Pure Motor Axonal Neuropathy Associated with Sjögren's Syndrome: A Rare Case Report.
Alyaa Kheirbek, Mohamad Yousef Almawaz, Yasmin Alsaadi et al.
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Case in brief
A 47-year-old man with a history of sicca symptoms presented with progressive, asymmetric motor weakness in all four limbs, without sensory involvement. Neurological examination revealed left-sided motor deficits, preserved reflexes, and normal sensory testing. Electrodiagnostic studies indicated a motor-predominant axonal neuropathy. Serological tests confirmed Sjögren's syndrome. Despite aggressive immunosuppressive treatment, including cyclophosphamide and rituximab, the patient experienced no significant clinical improvement.
What made this case unusual
This case highlights the rarity of pure motor axonal neuropathy associated with Sjögren's syndrome, which can mimic anterior horn cell disorders, complicating diagnosis and management.
Diagnostic / clinical pearl
Clinicians should consider pure motor axonal neuropathy as a potential manifestation of Sjögren's syndrome, especially in patients presenting with isolated motor weakness.
Why it matters
Recognizing this rare presentation can aid in timely diagnosis and management, although treatment responses may be limited.