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nÃo hà / Para analisar a histÃria natural da Esclerose MÃltipla (EM) no estado do CearÃ, Brasil, o autor estuda retrospectivamente 146 pacientes diagnosticados por critÃrios de Poser e / ou McDonald-2010. CasuÃstica e mÃtodos: dados biogrÃficos, clÃnicos e para-clÃnicos obtidos em visitas ambulatoriais e nos surtos. Considera como desfechos de incapacidade os marcos EDSS 4, 6 e 7. Com software estatÃstico R ( RKWard 0.5.3 ) faz anÃlise descritiva, teste exato de Fisher (p < 0,05) e curvas de anÃlise de tempo atà o evento ( Kaplan-Meier). Mais de 75 % dos pacientes sÃo acompanhados durante atà 15 anos. Resultados: EM predomina no sexo feminino (80,82 %); tem inÃcio antes dos 30 anos de idade em 47,9 % dos casos. Pacientes com educaÃÃo de nÃvel superior (33,5 %) representam mais do dobro do esperado na populaÃÃo em geral. Nas avaliaÃÃes inicial e evolutiva predominam os sintomas sensitivos, motores e esfincterianos. Pacientes mais jovens e com evoluÃÃo recorrente â remitente atingem EDSS 4 apÃs maior intervalo de tempo. A proporÃÃo de casos de EM benigna à de 4,7 %. A taxa anualizada de surtos à 0,6. Os dois primeiros surtos da maioria dos pacientes ocorreram nos primeiros 3 anos. O tempo entre 1 e 2 surtos tem relaÃÃo positiva com o tempo para atingir EDSS 4, 6 e 7. A duraÃÃo da doenÃa atà o Ãbito (8 casos, 5,4 %) foi em mÃdia de 14,4 anos. PrevalÃncia de EM no estado do Cearà à estimada em 2,9 / 100.000 habitantes. ConclusÃo: As caracterÃsticas clÃnicas e evolutivas da EM, no estado do Cearà (latitude sul entre 2o 46â e 7o 52â), sÃo semelhantes Ãs observadas mundialmente. / In order to study the natural history of Multiple Sclerosis (MS) in the state of CearÃ, Brazil, the author retrospectively analyzes 146 patients diagnosed according to Poser and/or McDonald-2010 criteria. Cases and methods: biographical, clinical and para-clinical data collected on outpatient visits and at relapses. EDSS scores 4, 6 and 7 used as disability outcomes. Statistical software R (RKWard 0.5.3) used to perform descriptive analysis, Fisher exact test (p< 0,05) and time-to-the-event curves (Kaplan-Meier). Results: over 75 % of the patients followed for as long as 15 years; disease onset before 30 years of age in 47,9 %, with female sex preponderance (80.82 %). Patients with university education (33,5 %) are in high proportion when compared to the general population. Sensory, motor and sphincter complaints are the most common, in both initial and final examinations. Younger patients with relapsing-remitting MS took a longer time to reach EDSS 4. The proportion of benign MS cases was 4, 7 %. Annualized relapse rate was 0,6 . For most patients, the first two relapses took place in the initial three years of illness.Time between 1st. and 2nd relapses bears a positive relationship with time to reach EDSS 4, 6 and 7. Disease duration until death (8 cases , 5,4 %) was an average of 14,4 years. The prevalence of MS in the state of CearÃ, Brazil, is estimated as 2,9 / 100.000 inhabitants. Conclusion: Clinical course of MS in the state of CearÃ, Brazil (south latitudes between 2o 46â and 7o 52â ) is similar to that observed worldwide.

Diss. (sammanfattning) Stockholm : Karol. inst., 2002. / Härtill 5 uppsatser.

