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         Porencephaly:     more detail
  1. The Official Parent's Sourcebook On Porencephaly: A Revised And Updated Directory For The Internet Age by Icon Health Publications, 2004-02-13
  2. Porencephaly: An entry from Thomson Gale's <i>Gale Encyclopedia of Neurological Disorders</i> by Larry, PhD Gilman, 2005

1. Porencephaly
porencephaly is an extremely rare disorder of the central nervous system involving cysts or cavities in a cerebral hemisphere.
http://healthlink.mcw.edu/article/921449639.html
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Porencephaly
Porencephaly is an extremely rare disorder of the central nervous system involving cysts or cavities in a cerebral hemisphere. The cysts or cavities are usually the remnants of destructive lesions, but are sometimes the result of abnormal development. The disorder can occur before or after birth. Most infants show symptoms of the disorder shortly after birth. Diagnosis is usually made before age 1. Signs may include delayed growth and development, spastic paresis (slight or incomplete paralysis), hypotonia (low muscle tone), seizures (often infantile spasms), and macrocephaly (large head) or microcephaly (small head). Individuals with porencephaly may have poor or absent speech development, epilepsy, hydrocephalus, spastic contractures (shrinkage or shortening of a muscle), and mental retardation. Treatment may include physical therapy, medication for seizure disorders, and a shunt for hydrocephalus.

2. NINDS Porencephaly Information Page
porencephaly information sheet compiled by NINDS.
http://www.ninds.nih.gov/health_and_medical/disorders/porencephaly.htm
National Institute of Neurological Disorders and Stroke Accessible version Science for the Brain The nation's leading supporter of biomedical research on disorders of the brain and nervous system Browse all disorders Browse all health
organizations
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Porencephaly
Studies with patients Research literature Press releases
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NINDS Porencephaly Information Page
Reviewed 06-11-2002 Get Web page suited for printing
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Table of Contents (click to jump to sections) What is Porencephaly?
Is there any treatment?

What is the prognosis?
What research is being done? ... Related NINDS Publications and Information What is Porencephaly? Porencephaly is an extremely rare disorder of the central nervous system involving cysts or cavities in a cerebral hemisphere. The cysts or cavities are usually the remnants of destructive lesions (due to stroke or infection), but are sometimes the result of abnormal development. The disorder can occur before or after birth. Most infants show symptoms of the disorder shortly after birth. Diagnosis is usually made before age 1. Signs may include delayed growth and development, spastic hemiplegia (slight or incomplete paralysis), hypotonia (low muscle tone), seizures (often infantile spasms), and macrocephaly (large head) or microcephaly (small head). Individuals with porencephaly may have poor or absent speech development, epilepsy, hydrocephalus, spastic contractures (shrinkage or shortening of a muscle), and mental retardation.

3. The Contact A Family Directory - PORENCEPHALY
printer friendly. porencephaly. home. more about us
http://www.cafamily.org.uk/Direct/p30.html
printer friendly PORENCEPHALY home more about us in your area conditions information ... how you can help search this site Porencephaly: porencephalic cyst This is a condition whereby there are isolated cavities within the cerebral hemisphere. They may or may not be single and there may be communication with ventricles and other subarachnoid space. The aetiology is thought to be that there is a localised destruction of brain tissue due to a number of causes but potentially including toxaemia, maternal injuries, infection, hypoxic injury or even intra-uterine intra-cerebral haemorrhage. Its overall incidence is unknown, aetiology must be multi-factorial and some series suggest that there is a risk that a patient with porencephaly has at 2-4% chance of having a child with a neural tube defect. There is no specific treatment other than symptomatic for conditions that may have caused it . There may be seizures but most of the time these present as incidental findings. Inheritance patterns
None.

