1/38. Congenital varicella syndrome diagnosed by polymerase chain reaction--scarring of the spinal cord, not the skin.A term infant with congenital varicella syndrome (CVS) is reported. Monoplegia of the left arm and paraplegia were present with no evidence of dermatomal skin scarring. Following death at 12 days of age, autopsy documented severe atrophy and gliosis of the spinal cord. Testing for varicella-zoster virus by the polymerase chain reaction method on brain tissue was positive. This case extends the current understanding of the clinical and pathological features of CVS.- - - - - - - - - - ranking = 1keywords = brain (Clic here for more details about this article) |
2/38. Fetal varicella syndrome: disruption of neural development and persistent inflammation of non-neural tissues.Primary varicella zoster virus (VZV) infection during pregnancy is rare. If it occurs between the 8th and 20th week of gestation, fetal varicella syndrome results in 1-2% of the fetuses. We report about a varicella infection that affected a pregnant mother in the 12th week of gestation. At 33 weeks, a premature girl was born with destruction of neurons in spinal cord, spinal ganglia and plexus myentericus, and secondary developmental disturbance including mummification of one arm and segmental intestinal atresia. The brain did not show any abnormalities. However, VZV dna could be detected by PCR in tissues from the brain and spinal ganglia. Chronic necrotizing inflammation was found in the placenta, fetal membranes, and one ovary. These locations showed nuclear inclusions which by in-situ-hybridization were proven to be VZV derived. This case demonstrates that in the fetal age, 'neurotropism' of VZV signifies severe destruction but not necessarily persistent inflammation of neural tissue. However, due to the inefficient fetal immune system, inflammation can go on for weeks, preferentially in non-neural tissues.- - - - - - - - - - ranking = 2keywords = brain (Clic here for more details about this article) |
3/38. Congenital varicella syndrome: a rare case of central nervous system involvement without dermatological features.An unusual case of congenital varicella syndrome with significant central nervous system involvement, but without dermatological features at birth is described. The mother contracted chicken pox at 15 weeks' gestation. Congenital varicella syndrome involves multiple systems, but rarely without skin lesions identifiable at birth. Although varicella infection in pregnant women is an uncommon complication, the fetal embryopathy that may result can be devastating. Antenatal diagnosis of fetal embryopathy during the first 20 weeks of pregnancy should be established by amniocentesis or cordocentesis when a mother presents in the first trimester with chicken pox, and appropriate risk counselling provided.- - - - - - - - - - ranking = 72.029512059024keywords = central nervous system, nervous system (Clic here for more details about this article) |
4/38. central nervous system and renal vasculitis associated with primary varicella infection in a child.A 7-year-old girl with primary varicella presented with encephalopathy and focal neurologic deficits 10 days after her first skin lesions appeared. She was discovered to have bilateral wedge-shaped renal infarctions, and ischemic lesions in the conus medullaris, cerebral cortex, and deep gray matter consistent with a medium and large vessel arteritis on magnetic resonance imaging. This complication has never before been reported in an immunocompetent child with primary varicella infection, and it represents a rare but serious complication of childhood chickenpox.- - - - - - - - - - ranking = 13.625980531961keywords = nervous system (Clic here for more details about this article) |
5/38. Varicella-associated acute necrotizing encephalopathy with a good prognosis.A patient with acute necrotizing encephalophathy (ANE) following varicella infection with a good prognosis is reported. A somatosensory evoked magnetic field (SEF) study using a 37-channel-magnetoencephalography system demonstrated normal latency and strength of the first component (N20m) elicited by median nerve stimulation, despite bilateral symmetrical thalamic lesions on MRI. The normal SEF findings and the good prognosis suggested a reversible breakdown of the blood-brain barrier, and an edematous process as the brain pathology. Furthermore, our results support the idea of distinct generators for the three earliest cortical SEF components (N20m, P30m, N45m).- - - - - - - - - - ranking = 2keywords = brain (Clic here for more details about this article) |
