Table 1 Clinical features of patients with small fibre ganglionopathy
Patient age/sexRelevant PMHSymptom duration (months)Initial symptomsOnsetImmune therapyResponseSymptomatic therapies offeredResponseOutcome at last follow-up
1/45/MNone84Burning feet, legs, hands, thighs, trunk, scalpAcutePrednisone, IVIG, CSANRPregabalin, tramadol, bupropion, fentanyl, methadoneSlightChronic, constant severe pain
2/56/MNone3Sunburn sensation over thighs, trunk, scalp, arms solesSubacuteNoGabapentinGoodStable 2/10* pain
3/55/FInfluenza vaccination; serum sickness25Itching, burning legs, arms, face, trunk and handsAcuteNoGabapentin, pregabalin duloxetineGoodStable 3/10 pain
4/46/MPreceding viral illness12Burning forearms, thighs, ventral hands, face, tongueAcuteNoMirtazapine, bupropion, pregabalinGoodStable 2/10 pain
5/65/FSicca symptoms9Burning feet, ankles, fingertips, face, mouth, tongue, scalpChronicNoDesipramine, gabapentin, mirtazapineGood50% pain reduction
6/50/MHepatitis C RA36Cold pain face, tongue, mouth, trunk, legsSubacutePrednisoneNRFentanyl, gabapentin, carbamazepine, amitriptylinePoorChronic severe pain
7/38/MType I DM4Sunburn pain and allodynia medial thighs, knees, back, chest, shoulders; hands/feet sparedAcuteIVIG30% pain reductionCarbamazepine, gabapentin, tramadolPoorFull recovery after pancreatic transplant
8/60/FNone84Painful dysaesthesias fingertips to elbowsChronicNoNoneLost to follow-up
9/57/MOnset after CABG24Numb feet, burning thighs, toes, fingertips, forearms, left faceAcuteNoTramadol, gabapentin, venlafaxineGoodStable 1/10 pain
10/55/MNone4Burning feet, legs, hands, thighs, anterior trunkAcutePrednisone, IVIGNRFentanyl, gabapentin, amitriptylineSlightSevere pain on chronic narcotics
11/47/MCLL24Dysaesthesias tongue, anterior trunk, face, hands, feetSubacutePrednisoneNRGabapentin, MS ContinGoodSlow recovery over 3 years; mild burning left foot persists
12/34/FCoeliac disease19Perineal hypersensitivity, dysaesthesias hands, face, mouth, feetChronicNoGabapentin, pregabalin, duloxetineGoodStable pain control
13/19/FHip arthralgias12Simultaneous painful hands, feet; spread to legs, thighs, forearmsChronicPrednisone, IVIGLess pain; relapse with taperingGabapentinGoodStable pain control. Relapsing course requiring periodic IVIG
14/46/MMalaise arthralgias12Allodynia, dysaesthesias, shooting pains face, trunk, arms, legsChronicIV medrol, IVIGNRDuloxetine, pregabalin, oxcarbazine, lamotrigine, gabapentin, nortriptyline, methadoneSlightWorsening pain
15/62/FNone30Hot/cold pain entire body; dysaesthesias scalp, thighs, arms, legsSubacuteNoGabapentin, pregabalin, duloxetine, nortriptylinePoorSevere pain unchanged
16/64/MNone12Severe burning, dysaesthesias feet, chest, face, tongue, limbs region, then arms, legs, hands, shouldersSubacuteIV MedrolNRGabapentin, oxycodone HCL, topiramate, pregabalin, tramadol, venlafaxine, methadone, duloxetineModeratePain reduced with stable pain control
17/55/MNone24Deep burning pain in all limbsChronicNoGabapentin, fentanyl, duloxetine, tramadolSlight6/10 pain persists
18/43/FSicca6Bilateral palm itching, burning, then spread to feet over 2 monthsAcuteNoGabapentinModerateReduced burning pain
19/64/FNone36Pricking, dull aching pain in perineal region, arms, legs, hands, shouldersSubacuteNoAmitriptyline, gabapentinModerateReduced pain
20/34/FMyelitis 15 y ago; recovered168Sunburn pain, aching in scalp, upper lip, 4th and 5th digits of hands, feetAcuteNoCarbamazepine, duloxetine, gabapentin, pregabalin, oxycodone HCL, methadone, valproic acidSlightChronic persistent pain, overall unchanged
21/53/FRemote SLE immune thyroid disease7Burning, paresthesias in thighs, legs, arms, neck, trunk; feet sparedChronicNoGabapentin, carbamazepine, duloxetine, amitriptyline, lidocaine patchesSlight7/10 chronic, severe pain
22/52/FNone6Burning, paresthesias in back, arms, legs, scalpSubacuteNoTramadol, gabapentinSlightPersistent chronic pain; mild improvement with medications
23/57/MSicca symptoms9Burning pain, dysaesthesias over forearms, legs, thighs, chest, abdomen, neck, mouth, earsSubacuteNopregabalin, gabapentin, duloxetinePoorPersistent chronic pain unchanged
  • CABG, coronary artery bypass surgery; CLL, chronic lymphocytic leukaemia; CSA, ciclosporin; DM, diabetes mellitus; IV, intravenous; IVIG, intravenous immune globulin; NR, no response; PMH, past medical history; RA, rheumatoid arthritis; SLE, systemic lupus erythematosus.

  • *Pain scores, when available, are expressed using a 0–10 verbal analogue scale.