La neuromielite ottica: Sindrome o malattia?

Abstract The association of unilateral or bilateral optic neuritis and acute or subacute transverse myelitis is known as neuromyelitis optica. It is a rare demyelinating disorder of unknown aetiology, which rarely occurs in western countries. Since its first descriptions (Devic, 1894) II, the problem concerning its nosographic position in the spectrum of the demyelinating diseases is at the present time unresolved, mainly regarding those cases presenting also cerebral involvement. We presented a longitudinal study of a case of neuromyelitis optica who suddenly developed cerebral signs such as delirium followed two weeks later by an episode of blurred vision and pyramidal weakness to the lower limbs. Five years later, the patient's status consisted of a spastic paraparesis along with an optic atrophy. At the onset, the cerebrospinal fluid analysis disclosed lymphocytic pleocytosis, moderate increase in protein, blood-brain barrier damage and oligoclonal bands which were successively normalized. Serial MRI revealed stable disseminated demyelinating areas. The relationship between neuromyelitis optica and demyelinating diseases is discussed hypothesizing the existence of a wide spectrum of the same disease group ranging from multiple sclerosis to neuromyelitis optica.