Table 1.

General findings.

	P III3	P III4
Sex	F	M
Age at onset	30	20
Age at exam	66	64
Cognitive deficits	+	+
Pyramidal system
UL/LL spasticity	−/+	−/+
UL/LL tendon reflexes	N	N
Babinski	+	+
Peripheral motor system
UL/LL weakness	N	N
Muscle atrophy	−	−
Cerebellar system
Saccadic pursuit	−	−
Dysartria	−	−
Tremor	−	−
Ataxia	−	−
Vibration/surface sensitive	D	D
Urinary/fecal urgency	N	N
Dentation abnormalities	−	−
SPRS score	39	28
Neurophysiology
Abnormal nerve conduction	−	−
Abnormal MEPs	+	+
Abnormal SEPs	+	+
Abnormal VEPs	+	+
Abnormal BAEPs	+	+
MRI
Spinal cord atrophy	n.d	+
Cerebellar atrophy	−	−
TCC	−	−
SCP hyperintensity (FLAIR)	+	+
Hypomyelination	−	−

BAEPs, Brainstem Auditory Evoked Potential; D, decrease of clinical sign; I, increase of clinical sign; F, female; LL, lower limb; M, male; MEP, motor evoked potential; n.d., not done; N, normal/unchanged findings; SCP, superior cerebellar peduncle; SEP, cortical latencies of somatosensory evoked potentials; SPRS: spastic paraplegia rating scale; TCC, thin corpus callosum; UL, upper limb; VEP, cortical latencies of visual evoked potentials. +, clinical sign is present; −, clinical sign is absent.