Sturge weber syndrome with phenytoin toxicity - A deeper pandora ’ s box
نویسندگان
چکیده
A neuro-cutaneous disorder with angiomas involving the leptomeninges and the face, Sturge weber syndrome is a crippling entity with devastating outcomes at times. Although known to conglomerate other markers like seizures, mental retardation, hemiplegia, intracerebral calcifications etc, atypical variants have been noted and we bring forth one such case complicated by the adverse effects of long term phenytoin use. Introduction: Sturge weber syndrome (SWS) is an encephalofacial angiomatosis with a facial capillary hemangioma, termed the Port wine stain (PWS) and angiomata of the leptomeninges. With an incidence of about 1 in 50,000 SWS is a rare entity with considerable morbidity. We report a case with the quintessential features of SWS associated with phenytoin toxicity and review the literature. Case report: A 22 year old male presented with gradually worsening shortness of breath and cough from 10 days. He had an erythematous patch on the left side of the face and left upper limb by birth (a full term normal vaginal delivery). He had been having recurrent seizures (starting 3 months of age) persisting despite treatment with phenytoin for more than 7 years (later switched to carbamazapine and sodium valproate due to gum hypertrophy). His milestones were delayed and sported short stature and mental retardation (IQ-40). He had repeated fractures (long bones and ribs) making him bedridden most of the time. His family history was not contributory. Examination revealed nonblanchable erythematous patches involving left side of the face, upper limb and the trunk (Figure 1).
منابع مشابه
معرفی دختر مبتلا به سندرم استورج وبر همراه با بدشکلی استخوانی در ناحیه دهان- فک بالا- صورت
Background: Sturge-Weber syndrome is one of the rare neurocutaneous disorders with frequency of approximately 1 per 50,000. Sturge-Weber syndrome consists of a constellation of symptoms and signs including a facial nevus (port wine stain), seizure and hemiparesis. In many cases it may associate with mental retardation. Case presentation: A 7- year old girl with mental retardation and a large fa...
متن کاملDermatological manifestations of epilepsy among adult Sudanese epileptic patients.
OBJECTIVES To study the pattern of dermatological changes associated with epilepsy among adult Sudanese epileptic patients. METHODS This non-interventional descriptive study included 360 adult Sudanese epileptic patients and was conducted at the El Shaab Teaching Hospital, Khartoum, Sudan, from February 2004 to August 2007. All patients had full detailed history and clinical examination. A de...
متن کاملPurple glove syndrome following intravenous phenytoin administration.
Purple glove syndrome (PGS) is a rare complication of intravenous phenytoin use that typically presents with pain, edema, and discoloration at the injection site that spreads to the distal limb. Several risk factors have been associated with the development of PGS; however, the etiology remains unknown. We present a case of PGS and briefly review the current understanding of this syndrome.
متن کاملPandora Syndrome – Beyond the Bladder and LUT Signs
What is Pandora Syndrome ? Is this terminology more helpful than FUS or FLUTD or IC ? Results of studies over the past 20 years indicate that idiopathic/interstitial cystitis in cats is the result of complex interactions between the bladder, nervous system, adrenal glands, husbandry practices, and the environment in which the cat lives. A recent review emphasizes that many cats with a diagnosis...
متن کاملذخیره در منابع من
با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید
عنوان ژورنال:
دوره شماره
صفحات -
تاریخ انتشار 2011