TABLE.
NAD | FAHN | |
---|---|---|
Neuromotor features | ||
Ataxia, dysmetria | Profound | Profound |
Dystonia | Mild | Mild-severe |
Spastic quadriplegia | Prominent | Prominent |
Pyramidal tract signs | Prominent | Prominent |
Axial hypotonia | Prominent | Not noted |
Neuro-ophthalmologic features | ||
Optic atrophy | Prominent | Mild-severe |
Nystagmus | Present | Present |
Acquired strabismus | Present | Present |
Neuroimaging findings | ||
Cerebellar atrophy | Prominent | Prominent |
Brainstem atrophy | Absent | Prominent |
Globus pallidus iron deposition | Variable | Variable |
Other features | ||
Seizures | Occasional | Occasional |
Peripheral neuropathy | Prominent | Absent |
Neuroaxonal spheroids | Prominent | ??? |
Excessive beta activity (EEG) | Prominent | Present |