Atypical imaging features of multiple sclerosis lesions include size >2 cm, mass effect, oedema and/or ring enhancement. This constellation is often referred to as 'tumefactive multiple sclerosis'. Previous series emphasize their unifocal and clinically isolated nature, however, evolution of these lesions is not well defined. Biopsy may be required for diagnosis. We describe clinical and radiographic features in 168 patients with biopsy confirmed CNS inflammatory demyelinating disease (IDD). Lesions were analysed on pre- and post-biopsy magnetic resonance imaging (MRI) for location, size, mass effect/oedema, enhancement, multifocality and fulfilment of Barkhof criteria. Clinical data were correlated to MRI. Female to male ratio was 1.2: 1, median age at onset, 37 years, duration between symptom onset and biopsy, 7.1 weeks and total disease duration, 3.9 years. Clinical course prior to biopsy was a first neurological event in 61%, relapsing-remitting in 29% and progressive in 4%. Presentations were typically polysymptomatic, with motor, cognitive and sensory symptoms predominating. Aphasia, agnosia, seizures and visual field defects were observed. At follow-up, 70% developed definite multiple sclerosis, and 14% had an isolated demyelinating syndrome. Median time to second attack was 4.8 years, and median EDSS at follow-up was 3.0. Multiple lesions were present in 70% on pre-biopsy MRI, and in 83% by last MRI, with Barkhof criteria fulfilled in 46% prior to biopsy and 55% by follow-up. Only 17% of cases remained unifocal. Median largest lesion size on T2-weighted images was 4 cm (range 0.5-12), with a discernible size of 2.1 cm (range 0.5-7.5). Biopsied lesions demonstrated mass effect in 45% and oedema in 77%. A strong association was found between lesion size, and presence of mass effect and/or oedema (P < 0.001). Ring enhancement was frequent. Most tumefactive features did not correlate with gender, course or diagnosis. Although lesion size >5 cm was associated with a slightly higher EDSS at last follow-up, long-term prognosis in patients with disease duration >10 years was better (EDSS 1.5) compared with a population-based multiple sclerosis cohort matched for disease duration (EDSS 3.5; P < 0.001). Given the retrospective nature of the study, the precise reason for biopsy could not always be determined. This study underscores the diagnostically challenging nature of CNS IDDs that present with atypical clinical or radiographic features. Most have multifocal disease at onset, and develop RRMS by follow-up. Although increased awareness of this broad spectrum may obviate need for biopsy in many circumstances, an important role for diagnostic brain biopsy may be required in some cases.

Diss. (sammanfattning) Stockholm : Karolinska institutet, 2005. / Härtill 6 uppsatser.

Brain atrophy has been used as a marker for disease progression in Multiple Sclerosis (MS). SIENA, an automated tool for measuring brain volume change, was tested to see whether MRI slice thickness and gap presence affect longitudinal atrophy measures. Isotropic global scan-rescan images were used to simulate 3 mm and 5 mm axial slice thicknesses with 1 and 2mm gaps, respectively. SIENA remained accurate and precise with increasing slice thickness and gap presence. Furthermore, symmetric pre-registration was crucial for scans with larger slice-thickness and gaps. / SIENA was used to observe atrophy in children who have experienced a Clinically Isolated Syndrome (CIS) of the type leading to MS (CIS-MS). Brain atrophy was present within the first three months after a CIS event, and then subsided over the rest of the year. If the first acute episode was excluded, there was no significant difference in atrophy rates between the CIS-MS group and the CIS group, and no significant difference between those with T2-weighted brain lesions versus those who had none.

Thesis (Ph.D.)--University of Florida, 2005. / Typescript. Title from title page of source document. Document formatted into pages; contains 123 pages. Includes Vita. Includes bibliographical references.

