TABLE 6-2 Vitamin E Deficiency Symptoms in Subjects with Ataxia with Vitamin E Deficiency

Reference

Country

Subjects

Clinical Features

Burck et al., 1981

Germany

n = 1 male; aged 12

Ataxia

Sensory neuropathy

Muscle hypertrophy

Laplante et al., 1984

Canada

n = 1 male; aged 10

Areflexia

Gait and limb ataxia

Muscle weakness

Decreased vibration sense

Decreased proprioception

Limb dysmetria

Babinski sign

Harding et al., 1985

United Kingdom

n = 1 female; aged 23 (aged 13 at onset)

Head titubation

Reduced muscle tone

Ataxia

Areflexia

Romberg sign

Defective or absent vibration sense

Impaired proprioception

Krendel et al., 1987

United States

n = 1 male; aged 19

Severe dysarthria

Unintelligible speech

Bradykinesia

Absent tendon reflexes

Stumpf et al., 1987

Italy

n = 1 female; aged 30 (aged 4 at onset)

Dysarthria

Dystonic smile

Absent position sensation in leg and hands

Absent tendon reflexes

Mildly dysmetric

Extensor plantar responses

Moderately ataxic heel to shin movements

Romberg sign



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