Table 2

Suggested distinguishing features between hereditary and inflammatory neuropathy and main findings indicative of overlap

Typical for hereditaryTypical for inflammatoryMain features in overlap
 Dysmorphic featuresYesNoYes
 WeaknessDistal/no proximalSymmetrical, proximal and distalProximal (recent change)
 Positive sensory symptomsNoYesYes (recent change)
 ProgressionSlowStepwise or rapidAcute, subacute or stepwise deterioration
 DemyelinatingYes (if demyelinating form, eg, CMT1, FAP)YesYes
 Internerve motor slowingHomogeneousHeterogeneous motor conduction slowing, distal latency prolongation and F-wave prolongation+ Heterogeneous (new vs comparative studies)
 Conduction block or temporal dispersionNo, except at entrapment sitesYes, outside entrapment sitesYes (new vs comparative studies)
Cerebrospinal fluid
 CSF proteinNormal or mildly elevated (<1 g/L)Elevated CSF protein above normal range, >0.50 g/LRaised; with suggested cut-off >1 g/L or 0.50–1 g/L, if highly suggestive clinical context
Imaging (MRI)
 Thickened nerve rootsYesYesYes
 Enhancing nerve roots/plexus with gadoliniumNoYesYes
Histopathological (nerve biopsy)
 Demyelinating: onion bulbs, loss of myelinated fibresYesYesYes
 Inflammatory cellsNoYes; macrophage-induced demyelination ++Yes; macrophage-induced demyelination ++
  • CMT, Charcot-Marie-Tooth disease; FAP, familial amyloid polyneuropathy.