To assess whether the month of birth in different latitudes of South America might influence the presence or severity of multiple sclerosis (MS) later in life.
Neurologists in four South American ...countries working at MS units collected data on their patients' month of birth, gender, age, and disease progression.
Analysis of data from 1207 MS patients and 1207 control subjects did not show any significant variation in the month of birth regarding the prevalence of MS in four latitude bands (0-10; 11-20; 21-30; and 31-40 degrees). There was no relationship between the month of birth and the severity of disease in each latitude band.
The results from this study show that MS patients born to mothers who were pregnant at different Southern latitudes do not follow the seasonal pattern observed at high Northern latitudes.
Some studies have suggested an inflammatory role of the choroid plexus (CP) in the pathophysiology of multiple sclerosis (MS), but mainly in adult patients. We aimed to evaluate clinical and MRI ...parameters in patients with pediatric-onset multiple sclerosis (POMS). We included 10 patients with POMS and 16 healthy controls (HC), evaluating clinical and neuroimaging variables (cerebral cortex, CP, deep gray matter structures, and demyelinating lesions). Most patients were girls (80%), with a mean age of 15.3 years. POMS individuals had a higher CP volume (
p
= 0.012) and lower thalamic volume (
p
= 0.038) compared to HC. This study shows an enlargement of the CP and lower thalamic volume in POMS patients compared to HC.
Sensory neuronopathies (SN) are peripheral nervous system disorders associated with degeneration of dorsal root ganglion neurons. Despite the evidence of a defective proprioceptive sensory input in ...SN,the prominent gait and truncal ataxia raises the question of a concomitant involvement of the cerebellum. OBJECTIVE: To evaluate cerebellar atrophy in SN. METHOD: We analyzed MRI-based volumetry of anterior lobe (paleocerebellum) and total cerebellum in patients with non-paraneoplastic chronic SN and compared to age- and gender-matched controls. RESULTS: Cerebellum and anterior lobe MRI volumetry were performed in 20 patients and nine controls. Mean anterior lobe and cerebellar volume were not statistically different. Three patients (15%), however, had an abnormal anterior lobe and cerebellar volume index (values outside 2.5 standard deviations). One of them also had a specific atrophy of the anterior lobe. All these patients had infectious or dysimmune associated SN. CONCLUSION: Cerebellar atrophy is infrequently associated with SN, but can be found in some patients with SN related to infectious or immune mediated conditions. It can be more prominent in the anterior lobe and may contribute to the ataxia seen in these patients.
Neuronopatias sensitivas (NS) são distúrbios do sistema nervoso periférico associados com a degeneração dos neurônios do gânglio da raiz dorsal. Apesar da evidência de um defeito das aferências proprioceptivas, a ataxia proeminente da marcha e do tronco levanta a questão de uma participação concomitante do cerebelo. OBJETIVO: Avaliar a atrofia cerebelar nas NS. MÉTODO: Foi analisada volumetria pela ressonância magnética do cerebelo total e do lobo anterior (paleocerebelo) em pacientes com NS crônica não-paraneoplásica e comparada a controles com idades e sexos correspondentes. RESULTADOS: A volumetria do cerebelo e lobo anterior foi realizada em 20 pacientes e nove controles. As médias do volume cerebelar e do lobo anterior não foram estatisticamente diferentes. Três pacientes, entretanto, tiveram um valor anormal do índice de volume cerebelar e do lobo anterior (valores fora de 2,5 desvios-padrão). Um deles tinha inclusive uma atrofia específica do lobo anterior. Todos esses pacientes tinham NS associada a doenças infecciosas ou disimunes. CONCLUSÃO: Atrofia cerebelar é raramente associada com SN, mas pode ser encontrada em alguns pacientes com NS relacionada com doenças infecciosas ou imunológicas. Esta atrofia pode ser mais proeminente no lobo anterior e pode contribuir para a ataxia observada nestes pacientes.
•Persons with MS have frequent impairments in daily occupations.•Cognitive/physical disability and MS-lesion burden are related to work-restriction.•Along with general cognition, social cognition ...plays a role in financial capacity.•Gray-matter atrophy also influence driving abilities.•Cognitive and brain reserve can help retain some of these daily occupations.
Multiple Sclerosis (MS) can impact performance of daily occupations in both relapsing-remitting (RRMS) and secondary-progressive (SPMS) clinical courses. Work force participation decreases with advancing physical disability but the influence of non-motor factors, neuroimaging, and reserve have been scarcely investigated. We aimed to evaluate MRI, clinical, and cognitive (social and general) factors associated with impairment in different daily occupations and address whether cognitive and brain reserve have a positive impact on the ability to maintain these activities.
