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Weber Syndrome (weber + syndrome)

Distribution by Scientific Domains
Medical Sciences100%

Selected Abstracts

Sturge,Weber syndrome and paroxysmal hemiparesis: epilepsy or ischaemia?

DEVELOPMENTAL MEDICINE & CHILD NEUROLOGY, Issue 11 2004
Floor E Jansen MD
Transient neurological deficits experienced by patients with Sturge,Weber syndrome can be caused by epilepsy, or may result from temporary ischaemia of the cortex underlying the vascular malformation. To show the difficulty in distinguishing seizures from ischaemic symptoms, two male children with episodes of acute unilateral weakness are presented here as well as a review of the literature. The first child presented at 2 years of age with a sudden increase in his pre-existing right hemiparesis accompanied by screaming. Ictal epileptiform activity was recorded at the moment of the attack, and subsequent seizures were controlled by adjustment of antiepileptic drug treatment. The second child presented at 4 years of age with attacks of vomiting and a coinciding increase in the pre-existing paresis of the left leg. Electroencephalogram (EEG) recording did not show ictal epileptiform activity. The origin was presumed to be vascular. Treatment with aspirin led to control of these transient ischaemic attacks. Ictal EEG is needed to differentiate between an epileptic and an ischaemic origin of transient focal deficit. Treatment with aspirin should be considered if an ischaemic origin cannot be excluded. [source]

Management of urethral hemangiomas associated with Klippel,Trenaunay,Weber syndrome by endoscopic sclerotherapy

INTERNATIONAL JOURNAL OF UROLOGY, Issue 7 2007
Naoki Terada
Abstract: Klippel,Trenaunay,Weber syndrome (KTS) is an unusual congenital anomaly characterized by cutaneous hemangiomas, varicosities and bony hypertrophy of the extremities. Herein the case is reported of a 24-year-old man with urethral bleeding from hemangiomas associated with KTS that were successfully managed by endoscopic sclerotherapy. A 23-G puncture needle was inserted into the bleeding vein to inject a 5% solution of monoethanolamine oleate (Oldamine), which is typically used for sclerotherapy of esophageal vasix. At a 4-month follow-up, the patient only had slightly bloodstained urethral discharge, and is doing well. This is the first case reporting endoscopic sclerotherapy for a KTS-associated urethral hemangioma. [source]

Phakomatosis pigmentovascularis type II b associated with Sturge,Weber syndrome

CLINICAL & EXPERIMENTAL DERMATOLOGY, Issue 4 2006
C. Ajith
No abstract is available for this article. [source]