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

Related articles:

• Approach to weakness, approach to sensory deficits, neuromuscular disease patterns, polyneuropathy,
• Sjogren syndrome neuropathy, paraneoplastic neuropathy, polyneuropathy associated with paraproteinemia, Refsum disease,