The pattern of an abnormal median-normal sural (AMNS) sensory response is associated with acute and chronic inflammatory demyelinating polyradiculoneuropathy (AIDP and CIDP) and considered unusual in other types of neuropathy, although specificity and sensitivity of this pattern have not been evaluated. We compared sensory responses (patterns and absolute values) in patients with AIDP, CIDP, diabetic polyneuropathy (DP), and motor neuron disease (MND). Using strict criteria, the AMNS pattern occurred more frequently in recent onset AIDP (39%) compared with CIDP (28%), DP (14%-23%), or MND (22%) patients. This pattern was found in 3% of control subjects. The extreme pattern of an absent median-present sural response occurred only in AIDP and CIDP patients and in no other groups. Abnormalities of both nerves were more common in long-standing polyneuropathies such as CIDP and DP compared with AIDP or MND. Median nerve amplitudes were reduced significantly in AIDP, CIDP, and DP patients compared with MND patients, whereas sural nerve amplitudes were significantly reduced only in DP and CIDP patients. These findings may reflect early distal nerve involvement particularly in AIDP patients which is highlighted by differences in median and sural nerve recording electrode placement. We conclude that, in the appropriate clinical setting, the AMNS pattern, an absent median-present sural response pattern, or a reduced median amplitude compared with the sural amplitude supports a diagnosis of a primary demyelinating polyneuropathy.