Cheiro-Oral Syndrome: A Clinical Analysis and Review of Literature

PURPOSE: After a century, cheiro-oral syndrome (COS) was harangued and emphasized for its localizing value and benign course in recent two decades. However, an expanding body of case series challenged when COS may arise from an involvement of ascending sensory pathways between cortex and pons and terminate into poor outcome occasionally. MATERIALS AND METHODS: To analyze the location, underlying etiologies and prognosis in 76 patients presented with COS collected between 1989 and 2007. RESULTS: Four types of COS were categorized, namely unilateral (71.1%), typically bilateral (14.5%), atypically bilateral (7.9%) and crossed COS (6.5%). The most common site of COS occurrence was at pons (27.6%), following by thalamus (21.1%) and cortex (15.8%). Stroke with small infarctions or hemorrhage was the leading cause. Paroxysmal paresthesia was predicted for cortical involvement and bilateral paresthesia for pontine involvement, whereas crossed paresthesia for medullary involvement. However, the majority of lesions cannot be localized by clinical symptoms alone, and were demonstrated only by neuroimaging. Deterioration was ensued in 12% of patients, whose lesions were large cortical infarction, medullary infarction, and bilateral subdural hemorrhage. CONCLUSION: COS arises from varied sites between medulla and cortex, and is usually caused by small stroke lesion. Neurological deterioration occurs in 12% of patients and relates to large vessel occlusion, medullary involvement or cortical stroke. Since the location and deterioration of COS cannot be predicted by clinical symptoms alone, COS should be considered an emergent condition for aggressive investigation until fatal cause is substantially excluded.

Factors Affecting the Designation of Cerebrovascular Diseases as Work-Related in Administrative Litigation

The purpose of this study was to identify factors that could be used as standardized criteria for evaluating occupational diseases in initial assessments or requests for examination. Using 100 administrative litigation cases on the work-relatedness of cerebrovascular diseases (CVDs) by the Seoul Branch of the Korea Labor Welfare Corporation (KLWC) from 1997 to 2002, we estimated the relationship between the investigated variables and designation of the work-relatedness of the CVD. As for the age, the odds ratio of the acceptance rate of a case as work-related in subjects over 60 yr of age was 0.08 (95% CI, 0.01-0.75), which was compared to subjects under 30 yr of age. Regarding working hours, the odds ratio of the acceptance rate of a case as work-related in CVDs in those over 56 hr was 9.50 (95% CI, 1.92-47.10) when compared to those less than 56 hr. As for the benefit type, the odds ratio of the acceptance rate of a case as work-related in medical benefits was 5.74 (95% CI, 1.29-25.54), compared to survivor benefits. As for the criteria for defining situations as work overload, the odds ratio of the acceptance rate of a case as work-related in injured workers was 12.06 (95% CI, 3.12-46.62), compared to that in non-injured workers. Our findings show that the criteria for defining situations of work overload played an important role in assessing the work-relatedness of CVDs in administrative litigation, and it is necessary to make the scientific evidence on judgement of workrelatedness on overwork.

Race, gender and stroke subtypes mortality in São Paulo, Brazil

Stroke mortality rates have a discrepant distribution according to socioeconomic variables as social exclusion in Brazil. Recently, data from race has been available from the official health statistics considering five categories: White, Mixed, Black, Asian and Native. We addressed in the city of São Paulo, Brazil, an analysis of cerebrovascular mortality according to race (excluding Asian and Native due to small number of events) and gender during 1999-2001 for people aged 30 to 79 years-old. For all cerebrovascular diseases, age-adjusted mortality rates (x 100,000) for men were higher for Black (150.2), intermediate for Mixed (124.2) and lower for White (104.5) people. These gradient patterns were similar for all stroke subtypes, except for subarachnoideal hemorrhage in which no differences were detected. For women, the rates were lower compared to men and the same pattern was observed among Black (125.4), Mixed (88.5) and White (64.1) women. Compared to White men, the risk ratio of Black men was 1.4. However, compared to White women, the risk ratio for Black women was 2.0. Concluding, there is a significant gradient of stroke mortality according to race, mainly among women.

