1/7. choroid plexus papilloma and aicardi syndrome: case report.A case of aicardi syndrome associated with a choroid plexus papilloma of the third and both lateral ventricles in a 15 months old baby girl is repported. The tumor was completely removed via three craniotomies. Reports of the literature with the association of choroid plexus papilloma and aicardi syndrome are rare. We suggest that children diagnosed with aicardi syndrome should routinely undergo imaging studies of the brain, such as computed tomography or magnetic resonance.- - - - - - - - - - ranking = 1keywords = choroid plexus papilloma, plexus papilloma, choroid plexus, choroid, papilloma, plexus (Clic here for more details about this article) |
2/7. Leptomeningeal angiomatosis with infantile spasms.We describe a 7-month-old female with leptomeningeal angiomatosis who developed infantile spasms. She did not manifest facial nevus or ocular choroidal angioma. Leptomeningeal angiomatosis is characterized by venous angiomas of leptomeninges and usually accompanied by facial nevus, a condition known as sturge-weber syndrome. In sturge-weber syndrome, leptomeningeal angiomas can cause infantile spasms but much less frequently than in other neurocutaneous syndromes, such as tuberous sclerosis. This patient is the first reported case of leptomeningeal angiomatosis without facial nevus who developed infantile spasms. Leptomeningeal angiomas should be taken into consideration as a cause of infantile spasms, even in the absence of facial nevus. We suggest that this case is clinically within the spectrum of sturge-weber syndrome, and that the embryologic origin of this case is similar to that of sturge-weber syndrome.- - - - - - - - - - ranking = 0.0050659473001999keywords = choroid (Clic here for more details about this article) |
3/7. aicardi syndrome. A clinicopathologic case report including electron microscopic observations.aicardi syndrome is characterized by infantile spasms, agenesis of the corpus callosum, severe mental retardation, and a characteristic chorioretinopathy with lacunar defects. The authors report on a 2-year-old girl with congenital hydrocephaly who was found unresponsive by the baby-sitter and died shortly thereafter. At autopsy, the histopathologic findings, which were confined to the brain and eyes, were found highly characteristic of AIC. The main abnormalities included agenesis of the corpus callosum, micropolygyria, bilateral papillomas of choroid plexi, bilateral microphthalmia, bilateral hypoplasia of the optic nerves, bilateral colobomas of the juxtapapillaris choroid and optic disc, bilateral total retinal detachment with dysplastic rosettes and chorioretinal lacunae with focal thinning, and atrophy of the retinal pigment epithelium and choroid. A detailed histopathologic study of the ocular findings and the brain anomalies is presented. The results of scanning electron microscopy of the chorioretinal lacunae demonstrated peculiar papillary proliferations of the retinal pigment epithelium in both eyes.- - - - - - - - - - ranking = 0.019235473240168keywords = choroid, papilloma (Clic here for more details about this article) |
4/7. A Golgi study of the polymicrogyric cortex in aicardi syndrome.The neuropathological examination of the brain of a 4-month-old girl with aicardi syndrome (infantile spasms, chorioretinal lacunae, psychomotor retardation, agenesis of the corpus callosum and vertebral anomalies) showed agenesis of the corpus callosum, agenesis of the anterior commissure and abnormal orientation of the hippocampal formation, in addition to periventricular cerebral nodules and extensive non-laminated cortical polymicrogyria of the fronto-parietal regions and gyrus cinguli of both hemispheres. A non-communicating medial supracollicular cyst, racemose cysts of the choroid plexus, underdevelopment of the inferior cerebellar vermis tonsils were also observed. The study of the polymicrogyric cortex with the Golgi method revealed an abnormal orientation of neurons; but the different varieties of cells were located at the depth corresponding to that were they are normally found. The Golgy study of the periventricular nodules disclosed the presence of different types of cortex-like neurons. On the basis of these data it may be suggested that polymicrogyria is not a migratory disturbance, but rather the result of a partial necrosis of the cortical mantle occurring before the 5th month of foetal life.- - - - - - - - - - ranking = 0.062431813670484keywords = choroid plexus, choroid, plexus (Clic here for more details about this article) |
5/7. Monozygotic twin patients with congenital ichthyosis, microcephalus, spastic quadriplegia, myoclonus, and EEG abnormalities.A pair of monozygotic twins manifested widespread ichthyosis on the trunk and extremities, mainly on the extensor surface of the lower legs. Both patients had severe developmental retardation, microcephalus, spastic quadriplegia, myoclonus, and hearing impairment. Cranial computed tomography and magnetic resonance imaging revealed diffuse cortical atrophy. electroencephalography documented diffuse slow wave dysrhythmia and marked epileptic discharges, namely bilateral occipital dominant, and low-voltage multifocal spikes which appeared asynchronously as a burst, mainly during sleep. Skin biopsy revealed hyperkeratosis with parakeratosis. Granular cell layer was not thickened and diminished. Papillomatosis was absent. Electron microscopy did not reveal any distinctive abnormalities. sjogren-larsson syndrome and other known ichthyosis syndromes were excluded because of the difference in localization of skin lesions, skin manifestations, lack of papillomatosis, the nature and severity of neurologic symptoms and marked electroencephalographic abnormalities.- - - - - - - - - - ranking = 0.0040376313395687keywords = papilloma (Clic here for more details about this article) |
6/7. aicardi syndrome: a morphologic description with particular reference to intracytoplasmic inclusions in cortical astrocytes.aicardi syndrome is characterized by agenesis of the corpus callosum, infantile spasms, and ocular anomalies. Very few morphologic descriptions have been made of the central nervous system of children with this syndrome. We performed a postmortem examination of the brain of a 13-year-old girl with clinically well-documented aicardi syndrome. Gross examination revealed a small brain (745 g) with the right cerebral and cerebellar hemispheres smaller than the left. There was agenesis of the corpus callosum, and ectopic gray matter was scattered throughout the cerebral hemispheres. Both choroid plexus and arachnoid cysts were present. Microscopic examination revealed indistinct cortical layering and multiple foci of ectopic gray matter. The cerebellar sections were altered by focal atrophy with gliosis and Purkinje cell dropout. Multiple sections of cerebrum contained astrocytes with coarse, paranuclear, eosinophilic inclusions. Electron microscopy, immunohistochemistry, and special stains further defined these inclusions, which we speculate represent a degenerative process.- - - - - - - - - - ranking = 0.062431813670484keywords = choroid plexus, choroid, plexus (Clic here for more details about this article) |
7/7. choroid plexus papilloma and cysts in the aicardi syndrome: case reports.Two female infants with callosal agenesis, infantile spasms, chorioretinal lacunae, optic disc colobomas and cortical heterotopias were diagnosed with aicardi syndrome. A choroid plexus papilloma was found in one patient, and choroid plexus cysts were found in the other. choroid plexus lesions are common findings in the aicardi syndrome and are discussed in this paper.- - - - - - - - - - ranking = 0.73441498211384keywords = choroid plexus papilloma, plexus papilloma, choroid plexus, choroid, papilloma, plexus (Clic here for more details about this article) |