4. Porencephaly-cerebellar Hypoplasia-internal Malformations Syndrome
A summary about porencephaly and a list of major features.
http://www.nlm.nih.gov/mesh/jablonski/syndromes/syndrome535.html
Multiple Congenital Anomaly/Mental Retardation (MCA/MR) Syndromes
View the Full Record
Syndrome porencephaly-cerebellar hypoplasia-internal malformations syndrome Summary A congenital syndrome of bilateral porencephaly, absence of the septum pellucidum, and cerebellar hypoplasia. Situs inversus and tetralogy of Fallot were observed in one of the original cases and atrial septal defect in the other. Both infants were retarded. Major Features Head and neck: Macrocephaly and mildly dysmorphic facies. Eyes: Hypertelorism, corneal clouding, pale optic nerve, and oval pupils. Mouth and oral structures: Broad alveolar ridges and highly arched palate. Muscles: Hypertonia. Nervous system: Porencephaly, absence of septum pellucidum, cerebellar hypoplasia, hydrocephalus, cerebral cortex abnormalities, hyperactive tendon reflexes, and seizures. Cardiovascular system: Situs inversus, tetralogy of Fallot, and atrial septal defect. Growth and development: Mental retardation. Behavior and performance: Feeding difficulty. Heredity: The syndrome is familial and is transmitted as an autosomal recessive trait.

5. Developmental Disorder - Online Neuropathology Atlas
Case 2 porencephaly Levels Septum Pellucidum. Cerebral Peduncle
http://www.neuropat.dote.hu/develop.htm
Developmental Brain Diseases
Case 1: Hemiatrophia cerebri (8-year-old girl)
Dorsal View Lateral View Ventral View Levels: Caudate Nucleus Septum Pellucidum Mammillary Body
Case 2: Porencephaly Levels:
Septum Pellucidum Cerebral Peduncle (click on any image for larger view)
Case 3: Agenesia vermis cerebelli
Ventral View Lateral View var site="sm5hegeduskdote" Last modified: February 17, 2001
If you have any comments, please, contact Neuroanatomy Structures Online Neuropathology Atlas Internet Handbook of Neurology

6. PORENCEPHALY
Pediatric Database (PEDBASE); Discipline CNS; Last Updated 5/22/94porencephaly. DEFINTION A congenital anomaly of the central nervous
http://www.icondata.com/health/pedbase/files/PORENCEP.HTM
  • Pediatric Database (PEDBASE)
  • Discipline: CNS
  • Last Updated: 5/22/94
    PORENCEPHALY
    DEFINTION:
    A congenital anomaly of the central nervous system (CNS) resulting in the formation of fluid-filled cysts or cavities within the cerebrum.
    EPIDEMIOLOGY:
    • incidence: ?
    • age of onset:
      • infancy to childhood
    • risk factors:
      • a well-defined destructive event (see below)
    • associated anomalies:
      • induction disorders
        • encephalocele
      • proliferation disorders
        • microcephaly
      • migration disorders
        • heterotopic grey matter
        • pachygyria
        • linear sebaceous nevi
      • developmental failure of cerebral venous sinuses
      • alopecia or other cranial defects
      TYPES:
      1. Type I - True Porencephalic Cysts
      • cysts arise by a faulty induction process and/or aberrant neuronal migration
      • associated with disorders of induction, proliferation, and other migration disorders
      • cysts most frequently located in region of the sylvian fissure
      • cysts may communicate with the ventricles, cerebral cortical surface, and/or subarachnoid space
      • a cyst communicating with the ventricles can act as a rapidly growing space-occupying lesion if a one-way valve mechanism occurs
      • associated with more severe neurologic manifestations
      2. Type II - Pseudoporencephalic Cysts
  • 7. NINDS Notes - November 2000
    Children with porencephaly, Stroke, and Cerebral Palsy Sought forStudy. Investigators at the National Institute of Neurological
    http://www.ninds.nih.gov/funding/nindsnotes11-00.htm
    National Institute of Neurological Disorders and Stroke Accessible version Science for the Brain
    The nation's leading supporter of biomedical research on disorders of the brain and nervous system
    Research funding NINDS funding strategy Funding opportunities ... Contact staff Training funding Research training and career development grants Special training and research funding Minorities and persons with disabilities Frequently asked ... Advisory council
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    NINDS Notes - November 2000 Get Web page suited for printing
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    Table of Contents
    Program Announcements (Grant Applications)
  • Basic and Translational Research on Emotion HIV-1 Infection and the Peripheral Nervous System Programs of Excellence in Biomedical Computing Research on Biomedical Information Science and Technology ... Research Relevant to Space Flight Request for Applications Functional MRI and Intervention for Cognitive Deficits After Traumatic Brain Injury Patient Volunteers Needed Menkes Disease Porencephaly, Stroke, and Cerebral Palsy
  • 8. Porencephaly
    porencephaly is an extremely rare disorder of the central nervoussystem involving a cyst or cavity in a cerebral hemisphere. The
    http://thecpnetwork.netfirms.com/porencephaly.html

