Improvement of Frontal Lobe Dysfunctions in Neuromyelitis Optica after Treatment: A Case Report

BACKGROUND: Neuromyelitis optica (NMO) is characterized by optic neuritis and longitudinally extensive transverse myelitis. Generally, the brain had been considered healthy in NMO patients, though recent studies have demonstrated that T2-weighted abnormalities may be observed in various brain regions. Logically, NMO brain lesions are localized at sites of high aquaporin-4 expression. CASE REPORT: A 68-year-old right-handed man with dysuria, weakness in the bilateral upper and lower limbs, and decreased sensation of the lower extremities, was diagnosed with neuromyelitis optica. The patient was gradually speaking less, was showing reduced interest in hobbies, and had undergone changes in character and behavior. An examination was performed using the Seoul Neuropsychological Screening Battery (SNSB), which revealed that the profile of frontal lobe dysfunctions was prominent as compared with other cognitive domains. The patient was treated with prednisolone and azathioprine for about 1 year without recurrence, and showed prognostic improvement according to further SNSB testing. CONCLUSIONS: Further studies are considered necessary in order to find the most effective medication regimen for improving cognitive functions in those accurately diagnosed with NMO, and to develop systematic treatment using even more diversified immune-related agents.

Posterior Cortical Atrophy with Acute Onset and Rapid Progressive Visual Symptoms: A Case Report

BACKGROUND: Posterior cortical atrophy (PCA) is characterized by slowly progressive early onset dementia with cortical visual dysfunction and disproportionate atrophy of the posterior cortex. CASE REPORT: A 55-year-old right-handed woman developed visuo-spatial impairments that progressed rapidly into cortical blindness over the following 3 months. Neuro-psychological evaluation revealed Gerstmann syndrome and severe constructional impairments with all components of Balint syndrome. However, her memory, insight, and judgment were preserved. Her brain MRI was normal. However, 18F fluorodeoxyglucose positron emission tomography revealed a marked hypometabolism in the bilateral parieto-occipital region. CONCLUSIONS: Although rapid progression of visuo-spatial dysfunction without memory impairment occurred, we considered PCA as well.