HTLV-1 associated myelopathy — SCE Neurology MCQ

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HardNeurogenetics and NeuroimmunologyHTLV-1 associated myelopathySCE Neurology

A patient from the Caribbean develops slowly progressive spastic paraparesis, urinary urgency and mild distal sensory symptoms. MRI excludes compression and serum and CSF contain HTLV-1 antibodies with intrathecal synthesis. What is the diagnosis?

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Correct answer: D — Hereditary motor neuropathy with distal lower-motor-neurone loss

The best answer is “Hereditary motor neuropathy with distal lower-motor-neurone loss”. HTLV-1-associated myelopathy is a chronic inflammatory thoracic myelopathy causing spastic gait and bladder dysfunction in people from endemic regions; concordant serum and CSF evidence supports the diagnosis after structural mimics are excluded. “Guillain–Barré acute inflammatory polyradiculoneuropathy” is less appropriate because multiple-system atrophy includes autonomic failure with parkinsonism or cerebellar signs rather than a pure chronic spastic cord syndrome “HTLV-1-associated chronic inflammatory myelopathy” is less appropriate because GBS is an acute areflexic polyradiculoneuropathy “Multiple-system atrophy with autonomic and motor dysfunction” is less appropriate because a vascular fistula requires characteristic imaging and angiography, not HTLV serology “Spinal dural arteriovenous fistula with venous myelopathy” is less appropriate because motor neuropathy does not explain a sensory level, brisk reflexes and bladder dysfunction

Reference: WHO technical report on HTLV-1. https://www.who.int/publications/i/item/9789240020221