The scale for the assessment and rating of ataxia correlates with dysarthria assessment in Friedreich's ataxia.

The scale for the assessment and rating of ataxia correlates with dysarthria assessment in Friedreich's ataxia. Eigentler A, Rhomberg J, Nachbauer W, Ritzer I, Poewe W, Boesch S.

Keywords: Dysarthria, neurogenic sensorimotor speech symptom, Frenchay Dysarthria Assessment, clinical and logopedic methodology, excellent clinical tool, ataxic speech disorders.

Clinical Monitoring in a Patient With Friedreich Ataxia and Osteogenic Sarcoma

Clinical Monitoring in a Patient With Friedreich Ataxia and Osteogenic Sarcoma . Deutsch EC, Seyer LA, Perlman SL, Yu J, Lynch DR. J Child Neurol 0883073812448460, first published on June 29, 2012 as doi:10.1177/0883073812448460

Keywords:  Friedreich ataxia, FXN, frataxin, lateral flow immunoassay, MAP chemotherapy.

Cross-Sectional Analysis of Electrocardiograms in a Large Heterogeneous Cohort of Friedreich Ataxia Subjects.

Cross-Sectional Analysis of Electrocardiograms in a Large Heterogeneous Cohort of Friedreich Ataxia Subjects.Schadt KA, Friedman LS, Regner SR, Mark GE, Lynch DR, Lin KY.J Child Neurol 0883073812448461, first published on June 29, 2012 as doi:10.1177/0883073812448461

Keywords:  Electrocardiographic (ECG) findings, Friedreich ataxia, ECG abnormalities, age, sex, shorter guanine-adenine-adenine triplet repeat length, age of disease onset, functional disability, nonspecific ST-T wave changes (53%), right axis deviation (32%), left ventricular hypertrophy (19%), right ventricular hypertrophy (13%).

Friedreich Ataxia: New Pathways.

Friedreich Ataxia: New Pathways.Pandolfo M., J Child Neurol 0883073812448534, first published on June 29, 2012 as doi:10.1177/0883073812448534

Keywords:  Friedreich ataxia, frataxin, neurologic, cardiac complications, mitochondrial biogenesis, iron-sulfur cluster synthesis, iron metabolism, antioxidant responses, mitophagy.

Cardiac Transplantation in Friedreich Ataxia

Cardiac Transplantation in Friedreich Ataxia. Yoon G, Soman T, Wilson J, George K, Mital S, Dipchand AI, McCabe J, Logan W, Kantor P. J Child Neurol 0883073812448229, first published on June 29, 2012 as doi:10.1177/0883073812448229

Keywords:  Friedreich ataxia, cardiac transplantation, left ventricular failure, dilated cardiomyopathy, neurological disease, dysarthria, weakness, mild gait impairment, limb ataxia, ethical issues.

Novel Diagnostic Paradigms for Friedreich Ataxia

Novel Diagnostic Paradigms for Friedreich Ataxia.. Brigatti KW, Deutsch EC, Lynch DR, Farmer JM., J Child Neurol 0883073812448440, first published on June 29, 2012 as doi:10.1177/0883073812448440

Keywords: Friedreich ataxia, lateral-flow immunoassay, quantify the frataxin protein, biomarker for clinical trials, gene dosage analysis.

Clinical Features of Friedreich Ataxia.

Clinical Features of Friedreich Ataxia. Martin B. Delatycki and Louise A. Corben. J Child Neurol 0883073812448230, first published on June 29, 2012 as doi:10.1177/0883073812448230

Keywords: Friedreich ataxia, FXN, central and peripheral nervous system, progressive ataxia, absent lower limb reflexes, upgoing plantar responses, peripheral sensory neuropathy, cardiomyopathy, diabetes mellitus.

Friedreich Ataxia Clinical Outcome Measures: Natural History Evaluation in 410 Participants

Friedreich Ataxia Clinical Outcome Measures: Natural History Evaluation in 410 Participants Sean R. Regner, Nicholas S. Wilcox, Lisa S. Friedman, Lauren A. Seyer, Kim A. Schadt, Karlla W. Brigatti, Susan Perlman, Martin Delatycki, George R. Wilmot, Christopher M. Gomez, Khalaf O. Bushara, Katherine D. Mathews, S. H. Subramony, Tetsuo Ashizawa, Bernard Ravina, Alicia Brocht, Jennifer M. Farmer, and David R. Lynch. J Child Neurol 0883073812448462, first published on June 29, 2012 doi:10.1177/0883073812448462

Keywords: Friedreich ataxia, ataxia, dysarthria, areflexia, large international noninterventional cohort (n = 410), Friedreich Ataxia Rating Scale.

Auditory Processing Deficits in Children With Friedreich Ataxia.

Auditory Processing Deficits in Children With Friedreich Ataxia. Rance G, Corben L, Delatycki M. J Child Neurol June 29, 2012 0883073812448963

Keywords: Friedreich ataxia, auditory neuropathy, personal FM-listening systems, auditory function tests.