1/26. incontinentia pigmenti: seven cases with dental manifestations.incontinentia pigmenti (Bloch-Sulzberger syndrome) is an uncommon genodermatosis that usually affects female infants. The condition is characterized by four cutaneous stages and is frequently associated with dental, ocular, central nervous system and structural anomalies. A large case series of seven patients, all female, who presented to the Department of Paediatric dentistry at the Eastman Dental Hospital over the last 16 years is reported. The dental features of these cases were typical and included missing teeth, microdontia and delayed eruption. In two of the seven cases, both maxillary canines were palatally impacted.- - - - - - - - - - ranking = 1keywords = central nervous system, nervous system (Clic here for more details about this article) |
2/26. Disappearance of a white matter lesion in incontinentia pigmenti.We report a 12-month-old Japanese female with incontinentia pigmenti, in whom magnetic resonance imaging (MRI) disclosed a small transient lesion in the white matter. After birth, she developed some vesicular skin eruptions that mainly involved the lower extremities. These skin lesions increased in size and number and became hyperpigmented within 2 weeks. At 1 month of age, MRI revealed a small hypointense lesion on T(1)-weighted imaging, with water density on T(2)-weighted imaging, in the right centrum semiovale. At 4 months of age, her hyperpigmented lesions had faded, and at 7 months of age, MRI disclosed the disappearance of the previously observed abnormality. She exhibited no neurologic abnormalities. No cases have been reported concerning a transient lesion in the white matter revealed by MRI in incontinentia pigmenti. Although the pathogenesis is unknown, transient central nervous system involvement might have occurred in early infancy as did the fading skin lesions.- - - - - - - - - - ranking = 1keywords = central nervous system, nervous system (Clic here for more details about this article) |
3/26. incontinentia pigmenti: a case associated with cardiovascular anomalies.Various cutaneous and developmental defects of the eyes, teeth, skeleton, and central nervous system have been detected in infants with incontinentia pigmenti. We report an isolated case of incontinentia pigmenti in a 6-month-old girl in association with tricuspid insufficiency, an abnormal shunt of the right pulmonary vein into the superior vena cava, and pulmonary hypertension. We believe that our findings will help to confirm the association of cardiovascular anomalies in IP.- - - - - - - - - - ranking = 1keywords = central nervous system, nervous system (Clic here for more details about this article) |
4/26. incontinentia pigmenti presenting as seizures.incontinentia pigmenti is a rare disorder that may affect many systems including the skin, central nervous system, bone, and eyes. We describe a 13-day-old girl who developed seizures on day 1 of life and was placed on antiseizure medication. On approximately day 4 of life, she developed a vesicular rash on her trunk and extremities. The pediatric team prescribed intravenous acyclovir and diphenhydramine cream. These were used without improvement. At dermatology consultation, linear and swirled vesicular lesions were seen. A skin biopsy specimen revealed eosinophils within intraepidermal vesicles consistent with a diagnoses of incontinentia pigmenti. This case of incontinentia pigmenti is of interest in that the initial symptom was a seizure disorder.- - - - - - - - - - ranking = 1keywords = central nervous system, nervous system (Clic here for more details about this article) |
5/26. Bilateral cerebrovascular accidents in incontinentia pigmenti.incontinentia pigmenti is an X-linked dominant neurocutaneous disorder with central nervous system manifestations in 30% of cases, including seizures and mental retardation. Ischemic or hemorrhagic cerebrovascular accidents have been reported rarely in incontinentia pigmenti. Chart review and literature search was performed following identification of the index case. We describe a patient with incontinentia pigmenti who developed bilateral cerebrovascular accidents in the neonatal period, with resultant severe neurologic sequelae. This is the second reported case of bilateral cerebrovascular accidents in a patient with incontinentia pigmenti. This finding may be secondary to cerebrovascular anomalies, similar to those observed in the retina. Recognition of cerebrovascular accidents as a complication of incontinentia pigmenti will hopefully lead to earlier recognition and treatment.- - - - - - - - - - ranking = 1keywords = central nervous system, nervous system (Clic here for more details about this article) |
6/26. incontinentia pigmenti associated with cerebral palsy and cerebral leukomalacia: a case report and literature review.incontinentia pigmenti (IP) is a multisystem disorder with characteristic cutaneous signs. After the skin, the central nervous system is the next most affected system. We report a child with IP and left-sided hemiparesis and cerebral periventricular leukomalacia on magnetic resonance imaging (MRI). The MRI findings would support ischemia sustained perinatally.- - - - - - - - - - ranking = 1keywords = central nervous system, nervous system (Clic here for more details about this article) |
7/26. Neonatal seizures in two sisters with incontinentia pigmenti.Familial incontinentia pigmenti (IP) (OMIM #308300) is a rare genetic disorder which segregates in an X-linked dominant way. The female-to-male ratio ranges from 20 to 37 : 1. In affected females IP causes highly variable abnormalities of the skin, hair, nails, teeth, eyes, and central nervous system (CNS). Cardiovascular anomalies, cerebral infarction, and immune dysfunction are rare complications of IP. The pathogenesis of cerebral changes in IP remains elusive. We report the case of two IP-affected sisters who presented in each case with neonatal seizures on the fifth day of life. Via cranial magnetic resonance tomographic imaging (MRI) different types of lesions in both hemispheres were demonstrable in both patients. To date the pathogenetic mechanisms for the cerebral lesions are not fully understood. However, multiple microscopic infarcts could serve as a possible explanation. The clinical course and the neurological development of the older child are favorable and so far the younger sibling appears to be developing normally, which is uncommon for patients with early onset of neurological symptoms. Symptomatic seizures in IP are an important differential diagnosis in benign non-familial and familial neonatal seizures.- - - - - - - - - - ranking = 1keywords = central nervous system, nervous system (Clic here for more details about this article) |
8/26. The importance of screening for sight-threatening retinopathy in incontinentia pigmenti.incontinentia pigmenti (IP) is an X-linked dominant disorder of ectodermal structures affecting the skin, hair, teeth, eyes, and central nervous system. The four classic cutaneous stages of the disorder are well known to pediatric dermatologists. However, ocular and neurologic sequelae represent the major morbidity in IP. The two patients reported here highlight some of the potential ocular manifestations of IP and emphasize the importance of early ophthalmologic assessment in this condition.- - - - - - - - - - ranking = 1keywords = central nervous system, nervous system (Clic here for more details about this article) |
9/26. A case of incontinentia pigmenti.incontinentia pigmenti is a multisystem disease that commonly involves the skin, eye, teeth and central nervous system. incontinentia pigmenti involving the skin and central nervous system is reported in a four-year-old female child from ethiopia. The clinical features of incontinentia pigmenti are discussed with literature review.- - - - - - - - - - ranking = 2keywords = central nervous system, nervous system (Clic here for more details about this article) |
10/26. Unusual neonatal presentation of incontinentia pigmenti with persistent pulmonary hypertension of the newborn: a case report.incontinentia pigmenti (Bloch-Sulzberger syndrome) is a multisystem disorder with classical changing skin lesions. The other systems that are involved include the central nervous system, eye, hair, teeth, musculoskeletal system and, occasionally, the cardiovascular system. We report a neonate with a diagnosis of incontinentia pigmenti who presented at birth with pulmonary hypertension. This presentation has not been described in the literature.- - - - - - - - - - ranking = 1keywords = central nervous system, nervous system (Clic here for more details about this article) |
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