We prospectively enrolled persons with MS (PwMS) who underwent clinical examination (Expanded Disability Status Scale – EDSS; Timed 25-Foot Walk Test - T25FW; and the Nine Hole Peg Test - 9HPT), general neuropsychological assessment (Brief Repeatable Battery of Neuropsychological Tests – BRBN, including the Symbol Digit Modalities Test - SDMT), social cognition evaluation (Reading the Mind in the Eyes Test), cognitive reserve questionnaire, and MRI (FreeSurfer). We also enrolled healthy subjects for comparison as a control group. Daily occupations (employment, money management, and driving abilities) were assessed in all individuals with questionnaires.
We included 62 PwMS (32 RRMS and 30 SPMS; mean age 42.8 years; median educational time 12.75 years) and 67 controls (mean age 39.7; median educational time 12.0 years) which were similar regarding demographics, education, and socioeconomic status (p > 0.1). Most PwMS (67.7%) had work-restrictions. They also reported fewer money management and driving abilities than controls (p < 0.001). Work-restriction was associated with physical disability (p = 0.006), SDMT and BRBN performance (p = 0.035 and p = 0.031, respectively), and T2-lesion volume (p = 0.022), with large effect sizes (d > 0.75). After hierarchical linear regression, money management was associated with hand dexterity, general and social cognition, and cognitive reserve (p < 0.03). Variables associated with driving abilities included fatigue, verbal fluency, striatum volume, and brain reserve (p < 0.05).
PwMS have more frequent work-restrictions and impairment in money management and driving abilities compared to controls. Cognitive function, physical disability, and MS-lesion burden are strongly associated with work-restriction. Social cognition can also influence financial capacity. Cognitive and brain reserve can help retain some of these daily occupations.
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Primitive reflexes and cognitive function Damasceno, Alfredo; Delicio, Adriane M; Mazo, Daniel F C ...
Arquivos de neuro-psiquiatria,
09/2005, Letnik:
63, Številka:
3A
Journal Article
Recenzirano
Odprti dostop
Data on the prevalence of primitive reflexes (PR) in adulthood, their pathological significance and relationship to age and cognition are controversial.
To study the relationship between PR and ...cognition in 30 patients with probable Alzheimer's disease (AD) and 154 control subjects.
Diagnosis of probable AD was based on DSM-IV, NINCDS-ADRDA, and CAMDEX criteria. Primitive reflexes were quantified from zero (absent) to 1 (mild) or 2 (markedly present). The Cognitive Abilities Screening Instrument-Short Form (CASI-S) was used to evaluate registration, temporal orientation, verbal fluency and recall. A drawing test was added.
Most frequent PR among demented and controls were suck (77% and 62%, respectively) and snout (60% and 27%), followed by glabellar (30% and 19%), paratonia (37% and 5%), and palmomental (23% and 5%). None of controls had more than three PR. Frequency of PR tended to increase with age and cognitive deterioration. Grasp and Babinski responses were found only in dementia patients. Primitive reflexes were not correlated with each other, except snout with suck, and snout with glabellar reflex.
The finding of grasp and Babinski sign, or the presence of more than three primitive signs, particularly the combination of paratonia, snout, suck, and palmomental reflexes strongly suggests brain dysfunction, especially when these signs are marked and accompanied by deficits in orientation, recall, verbal fluency, and constructional praxis.
•The definition of cognitive progression is not yet clear.•Fixed cutoffs (e.g. 4-point) may not address variability at the individual level.•Most patients classified with worsening using fixed ...cutoffs had later improvement.•Using a regression-based cutoff, subsequent improvement was noticeably reduced.•These data raise concerns about how we are defining cognitive worsening so far.
Although cognitive evaluation has been incorporated in recent MS clinical trials, the definition of cognitive progression is not clear and recent data are questioning the 4-point cutoff using the SDMT at the individual level. We aimed to evaluate the behavior of cognitive performance over time using different cutoffs.
Cognitive performance over six years was analyzed in a cohort of 42 relapsing-remitting MS patients and 30 controls using verbal/visual memory and information processing speed tests. Fixed cutoffs were: 10% and 20% change (all tests) and a 4- and 8-point change (SDMT). The relative cutoff established by regression-based models was a 1SD change.
