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Question Date: 16/07/2023

Question: A 35 year old male presents with subacute progressive ataxia and numbness. Examination reveals sensory ataxia without motor weakness. Electrophysiological studies reveal normal sensory and motor conduction with absent SSEP. CSF shows elevated protein and lymphocytic pleocytosis with normal cytology. Serum protein electrophoresis is normal and PET scan has excluded a malignancy, but shows increased metabolic activity in the spinal roots. What is the diagnosis?

Options:

• DADS (Distal acquired demyelinating, symmetric) neuropathy
• CISP (Chronic immune sensory polyradiculopathy)
• Sjogren syndrome
• CANOMAD (Chronic ataxic neuropathy with opthalmoplegia, M protein, cold agglutinins and Disialosyl antibodies)

Correct Answer: CISP (Chronic immune sensory polyradiculopathy)

Explaination: DADS neuropathy causes distal demyelination and is associated with IgM paraproteinemia. Sjogren syndrome may present with sensory ataxia and trigeminal neuropathy due to sensory ganglionopathy. CANOMAD is a rare paraproteinemic neuropathy presenting with sensory ataxia and is similar to miller fisher syndrome. It is associated with Ig M anti GQ1b antibody. Chronic immune sensory polyradiculopathy (CISP) presents with sensory ataxia and large fiber sensory loss. In this condition, inflammation is localized to sensory roots. Hence sensory and motor conduction is normal with abnormalities in SSEP. MRI shows enhancement of nerve roots and CSF is abnormal.

Reference: Gwathmey K. Sensory Polyneuropathies. Continuum 2017;23(5): 1411-1436