Heart disease in Friedreich's ataxia

Friedreich's Ataxia: Cardiac Evaluation of 25 Patients with Clinical Diagnosis and Literature Review

“…Therefore, the cardiac disturbs in Friedreich's ataxia appear to be much more frequent than previously believed [17][18][19] and it is accepted that the prominence of electrocardiographic defect parallels that of the neurologic deficit, in which the absent tendon reflexes in the lower extremities, extensor toe signs, scoliosis and pes cavus are most often correlated with electrocardiographic alterations 14 .…”

“…Cardiac enlargement when present, occurs late in the course of the illness. Anginal pain or decubitus angina has already been reported (12%) and no correlation was found between this symptom and the coronarial changes sometimes described in some patients [19][20][21] . About 73% of the patients either died of heart failure or showed some clinical evidence of cardiac dysfunction during life.…”

Die zerebelläre Dysarthrie - eine Literaturübersicht

“…Im fortgeschrittenen Stadium leiden wohl alle Patienten an einer Dysarthrie (Tyrer und Sutherland 1961, Heck 1964, Brain und Walton 1969, Geoffroy et al 1976). Gelegentlich sollen sprechmotorische Defizite sogar das Initialsymptom darstellen (Heck 1963). Das Vorkommen einer u. a. durch unregelmäßige und explosive Artikulation gekennzeichneten Dysarthrie wurde auch beim Roussy-Levy-Syndrom beobachtet (Spillane 1940, Lusso 1952. Aufgrund des zerebellären Charakters der Sprechstörungen wurde ein pathogenetischer Zusammenhang mit der FA angenommen.…”

Blood Flow in the Extremities at Rest, During Reactive Hyperaemia and after Ganglionic Block in Gases of Friedreich's Ataxia

“…Rest. The individual values are given in table 11. The blood flow in forearms and calves at rest in the different groups is illustrerad in fig.…”