6/38. Congenital varicella syndrome: case with isolated brain damage.We report a case of congenital varicella syndrome after maternal infection during the 10th week of pregnancy. None of the typical abnormalities were manifested at birth; however, the child experienced neonatal tonic convulsions that evolved to refractory focal epilepsy with adversive, complex partial, and secondarily generalized seizures. At the age of 5 years, after a prolonged generalized seizure following cranial trauma, cranial computed tomography (CT) was performed. It led to the misdiagnosis of post-traumatic intracerebral hemorrhage. Afterwards, because of persistent seizures and developmental delay with mild or slight intellectual deficit, the next CT and magnetic resonance imaging (MRI) investigation at 8 years of age disclosed massive malformation of the left cerebral hemisphere. The serologic evidence of varicella-zoster virus-specific IgG antibodies without a history of varicella disease after birth and maternal infection during early pregnancy were crucial to diagnosing the rare syndrome of congenital varicella consisting of isolated brain damage.- - - - - - - - - - ranking = 5keywords = brain (Clic here for more details about this article) |
7/38. Postvaricella angiopathy: report of a case with pathologic correlation.Varicella is a common childhood illness, and central nervous system complications occur frequently. Delayed angiopathy has been described, although there are few reports of clinicopathologic correlation. A previously well 4-year-old male is presented. He suffered varicella 2 months before presentation with extensive right middle cerebral artery (MCA) territory infarction. cerebral angiography demonstrated an isolated 89% stenosis of the right proximal MCA. He developed cerebral edema refractory to medical treatment and progressed to transtentorial herniation. Right frontal temporoparietal craniotomies were performed with evacuation of infarcted brain tissue. Pathologic studies revealed small vessel vasculitis with lymphocytic infiltration of the vessel wall. Areas of demyelination were present within the white matter. polymerase chain reaction for varicella was negative on brain tissue. Postvaricella angiopathy, although an uncommon complication, may affect both small and large blood vessels, with catastrophic results.- - - - - - - - - - ranking = 16.405902411805keywords = central nervous system, nervous system, brain (Clic here for more details about this article) |
8/38. Rasmussen's syndrome associated with chronic brain stem encephalitis.We report a 3-year-old boy with a clinical picture of Rasmussen's encephalitis who had clinical, radiological and pathological evidence of brain stem involvement resulting in death. In addition, there were unexpected neuropathological findings of severe bilateral mesial temporal sclerosis. We discuss the novel finding of brain stem involvement in this condition and the association with mesial temporal sclerosis.- - - - - - - - - - ranking = 6keywords = brain (Clic here for more details about this article) |
9/38. cerebral infarction in a child. A case report.Cerebral infarcts in children are rather rare and in most cases no precise etiology is established. The authors describe a case of cryptogenetic cerebral infarction in a 9-year-old boy. The child presented an acute onset of hemiplegia in the right arm and leg, central facial palsy, dysarthria and steppage. The infarction was proved by Computed tomography (CT) and magnetic resonance imaging (MRI). Laboratory and instrumental studies rule out all known causes of brain infarction. The only possible etiopathogenetic hypothesis was a varicella arteritis which occurred 45 days before the clinical manifestation.- - - - - - - - - - ranking = 1keywords = brain (Clic here for more details about this article) |
10/38. Intracerebral varicella-zoster virus reactivation in congenital varicella syndrome.patients with congenital varicella syndrome (CVS) typically present with clinical symptoms consisting of skin lesions, neurological defects, eye diseases, and/or limb hypoplasia. In rare cases, isolated manifestations in the brain or eye have been reported. The varicella-zoster virus (VZV), as the causative agent of CVS, could only be detected in a few infants with CVS. In addition, there is little in the literature on antiviral treatment of infants born with signs of CVS. We report a case of CVS in a male infant who presented with generalized clonic cerebral seizures at age 4 months. An endogenous intracerebral viral reactivation following intrauterine VZV infection was assumed. After the diagnosis was confirmed virologically, acyclovir was administered intravenously for 10 days and afterwards orally for 3 weeks. This antiviral treatment was aimed at preventing progression of the disease. We concluded from this case that infants with intrauterine VZV infection can suffer intracerebral VZV reactivations that require antiviral treatment.- - - - - - - - - - ranking = 1keywords = brain (Clic here for more details about this article) |
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