Neuropathology in juvenile Huntington disease
نویسندگان
چکیده
منابع مشابه
A comparison of huntington disease and huntington disease-like 2 neuropathology.
Huntington disease-like 2 (HDL2) is an autosomal dominant disorder characterized by adult-onset, progressive motor abnormalities, psychiatric disturbances, and dementia ending in premature death. Clinically, it most closely resembles Huntington disease (HD), although a subset of affected individuals have parkinsonian features. Here, we systematically compare 5 HDL2 and 5 HD brains with the hypo...
متن کاملJuvenile Huntington disease: CT and MR features.
PURPOSE To describe the clinical and radiologic manifestations of juvenile Huntington disease and to determine whether adult imaging criteria for Huntington disease are helpful for pediatric patients. METHODS Six patients (3 to 18 years of age; mean age, 9.8 +/- 5.6 years; 3 female, 3 male) with juvenile Huntington disease were studied with CT (n = 6) and/or MR (n = 3). CT and MR studies were...
متن کاملClinical presentation of juvenile Huntington disease.
OBJECTIVE To describe the clinical presentation a group of patients with juvenile onset of Huntington disease. METHOD All patients were interviewed following a structured clinical questionnaire. Patients were genotyped for the trinucleotide cytosine-adenine-guanine (CAG) repeat in the Huntington Disease gene. High resolution brain MRI was performed in all patients. RESULTS We identified 4 p...
متن کاملCholinergic neuropathology in a mouse model of Alzheimer's disease
Transgenic mice over-expressing mutant human amyloid precursor protein (PDAPP mouse) develop several Alzheimer’s disease (AD)-like lesions including an age-related accumulation of amyloid-?-containing neuritic plaques. Although aged, heterozygous PDAPP mice also exhibit synaptic and glial cell changes, that is characteristic of AD pathology, no evidence of neurodegeneration has been observed. T...
متن کاملCholinergic neuropathology in a mouse model of Alzheimer's disease
Transgenic mice over-expressing mutant human amyloid precursor protein (PDAPP mouse) develop several Alzheimer’s disease (AD)-like lesions including an age-related accumulation of amyloid-?-containing neuritic plaques. Although aged, heterozygous PDAPP mice also exhibit synaptic and glial cell changes, that is characteristic of AD pathology, no evidence of neurodegeneration has been observed. T...
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ژورنال
عنوان ژورنال: The FASEB Journal
سال: 2007
ISSN: 0892-6638,1530-6860
DOI: 10.1096/fasebj.21.5.a23