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1.
Eur J Neurol ; 14(7): 826-8, 2007 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-17594344
2.
Rev Neurol ; 39(8): 731-3, 2004.
Artigo em Espanhol | MEDLINE | ID: mdl-15514901

RESUMO

INTRODUCTION: Sneddon's syndrome is the association between livedo reticularis and stroke. Hemorrhagic strokes in Sneddon's syndrome are exceptional. CASE REPORT: A 39-year-old woman who had had a livedo reticularis for about 14 years attended the Emergency Unit complaining of sudden, severe headache and numbness and weakness in her left extremities. Physical examination revealed left supranuclear facial palsy and left crural hemiparesis and hemihypoalgesia. Cranial CT showed a right parieto-occipital lobar haemorrhage. Immunological studies, including antiphospholipid antibodies, were negative. A cerebral angiography showed anomalies in most distal branches of intracranial arteries. Biopsies of the skin and digital arteries yielded non conclusive results. CONCLUSION: The association between livedo reticularis and stroke, together with the angiographic findings led us to the diagnosis of Sneddon's syndrome. When comparing our case with similar ones, we must point out to its onset with a hemorrhagic stroke and to the lobar location of the bleeding.


Assuntos
Hemorragia Cerebral/etiologia , Hemorragia Cerebral/patologia , Síndrome de Sneddon , Adolescente , Adulto , Angiografia Cerebral , Artérias Cerebrais/patologia , Circulação Cerebrovascular/fisiologia , Feminino , Humanos , Fluxo Sanguíneo Regional , Síndrome de Sneddon/complicações , Síndrome de Sneddon/diagnóstico
8.
Neurologia ; 23(3): 152-6, 2008 Apr.
Artigo em Espanhol | MEDLINE | ID: mdl-18370334

RESUMO

INTRODUCTION: There is a strong association between the e4 allele of apolipoprotein E (APOE) and Alzheimer's disease (AD). This converts this allele into a risk factor for the development of AD. The association between APOE4 and dementia with Lewy bodies (DLB) is under discussion. In DLB, the presence of APOE4 has been related with a greater amount of senile plaques and neurofibrillary tangles. METHOD: This is a case-control study in which the APOE genotype was determined using the modified PCR technique of Wenham in 306 patients with diagnosis of probably AD, NINCDS-ADRDA criteria, 58 cases of probably DLB, McKeith et al. consensus criteria (1996), all of them with SPECT with pathological 123I-FP-CIT and 80 normal controls (NC) having similar age and gender distribution. RESULTS: The frequency of alleles was: DLB group epsilon4: 16%; epsilon3: 75%; epsilon2: 9%; AD: epsilon4: 32%; epsilon3: 67%; epsilon2: 1%; and in the normal control group: epsilon4: 12%; epsilon3: 83%; epsilon2: 5%. The percentage of alleles in both genders was similar in the three groups. CONCLUSIONS: APOE4 percentage in DLB group (16%) was lower than in AD group (32%), and similar to the control group (12%). Considering that the presence of morphopathological Alzheimer type alterations in DBL, essentially neurofibrillary tangles, is inversely correlated with the presence of Parkinsonian signs, this group may represent pure forms of the disease, although the lack of neuropathological demonstration does not make it possible to confirm this hypothesis.


Assuntos
Apolipoproteína E4 , Doença por Corpos de Lewy/genética , Doença por Corpos de Lewy/metabolismo , Doença de Alzheimer/genética , Doença de Alzheimer/metabolismo , Doença de Alzheimer/patologia , Apolipoproteína E4/genética , Apolipoproteína E4/metabolismo , Radioisótopos de Carbono/metabolismo , Feminino , Frequência do Gene , Genótipo , Humanos , Radioisótopos do Iodo/metabolismo , Doença por Corpos de Lewy/diagnóstico , Doença por Corpos de Lewy/patologia , Masculino , Tomografia Computadorizada de Emissão de Fóton Único , Tropanos/metabolismo
9.
Neurologia ; 22(1): 54-7, 2007.
Artigo em Espanhol | MEDLINE | ID: mdl-17315105

