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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