of the Na(+),K(+)-ATPase's catalytic subunit, thus classifying FHM primarily as an ion channel/ion transporter pathology. FHM type 4 is attributed to mutations in the PRRT2 gene, which encodes a proline-rich transmembrane protein of as yet unknown function...
2016: Frontiers in Physiology
https://www.readbyqxmd.com/read/27435762/-missense-mutation-r1345q-in-cacna1a-gene-causes-a-new-type-of-ataxia-with-episodic-tremor-clinical-features-genetic-analysis-and-treatment-in-a-familial-case
#18
Hai-Shan Jiang, Dong-Mei Wang, Qun Wang, Man Yang, Wei Wang, Su-Yue Pan, Ya-Fang Hu
OBJECTIVE: Mutations in CACNA1A, which encodes the P/Q-type calcium channel subunit, are responsible for at least 3 allelic diseases, namely type 2 episodic ataxia (EA-2), familial hemiplegic migraine?type-1 (FHM1), and spinocerebellar ataxia type-6?(SCA 6). Herein we present a case of ataxia with episodic tremors in a 19-year-old man with a missense mutation of CACNA1A gene and summarize the clinical features, genetic analysis and treatment in this case and in his affected family members...
June 20, 2016: Nan Fang Yi Ke da Xue Xue Bao, Journal of Southern Medical University
https://www.readbyqxmd.com/read/27354390/defective-glutamate-and-k-clearance-by-cortical-astrocytes-in-familial-hemiplegic-migraine-type-2
#19
Clizia Capuani, Marcello Melone, Angelita Tottene, Luca Bragina, Giovanna Crivellaro, Mirko Santello, Giorgio Casari, Fiorenzo Conti, Daniela Pietrobon
Migraine is a common disabling brain disorder. A subtype of migraine with aura (familial hemiplegic migraine type 2: FHM2) is caused by loss-of-function mutations in α2 Na(+),K(+) ATPase (α2 NKA), an isoform almost exclusively expressed in astrocytes in adult brain. Cortical spreading depression (CSD), the phenomenon that underlies migraine aura and activates migraine headache mechanisms, is facilitated in heterozygous FHM2-knockin mice with reduced expression of α2 NKA The mechanisms underlying an increased susceptibility to CSD in FHM2 are unknown...
2016: EMBO Molecular Medicine
https://www.readbyqxmd.com/read/27346147/loss-of-inhibition-by-brain-natriuretic-peptide-over-p2x3-receptors-contributes-to-enhanced-spike-firing-of-trigeminal-ganglion-neurons-in-a-mouse-model-of-familial-hemiplegic-migraine-type-1
#20
Anna Marchenkova, Arn M J M van den Maagdenberg, Andrea Nistri
Purinergic P2X3 receptors (P2X3Rs) play an important role in pain pathologies, including migraine. In trigeminal neurons, P2X3Rs are constitutively downregulated by endogenous brain natriuretic peptide (BNP). In a mouse knock-in (KI) model of familial hemiplegic migraine type-1 with upregulated calcium CaV2.1 channel function, trigeminal neurons exhibit hyperexcitability with gain-of-function of P2X3Rs and their deficient BNP-mediated inhibition. We studied whether the absent BNP-induced control over P2X3Rs activity in KI cultures may be functionally expressed in altered firing activity of KI trigeminal neurons...
September 7, 2016: Neuroscience
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