Synonyms:
a.k.a. chronic ataxic neuropathy
Diagnosis:
Clinical features +NCS/EMG findings +Exclusion of other causes: Sjogren syndrome, paraneoplastic syndrome, paraproteinemia, Refsum disease etc.
Clinical features:
Global sensory loss (especially proprioception), sensory ataxia (pseudoathetosis), normal power
Exclusion of other causes: Sjogren syndrome, paraneoplastic syndrome, paraproteinemia, Refsum disease etc.
Findings on investigations:
+NCS +EMG:
- Axonal Sensory neuropathy (may be normal initially)
- Normal motor,
Pathology, nerve biopsy:
Dorsal root ganglia: lymphocytic infiltration, destruction of nerve cells,
Sural nerve biopsy:
- Loss of myelinated axons