Thesis (Ph.D.)--Uniformed Services University of the Health Sciences, 2006 / Typescript (photocopy)

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Introdução: perda da sensibilidade axial é bem descrita nas polineuropatias axonais comprimento-dependentes (PACD). O padrão da perda de sensibilidade axial nas polineuropatias desmielinizantes adquiridas (PDA) é menos reconhecido na prática clínica e tem sido descrito como um padrão não-comprimentodependente. Nas PDAs, a perda de sensibilidade axial pode, somada às alterações da sensibilidade nos membros, envolver as regiões anteriores do tronco, da face, do couro cabeludo e do períneo. Eventualmente, pode ocorrer apenas no eixo do corpo sem o envolvimento dos membros. O padrão da perda de sensibilidade axial observado ao exame físico neurológico pode auxiliar no diagnóstico diferencial entre as PACDs e as PDAs. Objetivos: analisar as diferentes apresentações clínicas das alterações da sensibilidade axiais nos indivíduos acometidos por neuropatias desmielinizantes adquiridas. Analisar a resposta de curto prazo das alterações da sensibilidade nas porções axiais e suas relações com o gênero, idade, diagnóstico e com a presença ou não e comorbidades. Analisar se a resposta das alterações de sensibilidade axiais acompanham a resposta da força muscular ao tratamento, a fim de agilizar a avaliação clinica, o início do tratamento e o estabelecimento de prognóstico, visto não haver dados prévios na literatura médica a este respeito. Métodos: trata-se de estudo observacional analítico por meio de coorte retrospectiva não-controlada. Foram revisados 460 prontuários médicos de indivíduos com possíveis diagnósticos de PDAs. Dentre os 460, foram selecionados 284 prontuários de indivíduos que preencheram critérios diagnósticos recomendados para polirradiculoneuropatia inflamatória desmielinizante crônica (PIDC) ou para síndrome de Guillain-Barré (SGB) na sua forma de polirradiculoneuropatia inflamatória 5 desmielinizante aguda (PIDA). Registros médicos de indivíduos com comorbidades que poderiam cursar com neuropatias axonais, como a diabetes mellitus, por exemplo, não foram incluídos para avaliação. Deste modo, foram selecionados para a análise estatística 49 indivíduos. Após a revisão, foram analisadas a prevalência e as alterações clínicas da sensibilidade superficial, tátil e dolorosa, e força muscular que ocorreram durante e após o tratamento, a fim de avaliar a influência de dados demográficos, tais como a idade, o gênero, a presença ou não de comorbidades e o diagnóstico dos indivíduos sobre as variáveis clínicas acima descritas. Resultados: alterações da sensibilidade axial ocorreram em 64 (22,5%) dos 284 indivíduos que preencheram os critérios diagnósticos de PIDC e SGB. Destes, 49 (17,25%) foram selecionados por apresentarem alteração da sensibilidade axial, em algum momento da doença, e não preencherem os critérios de não-inclusão. 31 indivíduos (63,26%) eram do gênero masculino e 18 (36,74%) do gênero feminino, com mediana de idade em 45 anos (variando de 11 a 75 anos). Alteração da sensibilidade axial foi observada acometendo a região central do abdome (n=48, 97,9%), couro cabeludo (n=11, 22,4%), porção central da face (n=18, 36,7%), do dorso (n=3, 6,2%), da linha média anterior do tórax (n=2, 4,1%) ou do períneo (n=4, 8,2%). A perda de sensibilidade nos membros acometeu as regiões distais desses, embora o padrão da perda de sensibilidade não tenha ocorrido de forma comprimento-dependente. Após o primeiro curso de tratamento (com imunoglobulina humana ou plasmaférese nos casos de SGB, e corticoide, imunoglobulina humana ou plasmaférese, nos casos de PIDC), 29 (59,18%) dos indivíduos apresentaram melhora da perda da sensibilidade axial e 32 (61,5%) obtiveram melhora na força muscular. Em adição aos critérios clínicos recomendados, atualmente, para o diagnóstico destas doenças, a ausência do padrão comprimento-dependente de acometimento da sensibilidade pode auxiliar no diagnóstico clínico de ambas PDAs avaliadas. Conclusões: perda de sensibilidade axial ocorre em cerca de um a cada 5 indivíduos acometidos de PDAs. A ausência de relação da perda de sensibilidade com o comprimento do nervo acometido pode ser de suporte ao diagnóstico das neuropatias