𝔖 Bobbio Scriptorium
✦   LIBER   ✦

Alterations of striatal neurons in benign hereditary chorea

✍ Scribed by Galit Kleiner-Fisman; Noel Y. Calingasan; Mary Putt; June Chen; M.Flint Beal; Anthony E. Lang

Publisher
John Wiley and Sons
Year
2005
Tongue
English
Weight
217 KB
Volume
20
Category
Article
ISSN
0885-3185

No coin nor oath required. For personal study only.

✦ Synopsis

Abstract

Benign hereditary chorea (BHC) recently has been associated with mutations in TITF‐1 gene, although a pathological study of an individual with BHC and a TITF‐1 mutation revealed no significant gross or microscopic abnormalities using standard methods. Immunohistochemical staining of striatal tissue from a BHC‐affected postmortem brain was performed using antibodies against neurotransmitters of interneurons whose tangential migration is mediated by TITF‐1. There was a loss of most TITF‐1–mediated striatal interneurons in the BHC specimen compared to four matched control brains. © 2005 Movement Disorder Society

📜 SIMILAR VOLUMES

Cerebral metabolism of glucose in benign
Cerebral metabolism of glucose in benign hereditary chorea
✍ Oksana Suchowersky; Dr. Michael R. Hayden; W. R. Wayne Martin; A. Jon Stoessl; A 📂 Article 📅 1986 🏛 John Wiley and Sons 🌐 English ⚖ 700 KB

Snmmerp: Benign hereditary chorea (BHC) is an autosomal dominant disorder characterized by chorea of early onset with little or no progression. There is marked clinical variability in this disease with some subjects having onset in infancy and others with onset in early adulthood. In contrast to Hun

Normal striatal glucose consumption in t
Normal striatal glucose consumption in two patients with benign hereditary chorea as measured by positron emission tomography
✍ T. Kuwert; H. W. Lange; K. -J. Langen; H. Herzog; H. Hefter; A. Aulich; L. E. Fe 📂 Article 📅 1990 🏛 Springer 🌐 English ⚖ 951 KB

Positron emission tomography (PET) with [18F]-2-fluoro-2-deoxy-D-glucose (FDG) was used to investigate the regional cerebral metabolic rate of glucose consumption (rCMRGlc) in two patients with benign hereditary chorea (BHC) and 21 normal subjects. Relative and absolute values of cerebellar, striata

Benign hereditary chorea: Clinical and n
Benign hereditary chorea: Clinical and neuroimaging features in an Italian family
✍ Elena Salvatore; Luigi Di Maio; Alessandro Filla; Alfonso M. Ferrara; Carlo Rina 📂 Article 📅 2010 🏛 John Wiley and Sons 🌐 English ⚖ 401 KB

## Abstract Benign hereditary chorea is an autosomal dominant disorder characterized by early onset nonprogressive chorea, caused by mutations of the __thyroid transcription factor‐1 (TITF‐1)__ gene. Clinical heterogeneity has been reported and thyroid and respiratory abnormalities may be present.

Clinical differentiation of genetically
Clinical differentiation of genetically proven benign hereditary chorea and myoclonus-dystonia
✍ Friedrich Asmus; Anita Devlin; Marita Munz; Alexander Zimprich; Thomas Gasser; P 📂 Article 📅 2007 🏛 John Wiley and Sons 🌐 English ⚖ 229 KB

## Abstract Because of clinical similarities, benign hereditary chorea and myoclonus‐dystonia (DYT11) might be confused. No systematic comparisons of genetically proven cases with thyroid transcription factor‐1 (__TITF‐1__) and ε‐sarcoglycan (__SGCE__) mutations have been performed to date. Three i

Advanced magnetic resonance imaging in b
Advanced magnetic resonance imaging in benign hereditary chorea: Study of two familial cases
✍ Gloria Maccabelli; Anna Pichiecchio; Andrea Guala; Michela Ponzio; Fulvia Palesi 📂 Article 📅 2010 🏛 John Wiley and Sons 🌐 English ⚖ 645 KB

## Abstract No brain abnormalities are usually detected on conventional magnetic resonance imaging (MRI) in benign hereditary chorea (BHC); there are currently no studies with advanced techniques in literature. We investigated whether conventional and advanced MRI techniques could depict regional b