RESUMO

Monoclonal and polyclonal immunoglobulinemia, including lymphoma, Waldenström's macroglobulinemia and less commonly multiple myeloma (MM), are considered as infrequent causes of ischemic stroke. Hyperviscosity states, as well as procoagulant disturbances, both potentially treatable, have been implicated in its etiopathogenesis. The so-called "hyperviscosity syndrome" is a clinical syndrome consisting of headache, visual, auditory and vestibular disturbances, confusion and decreased level of consciousness, and is caused by extreme hypervolemia with a high degree of erythrocyte aggregation caused by paraproteinemia. However, in addition to this global cerebral ischemia syndrome, increase in blood viscosity (BV) can also be a cause of focal ischemia. We report a case of a patient diagnosed with IgG type MM, who suffered multiple vertebrobasilar transient ischemic attacks and minor ischemic strokes concurrent with a reactivation of his hematological disease. He became completely asymptomatic after specific treatment with dexamethasone of the paraproteinemia associated with MM. We discuss its pathophysiology in this report.


Assuntos
Mieloma Múltiplo/complicações , Acidente Vascular Cerebral/etiologia , Humanos , Masculino , Pessoa de Meia-Idade , Acidente Vascular Cerebral/terapia
16.
Neurología (Barc., Ed. impr.) ; 23(3): 152-156, abr. 2008. ilus, tab
Artigo em Espanhol | IBECS (Espanha) | ID: ibc-75978

RESUMO

Introducci¨®n. Existe una fuerte asociaci¨®n entre el alelo¦Å4 de la apolipoprote¨ªna E (APOE) y enfermedad de Alzheimer(EA), constituyendo este alelo un factor de riesgopara padecer esta enfermedad. Su asociaci¨®n con la demenciacon cuerpos de Lewy (DCL) es objeto de controversia. Enla DCL la presencia de APOE4 se ha relacionado con unamayor carga de placas seniles y degeneraci¨®n neurofibrilar.M¨¦todo. Estudio de casos y controles en el que se determin¨®el genotipo APOE mediante la t¨¦cnica de reacci¨®nen cadena de la polimerasa (PCR) modificada de Wenhamen 306 pacientes diagnosticados de EA probable, criteriosNINCDS-ADRDA, 58 casos de DCL probable, criterios de consensode McKeith et al. (1996), todos ellos con SPECT con 123IFP-CIT patol¨®gico, y 80 controles normales (CN) de edad y distribuci¨®npor sexos similares.Resultados. La frecuencia de alelos fue la siguiente:DCL ¦Å4: 16%; ¦Å3: 75%; ¦Å2: 9%; EA ¦Å4: 32%; ¦Å3: 67%; ¦Å2:1%; CN ¦Å4: 12%; ¦Å3: 83%; ¦Å2: 5%. En los tres grupos la distribuci¨®nde alelos en ambos sexos fue similar.Conclusiones. En la DCL la frecuencia de ¦Å4 (16%) esmuy inferior a la de la EA (32%) y muy pr¨®xima a la cifra delos CN (12%). Teniendo en cuenta que la presencia de alteracionesmorfopatol¨®gicas tipo Alzheimer en la DCL, fundamentalmentedegeneraci¨®n neurofibrilar, se correlacionainversamente con la presencia de signos parkinsonianos, esposible que este grupo represente a las formas puras de laenfermedad, aunque la falta de comprobaci¨®n neuropatol¨®gicano permite confirmar esta hip¨®tesis (AU)


