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Neurologia Medico-chirurgica Apr 1989A case of porencephaly in a 33-year-old male is presented. The patient exhibited right hemiparesis and generalized seizures. Computed tomography and, subsequently,...
A case of porencephaly in a 33-year-old male is presented. The patient exhibited right hemiparesis and generalized seizures. Computed tomography and, subsequently, craniotomy revealed a cerebral defect in the left parietal lobe, which communicated with the lateral ventricle. On angiography and at surgery, the cortical branches of the middle cerebral artery were found to be stretched over the surface of the cyst. Histopathological examination showed the outer membrane of the cyst to consist of arachnoid, subarachnoid space, vessels, pia mater, and a layer of degenerative brain tissue on histology. The diagnosis of porencephaly and its differentiation from arachnoid cyst are discussed.
Topics: Adult; Arachnoid; Brain Diseases; Cerebral Angiography; Cerebral Ventricles; Cysts; Diagnosis, Differential; Humans; Male; Parietal Lobe; Tomography, X-Ray Computed
PubMed: 2478920
DOI: 10.2176/nmc.29.347 -
The Journal of Veterinary Medical... Dec 2016A postmortem examination revealed a large brain cavity in the right cerebral hemisphere of a 9-year-old male fennec (Vulpes zerda). The cavity was filled with...
A postmortem examination revealed a large brain cavity in the right cerebral hemisphere of a 9-year-old male fennec (Vulpes zerda). The cavity was filled with cerebrospinal fluid and extended to the right lateral ventricle. Swelling and displacement of the right hippocampal area were also observed. Histologic examination revealed no evidence of previous infarct lesions, hemorrhage, inflammation or invasive tumor cells. Observation of the defective part suggested a local circulatory disorder during the fetal stage, although the cause was not detected. No neurological symptoms that could enable a provisional diagnosis were observed during the course of his life. This is the first report of asymptomatic porencephaly in a fennec fox.
Topics: Animals; Cerebrum; Fatal Outcome; Foxes; Lymphoma, T-Cell; Male; Porencephaly
PubMed: 27523321
DOI: 10.1292/jvms.16-0121 -
Medicine Jul 2020Foetal alcohol spectrum disorder (FASD) is a complex malformative disease caused by the teratogenic effect of alcohol consumed during pregnancy. Mothers are frequently...
INTRODUCTION
Foetal alcohol spectrum disorder (FASD) is a complex malformative disease caused by the teratogenic effect of alcohol consumed during pregnancy. Mothers are frequently reluctant to admit alcohol consumption during pregnancy. During infancy and particularly during neonatal period, differential diagnosis is difficult.
PATIENT CONCERNS
This case is represented by an Italian neonate boy small for gestational age, born by caesarean section at a gestational age of 37 weeks + 6 days by neglect and single-parent pregnancy. On physical examination, he presented particular facial features: microcephaly, epicanthal folds, flat midface, low nasal bridge, indistinct philtrum, and thin upper lip; moreover, examination revealed a macro-penis and recurvation without evidence of glans.
DIAGNOSIS
Echocardiogram showed an inter-ventricular defect of medium-muscular type and brain magnetic resonance imaging showed asymmetry of the cerebral hemispheres with hypoplasia of the left cerebral hemisphere, dilatation of the left ventricle, cerebrospinal fluid cavity, and porencephaly.
INTERVENTIONS
We investigated the ethylglucuronide (EtG) concentration in the neonate's hair by liquid chromatography-tandem mass spectrometry and we detected EtG in the infant's hair (normal value, 30 pg/mg), demonstrating prenatal alcohol exposure.
OUTCOMES
In this neonate, EtG measure in hairs permitted the diagnosis of FASD, so allowing to exclude genetic diseases associated with similar clinical findings. After this result the mother admitted that she drunk alcohol during pregnancy (she declared 3 glasses of wine every day). At the age of 6 months, the child showed a moderate neurodevelopmental delay.
CONCLUSION
This case shows that FAD should be considered in neonates with rare neurological diseases as porencephaly. In neonates and infants born to a mother who did not report alcohol use, EtG measure in hairs can significantly improve diagnosis of FASD, so allowing to exclude genetic diseases associated with similar clinical findings.
Topics: Female; Fetal Alcohol Spectrum Disorders; Glucuronates; Hair; Humans; Infant, Newborn; Italy; Male; Porencephaly; Pregnancy
PubMed: 32756128
DOI: 10.1097/MD.0000000000021384 -
The Veterinary Record Feb 2012A retrospective study was performed to identify dogs with cerebrospinal fluid-filled cavitatory lesions on MRI. Six dogs were included and the lesions were classified....
A retrospective study was performed to identify dogs with cerebrospinal fluid-filled cavitatory lesions on MRI. Six dogs were included and the lesions were classified. In the three dogs in the present study with hydranencephaly, unilateral but complete loss of the temporal and parietal lobes was noted and had almost complete loss of the occipital and frontal lobes of a cerebral hemisphere. In the three dogs with porencephaly, there was unilateral incomplete loss of the parietal lobe and one dog had additional partial loss of the temporal and frontal lobes. Two of the dogs with porencephaly had seizures; the third showed no associated clinical signs. The dogs with hydranencephaly had mentation changes and circled compulsively. The two porencephalic dogs with seizures were treated with phenobarbitone. One of the dogs with hydranencephaly showed increased frequency and duration of circling; one dog's clinical signs did not progress and the third dog was euthanased due to increasing aggression. The dog with increased circling had ventriculoperitoneal shunt placement and the circling frequency reduced.
