We report the observation of a child born at 38 5/7 weeks’ gestation, who presented with marked microcephaly and suffered from persistent intractable epilepsy up to his death at the age of 5 days. Magnetic resonance imaging suggested lissencephaly. But interestingly no submicroscopical deletion in LIS-1 gene could be detected and true lissencephaly could not be confirmed histologically. Instead, primary degenerative process was considered the most probable cause of congenital pontocerebellar hypoplasia II in this case, which may be associated with a maturation delay of the cerebral hemispheres. The present case points to possible pitfalls in neuroradiological interpretation of simplified cerebral gyration and to the necessity to take into account cerebral pathologies leading to slowing or arrest of intrauterine cerebral development and delay of cerebral gyration.
The Comparison of Balloon versus Rotational Angioplasty (COBRA) study is a prospective, randomized study to compare short‐ and long‐term effects of PTCA and high speed PTCRA (or rotablation) in patients with complex coronary stenoses. Patients with angiographically defined complex coronary stenoses (> 70% diameter reduction) are included in the study. The trial has recruited 501 patients who will have detailed follow‐up for 6 months. The primary end points are: (1) procedural success, defined as angiographically proven residual stenoses < 50% and stenosis reduction of ± 20% in absence of new myocardial infarction, emergency CABG, or death; (2) 6‐month restenosis in the treated segment; and (3) major cardiac events during the follow‐up period. Additionally, improved exercise tolerance will be scored. The final results of the study are expected by late 1997.
A 4 year-old boy was referred for diagnostic reevaluation with known pulmonary valve stenosis. Physical examination revealed multiple cafe-au-lait spots, inguinal freckling and on the right side in supraclavicular region a softly, non-painful tumour. The boy showed a mild mental and language retardation. Ultrasound and MRT demonstrated supraclavicular a plexiform neurofibroma and intracranial increased intensity lesions in basal ganglia and mesencephalon. In our patient, we have diagnosed a Watson-Syndrome, the overlap and differences to neurofibromatosis type I is discussed.
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