Introduction. There is a strong association betweenthe ¦Å4 allele of apolipoprotein E (APOE) and Alzheimer¡¯sdisease (AD). This converts this allele into a risk factorfor the development of AD. The association between APOE4and dementia with Lewy bodies (DLB) is under discussion.In DLB, the presence of APOE4 has been related with a greateramount of senile plaques and neurofibrillary tangles.Method. This is a case-control study in which the APOEgenotype was determined using the modified PCR techniqueof Wenham in 306 patients with diagnosis of probably AD,NINCDS-ADRDA criteria, 58 cases of probably DLB, McKeithet al. consensus criteria (1996), all of them with SPECT withpathological 123I-FP-CIT and 80 normal controls (NC) havingsimilar age and gender distribution.Results. The frequency of alleles was: DLB group ¦Å4:16%; ¦Å3: 75%; ¦Å2: 9%; AD: ¦Å4: 32%; ¦Å3: 67%; ¦Å2: 1%;and in the normal control group: ¦Å4: 12%; ¦Å3: 83%; ¦Å2:5%. The percentage of alleles in both genders was similarin the three groups.Conclusions. APOE4 percentage in DLB group (16%)was lower than in AD group (32 %), and similar to thecontrol group (12 %). Considering that the presence ofmorphopathological Alzheimer type alterations in DBL,essentially neurofibrillary tangles, is inversely correlatedwith the presence of Parkinsonian signs, this group mayrepresent pure forms of the disease, although the lack ofneuropathological demonstration does not make it possibleto confirm this hypothesis (AU)


Assuntos
Humanos , Masculino , Feminino , Adulto , Pessoa de Meia-Idade , Apolipoproteína E4 , Doença por Corpos de Lewy/diagnóstico , Estudos de Casos e Controles , Reação em Cadeia da Polimerase/métodos
17.
Neurología (Barc., Ed. impr.) ; 22(1): 54-57, ene.-feb. 2007. ilus, tab
Artigo em Es | IBECS (Espanha) | ID: ibc-054534

RESUMO

Las inmunoglobulinemias mono y policlonales, incluyendo el linfoma, la macroglobulinemia de Waldenström y, menos comúnmente, el mieloma múltiple (MM), se consideran causas inhabituales de ictus isquémicos. En su etiopatogenia se han implicado tanto estados de hiperviscosidad como alteraciones procoagulantes, ambas potencialmente tratables. El llamado «síndrome de hiperviscosidad» es un cuadro clínico formado por cefalea, alteraciones visuales, auditivas y vestibulares, confusión y disminución del nivel de conciencia y causado por una hipervolemia extrema con una gran agregación eritrocitaria producida por la paraproteinemia. Pero junto a este síndrome de isquemia cerebral global el aumento de la viscosidad sanguínea (VS) puede también ser causa de isquemia focal. Se presenta el caso de un paciente diagnosticado de un MM tipo IgG que sufrió múltiples ataques isquémicos transitorios e ictus isquémicos menores vertebrobasilares coincidiendo con una reactivación de su enfermedad de base y quedó completamente asintomático tras la instauración del tratamiento específico con dexametasona frente a la paraproteinemia asociada al MM. En el presente trabajo se discute su fisiopatología


Monoclonal and polyclonal immunoglobulinemia, including lymphoma, Waldenström's macroglobulinemia and less commonly multiple myeloma (MM), are considered as infrequent causes of ischemic stroke. Hyperviscosity states, as well as procoagulant disturbances, both potentially treatable, have been implicated in its etiopathogenesis. The so-called «hyperviscosity syndrome» is a clinical syndrome consisting of headache, visual, auditory and vestibular disturbances, confusion and decreased level of consciousness, and is caused by extreme hypervolemia with a high degree of erythrocyte aggregation caused by paraproteinemia. However, in addition to this global cerebral ischemia syndrome, increase in blood viscosity (BV) can also be a cause of focal ischemia. We report a case of a patient diagnosed with IgG type MM, who suffered multiple vertebrobasilar transient ischemic attacks and minor ischemic strokes concurrent with a reactivation of his hematological disease. He became completely asymptomatic after specific treatment with dexamethasone of the paraproteinemia associated with MM. We discuss its pathophysiology in this report


Assuntos
Masculino , Pessoa de Meia-Idade , Humanos , Acidente Vascular Cerebral/etiologia , Mieloma Múltiplo/complicações , Acidente Vascular Cerebral/prevenção & controle , Ataque Isquêmico Transitório/etiologia , Dexametasona/uso terapêutico , Paraproteinemias/tratamento farmacológico
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