ABSTRACT
Dyke-Davidoff-Masson syndrome (DDMS) was first described in 1933 as a clinical condition characterized by hemiatrophy, hyperpneumatization of paranasal sinuses, contralateral hemiparesis, facial asymmetry, seizures, and mental retardation.1 DDMS can be of 2 types: congenital and acquired. The congenital type can be caused by various conditions experienced during fetal or early childhood development, including ischemia, infarction, trauma, infections, and hemorrhage. The acquired type is mostly associated with hemorrhage, trauma, and infections experienced after 1 month of age. DDMS can manifest alone or can be accompanied by crossed cerebellar atrophy (CCA) which is a newly discovered radiological marker characterized by prominent cortical sulci and loss of cerebellar parenchyma. The congenital type of DDMS is known to be accompanied by ipsilateral cerebellar atrophy and the acquired type is known to be accompanied by contralateral cerebellar atrophy.2,3 Supratentorial events may lead to destruction in the cortico-ponto-cerebellar pathways, mostly in the contralateral side of the body (80%) due to decussation.4 In this report, we present 2 cases of DDMS accompanied by CCA to emphasize the possibility that the DDMS cases with severe intrauterine hemorrhage can be accompanied by contralateral CCA and migratory abnormalities rather than ipsilateral CCA and clinical survey.
Subject(s)
Cerebellar Diseases/complications , Epilepsy, Generalized/complications , Intracranial Hemorrhages/etiology , Seizures, Febrile/complications , Adolescent , Anticonvulsants/therapeutic use , Atrophy , Cerebellar Diseases/congenital , Cerebellar Diseases/diagnostic imaging , Cerebellar Diseases/drug therapy , Epilepsy, Generalized/congenital , Epilepsy, Generalized/diagnostic imaging , Epilepsy, Generalized/drug therapy , Female , Humans , Infant , Intracranial Hemorrhages/diagnostic imaging , Intracranial Hemorrhages/drug therapy , Magnetic Resonance Imaging , Risk Factors , Seizures, Febrile/congenital , Seizures, Febrile/diagnostic imaging , Seizures, Febrile/drug therapy , Steroids/therapeutic use , Syndrome , Treatment OutcomeSubject(s)
Aneurysm, Ruptured/diagnostic imaging , Aortic Dissection/diagnostic imaging , Pregnancy Complications, Cardiovascular/diagnostic imaging , Subarachnoid Hemorrhage/diagnostic imaging , Adult , Aortic Dissection/complications , Aneurysm, Ruptured/complications , Female , Humans , Pregnancy , Thrombosis/etiologyABSTRACT
BACKGROUND: The major cause of hereditary renal failure is autosomal dominant polycystic kidney disease (ADPKD). Many factors affect renal progression in these patients. Among these, hypertension and an increase in renal volume are interrelated in terms of their effects on renal progression. We aimed to investigate the effects of losartan and ramipril on renal volume and progression in patients with ADPKD. MATERIALS AND METHODS: Data from 18 hypertensive patients with ADPKD were evaluated. Eleven of the 18 hypertensive patients were on losartan and 7 on ramipril treatment. Demographic parameters, use of antihypertensives and other medications, the course of blood pressure (BP), biochemical parameters, creatinine clearance (CrCL), findings at computed tomography and renal volume were recorded at baseline and at 1 and 5 years. RESULTS: Target BP values were maintained over 5 years. The annual decrease in CrCL was 1.33 mL/min in the losartan group compared with 6.59 mL/min in the ramipril group. There was no significant difference between the groups in terms of annual decrease in CrCL. Annual increase in renal volume was 252.04 cm³ in the losartan group and 167.36 cm³ in the ramipril group. There was no significant difference between the groups in terms of the increase in renal volumes at 1 and 5 years. CONCLUSION: Our study demonstrated that losartan and ramipril provided effective BP control. In addition, the results of our study demonstrated that despite the increase in renal volume, losartan and ramipril may have regressed renal progression via other factors.