Table 3.

Reported diagnostic criteria, clinical characteristics and response to treatment for focal chronic inflammatory demyelinating polyradiculoneuropathy

References	Number of patients or frequency	Clinical definition	Electrodiagnostic criteria for diagnosis	Exclusion criteria with respect to the specific variant	Disability (compared with typical CIDP)	Response to treatment
1996	9	Focal UL demyelinating neuropathy [25]	NM	NM	NM	3/5 (60%) patients improved after steroids, 6/6 (100%) after IVIg
2000	1	UL sensorimotor deficit [15]	CB	NM	NM	Improved after IVIg
2013	1	As per 2010 EFNS/PNS guidelines [26]	NM	NM	NM	Improved after IVIg
2019	1%	Included under the definition of LSS [7▪▪]	With or without motor CB	1. Weakness in isolation, without sensory symptoms. 2. Symptoms/signs in a polyneuropathic distribution	NM	NM
2019	1%	Motor or sensorimotor neuropathy confined to one limb [8▪▪]	NM	Clinical picture of typical CIDP or other atypical variants	NM	NM
2020	1	As per 2010 EFNS/PNS guidelines [27]	NM	NM	NM	Improved after IVIg and steroids in combination
2021	1	As per 2010 EFNS/PNS guidelines [28]	NM	NM	NM	Improved after IVIg
2021	30	Sensory or motor or sensorimotor neuropathy involving the brachial or lumbosacral plexus, or one or more peripheral nerves in one UL or one LL (monomelic distribution) [29]	NM	NM	NM	16/19 (84%) improved after IVIg, 0/5 after steroids

CB, conduction blocks; CIDP, chronic inflammatory demyelinating polyradiculoneuropathy; EFNS/PNS, European Federation of Neurological Societies/Peripheral Nerve Society; IVIg, intravenous immunoglobulin; LL, lower limbs; LSS, Lewis-Sumner syndrome; NM, not mentioned; UL, upper limbs.