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Syringomyelia – Classical Presentation — SCE Neurology MCQ

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ModerateNeuro-oncologySyringomyelia – Classical PresentationSCE Neurology

A 55-year-old man presents with a 3-month history of bilateral upper limb pain and weakness with wasting affecting the hands and forearms. Reflexes are absent in the upper limbs but brisk in the lower limbs with extensor plantar responses bilaterally. He has a dissociated sensory loss (loss of pain and temperature sensation in a cape-like distribution across the shoulders and upper trunk, with preserved light touch and proprioception). MRI cervical spine shows a central intramedullary cystic lesion extending from C3 to T1. What is the most likely diagnosis?

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Correct answer: C — Syringomyelia

Syringomyelia (a fluid-filled cavity within the spinal cord) classically presents with: dissociated sensory loss ('cape distribution' — loss of pain and temperature with preserved light touch/proprioception, due to crossing spinothalamic fibres being disrupted while the dorsal columns are spared), lower motor neurone signs at the level of the syrinx (hand and forearm wasting from anterior horn cell damage), and upper motor neurone signs below the syrinx (brisk leg reflexes, extensor plantars). MRI shows a central intramedullary cystic cavity. Causes include Chiari I malformation, post-traumatic, and tumour-associated. A: CSM does not cause central cystic cavitation or cape distribution sensory loss. C: MS has different imaging pattern. D: Tumour would show solid enhancement. E: TM is acute.

Reference: ABN Myelopathy Guidelines; Clinical Neuroanatomy