Phenotypic Variability Within the Inclusion Body Spectrum of Basophilic Inclusion Body Disease and Neuronal Intermediate Filament Inclusion Disease in Frontotemporal Lobar Degenerations With FUS-Positive Inclusions
نویسندگان
چکیده
منابع مشابه
Phenotypic variability within the inclusion body spectrum of basophilic inclusion body disease and neuronal intermediate filament inclusion disease in frontotemporal lobar degenerations with FUS-positive inclusions.
Basophilic inclusion body disease and neuronal intermediate filament inclusion disease (NIFID) are rare diseases included among frontotemporal lobar degenerations with FUS-positive inclusions (FTLD-FUS). We report clinical and pathologic features of 2 new patients and reevaluate neuropathologic characteristics of 2 previously described cases, including an early-onset case of basophilic inclusio...
متن کاملClinical and pathological heterogeneity of neuronal intermediate filament inclusion disease.
OBJECTIVE To report new cases of neuronal intermediate filament inclusion disease (NIFID). DESIGN Case report. PATIENTS Pathologically proved NIFID was found in 2 patients from the Universitat de Barcelona-Hospital Clínic Brain Bank. The findings of a neuropathological examination in both patients revealed intracellular inclusions that were detected with hematoxylin-eosin and stained positi...
متن کاملClinical and neuropathologic variation in neuronal intermediate filament inclusion disease.
BACKGROUND Recently described neuronal intermediate filament inclusion disease (NIFID) shows considerable clinical heterogeneity. OBJECTIVE To assess the spectrum of the clinical and neuropathological features in 10 NIFID cases. METHODS Retrospective chart and comprehensive neuropathological review of these NIFID cases was conducted. RESULTS The mean age at onset was 40.8 (range 23 to 56)...
متن کاملChorea as a clinical feature of the basophilic inclusion body disease subtype of fused-in-sarcoma-associated frontotemporal lobar degeneration.
Choreoathetoid involuntary movements are rarely reported in patients with frontotemporal lobar degeneration (FTLD), suggesting their exclusion as a supportive feature in clinical diagnostic criteria for FTLD. Here, we identified three cases of the behavioral variant of frontotemporal dementia (bvFTD) that display chorea with fused in sarcoma (FUS)-positive inclusions (FTLD-FUS) and the basophil...
متن کاملCytomegalic Inclusion-Body Disease
Premature baby unit at Southmead Hospital where she arrived 1J hours after birth. The whole of her body was found to be covered by petechial haemorrhages which in Places merged to form larger lesions resembling bruises. She had a very large spleen , and a very large liver and was thought to be very slightly jaundiced. It was soon realized that this baby had some quite unusual problem and our fi...
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ژورنال
عنوان ژورنال: Journal of Neuropathology & Experimental Neurology
سال: 2012
ISSN: 0022-3069,1554-6578
DOI: 10.1097/nen.0b013e318266efb1