Forty-six patients with syncope and/or black-out episodes due to sinoatrial block are presented. Male-tofemale ratio was i to i, and mean age at onset of symptoms approximately 63 years in both sexes. Additional heart disease, especially coronary artery disease, was frequently, but not constantly, found. Twenty-five per cent had sinus node dysfunction as the only manifestation of cardiac diseas...

We report an autopsy case of a male fetus with Adams-Oliver syndrome. His mother was a healthy, 31-year-old woman and her family and past histories were unremarkable. Therapeutic termination was done at 28(+6) weeks gestational age due to oligohydramnios detected by antenatal ultrasonography. Chromosomal study revealed normal karyotype. On autopsy, characteristic transverse terminal defect of f...

We report the case of an infant with Adams-Oliver syndrome, a rare disorder characterized by aplasia cutis congenita, defects of the limbs and extremities, and cutis marmorata telangiectatica. Other associated anomalies have been reported, such as facial dysmorphism, heart defects, and disorders of the central nervous system.

INTRODUCTION Chronic post-anoxic myoclonus, also known as Lance-Adams syndrome, may develop following hypoxic brain injury, and is resistant to pharmacological therapy. CASE REPORT The patient we presented developed post-anoxic action myoclonus with severe, completely incapacitating myoclonic jerks. Myoclonus did not respond to the treatment with commonly used agents, i.e. valproate and clona...

Adams-Oliver syndrome (AOS) is a rare congenital disorder with unknown etiology commonly presented with aplasia cutis and terminal limb defects. Central nervous and cardiopulmonary systems may also be affected. It is commonly inherited as an autosomal dominant disorder but autosomal recessive and sporadic cases have also been reported. Here, we present a 10-year-old boy with extensive aplasia c...

Lance-Adams syndrome (LAS) is an uncommon neurological disorder characterized by the development of chronic post-hypoxic myoclonus. There are relatively few cases described following successful cardiopulmonary resuscitation. We report a patient who developed LAS 3 months after successful resuscitation. Cerebral imaging studies indicate that brain pathology in LAS patients is not uniform, sugges...

Received March 19, 2009, Revised July 2, 2009, Accepted for publication July 27, 2009 Reprint request to: Deborah Lee, M.D., Department of Dermatology, Busan Paik Hospital, College of Medicine, Inje University, 633-165, Gaegeum-dong, Busanjin-gu, Busan 614-735, Korea. Tel: 82-51-8906135, Fax: 82-51-897-6391, E-mail: [email protected] Ann Dermatol Vol. 22, No. 1, 2010 DOI: 10.5021/ad.2010.22...

Results. As shown in the Table, by comparison with the GG genotype, the TT genotype was associated with very high risk for all types of ARM, with increasing odds ratios according to the severity of ARM (from 4.60 for early ARM1 to 23.63 and 16.15 for late atrophic and neovascular ARM, respectively). By contrast, associations of the different types of ARM with the GT genotype were modest (OR, 1....