B. Lemieux

Hôpital Notre-Dame

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A. Barbeau

Montreal Clinical Research Institute

G. Geoffroy

Institut de Recherche en Sciences de la Santé

D. Shapcott

Centre Hospitalier Universitaire de Sherbrooke

S. Melançon

The University of Texas MD Anderson Cancer Center

Roger F. Butterworth

Western University

Guillaume Breton

Hôpital Pellegrin

Michel Côté

Université de Montréal

A Davignon

Washington University in St. Louis

Louis Dallaire

Centre Hospitalier Universitaire Sainte-Justine

Robert Giguère

Université de Sherbrooke

Cooperative Institutions

Centre Hospitalier Universitaire de Sherbrooke

Université de Montréal

McGill University

Université de Sherbrooke

Centre Hospitalier Universitaire Sainte-Justine

Montreal Clinical Research Institute

Université Laval

University of Toronto

Hôtel-Dieu de Montréal

Montreal Children's Hospital

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Echocardiographic Findings in Friedreich's Ataxia

Canadian Journal of Neurological Sciences / Journal Canadien des Sciences Neurologiques (1976)

H Gattiker A Davignon A Bozio J. Batlle-Diaz G. Geoffroy

Echocardiographic examination of 21 patients with Friedreich's ataxia (age 7 to 28 years) showed cardiac abnormalities in 90% of the cases. They were characterized by varying degrees of septal hypertrophy in 81%, left ventricular free wall hypertrophy in 61%, and a slight reduction of left ventricular internal dimension in 57% of the cases. Asymmetric septal hypertrophy (ASH) with a septal/left ventricular free wall ratio of over 1.3 was found in 29% of the cases, and systolic anterior motion (SAM) of the mitral valve in three patients. Two other patients showed evidence of a different type of cardiomyopathy with marked symmetric left ventricular hypertrophy and marked left ventricular enlargement.

10.1017/s0317167100025543

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Citations (31)

Cardiological Signs and Symptoms in Friedreich's Ataxia

Canadian Journal of Neurological Sciences / Journal Canadien des Sciences Neurologiques (1976)

Michel Côté A Davignon K. Pecko-Drouin A Solignac G. Geoffroy

The cardiovascular signs and symptoms were recorded in 36 patients with typical Friedreich's Ataxia (Group Ia, Ib). Seventeen patients were asymptomatic and this did not correlate with the severity of the disease. No pathognomonic clinical constellation was found to reveal the underlying cardiomyopathy.

Pathognomonic

Signs and symptoms

10.1017/s031716710002552x

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Citations (31)

Bilirubin Metabolism — Preliminary Investigation

Canadian Journal of Neurological Sciences / Journal Canadien des Sciences Neurologiques (1976)

A. Barbeau Guillaume Breton B. Lemieux Roger F. Butterworth

SUMMARY: In our studies, high total bilirubin values in the plasma were noted in cases of Friedreich's ataxia. A bimodal distribution of the values indicated the possible presence of two subgroups of patients. In these kindred, we demonstrated an elevation in unconjugated bilirubin with features similar to those reported in Gilbert's syndrome: normal liver function tests, elevation after fasting and day to day variability. We also report preliminary experiments indicating that bilirubin levels may be taurine dependent. We postulate that the defect could be a secondary component of the ataxic disease, possibly indicating a defect in membrane transport.

10.1017/s0317167100025610

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Citations (14)

Purine Metabolism in Friedreich's Ataxia

Canadian Journal of Neurological Sciences / Journal Canadien des Sciences Neurologiques (1978)

P. Draper B. Lemieux Irving H. Fox D. Shapcott

In a detailed investigation of nucleotide synthesis, interconversion and degradation, no difference was found between subjects with Friedreich's Ataxia and normal controls. It appears improbable that this disorder is related to a primary defect in purine metabolism.

10.1017/s031716710002494x

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Citations (1)

The Hartnup phenotype: Mendelian transport disorder, multifactorial disease.

PubMed (1987)

C. R. Scriver Barbara E. Mahon Harvey L. Levy C Clow Terry M. Reade

Succinonitrile

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Citations (78)

Ultrastructural Observations on Spinal Ganglion Biopsy in Friedreich’s Ataxia: A Preliminary Report

Canadian Journal of Neurological Sciences / Journal Canadien des Sciences Neurologiques (1982)

Jacques Lamarche C. Luneau B. Lemieux

We report the preliminary study of a dorsal root ganglion obtained at biopsy during a surgical intervention in a patient with Friedreich's ataxia. There was an apparent decrease in the number of large myelinated fibers without necrosis, but with numerous axonal swellings consisting mainly of dense accumulated neurofilaments. Large amounts of lipofuscin were also found as well as some onion bulb formations suggesting a process of axonal atrophy.

Dorsal root ganglion

Neurofilament

10.1017/s0317167100043833

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Citations (24)

Cardiovascular Evaluation of Obligate Hétérozygotes in Friedreich's Ataxia

Canadian Journal of Neurological Sciences / Journal Canadien des Sciences Neurologiques (1979)

Michel Côté A. Larose M. Cimon B. Lemieux

A study of 17 parents (obligate heterozygotes) of children with Friedreich's ataxia was carried out. In addition to medical histories and physical examinations, a standard 12 lead ECG tracing was obtained. In the age group below 50, there was no significant evidence of ischaemic or primary cardiomyopathy. Older subjects had more frequent risk factors for arteriosclerotic heart disease.

Obligate

10.1017/s0317167100119559

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Citations (1)

Serum and Platelet Lipoamide Dehydrogenase in Friedreich's Ataxia

Canadian Journal of Neurological Sciences / Journal Canadien des Sciences Neurologiques (1978)

A Filla Roger F. Butterworth G. Geoffroy B. Lemieux A. Barbeau

Pyruvate dehydrogenase (PDH), alpha-keto glutarate dehydrogenase (alpha-KGDH) and lipoamide dehydrogenase (LAD) were measured in platelets of 11 patients with typical Friedreich's ataxia and 10 normal control subjects. Serum LAD was also evaluated in the same patients. No statistically significant changes were found in platelets for the group as a whole, although some patients had low values (more than one standard deviation below control mean). Serum LAD was significantly reduced in the patients with Friedreich's ataxia. This was not due to associated diabetes.

10.1017/s0317167100024872

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Citations (29)

[Linkage studies in Emery-Dreifuss muscular dystrophy].

PubMed (1989)

Paquis Nisha Philip Voelckel Ma Jennie G. Pouget B. Lemieux

We report linkage studies between Emery-Dreifuss muscular dystrophy (EDMD) and polymorphic probes from the long arm of chromosome X in two pedigrees. The results don't show significant linkage but are consistent with previous localisation of EDMD in Xq28. Further studies will be necessary to apply molecular biology to genetic counselling and prenatal diagnosis of this disease.

Pedigree chart

Emerin

Linkage (software)

Genetic linkage

Xq28

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Citations (3)

[You and digoxin].

PubMed (1985)

M. Laflamme L Leclerc B. Lemieux

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