The impact of multiple sclerosis on the size and morphology of corpus callosum: an MRI-based retrospective study / Impacto de la esclerosis múltiple en el tamaño y la morfología del cuerpo calloso: un estudio retrospectivo basado en resonancia magnética

SUMMARY: The corpus callosum (CC) includes the majority of fibers linking the two brain hemispheres. Several cross sectional studies showed an association between callosal atrophy and malfunction and neurodegenerative diseases, which may play a role in their pathological manifestations. As a result, the accurate quantification of the corpus callosum is important to have normative values according to sex, age and ethnicity. The purpose of this study is to determine the size of CC in patients suffering from multiple sclerosis, and compare it to CC size in healthyindividuals. Midsagittal size of CC were recorded prospectively from 404 routine MR brain examinations in normal individuals. The internal skull surface was measured to calculate CC/ internal skull surface ratio. Two groups of patients were studied: 200 (100 male /100 female) healthy individuals and 204 (101 males/103 females) with multiple sclerosis (MS). Mean surface area of CC in controls was 6.58±1.04 cm2 and there was no significant difference between males and females (P< 0.627). CC/ internal skull surface ratio was 4.44±0.77 %. MS patients showed a significant decrease in CC size compared to normal controls. Using MR imaging, we measured the mean sizes of the various portions of the CC in normal individuals, in addition to MS patients; these values may provide a useful basis to determine changes occurring in CC structures.

El cuerpo calloso (CC) incluye la mayoría de las fibras que unen los dos hemisferios cerebrales. Varios estudios transversales mostraron una asociación entre la atrofia y el mal funcionamiento calloso y las enfermedades neurodegenerativas, lo que puede desempeñar un papel en sus manifestaciones patológicas. En consecuencia, la cuantificación precisa del cuerpo calloso es importante para tener valores normativos según sexo, edad y etnia. El propósito de este estudio fue determinar el tamaño de CC en pacientes que padecen esclerosis múltiple y compararlo con el tamaño de CC en individuos sanos. El tamaño sagital medio del CC se registró prospectivamente a partir de 404 exámenes cerebrales de RM de rutina en individuos normales. Se midió la superficie interna del cráneo para calcular la relación CC/superficie interna del cráneo. Se estudiaron dos grupos de pacientes: 200 (100 hombres/100 mujeres) sanos y 204 (101 hombres/103 mujeres) con esclerosis múltiple (EM). El área superficial media de CC en los controles fue de 6,58±1,04 cm2 y no hubo diferencia significativa entre hombres y mujeres (P< 0,627). La relación CC/superficie interna del cráneo fue de 4,44±0,77 %. Los pacientes con EM mostraron una disminución significativa en el tamaño de CC en comparación con los controles normales. Usando imágenes de RM, medimos los tamaños medios de las diversas porciones del CC en individuos normales, además de pacientes con EM; estos valores pueden proporcionar una base útil para determinar los cambios que ocurren en las estructuras CC.

Hereditary spastic paraplegia: a clinical and epidemiological study of a Brazilian pediatric population / Paraplegia espástica hereditária: estudo clínico e epidemiológico em uma população pediátrica brasileira

ABSTRACT Aims: To investigate hereditary spastic paraplegia (HSP) in a pediatric Brazilian sample. Methods: Epidemiological, clinical, radiological and laboratory data were analyzed in 35 patients. Results: Simple HSP (HSP-S) was detected in 12 patients, and complicated HSP (HSP-C) was detected in 23 patients. The mean age of onset of symptoms was 2.9 years in HSP-S and 1.6 years in HSP-C (p = 0.023). The disease was more severe in HSP-C. There were no differences in sex, ethnic background, or family history between groups. Intellectual disability was the most frequent finding associated with HSP-C. Peripheral axonal neuropathy was found in three patients. In the HSP-C group, MRI was abnormal in 13 patients. The MRI abnormalities included nonspecific white matter lesions, cerebellar atrophy, thinning of the corpus callosum and the "ear of the lynx sign". Conclusions: In children with spastic paraplegia, HSP must be considered whenever similar pathologies, mainly diplegic cerebral palsy, are ruled out.

