- Discussion:
- most common form of spinal cerebellar degenerative dz;
- onset usually before 10 years old;
- tends to have acute onset;
- Clinical Manifestations:
- staggering gait
-
pes cavus:
- cavus foot deformity is symmetric and progressive;
- initially, it is flexible but soon becomes fixed & may develop heel varus component;
- intrinsic muscle wasting adds to the extrinsic muscle imbalance and contributes to
the developing cavus deformity;
- progressive ataxia
- dysarthria;
- diminished proprioception or vibratory sense;
- muscle weakness and absent deep tendon reflexes;
- scoliosis,
- associated w/ hyperkyphosis, occurs in all pts;
- onset of scoliosis before puberty is a major factor in prognosis;
- Babinski's sign;
- cardiomyopathy;
Natural history of muscle weakness in Friedreich's Ataxia and its relation
to loss of ambulation.
