Table E-1 Patient Demographics
PatientSex/Age / Earliest clinical symptoms by history; onset
G=gastrointestinal
N=neurologic / Neuropathic symptoms
at time of presentation; duration / Neurological Exam
(vib=vibration; lt=light touch)
Sensory dysfunction in extremities
+ = mild
++ = mild-moderate
+++ = moderate
++++ = severe / Electrodiagnostic features / Treatment trials/
Associated medical conditions
#1 MA
F/63 / G; age 40 / Multifocal body pains, facial dysesthesias;
1 yr / ++ (vib/lt) / Severely prolonged bilateral R1 blink latencies; moderate-severely low amplitude peroneal motor responses / Anxiety
#2 AB
F/63 / G; childhood / Distal lower extremity dysesthesias; left lower back pain with radiation down lateral aspect of left thigh; 1 yr / + (vib/pin/lt) / Normal
#3 JG
F/84 / G; adulthood / Lower extremity dysesthesias/paresthesias; occasional facial flushing; mild gait unsteadiness; 4 yrs / ++ (vib); except for absent vibration in right great toe
Absent knee/ankle reflexes; positive Romberg / Mild motor polyneuropathy, borderline prolonged bilateral R1 blink latencies / Dermatitis herpetiformis, eczema
#4 LB
F/44 / G; childhood / Intermittent extremity numbness; 20 yrs / + (lt/vib/pin) in toes
+ (lt) in fingers
Mildly positive Romberg / Normal / Positive family history
#5 CB
F/55 / G; age 55 / Multifocal body pains, facial dysesthesias;
1 month / + (vib) in legs / Bilateral median neuropathies at the wrist / Low normal vitamin B12 level
#6 KC
F/53 / G; childhood / Distal extremity dysesthesias; 30 yrs / ++ (vib/pin/lt) / Normal / Elevated anti-GM1 antibody titer
#7 MG
M/44 / N; age 37
GI symptoms occurred 8 years later / Distal extremity dysesthesias, intermittent skin eruptions; 7 yrs / + (vib/lt) / Mild median neuropathy at the wrist; mildly low amplitude right tibial motor response / Depression, sarcoidosis
#8 PK
F/49 / N; age 47
CD diagnosed during workup for gastroesophageal reflux disease / Multifocal body pains, facial dysesthesias, tracheal pain; 2 yrs / + (vib/pin/lt) / Normal / Depression
#9 VM
M/79 / N; age 59 / Right foot drop with gait unsteadiness;
20 yrs / Right foot drop;
+ (vib/lt) in left leg
+++ (vib/lt) in right leg
Mildly positive Romberg / Mild sensorimotor polyneuropathy / IVIG X 3 months; no benefit
IgM monoclonal gammopathy
#10 SM
M/72 /
G; age 62
/ Distal extremity dysesthesias, facial dysesthesias; 2 yrs / + (vib/lt) / Mild sensorimotor polyneuropathy / IVIG X 3 months;no benefit
Psoriasis, gout, pericarditis
#11 MM
F/68 /
G; childhood
/Distal extremity dysesthesias; 14 yrs
/ +++ / NormalSural nerve bx**
#12 SM
F/68 / G; age 52 / Distal upper extremity dyesthesias, lower extremity pains; 16 yrs / + (vib/lt) / Mild right median neuropathy at the wrist; mildly low amplitude right peroneal motor response / Hyperthyroidism
#13 AR
M/55 / N; age 54 / Distal lower extremity dyesthesias/numbness, multifocal body pains; 1 yr / + (lt/pin) in feet
++++ (vib) in feet
Absent reflexes; poor tandem gait / Normal
Sural nerve bx** / IVIg, etanercept; no benefit
Attention deficit hyperactivity disorder
#14 DS
M/48 / N; age 47 / Distal lower extremity dysesthesias; 1 yr / ++ (vib) / Absent bilateral H-reflexes / Low normal vitamin B12 level, diabetes mellitus
#15 DT
F/47 / N; age 43 / Distal lower extremity dysesthesias,
multifocal body pains, facial dysesthesias;
4 yrs / ++ (vib) / Normal / IVIg; no benefit
Anxiety, hypothyroidism, pericarditis, iron-deficiency anemia
Positive family history
#16 FT
F/77 / N; age 72
CD diagnosed during workup for anemia / Lower extremity dysesthesias/numbness;
5 yrs / ++ (vib/pin)
Impaired tandem gait; positive Romberg / Normal / Iron-deficiency anemia
#17 AU
F/70 / G; childhood / Intermittent mild paresthesias in distal extremities; 15 yrs / ++ (vib)
Absent lower extremity reflexes; impaired tandem gait; positive Romberg / Mild motor polyneuopathy; left L4/5 radiculopathy / Positive family history
#18 CW
M/31 / N; age 30 / Distal extremity dysesthesias, facial dysesthesias; 1yr / + (vib/lt) in legs / Normal
#19 AC
M/54 / N; age 52 / Distal extremity dysesthesias/numbness, gait instability; 2 yrs / Bilateral dorsiflexor weakness,
Absent vib/pin/lt in legs;
+ (vib/pin) in hands
Absent reflexes, impaired gait, positive Romberg / Severe sensorimotor polyneuropathy affecting the legs; severe sensory polyneuropathy in arms;
prolonged bilateral R1 blink latencies
Sural nerve bx** / Ongoing IVIg therapy; improved balance
Anxiety/depression
#20 MN
F/53 / G; childhood / Distal extremity dysesthesias/numbness;
2 yrs / + (lt/pin) diffusely below neck / Normal / Mood disorder
** Sural nerve biopsy results: Patient #11 = chronic moderately-severe axonopathy; mild neurogenic abnormalities;
Patient #13 = chronic axonopathy; Patient #19 = chronic, severe axonal neuropathy
For further information contact:
(Russell L. Chin, M.D.)