As taxas de mortalidade pela doença cerebrovascular apresentam distribuição diferenciada de acordo com variáveis socioeconômicas. Informação sobre raça é nova no sistema de informação de mortalidade do Ministério da Saúde. Na cidade de São Paulo foi verificada entre três categoria de raça - branca, parda e negra - a taxa específica de mortalidade nos anos de 1999-2001 para pessoas entre 30 e 79 anos. Para o conjunto das doenças cerebrovasculares as taxas de mortalidade ajustadas para idade (x 100.000) para homens foram maiores entre os negros (150,2), intermediária para os pardos (124,2) e menor para brancos (104,5). Esse gradiente foi o mesmo para todos os subtipos, excluindo a hemorragia subaracnoídea. Para as mulheres, as taxas foram menores quando comparada aos homens e, o mesmo padrão foi observado para negras (125,4), pardas (88,5) e brancas (64,1). A razão de risco para homens negros quando comparado aos brancos foi 1.4, mas entre as mulheres negras e as brancas foi o dobro. Concluindo, houve um gradiente significativo da mortalidade cerebrovascular de acordo com raça, principalmente entre mulheres.

Trends of stroke subtypes mortality in Sao Paulo, Brazil (1996-2003)

O declínio da mortalidade pela doença cerebrovascular no Brasil é conhecido, porém há poucos dados sobre a evolução temporal dos dois principais subtipos, a hemorragia parenquimatosa e o infarto cerebral. As modificações temporais dos subtipos de doença cerebrovascular foram estudadas na cidade de São Paulo entre 1996 e 2003 por gênero e faixa etária decenal entre os 30 e 79 anos. Para os homens detectou-se redução anual para todo os tipos (-3,9%), para hemorragia parenquimatosa (-3,0%), para infarto cerebral (-2,7%) bem como para os casos mal definidos (-7.4%). Para as mulheres somente houve variação significativa para todos os tipos da doença cerebrovascular (-3,3%) e para os casos mal definidos (-12%). Concluindo, as taxas de doença cerebrovascular estão em queda, porém entre as mulheres devido à melhoria do diagnóstico clínico houve migração de casos mal definidos para casos bem definidos. Devido a isso não foi possível detectar declínio nas taxas de mortalidade pelos subtipos de doença cerebrovascular.

Posibles hallazgos neurorradiológicos en pacientes con hipertensión arterial / Possible neuroradiological findings in patients with arterial hypertension

La hipertensión arterial (HA) está asociada a una variedad de diferentes enfermedades neurológicas de orígen vascular, las cuales pueden ser bien evaluadas con diferentes y avanzados métodos de neuroimágenes. A continuación se analizará brevemente, mostrando algunos ejemplos, diferentes entidades asociadas a la HA que afectan al cerebro, como la leukoaraiosis, micro-hemorragias cerebrales, infartos lacunares, enfermedad de grandes vasos cerebrovasculares, rescate del infarto cerebral agudo así como también se revisarán ejemplos de disección arterial, aneurismas cerebrales y eclampsia. Por último, hay interesantes nuevos trabajos sobre la compresión neurovascular ventrolateral de la médula oblongata como causante de la HA.

Vein of Galen malformations: review.

Vein of Galen malformations are unique congenital malformations of the cerebral vasculature that result in persistence and 'aneurysmal' dilatation of the venous structures. The varied clinical presentations and their distinctive and complex angioarchitecture make it important for the caring physician to understand their embryological and pathophysiological aspects. Management of these lesions--both in the neonatal period and at the time of definitive intervention, is challenging. Considering the rarity of these lesions, there are very few studies that have been able to compare the results of different techniques in the management. Continuing developments in the diagnostic as well as interventional aspects during the last two decades have radically changed the management of these lesions. Antenatal diagnosis and referral to a center with facilities for advanced neonatal cardiac care as well as for interventional neuroradiological therapy can go a long way in improving the prognosis in these children.

Hallazgos angiotomográficos en pacientes con diagnóstico clínico de enfermedad vascular cerebral / Angiotomography in patients with cerebral vascular disease

Se describe, retrospectivamente los hallazgos angiotomográficos en 58 pacientes con diagnóstico de enfermedad vascular cerebral consistentes en isquemia cerebral transitoria, insuficiencia vértebrobasilar, ictus completo, alteraciones visuales y cefalea. Las ventajas de este procedimiento son su rapidez, alta sensibilidad para detectar placas de ateroma calcificadas y excelente demostración de la anatomía vascular intra y extracraneal. La aterosclerosis (31 por ciento) y los infartos lacunares (21 por ciento) fueron los hallazgos más comunes sobrre todo en pacientes mayores a los 50 años de edad. El 20 por ciento de los estudios no presentaron anormalidades, 8 por ciento correspondieron a aneurismas y el restante 20 por ciento correspondió a infartos crónicos, trombosis arterial, vasculitis, angiomas venosos, lesión selar y hemorragia subaracnoidea. Por último, considerando únicamente los estudios de cráneo (50 pacientes) los aneurismas saculares a nivel del polígono de Willis fueron el 12 por ciento

