Abstract
Objective: To report a case of CANOMAD syndrome
Background: CANOMAD syndrome is a rare chronic neuropathy characterized by sensory ataxia, ophthalmoplegia, IgM paraprotein, cold agglutinins and antidisialosyl antibodies. The condition is associated with significant disability.
Design/Methods: Case report.
Results: 65 year-old Caucasian male presented with gait imbalance and vision disturbance for 3 years. He also reported paresthesias in fingers and toes. He was subsequently diagnosed with idiopathic neuropathy which became progressively worse. He had difficulty focusing his vision but denied diplopia or pain with eye movement. He was diagnosed with decompensated alternating esotropia vs sixth nerve palsy by ophthalmology who recommended strabismus surgery. He also reported unintentional 60–70lbs weight loss in the past 2 years. Malignancy workup was negative. During a neurology consultation, his exam showed bilateral limited eye abduction, R>L; mild symmetric distal upper and lower extremities weakness and muscle atrophy, with marked impairment of vibration and joint positional sense in hands and feet. He also had diffuse areflexia, a positive Romberg sign and a wide-based ataxic gait. Labs were significant for positive GD1B IgM antibody, IFE with monoclonal IgM Kappa and Lambda band in gamma region. Anti-Hu, VGCC, anti-AchR, ANA, SSA/SSB antibodies and B12 level were negative or normal. MRI brain and orbit were unremarkable. There was electrophysiologic evidence of an asymmetric, sensorimotor polyneuropathy that is predominantly axonal with demyelinating features. Patient was treated with IVIG with improvement of his vision and gait. He will receive IVIG monthly and a hematology consult is pending.
Conclusions: CANOMAD syndrome should be considered when patient presents with polyneuropathy with sensory ataxia and ophthalmoparesis. This consideration should prompt a search for monoclonal protein and ganglioside antibodies. Immunomodulation treatment such as IVIG is indicated for this condition.
Disclosure: Dr. Bai has nothing to disclose. Dr. Christiana has nothing to disclose. Dr. Jiang has nothing to disclose.
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