Table 1

Patient	Age	Sex	Clinical	RNS 20 Hz	RNS 50 Hz	Anti-GQ1b IgG titer	CMAP	Imaging	Remarks
Group 1 BBE
1	31	M	BBE: drowsy, ataxia, bilateral ptosis, weakness & brisk reflexes in upper & lower limbs; power 4/5 in all limbs	1^st: +90% (87-93)(1.5) 2nd: +38% (36-40)(1)	1^st: +69% (66-71)(1) 2nd: +35% (32-38)(1.5)	2168	11.1	MRI brain: normal	NCS normal; F and H responses normal Had 5 days of immunoglobulin infusion Complete recovery in 3 momonths
2	60	M	MFS: Diplopia after URTI, complete ophthalmoplegia ; areflexia of all limbs	1^st: +69% (67-71)(1) 2nd: +26% (24-28)(1)	1^st: +72% (70-74)(1) 2nd: +28% (25-31)(1.4)	4855	7.4	MRI brain: normal	NCS; absent H reflexes Complete recovery in 3 months
3	27	M	MFS: complete ophthlmoplegia, ataxia, generalized areflexia	1^st: +97% (95-99)(1) 2nd: +38%(36-40)(1.1)	1^st: +85% (83-87)(1) 2nd: +41%(39-43)(1.1)	12840	9.1	MRI brain: normal	NCS: absent H reflexes
Group 2 GBS
4	71	M	GBS: generalized areflexia, ataxia, dysmetria	1^st: +34%(32-36)(1)	1^st: +33%(31-35)(1)	16	6.7	MRI brain: left basal ganglia hyperintensity	NCS: abnormal F latencies in 4 limbs (upper > 34 ms; lower > 56 ms); H reflexes absent Complete recovery in 3 months
5	51	F	GBS: bilateral lower VII palsy, generalized areflexia	1^st: +29%(26-32)(1.3)	1^st: +40%(38-42)(1)	3	6.5	CT brain: normal MRI brain: normal	NCS: absent H reflexes & prolonged F latencies in 4 limbs (upper > 36 ms; lower > 60 ms)) Clinical deficits resolved in 6 weeks
6	56	F	GBS: lower limb weakness, numbness & generalized areflexia	1^st: +9% (8-10)(0.5)	1^st: +4% (2-6)(1)	65	8.9	MRI brain: normal MRI lumbosacral spine: hyperintense conus medullaris	NCS: absent H reflexes & prolonged lower limb F latencies (all > 36 ms) Clinical deficits resolved in 2 months
7	57	M	GBS: pharyngeal-cervical-brachial variant	1^st: + 10% (8-12)(1.1)	1^st: +7% (4-10)(1.5)	1	2.7	MRI brain: normal MRI cervical spine: normal	NCS: prolonged upper limb F latencies ( all > 35 ms) Clinical deficits resolved in 2 months
8	61	M	GBS: bilateral footdrop; lower limb weakness, numbness & generalized areflexia	1^st: + 38% (36-40)(1)	1^st: +29% (26-32)(1.4)	10	10.8	MRI lumbar spine: mild L3 & L4 spondylosis	NCS: prolonged upper & lower limb F latencies (upper > 36 ms; lower > 56 ms) Clinical deficits resolved in 2 months
9	61	F	MFS variant: ataxia; bilateral VII palsy, reduced lower limb reflexes	1^st: +48%(46-50)(1)	1^st: +50%(47-63)(2.3)	115	7.5	MRI brain: old right parietal infarct	NCS: absent H reflexes & prolonged F latencies Clinical deficits resolved in 2 weeks
10	53	F	MFS variant: Diplopia; reduced vertical eye movements; absent lower limb reflexes; mild dysmetria	1^st: +38% (35-41)(1.5)	1^st: +40% (38-42)(1)	7	6.8	MRI brain: hyperintense midbrain signal	NCS: normal Complete recovery in 3 months

M: male; F: female; RNS: repetitive nerve stimulation; URTI: upper respiratory tract infection
1^st: initial RNS study; 2^nd: repeat RNS study at 6 months; values in brackets indicate range of 5 RNS values (Second bracket denote standard deviation value)
Upper limit of normality: 56% for 20 Hz RNS; 52% for 50 Hz RNS
CMAP: compound muscle action potential amplitude (mV); refers to amplitude at initial study
CMAP & RNS values were measured from the right abductor digiti minimi muscle; abnormal values underlined
Upper limit of normal for anti-GQ1b IgG titer: 534

Table 1: Summary of clinical, electrophysiological and imaging data.