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Neurology India 2021The prevalence of hydrocephalus among patients with neurofibromatosis type I (NF1) is estimated to be between 1 and 13%. Aqueductal webs, chiasmatic-hypothalamic tumors,... (Review)
Review
The prevalence of hydrocephalus among patients with neurofibromatosis type I (NF1) is estimated to be between 1 and 13%. Aqueductal webs, chiasmatic-hypothalamic tumors, and thalamic mass effect related to NF changes are the common causes of NF1-related hydrocephalus. Brain tumors and moyamoya syndrome may mimic the clinical presentation of hydrocephalus in children with NF1, and should be ruled out while evaluating children with headaches. Treatment of NF1-related hydrocephalus should be personally tailored, including shunts, endoscopic procedures such as septostomy and third ventriculostomy, and tumor resection or debulking. Despite these personalized treatments, many of the primary treatments (including shunts and endoscopic procedures) fail, and patients should be screened and followed accordingly. In the current manuscript, we review the causes of NF1-related hydrocephalus, as well as treatment options.
Topics: Brain Neoplasms; Cerebral Aqueduct; Child; Humans; Hydrocephalus; Neurofibromatosis 1; Ventriculostomy
PubMed: 35102991
DOI: 10.4103/0028-3886.332254 -
Neurology India 2021Vein of Galen malformation (VOGM) is a fistulous arteriovenous malformation presenting in the early childhood. Hydrocephalus with VOGM develops in one half of patients... (Review)
Review
Vein of Galen malformation (VOGM) is a fistulous arteriovenous malformation presenting in the early childhood. Hydrocephalus with VOGM develops in one half of patients during the course of the disease. The concept of hydrodynamic disorders is the key to understand the development of hydrocephalus. VOGM results in venous hypertension that secondarily disturbs cerebrospinal fluid (CSF) absorption leading to hydrocephalus and occurs frequently in infants and young children. The medullary veins are thought to be the main pathway for absorption of CSF by the cerebrofugal gradient act as the driving force. In neonates, the cavernous sinus is poorly developed and brain does not use it for venous drainage along with poor jugular bulb maturation results in poor venous drainage reserve. The presence of high flow vascular malformation with poor venous drainage reserve leads to hydrodynamic disorder, poor CSF absorption, and hydrocephalus. Apart from this, hydrocephalus secondary to intraventricular hemorrhage and physical obstruction of the enlarged VOGM at the aqueduct has been proposed. The management strategy is to perform timely endovascular treatment to correct the hydrodynamic disorder and avoid ventricular shunting. Trans-arterial embolization is the effective way, as it decreases flow in the malformation, secondarily the venous hypertension, and thereby improving the clinical symptoms related to hydrodynamic disorder. Ventricular diversion procedure is indicated in symptomatic hydrocephalus after exhausting our effort to reduce hydrodynamic pressure by endovascular embolization.
Topics: Cerebral Aqueduct; Cerebral Veins; Child; Child, Preschool; Humans; Hydrocephalus; Infant; Infant, Newborn; Intracranial Arteriovenous Malformations; Vein of Galen Malformations
PubMed: 35102992
DOI: 10.4103/0028-3886.332279 -
Neurology India 2021Hydrocephalus is a neurological disorder with an incidence of 80-125 per 100,000 live births in the United States. The molecular pathogenesis of this multidimensional... (Review)
Review
Hydrocephalus is a neurological disorder with an incidence of 80-125 per 100,000 live births in the United States. The molecular pathogenesis of this multidimensional disorder is complex and has both genetic and environmental influences. This review aims to discuss the genetic and molecular alterations described in human hydrocephalus, from well-characterized, heritable forms of hydrocephalus (e.g., X-linked hydrocephalus from L1CAM variants) to those affecting cilia motility and other complex pathologies such as neural tube defects and Dandy-Walker syndrome. Ventricular zone disruption is one key pattern among congenital and acquired forms of hydrocephalus, with abnormalities in cadherins, which mediate neuroepithelium/ependymal cell junctions and contribute to the pathogenesis and severity of the disease. Given the relationship between hydrocephalus pathogenesis and neurodevelopment, future research should elucidate the genetic and molecular mechanisms that regulate ventricular zone integrity and stem cell biology.