The distributions of “worsening”, “stability”, and “improvement” showed low concordance rates across the cutoffs (p < 0.001 for most comparisons). Most patients classified with worsening initially using fixed cutoffs had subsequent improvement in all cognitive tests, yielding a low sensitivity to predict later cognitive worsening. Using the relative cutoff, the proportion of patients with subsequent improvement was noticeably smaller.
Fixed cutoffs classify a high proportion of patients with cognitive improvement. Most patients categorized with worsening initially presented subsequent improvement. Instead, the relative cutoff generally had a better performance. These data raise concerns about how we are defining cognitive worsening so far, especially at the individual level.
•PMS had more frequent impairment in combined cognitive domains compared to RRMS.•PMS had a higher prevalence of verbal memory and verbal fluency deficits.•Between-group cognitive differences were ...not significant after controlling for EDSS.•Worse cognitive performance was associated with increased physical disability.•Thalamic and T1-lesion volumes were consistent MRI predictors of cognitive disability.
Cognitive impairment (CI) is present in all stages and subtypes of multiple sclerosis (MS). However, the majority of studies examined relapsing-remitting (RRMS) patients, and did not address cognitive phenotyping. Is still not clear whether patients with progressive MS (PMS) have a distinct pattern of CI compared to RRMS. In addition, there is conflicting data regarding the correlation between clinical and cognitive disability.
To investigate the differences of CI between PMS and RRMS patients, evaluating cognitive phenotypes. We also aimed to analyze the association between physical and cognitive disability with MRI measures of grey-matter atrophy and lesion burden.
Thirty patients with PMS and twenty-four with RRMS underwent neurological, neuropsychological (BRB-N, Boston Naming, and Tower of London), and MRI assessments (3T). Brain volume evaluations were performed using FreeSurfer. Principal Components Analysis on neuropsychological yielded six principal cognitive domains. Cognitive deficits were classified according to three categories: no CI, impairment in isolated cognitive domain, or impairment in combined domains.
In the overall sample, the most frequently impaired cognitive domains were information processing speed (IPS) and visual memory. PMS patients had a higher prevalence of verbal memory and verbal fluency deficits, and more frequent impairment in combined cognitive domains compared to RRMS individuals. After multivariable regression analysis with clinical variables, EDSS was associated with most cognitive domains. Nevertheless, after including T1-lesion volume in the model, it was the most consistent predictor of cognitive performance. To further analyze the interaction between EDSS and T1-lesions, we performed GLM analysis with EDSS and T1-hypointense lesion volume as covariates, and T1-lesion volume adjusted better the model for verbal memory (p = 0.013), IPS (p = 0.021) and total number of impaired cognitive domains (p = 0.021).
RRMS and PMS patients tend to have a similar neuropsychological profile in general, but the extent of CI was greater in PMS patients. Worse cognitive performance was associated with increased physical disability, but this correlation was no longer significant after controlling for T1-lesion volume, suggesting that the underlying MS pathology might be involved in this relationship. Thalamic and T1-lesion volumes were the most consistent MRI predictors associated with cognitive disability.
Abstract Background The pathological mechanisms underlying cognitive dysfunction in multiple sclerosis (MS) are not yet fully understood and, in addition to demyelinating lesions and gray-matter ...atrophy, subclinical disease activity may play a role. Objective To evaluate the contribution of asymptomatic gadolinium-enhancing lesions to cognitive dysfunction along with gray-matter damage and callosal atrophy in relapsing-remitting MS (RRMS) patients. Methods Forty-two treated RRMS and 30 controls were evaluated. MRI (3 T) variables of interest were brain white-matter and cortical lesion load, cortical and deep gray-matter volumes, corpus callosum volume and presence of gadolinium-enhancing lesions. Outcome variables included EDSS, MS Functional Composite (MSFC) subtests and the Brief Repeatable Battery of Neuropsychological tests. Cognitive dysfunction was classified as deficits in two or more cognitive subtests. Multivariate regression analyses assessed the contribution of MRI metrics to outcomes. Results Patients with cognitive impairment (45.2%) had more cortical lesions and lower gray-matter and callosal volumes. Patients with subclinical MRI activity (15%) had worse cognitive performance. Clinical disability on MSFC was mainly associated with putaminal atrophy. The main independent predictors for cognitive deficits were high burden of cortical lesions and number of gadolinium-enhancing lesions. Conclusions Cognitive dysfunction was especially related to high burden of cortical lesions and subclinical disease activity. Cognitive studies in MS should look over subclinical disease activity as a potential contributor to cognitive impairment.
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