    This site is hosted by
    Netfirms Web Hosting *~Porencephaly~* Directory: Cephalic Disorders Home Anecephaly Cebocephaly ... Research PORENCEPHALY is an extremely rare disorder of the central nervous system involving a cyst or cavity in a cerebral hemisphere. The cysts or cavities are usually the remnants of destructive lesions, but are sometimes the result of abnormal development. The disorder can occur before or after birth. Porencephaly most likely has a number of different, often unknown causes, including absence of brain development and destruction of brain tissue. The presence of porencephalic cysts can sometimes be detected by transillumination of the skull in infancy. The diagnosis may be confirmed by CT, MRI, or ultrasonography. More severely affected infants show symptoms of the disorder shortly after birth, and the diagnosis is usually made before age 1. Signs may include delayed growth and development, spastic paresis (slight or incomplete paralysis), hypotonia (decreased muscle tone), seizures (often infantile spasms), and macrocephaly or microcephaly. Individuals with porencephaly may have poor or absent speech development, epilepsy, hydrocephalus, spastic contractures (shrinkage or shortening of muscles), and mental retardation. Treatment may include physical therapy, medication for seizure disorders, and a shunt for hydrocephalus. The prognosis for individuals with porencephaly varies according to the location and extent of the lesion. Some patients with this disorder may develop only minor neurological problems and have normal intelligence, while others may be severely disabled. Others may die before the second decade of life.

    9. DIRECTORY.TERADEX.COM - Health/Fitness/Diseases/Neurological Disorders/Porenceph
    MCW Health Link An article about porencephaly, which is an extremely rare disorder of the central nervous system
    http://directory.teradex.com/Health_Fitness/Diseases/Neurological_Disorders/Pore
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    Search through: Entire site Porencephaly DIRECTORY Health/Fitness Diseases Neurological Disorders ... MCW Health Link - An article about porencephaly, which is an extremely rare disorder of the central nervous system involving cysts or cavities in a cerebral hemisphere. National Institute of Neurological Disorders and Stroke - Porencephaly information sheet compiled by NINDS. National Library of Medicine - A summary about porencephaly and a list of major features. The CaF Directory - A brief description of porencephaly along with a contact group for those residing in the UK.
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    10. Hydranencephaly
    Hydranencephaly is an extreme form of porencephaly (a rare disorder, discussedlater in this fact sheet, characterized by a cyst or cavity in the cerebral
    http://thecpnetwork.netfirms.com/hydranencephaly.html

    This site is hosted by
    Netfirms Web Hosting *~Hydranencephaly~* Directory: Cephalic Disorders Home Anecephaly Cebocephaly ... Research HYDRANENCEPHALY After several months of life, seizures and hydrocephalus may develop. Other symptoms may include visual impairment, lack of growth, deafness, blindness, spastic quadriparesis (paralysis), and intellectual deficits. Hydranencephaly is an extreme form of porencephaly (a rare disorder, discussed later in this fact sheet, characterized by a cyst or cavity in the cerebral hemispheres) and may be caused by vascular insult or injuries, infections, or traumatic disorders after the 12th week of pregnancy. There is no standard treatment for hydranencephaly. Treatment is symptomatic and supportive. Hydrocephalus may be treated with a shunt. The outlook for children with hydranencephaly is poor. Death generally occurs before age 1. D Angel's info@cp-network.org for further information. Last updated

    11. Porencephaly
    porencephaly Directory Cephalic Disorders.
    http://www.geocities.com/Heartland/Plains/8950/porencephaly.html

    12. The Cerebral Palsy Network
    Hydranencephaly is an extreme form of porencephaly (a rare disorder, discussedlater in this fact sheet, characterized by a cyst or cavity in the cerebral
    http://www.geocities.com/Heartland/Plains/8950/hydranencephaly.html
    The Cerebral Palsy Network
    Cephalic Disorders Anencephaly Cebocephaly Colpocephaly Ethmocephaly ... Research
    About CPN
    About CPN Join CPN Member's Center CPN's Committees ... Email Us
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    Amanda's Friends Adults with CP CPN Moms CPN UPDATES ... Award of Excellence
    Resource Center CPN's Bookstore CPN's V Store Regional Directors Disability Library ... View Our Guest book HYDRANENCEPHALY is a rare condition in which the cerebral hemispheres are absent and replaced by sacs filled with cerebrospinal fluid. Usually the cerebellum and brainstem are formed normally. An infant with Hydranencephaly may appear normal at birth. The infant’s head size and spontaneous reflexes such as sucking, swallowing, crying, and moving the arms and legs may all seem normal. However, after a few weeks the infant usually becomes irritable and has increased muscle tone (hypertonia) After several months of life, seizures and hydrocephalus may develop. Other symptoms may include visual impairment, lack of growth, deafness, blindness, spastic quadriparesis

