PubMedFortschritte der Neurologie-Psychiatrie2026-06-09
[Rare very late-onset neuromyelitis optica].
Maximov Maxim M, Rodriguez Lago Alberto A
Neuromyelitis optica (NMO) and its related forms, collectively referred to as neuromyelitis optica spectrum disorders (NMOSD), are rare but often severe autoimmune diseases of the central nervous system (CNS). NMOSD predominantly affects women, most commonly between the ages of 30 and 40 years. Aquaporin-4 antibodies (AQP4-IgG) are detectable in approximately 80% of patients with NMOSD.The cardinal clinical manifestations include transverse myelitis, optic neuritis (ON), as well as brainstem and diencephalic involvement.Most NMOSD cases follow a relapsing-remitting course without progression between relapses. NMOSD relapses are often severe and result in incomplete remission and permanent disability. Monophasic courses are rare.
We report the case of an 86-year-old female patient who presented with subacute occipital headache and right leg hemiparesis, which progressed to tetraparesis.
Magnetic resonance imaging (MRI) revealed a longitudinally extensive transverse myelitis (LETM) from cervical vertebrae C1 to C5. Cerebrospinal fluid (CSF) analysis demonstrated mild pleocytosis, elevated protein levels, and the presence of oligoclonal bands. Aquaporin-4 antibody testing returned strongly positive, confirming the diagnosis of NMOSD. The disease was classified as very late-onset NMOSD (VLO-NMOSD). Treatment with high-dose intravenous methylprednisolone for five days resulted in symptom improvement. Approximately eleven weeks post-discharge, therapy with the anti-CD20 monoclonal antibody rituximab was initiated. The patient's condition remained stable under maintenance therapy, with no further relapses.
NMOSD can manifest even in very advanced age, beyond 75 years. Given the patient's initial presentation with right leg hemiparesis, advanced age, and multiple vascular risk factors, NMOSD was not initially suspected. The accompanying occipital headaches often exhibit migraine-like characteristics.Treatment with intravenous methylprednisolone resulted in symptom improvement, and rituximab was well tolerated.