desmielinizantes adquiridas. A resolução das alterações da sensibilidade axial foi observada em 60,8% dos indivíduos após o tratamento. Esta melhora foi mais expressiva em casos de indivíduos com SGB (com 80% de melhora) do que em indivíduos com PIDC (com 33,3% de melhora). Por outro lado, indivíduos com menos de 45 anos de idade e acometidos por SGB apresentaram 6 a pior resposta ao tratamento. Assim, recomenda-se a busca ativa e sistemática por alterações da sensibilidade axial, visto que não são alterações frequentemente relatadas pelos pacientes no contexto do atendimento medico / Background: axial sensory loss (ASL) is well known in length-dependent axonal polyneuropathies (LDAP). The pattern of ASL in acquired demyelinating polyneuropathies (ADP) is less known in clinical practice but it was referred as a nonlength dependent. In ADPs, ASLs may, in addition to the upper and lower limbs, involve the anterior region of the trunk, face, scalp, and perineum. Eventually, it occurs only in the body axis without limbs involvement. The ASLs pattern could help in the differential diagnosis between ADPs and LDAP. Objectives: this study aims to analyze the different clinical presentation of the ASL in ADP patients; to analyze the clinical response in a short time to treatment of ASL and their relation with the gender, age, diagnosis and the presence, or not, of comorbidities; and to analyze the relation between the improvement of the ASL\'s and the improvement of the muscular weakness after treatment, in order to expedite the clinical evaluation, the beginning of the treatment and the evaluation of the prognosis, since there is no previous data in the medical literature in this regard. Methods: it is a observational, analytical and uncontrolled study using a retrospective cohort. Were reviewed 460 medical records with possible ADPs and selected 284 with the recommended diagnostic criteria for chronic inflammatory demyelinating polyneuropathies (CIDP) or Guillain-Barré syndrome (GBS), in acute inflammatory demyelinating polyneuropathy (AIDP) form. Medical records of patients with comorbidities that could course with neuropathies, like diabetes mellitus, were excluded. Thus, 49 subjects were enrolled for the statistical analysis. We checked the prevalence and the changes that happened with ASL (pain and tactile sensitivity), during and after treatment and then we analyzed the influence of demographic data, such as age at the onset, gender, diagnosis and comorbidities over the clinical variables described above. Results: partial ASL occurred in 64 8 (22,5%) of the 287 subjects who met the diagnostic criteria for CIDP and SGB. Of there, 49 subjects (17,25%) were accepted for the present study because they had ASL and did not fulfilled the non-inclusion criteria. 31 subjects (63,26%) were male and 18 (36,74%) were female with median age of 45 years old (11 - 75 years old). ASLs were found in the periumbilical region of the abdomen (n=48, 97,9%), scalp (n=11, 22,4%), central portion of the face (n=18, 36,7%), dorsum (n=3, 6,2%), anterior portion of the thorax (n=2, 4,1%) and perineum (n=4, 8,2%). The sensory loss in the patients\' limbs were very distal, though non-length-dependent. After the first course of treatment (intravenous human immunoglobulin - IgIV - or plasmapheresis for GBS, and steroids or IgIV for CIDP), 29 (59,18%) subjects showed improvement of ASL after treatment and 32 (61,5%) got a better motor strength. In addition to the clinical criteria recommended for diagnosis of CIDP or GBS, the absence of length dependency also supported the clinical diagnosis of both ADPs. Conclusions: ASLs occur in about one among five patients with ADPs and its non-length-dependent pattern can support their diagnosis. The absence of relationship between the sensory loss and the length of the nerve can be supportive to the diagnosis of ADPs. In 60,8% of the subjects, resolution of ASL were observed after the treatment. This was more expressive in GBS than in CIDP (80% after 33,3%). On the other hand, subjects with GBS younger than 45 years old showed poorer response to treatment. Yet, it is recommended to look for ASL since it is not frequently reported complaints by patients in the context of medical care