    13. Porencephaly

    http://spinwarp.ucsd.edu/NeuroWeb/Key-Facts/br-140por.html

    14. Tuberous Sclerosis Of The Brain
    porencephaly of the Brain From the Virtual Pathology Museum, Department ofPathology, University of Connecticut Health Center. porencephaly, Brain.
    http://155.37.5.42/Code/1575.htm
    Tuberous sclerosis, Brain Tuberous Sclerosis •View of the base and frontal lobes of the brain showing tubers or hard, potato like gyri at the arrows seen in tuberous sclerosis. •Tubers contain malformed neurons and astrocytes which form the nidus for seizures often found in patients with this disease. Image Contrib. by:Margaret Grunnet, M.D. UCHC Description by: Margaret Grunnet, M.D. ( 672-6357) More Information

    15. Porencephaly
    porencephaly. What is porencephaly? The prognosis for individuals with porencephalyvaries according to the location and extent of the lesions.
    http://www.clevelandclinic.org/health/health-info/docs/1300/1324.asp?index=6099

    16. Show-documents.asp
    porencephaly Written Information. Care Treatment., porencephaly. New Search. Health Extra Menu.
    http://www.clevelandclinic.org/healthextra/do-query.asp?TopicId=1343

    17. Porencephaly Of The Brain
    porencephaly of Brain From the Virtual Pathology Museum, Departmentof Pathology, University of Connecticut Health Center.
    http://radiology.uchc.edu/eAtlas/CNS/671.htm

    18. NINDS Porencephaly Information Page
    porencephaly information sheet compiled by the National Institute of NeurologicalDisorders and Stroke (NINDS). More about NINDS porencephaly Information Page.
    http://accessible.ninds.nih.gov/health_and_medical/disorders/porencephaly.htm
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    More about NINDS Porencephaly Information Page
  • Studies with patients Research literature Press release
  • NINDS Search (search help) Contact us My privacy NINDS is part of the National Institutes of Health Contact us
    Content for this page
    NINDS Porencephaly Information Page
    Reviewed 06-11-2002 Get Web page suited for printing
    Email this to a friend or colleague

    Table of Contents (click to jump to sections)
    What is Porencephaly?

    Is there any treatment?

    What is the prognosis?

    What research is being done?
    ...
    Related NINDS Publications and Information

    What is Porencephaly?
    Porencephaly is an extremely rare disorder of the central nervous system involving cysts or cavities in a cerebral hemisphere. The cysts or cavities are usually the remnants of destructive lesions (due to stroke or infection), but are sometimes the result of abnormal development. The disorder can occur before or after birth. Most infants show symptoms of the disorder shortly after birth. Diagnosis is usually made before age 1. Signs may include delayed growth and development, spastic hemiplegia (slight or incomplete paralysis), hypotonia (low muscle tone), seizures (often infantile spasms), and macrocephaly (large head) or microcephaly (small head). Individuals with porencephaly may have poor or absent speech development, epilepsy, hydrocephalus, spastic contractures (shrinkage or shortening of a muscle), and mental retardation.

    19. Porencephaly
    HOME porencephaly. porencephaly of the Brain (Including Photo); A Pilot Study ofAbnormal Acquired and Genetic Coagulation Factors in Children with porencephaly;
    http://www.bdid.com/porencephaly.htm

    HOME
    Porencephaly

    HOME

    20. ABSENT SEPTUM PELLUCIDUM-PORENCEPHALY
    Features Listed For ABSENT SEPTUM PELLUCIDUMporencephaly. McKusickHydranencephaly/porencephaly/arachnoid cyst; Optic atrophy; Ptosis
    http://www.hgmp.mrc.ac.uk/dhmhd-bin/hum-look-up?2018

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