Topics: Cerebral Aqueduct; Genetic Diseases, X-Linked; Humans; Hydrocephalus; Neural Tube Defects
PubMed: 35102976
DOI: 10.4103/0028-3886.332249 -
Eye and Brain 2023Pineal germinomas can be very complex in terms of presentation, diagnosis, and management. This review attempts to simplify this complexity in an organized manner,... (Review)
Review
Pineal germinomas can be very complex in terms of presentation, diagnosis, and management. This review attempts to simplify this complexity in an organized manner, addressing the anatomic relationships that provide the basis for the uniqueness of pineal germinoma. Ocular findings and signs and symptoms of elevated intracranial pressure are the keys to suspecting the diagnosis and obtaining the necessary imaging and cerebrospinal fluid studies. Other symptoms can suggest spread beyond the pineal region. Surgery may only be needed to obtain tissue for a definitive diagnosis, as germinoma is highly responsive to chemotherapy and focused radiation therapy. Hydrocephalus, usually related to tumor obstruction of the cerebral aqueduct, may also need to be addressed. Outcome for pineal germinoma is usually excellent, but relapse can occur and may require additional intervention. These issues are detailed in this review.
PubMed: 37077304
DOI: 10.2147/EB.S389631 -
The Journal of Physiology May 19741. To find the site where morphine acts when producing hyperglycaemia on injection into the cerebral ventricles in unanaesthetized cats, morphine sulphate was infused or...
1. To find the site where morphine acts when producing hyperglycaemia on injection into the cerebral ventricles in unanaesthetized cats, morphine sulphate was infused or injected through an implanted Collison cannula into different parts of the liquor space in an amount of 0.75 mg except on microinfusion into the posterior hypothalamus, when the amounts were 80 or 160 mug. The glucose was determined in blood samples collected from the inferior vena cava.2. Microinfusions of morphine into the posterior hypothalamus did not produce hyperglycaemia.3. Infusion of morphine into the liquor space at the entrance of the aqueduct or of the fourth ventricle produced hyperglycaemia. Any structures in the walls of the third ventricle as well as the peri-aqueductal grey are thus excluded as the site of action.4. Infusion of morphine into the subarachnoid space just above the corpora quadrigemina or below the ventral surface of the brain stem produced hyperglycaemia. With these routes the morphine does not enter any part of the ventricular cavities and the action would appear to be on structures at the ventral surface of the brain stem.5. Injection of morphine into the cisterna magna produces hyperglycaemia when the doses are larger than those already effective on injection into the cerebral ventricles. This also suggests an action on structures at the ventral surface of the brain stem, as this surface is reached more readily from the ventricles than from the cisterna.6. It is concluded that on injection into the cerebral ventricles, the morphine has to pass into the subarachnoid space, through the foramina of Luschka, in order to produce hyperglycaemia. It then reaches the ventral surface of the brain stem and probably acts there on structures in the upper part of the medulla oblongata.7. Infusion of morphine into the corpora quadrigemina near the caudal end of the superior corpora can produce profound hypoglycaemia.8. Anaesthesia depresses the morphine hyperglycaemia, but when the dose injected into the cerebral ventricles is increased four times or more, hyperglycaemia is also produced in pentobarbitone sodium anaesthesia.
Topics: Anesthesia; Animals; Blood Glucose; Brain Stem; Catheterization; Cats; Cerebral Ventricles; Consciousness; Dose-Response Relationship, Drug; Female; Hyperglycemia; Hypothalamus; Injections; Male; Morphine; Pentobarbital; Subarachnoid Space; Tectum Mesencephali
PubMed: 4853259
DOI: 10.1113/jphysiol.1974.sp010539