Prune belly and schizencephaly.
نویسندگان
چکیده
A 45-year-old man without relevant family history presented with prune belly syndrome (PBS). He had a 34year history of right simple partial motor seizures with and without secondary generalization, with a good response to carbamazepine monotherapy; there was no cognitive or social impairment. MRI showed global brain atrophy, ventricular asymmetry, left hemisphere schizencephaly, and bihemispheric heterotopias (figure, A–C). Although PBS is considered a mesoderm layer defect characterized by total or partial absence of abdominal muscles (figure, D), urinary tract abnormalities, and cryptorchidism of unknown etiology, involvement of other embryologic tissues has also been reported, including the ectodermic layer, as in this case.
منابع مشابه
Concurrence of prune belly syndrome with meningomyelocele
Prune Belly Syndrome (PBS) comprises a triad of abdominal wall deficiency, urogenital abnormalities and cryptorchidism. Neurologic abnormalities are unusual in prune belly syndrome. Here, we report a rare association of meningomyelocele with prune belly syndrome and provide an embryologic perspective to this concurrence.
متن کاملPrune Belly Syndrome in Adolescence: A Case Report
The Prune Belly syndrome also known as Eagle Barret syndrome is a rare disorder. We report a rare case of Prune Belly syndrome in 17 year old boy. Patient presented with complains of absence of both testis in scrotum since birth. On examination patient was found to have lax abdominal wall. Patient was further evaluated and found to have shrunken small right kidney and left hydroureteronephrosis...
متن کاملStructural study of gubernaculum testis in fetuses with prune belly syndrome.
PURPOSE We compared and contrasted the structure of the gubernaculum testis in fetuses with prune belly syndrome and normal controls. MATERIALS AND METHODS We studied a total of 6 gubernacula from 3 male fetuses with prune belly syndrome and a total of 14 from 7 male fetuses without an anomaly. Gubernacular specimens were cut into 5 μm sections and stained with Masson trichrome to quantify co...
متن کاملIt's not All Doom and Gloom: Prune Belly Syndrome Associated with VACTERL
Prune belly syndrome is a rare abnormality; its association with VACTERL is even rarer. This association has been reported in literature a few times since first reported in 1993 and so far the majority have either been stillbirths or died shortly after birth. We present a case of Prune belly syndrome associated with VACTERL who is now one year old.
متن کاملPrune Belly Syndrome: Errors in Management and Complications of Treatment
Prune Belly Syndrome is a condition in which the abdominal wall, urinary tract and testes are affected resulting in a severely curtailed life span. With proper management these patients could have a normal, productive life.
متن کاملذخیره در منابع من
با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید
برای دانلود متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید
ثبت ناماگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید
ورودعنوان ژورنال:
- Neurology
دوره 83 7 شماره
صفحات -
تاریخ انتشار 2014