RESUMO Objetivo: Investigar paraplegia espástica hereditária (PEH) em uma amostra brasileira de pacientes pediátricos. Métodos: Foram colhidos dados clínicos, epidemiológicos, radiológicos e laboratoriais de 35 pacientes. Resultados: Doze pacientes foram classificados como PEH simples (PEH-S), e 23 como PEH complicada (PEH-C). A média de idade de início dos sintomas foi de 2,9 anos na PEH-S e 1,6 anos na PEH-C (p = 0,023). A doença foi mais grave na PEH-C. Não houve diferença de sexo, etnia e histórico familial entre os dois grupos. Deficiência intelectual foi a associação clínica mais frequente na PEH-C. Neuropatia periférica axonal foi encontrada em três pacientes. A RM foi normal em 13 casos de PEH-C. Anormalidades de RM incluiram alterações inespecíficas da substância branca, atrofia de cerebelo, afilamento de corpo caloso e o "sinal da orelha de lince". Conclusões: PEH deve ser considerada em crianças com paraparesia espástica sempre que descartadas condições patológicas similares, principalmente paralisia cerebral.

White matter volume is decreased in bipolar disorder at early and late stages / O volume da substância branca está reduzido tanto nos estágios iniciais quanto nos estágios avançados do transtorno bipolar

Abstract Introduction: Bipolar disorder (BD) is a debilitating mood condition that affects approximately 1.3% of people worldwide, although some studies report up to 3.9% lifetime prevalence and 4-6% in adults when broad diagnostic criteria are applied. Objective: To compare differences in total white matter (WM), corpus callosum (CC) and total gray matter (GM) volumes in patients with type I BD at early and late stages compared with controls. Methods: Fifty-five subjects were enrolled in this study protocol. The double case-control design included 14 patients with BD at early stage; 15 patients at late stage; and their respective matched controls (14 and 12 subjects). Results: CC and total WM volumes were significantly smaller in patients with BD at early and late stages vs. controls. There was no difference for total GM volume in the early stage group, but in patients at late stage total GM volume was significantly smaller than in controls. The total GM volume reduction in patients at late stage is in agreement with the neuroprogression theory of BD. The reduction of WM volumes in total WM and in the CC at early and late stages supports the possibility that an early demyelination process could occur underlying the clinical manifestation of BD. Conclusion: Our findings may direct to the investigation of WM abnormalities in populations at high risk to develop BD, perhaps as early biomarkers before the overt syndrome.

Resumo Introdução: O transtorno do humor bipolar (THB) é uma condição debilitante que afeta aproximadamente 1,3% das pessoas em todo o mundo, embora alguns estudos relatem uma prevalência acumulada de até 3,9% e de 4-6% em adultos quando os critérios diagnósticos mais abrangentes são aplicados. Objetivo: Comparar as diferenças nos volumes totais de substância branca (SB), corpo caloso (CC) e volume total de substância cinzenta (SC) em pacientes com THB tipo I em estágios iniciais e tardios em comparação com controles. Métodos: Cinquenta e cinco sujeitos foram incluídos neste protocolo de estudo. O desenho de caso com duplo controle incluiu 14 pacientes com THB em estágio inicial; 15 pacientes com THB em fase tardia; e seus respectivos controles correspondentes (14 e 12 sujeitos). Resultados: Os volumes do CC e total de SB foram significativamente menores nos pacientes com THB nos estágios iniciais e tardios vs. controles. Não houve diferença para o volume total de SC no grupo em estágio inicial, mas em pacientes em fase tardia o volume total de SC foi significativamente menor do que nos controles. A redução do volume total de SC em pacientes em fase tardia está de acordo com a teoria da neuroprogressão do THB. A redução dos volumes de SB em SB total e no CC em fases precoces e tardias suporta a possibilidade de que um processo de desmielinização precoce poderia ocorrer subjacente à manifestação clínica de THB. Conclusão: Nossos achados podem direcionar a investigação de anormalidades da SB em populações de alto risco para o desenvolvimento de THB, talvez como biomarcadores precoces antes da síndrome aberta.

