ABSTRACT
Background: Compared with the Western population, central demyelinating disorders are relatively rare while the data on the prognostic value of autoantibodies together with clinical characteristics and cognitive dysfunction has rarely been explored in neuromyelitis optica (NMO) and multiple sclerosis (MS). Methods: Nineteen patients with MS and 14 with NMO underwent clinical profiling and cognitive assessment. According to serology tests, they are divided into four subgroups for further analysis. Results: There was higher frequency of aquaporin-4 immunoglobulin G. sero-positivity (64.3% vs. 10.5%; p=0.003) and antinuclear antibodies (ANA) and/or antibodies to extractable nuclear antigens (anti-ENA) in NMO compared to MS (42.9% vs. 5.2%; p=0.026). The presence of anti-ENA represented a unique clinical phenotype, with longer segment of myelitis (p=0.049), female preponderance, and an inverse correlation between age-of-onset and annual relapse rate (ρ= -0.88, p=0.021). Among patients with anti-ENA positivity, comprehensive serology panels revealed Sjögren’s syndrome A antibodies as the most common (83%), in contrast to limited clinical documentation of Sjögren’s syndrome (16%). There was no significant difference in cognitive assessment by anti-ENA status. MS and NMO represent two different serologic entities. Conclusions: Anti-ENA may have prognostic value for its linkage to a unique clinical phenotype, which has longer initial segment of myelitis, female preponderance, and higher annual relapse rate on earlier age-of-onset, but has limited clinical impact on cognition. Further studies are warranted to investigate whether anti-ENA represents an epiphenomenon of myelitis or simply a systemic inflammatory state.
ABSTRACT
Background:Reports on the aquaporin-4 immunoglobulin G (AQP4-IgG) status for cognitive performance and neuroimaging correlations are limited in neuromyelitis optica (NMO) and multiple sclerosis (MS) literature. Methods: Cognitive results of 19 MS and 15 NMO patients were compared with 47 agematched controls. Apparent diffusion coeffi cient (ADC) values were used to delineate gray matter and white matter damages and correlate with neuropsychological results. Results: Verbal memory test showed signifi cant differences between MS and NMO in the late registration, early and delay recall (p<0.05), while their retention rates were even. In MS, ADC values were signifi cantly elevated in the dorsolateral prefrontal and occipital gray matter which was in contrast with NMO group that showed elevation in the dorsolateral prefrontal gray matter and parieto-occcipital white matter. AQP4-IgG status exerted a limited effect on ADC values and neuropsychological results. Conclusions: Verbal memory test might be helpful in differentiating NMO and MS. ADC values can be used as a surrogate marker for tissue injury in NMO and MS since they were in line with the cognition scores. Anatomical regions with elevated ADC values were different in NMO and MS.
ABSTRACT
Exertional headache is one entity of Valsalva maneuver-related headache syndrome. It is usually idiopathic, but has occasionally been reported to be associated with secondary causes. However, central nervous system infection has not been mentioned before. We encountered a young man who suffered an isolated exertional headache and was found to have an active varicella-zoster virus central nervous system infection without typical intracranial hypertension or outfl ow obstruction. Intracranial vasoconstriction was detected during headache when the patient performed acute lifting or heavy exertion. The fi ndings in this patient suggest a specifi c relationship between varicella-zoster virus-related vasculopathy and exertional headache from other Valsalva maneuver-related headache syndrome