Table 1.

Patients demographics and neurological examination

	n = 100
Demographics
Male	45
Positive family history	45
Age of onset	52 (range 19–76)
Age at examination	63 (range 33–82)
History of cancer	12
Previous misdiagnosis	32
Neurological examination	79
Ataxic gait
Positive Romberg	83
Dysarthria	40
Abnormal eye movements	65
Nystagmus	55
Broken pursuit	42
Dysmetric saccades	41
Abnormal head impulse test	28/32 (87%)
Sensation
Abnormal pinprick face	22
Abnormal pinprick upper limbsa	55
Abnormal pinprick lower limbsb	74
Abnormal vibration upper limbs	41
Abnormal vibration lower limbs	84
Abnormal joint position upper limbs	10
Abnormal joint position lower limbs	48
Muscle strength
Distal weakness upper limbs	0
Distal weakness lower limbs	0
Reflexes
Upper limb reflexes
Normal	54
Brisk	26
Reduced	12
Absent	8
Knee reflexes
Normal	47
Brisk	28
Reduced	13
Absent	12
Ankle reflexes
Normal	30
Brisk	15
Reduced	6
Absent	49
Coordination
Abnormal finger-nose	49
Abnormal heel-shin	52

^aAbsent = 12/55 (22%), patchy = 2/55 (4%), length dependent pattern n = 41/55 (74%).

^bAbsent = 13/74 (18%), patchy = 4/74 (5%), length dependent pattern n = 57/74 (77%).