Avaliação microestrutural do corpo caloso em pacientes com lúpus eritematoso sistêmico: um estudo por imagem de tensor de difusão pela ressonância magnética / Microstructural evaluation of the corpus callosum in patients with systemic lupus erythematosus: a magnetic resonance diffusion tensor imaging study

Resumo: Determinar as alterações microestruturais do corpo caloso (CC) em pacientes com lúpus eritematoso sistêmico (LES) pelas imagens de ressonância magnética, através da técnica de tensor de difusão (DTI) e determinar associações com as manifestações clínicas e laboratoriais e com o tratamento. Material e métodos: O estudo avaliou 116 pacientes com LES (110 mulheres, média de idade 39,4 anos) e 48 controles saudáveis (40 mulheres, média de idade 35,5 anos). Todos os pacientes preenchiam 4 ou mais critérios classificatórios de LES do Colégio Americano de Reumatologia (ACR) e as manifestações neuropsiquiátricas foram analisadas segundo os critérios do ACR para o acometimento neuropsiquiátrico, classificadas em ativas, inativas ou ausentes na data de inclusão do estudo. Foram avaliadas as manifestações clínicas, laboratoriais e atividade de doença (SLEDAI - Systemic Lupus Erythematosus Disease Activity Index) e índice de dano (SLICC- Systemic Lupus International Collaborating Clinics /ACR). Todos os pacientes e controles foram submetidos ao exame de RM de crânio utilizando a técnica de tensor de difusão (DTI) e os valores de anisotropia fracionada (FA), difusividade média (MD), difusividade axial (AD) e radial (RD) foram calculados. O CC foi segmentado em 5 sub-regiões, segundo classificação validada. Resultados: Os pacientes com LES apresentaram valores significativamente menores de FA (0,629±0,0612 vs 0,6781±0,0154; p<0,001) e significativamente maiores de MD (0,0023±0,0036 vs 0,0010±0,0001; p=0,020), AD (0,0018±0,0001 vs 0,0017±0,0002; p=0,040) e RD (0,0008±0,0006 vs 0,0005±0,0001; p=0,024) quando comparados aos controles. Os pacientes com LESNP ativo quando comparado aos LESNP inativo apresentaram valores menores de FA [(0,590±0,072 vs 0,661±0,021); (0,590±0,072 vs 0,646±0,045); (0,590±0,072 vs 0,678±0,015)] e maiores de MD [(0,0044±0,005 vs 0,0010±0,0001); (0,0044±0,005 vs 0,001±0,001); (0,0044±0,005 vs 0,001±0,0001)], AD [(0,0018±0,001 vs 0,0017±0,00009); (0,0018±0,001 vs 0,0017±0,0002)] e RD que os controles (0,0007±0,002 vs 0,0011±0,0006). O SLEDAI apresentou correlação negativa com FA (r=-0,346; p<0,001) e positiva com MD (r=0,239; p=0,010), AD (r=0,223; p=0,016) e RD (r=0,218; p=0,018). O SLICC apresentou correlação negativa com FA (r=-0,314; p= 0,001) e positiva com MD (r=0,275; p=0,002) e AD (r=0,193; p=0,03). Na avaliação parcelada do CC, observamos comprometimento das parcelas anteriores do corpo caloso nos LESNP ativo quando comparado aos controles. Conclusão: Nosso estudo indica que há comprometimento microestrutural em pacientes com LESNP ativo e em pacientes com atividade sistêmica da doença. Não observamos dano microestrutural nos pacientes com LESNP inativo quando comparados aos LES sem MNP e controle, no CC total. Observamos na análise parcelada do CC que as parcelas anteriores foram as que apresentaram alteração microestrutural quando analisadas individualmente. O estudo DTI pela RM pode fornecer elementos para uma análise mais abrangente da microestrutura da substância branca nos pacientes com LES com MNP(AU)

