Search results for "Familial form"

showing 2 items of 2 documents

Accuracy of SWI sequences compared to T2*-weighted gradient echo sequences in the detection of cerebral cavernous malformations in the familial form

2016

Purpose The purpose of this study was to assess the accuracy of susceptibility-weighted imaging (SWI), compared with T2*-weighted gradient echo (GRE) imaging in assessing cerebral cavernous malformations. Materials and methods We retrospectively evaluated 21 patients with a familial form of cavernous malformation. Magnetic resonance (MR) protocol included non-enhanced and contrast-enhanced fast-spin echo (FSE) T1-weighted sequences, FSE T2-weighted sequences, fluid-attenuated inversion-recovery (FLAIR), GRE T2*-weighted and SWI sequences. Images were reviewed in consensus by two expert neuroradiologists to assess the location, number, size and conspicuity of the lesions on T2*-weighted GRE …

AdultMaleHemangioma Cavernous Central Nervous SystemCerebrovascular DiseasesStatistics as TopicSensitivity and SpecificityCerebral cavernous malformations030218 nuclear medicine & medical imaging03 medical and health sciences0302 clinical medicineNuclear magnetic resonanceImage Processing Computer-AssistedHumansMedicineRadiology Nuclear Medicine and imagingAgedRetrospective StudiesCerebral cavernous malformationFamilial formEcho-Planar Imagingbusiness.industryimagingSettore MED/37 - NeuroradiologiaT2*-weighted gradient echo sequenceGeneral MedicineMiddle AgedImage EnhancementMagnetic Resonance Imagingdiagnosisusceptibility-weighted imagingSusceptibility weighted imagingFemaleNeurology (clinical)T2 weightedbusiness030217 neurology & neurosurgeryGradient echoThe Neuroradiology Journal
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Identification of the novel D297fsX318 PINK1 mutation and phenotype variation in a family with early-onset Parkinson's disease

2008

Herein we first describe a novel homozygous single nucleotide deletion in PINK1 exon 4 (889delG) which results in a loss of kinase domain on the PINK1 protein (D297fsX318). This mutation was identified in two brothers with early-onset Parkinson disease (EOPD) from a Sicilian consanguineous family. Of note, while one of the two patients developed mental deterioration and psychiatric problems, the other showed no cognitive decline. The present study supports the view that PINK1 is a pathogenic gene in some Italian families with EOPD and contributes to define the PINK1-associated phenotype. Herein we first describe a novel homozygous single nucleotide deletion in PINK1 exon 4 (889delG) which r…

MaleParkinson's diseaseGenotypeParkinson's diseaseMolecular Sequence DataPINK1DiseaseBiologyAntiparkinson AgentsLevodopaExonmedicineHumansAmino Acid SequenceAge of OnsetCognitive declineGeneAgedGeneticsGenotype–phenotype correlationPINK1Parkinson DiseaseExonsFamilial formmedicine.diseasePhenotypePedigreeSettore BIO/18 - GeneticaPhenotypeNeurologyMutationMutation (genetic algorithm)Settore MED/26 - NeurologiaNeurology (clinical)Geriatrics and GerontologyCognition DisordersProtein KinasesGene DeletionParkinsonism & Related Disorders
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