Topics: Animals; Cerebellar Diseases; Cerebellum; Dog Diseases; Dogs; Female; Hydranencephaly; Male; Phenobarbital; Porencephaly; Retrospective Studies; Seizures; Treatment Outcome; Ventriculoperitoneal Shunt
PubMed: 22186380
DOI: 10.1136/vr.100109 -
Best Practice & Research. Clinical... Jan 2017Cytomegalovirus (CMV) congenital infection affects 0.7% of live births worldwide and is the leading cause of congenital neurological handicap of infectious origin.... (Review)
Review
Cytomegalovirus (CMV) congenital infection affects 0.7% of live births worldwide and is the leading cause of congenital neurological handicap of infectious origin. However, systematic screening for this infection has not been implemented in pregnancy or at birth in any country. This apparent paradox had been justified by persisting gaps in the knowledge of this congenital infection: uncertain epidemiological data, difficulty in the diagnosis of maternal infection, absence of validated prenatal prognostic markers, unavailability of an efficient vaccine and scarcity of data available on the treatment. However, in the last decade, new data have emerged towards better management of this congenital infection, including solid epidemiological data, good evidence for the accuracy of diagnosis of maternal CMV infection and good evidence for the feasibility of predicting the outcome of fetal infection by a combination of fetal imaging and fetal laboratory parameters. There is also some evidence that valaciclovir treatment of mothers carrying an infected fetus is feasible, safe and might be effective. This review provides an update on the evidence for diagnosis, prognosis and treatment of congenital infection in the antenatal period. These suggest a benefit to a proactive approach for prenatal congenital infections.
Topics: Acyclovir; Amniotic Fluid; Antibodies, Viral; Antiviral Agents; Ascites; Cytomegalovirus Infections; DNA, Viral; Female; Fetal Diseases; Humans; Hydrocephalus; Hydrops Fetalis; Immunization, Passive; Immunoglobulin G; Immunoglobulin M; Infectious Disease Transmission, Vertical; Lissencephaly; Microcephaly; Oligohydramnios; Polyhydramnios; Porencephaly; Pregnancy; Pregnancy Complications, Infectious; Seroconversion; Ultrasonography, Prenatal; Valacyclovir; Valine
PubMed: 27923540
DOI: 10.1016/j.bpobgyn.2016.10.005 -
Journal of the American Veterinary... Jun 2013
Topics: Animals; Brain; Brain Diseases; Cat Diseases; Cats; Male; Seizures
PubMed: 23725423
DOI: 10.2460/javma.242.12.1641 -
The Journal of Veterinary Medical... Jul 2015Porencephaly is the congenital cerebral defect and a rare malformation and described few MRI reports in veterinary medicine. MRI features of porencephaly are recognized...
Porencephaly is the congenital cerebral defect and a rare malformation and described few MRI reports in veterinary medicine. MRI features of porencephaly are recognized the coexistence with the unilateral/bilateral hippocampal atrophy, caused by the seizure symptoms in human medicine. We studied 2 dogs and 1 cat with congenital porencephaly to characterize the clinical signs and MRI, and to discuss the associated MRI with hippocampal atrophy. The main clinical sign was the seizure symptoms, and all had hippocampal atrophy at the lesion side or the larger defect side. There is association between hippocampal atrophy or the cyst volume and the severe of clinical signs, and it is suggested that porencephaly coexists with hippocampal atrophy as well as humans in this study.
Topics: Animals; Atrophy; Cat Diseases; Cats; Dog Diseases; Dogs; Female; Hippocampus; Magnetic Resonance Imaging; Male; Porencephaly
PubMed: 25786357
DOI: 10.1292/jvms.14-0359 -
Proceedings of the Royal Society of... Feb 1929
PubMed: 19986820
DOI: No ID Found -
Journal of Comparative Pathology May 2019A 4-month-old puppy died after showing intracranial signs a few days after a suspected viral enteritis. Grossly, the right cerebral hemisphere had a large irregular...
A 4-month-old puppy died after showing intracranial signs a few days after a suspected viral enteritis. Grossly, the right cerebral hemisphere had a large irregular cavity external to the internal capsule. Histopathological examination revealed a cystic lesion in the right hemisphere and non-suppurative inflammation of the diencephalon and periaqueductal nervous tissue. Porencephaly associated with periventricular non-suppurative encephalitis was diagnosed. A nested polymerase chain reaction (PCR) identified the presence of parvovirus DNA in the brain and real-time PCR typed this as canine parvovirus (CPV) type 2a. Immunohistochemistry revealed the presence of CPV antigen in the cytoplasm of scattered cells in the subependymal layers and choroid plexus epithelium. The porencephaly was not associated with inflammatory lesions or CPV antigen and was considered to have preceded the neurological signs. In contrast, the detection of CPV antigen in the subependymal layers and choroid plexus epithelium supported the association of this virus with the periventricular encephalitis.
Topics: Animals; Dog Diseases; Dogs; Encephalitis, Viral; Female; Parvoviridae Infections; Parvovirus, Canine; Porencephaly
PubMed: 31159946
DOI: 10.1016/j.jcpa.2019.03.005