Abstract: The aim of this study was to determine the microstructural changes of the corpus callosum in patients with systemic lupus erythematosus by magnetic resonance imaging using the diffusion tensor technique (DTI) and to determine associations with neuropsychiatric, clinical and laboratory manifestations and treatment. Material and method: The study evaluated 116 patients with SLE (110 women, mean age 40 years) and 48 healthy controls (40 women, mean age 35.5 years). All patients fulfilled 4 or more American College of Rheumatology (ACR) SLE criteria and neuropsychiatric manifestations (NPM) were analyzed based on the ACR diagnostic criteria for neuropsychiatric impairment, classified as active, inactive, or absent on the date of inclusion of the study. Clinical, laboratory and disease manifestations (SLEDAI) and SLICC (Systemic Lupus International Collaborating Clinics / ACR) were evaluated. All patients and controls underwent cranial MRI examination using diffusion tensor technique (DTI) and fractional anisotropy (FA), mean diffusivity (MD), axial diffusivity (AD) and radial (RA) values were calculated. The CC was segmented into 5 sub-regions, according to the validated classification. Results: Patients with SLE presented significantly lower values of FA (0.629 ± 0.0612 vs 0.67781 ± 0.0154, p <0.001) and significantly higher MD values (0.0023 ± 0.0036 vs 0.0010 ± 0.0001 , p = 0.020), and AD (0.0018 ± 0.0001 vs. 0.0017 ± 0.0002, p = 0.040) and RD (0.0008 ± 0.0006 vs 0.0005 ± 0.0001, p = 0.024 ) when compared to controls. Patients with active NPSLE when compared to inactive NPSLE had lower values of FA [(0.590 ± 0.072 vs 0.661 ± 0.021); (0.590 ± 0.072 vs. 0.646 ± 0.045); (0.590 ± 0.072 vs. 0.678 ± 0.015)] and higher MD [(0.0044 ± 0.005 vs. 0.0010 ± 0.0001); (0.0044 ± 0.005 vs. 0.001 ± 0.001); (0.0044 ± 0.005 vs 0.001 ± 0.0001)], AD [(0.0018 ± 0.001 vs. 0.0017 ± 0.00009); (0.0018 ± 0.001 vs. 0.0017 ± 0.0002)] and RD than the controls (0.0007 ± 0.002 vs 0.0011 ± 0.0006). The SLEDAI had a negative correlation with FA (r = -0.346, p <0.001) and positive with MD (r = 0.239, p = 0.010), AD (r = 0.223, p = 0.016) and RD 0.018). The SLICC had a negative correlation with FA (r = -0.314, p = 0.001) and positive with MD (r = 0.275, p = 0.002) and AD (r = 0.193, p = 0.03). In the partial evaluation of CC, we observed impairment of the anterior portion of the corpus callosum in active NPSLE when compared to controls. Conclusion: Our study indicates that there is microstructural impairment in patients with active NPSLE and in patients with systemic disease activity. We did not observe microstructural damage in patients with inactive NPSLE when compared to SLE without NPM and control, in the total CC. We observed in the split analysis of CC that the previous plots were those that presented microstructural alteration when analyzed individually. The DTI MRI study may provide elements for a more comprehensive analysis of the microstructure of the white matter in patients with SLE with NPM(AU)

Reversible splenial lesion on the corpus callosum in nonfulminant hepatitis A presenting as encephalopathy

Reversible focal lesions on the splenium of the corpus callosum (SCC) have been reported in patients with mild encephalitis/encephalopathy caused by various infectious agents, such as influenza, mumps, adenovirus, Varicella zoster, Escherichia coli, Legionella pneumophila, and Staphylococcus aureus. We report a case of a reversible SCC lesion causing reversible encephalopathy in nonfulminant hepatitis A. A 30-year-old healthy male with dysarthria and fever was admitted to our hospital. After admission his mental status became confused, and so we performed electroencephalography (EEG) and magnetic resonance imaging (MRI) of the brain, which revealed an intensified signal on diffusion-weighted imaging (DWI) at the SCC. His mental status improved 5 days after admission, and the SCC lesion had completely disappeared 15 days after admission.