Version June 2024
| Clinical Criteria |
| Age and sex incidence |
| Mostly sporadic and adult, but sometimes familial; occasionally seen in childhood; females predominant |
| Onset |
| Usually insidious but may be sudden |
| Main neurological manifestations |
| Chronic spastic paraparesis, which usually progresses slowly, sometime remains static after initial progression |
| Weakness of the lower limbs, more marked proximally |
| Bladder disturbance usually an early feature; constipation usually occurs later; impotence or decreased libido is common |
| Sensory symptoms such as tingling, pins and needles, burning, etc., are more prominent than objective physical signs |
| Low lumbar pain with radiation to the legs is common |
| Vibration sense is frequently impaired; proprioception is less often affected |
| Hyperreflexia of the lower limbs, often with clonus and Babinski's sign |
| Hyperreflexia of the upper limbs; positive Hoffman's and Tromner signs frequent; weakness may be absent |
| Exaggerated jaw jerk in some patients |
| Less frequent neurological findings |
| Cerebellar signs, optic atrophy, deafness, nystagmus, other cranial nerve deficits, hand tremor, absent or decreased ankle jerk. Convulsions, cognitive impairment, dementia or impaired consciousness are rare. |
| Muscular atrophy, fasciculations (rare), polymyositis, peripheral neuropathy, polyradiculopathy, cranial neuropathy, meningitis, encephalopathy |
| Systemic non-neurological manifestations |
| Pulmonary alveolitis, uveitis, Sjögren's disease, arthropathy, vasculitis, ichthyosis, cryoglobulinemia, monoclonal gammopathy, adult T-cell leukemia/lymphoma |
| Laboratory Diagnosis |
| Presence of HTLV-1 antibodies or antigens in blood and CSF |
| CSF may show mild lymphocyte pleiocytosis |
| Lobulated lymphocytes may be present in blood and/or CSF |
| Mild to moderate increase of protein may be present in CSF |
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