Factores de riesgo cerebrovacular y cardiovascular en el medio rural de la zona semiárida, Venezuela / Factors of risk to cerebrovascular and cardiovascular disease in the semiarid zona, Venezuela

Los pobladores de la zona semiárida del Estado Falcón (Municipio Democracia (se caracteria por una elevada consanguinidad entre sus integrantes, esta particularidad podría influir en un mayor riesgo a las enfermedades cerebrovasculares y cardiovasculares. Con el propósito de establecer los factores que afectan a esta población se diseño un estudio univariante de campo de un universo de 5407 personas y se obtuvo una muestra aleatoria y estratificada en 17 caseríos del municipio de 540 individuos; evaluados en una encuesta individual y domiciliaria. Se incluyeron en el estudio 482 personas en una relación 1:1 hombre-mujer, 263 adultos, 61,9 por ciento pertenecen a la clase obrera según Graffar, 64,3 por ciento consumen grasa de origen animal (> 3 días a la semana), 46,4 por ciento no consumen pescado, 25 por ciento fuman y consuman alcohol simultáneamente, 52,4 por ciento tienen constumbres sedentarias, 15,2 por ciento de los adultos padecen de hipertención arterial. Los resultados indican porcentajes inferiores al promedio nacional que podría explicar una probable resistencia a estas enfermedades lo cual abre la posibilidad de estudios epidemiológicos y genéticos en esta zona para establecer el papel de la heredabilidad en el riesgo

Accidente vascular encefálico, hipertensión arterial y ritmo circadiano / Stroke hypertension and circadian rhythm

Con el fin de estudiar un posible ritmo circadiano en la hora de presentación de los accidentes vasculares encefálicos (AVEs) y la influencia que en el tiene la hipertensión arterial, se analizaron 144 pacientes, ingresados al Hospital A Nef entre enero de 1988 y julio de 1992. De los AVEs, el 72 por ciento eran isquémicos y el 28 por ciento hemorrágicos. Estaban asociados a hipertensión arterial el 79 por ciento de ellos. Para el estudio del ritmo circadiano se esatablecieron 2 períodos: A.- 08.00-21.00 horas (diurno) y B.- 21.01-07.59 horas (nocturno). el 73 por ciento de los AVEs se presentaron en el período A o diurno (p<0.01) y el análisis realizado según tipo de accidente vascular encefálico, reveló un predominio diurno en la hora de presentación de los isquémicos (p<0.01) y la ausencia de un predominio por alguno de los períodos analizados para los hemorrágicos (NS). La presencia de hipertensión arterial no fue un factor determinante en la hora de presentación de los AVEs

Estudio multidisciplinario de los accidentes vasculares encefálicos: II perfil cardiovascular de los accidentes vasculares oclusivos / Multidisciplinary study of encephalic vascular accidents: II cardiovascular profile of occlusive vascular accidents

Ischemic stroke constitute a major cause of morbidity and mortality in the adult population, particulary in the elderly. Heart disease may predispose to ischemic stroke, especially in the presence of transient or permanent precipitating factors such as atrial fibrillation. To elucidate the role of heart disease in predisposing to ischemic stroke we studied the clinical and non invasive cardiac profile (EKG, 2D-Echo, Holter) of 186 consecutive patients, 91 of them embolic (Gl) and 96 non embolic (lacunar, atherotrombotic, others) (Gll), as determided by brain CT scan and through clinical evaluation. Age and male/female ratio were significantly different (71 + 13 vs 65 + 12 years, 40/60 vs 65/35, p <0.003). Hypertension was equally common in both groups (38 and 40 percent). Patients in Gl had higher prevalence of valvular heart disease (23 vs 1 percent), and atrial fibrillation (67 vs 10 percent), 2D Echo left atrial enlargement (45 vs 16 percent) and supraventricular ectopy in Holter (59 vs 32 percent) p< 0,001. By contrast absence of heart disease (45 vs 19 percent), ST-T changes in EKG (28 vs 14 percent), left ventricular hypertrophy in 2D Echo (28 vs 9 percent) and ventricular ectopy in Holter (54 vs 23 percent) were more prevalent in Gll patients, p<0.001. Multiple stepwise logistic regression analysis showed that age> 70years (relative risk (RR) 1.67), valvular heart disease (RR 2.25), chronic AF (RR 2.44) and paroxysmal AF (RR 1.89) were significant independient predictors of embolic stroke, whereas the presence of left ventricular hypertrophy in 2D-Echo (RR 0.76) and frequent ventricular premature beats in Holter (RR 0.47) were predictors of occlusive non embolic stroke. Thus, the clinical and non invasive cardiac profile of embolic and non embolic ischemic stroke is significantly different, which is relevant to preventive strategies