Subcortical band heterotopia
Zuletzt aktualisiert 18. Aug 2020
Erfüllt die redaktionelle Richtlinien
- HerunterladenHerunterladen
- Teilen
- Language
- Diskussion
- Audio-Version
- Zu bevorzugten Quellen bei Google hinzufügen
Subcortical band heterotopia is a condition in which nerve cells (neurons) do not move (migrate) to their proper locations in the fetal brain during early development. (Heterotopia means "out of place.") Normally, the neurons that make up the outer surface of the brain (cerebral cortex) are distributed in a well-organized and multi-layered way. In people with subcortical band heterotopia, some neurons that should be part of the cerebral cortex do not reach it. These neurons stop their migration process in areas of the brain where they are not supposed to be and form band-like clusters of tissue. Since these bands are located beneath the cerebral cortex, they are said to be subcortical. In most cases, the bands are symmetric, which means they occur in the same places on the right and left sides of the brain.
Beschreibung
Subcortical band heterotopia is a condition in which nerve cells (neurons) do not move (migrate) to their proper locations in the fetal brain during early development. (Heterotopia means "out of place.") Normally, the neurons that make up the outer surface of the brain (cerebral cortex) are distributed in a well-organized and multi-layered way. In people with subcortical band heterotopia, some neurons that should be part of the cerebral cortex do not reach it. These neurons stop their migration process in areas of the brain where they are not supposed to be and form band-like clusters of tissue. Since these bands are located beneath the cerebral cortex, they are said to be subcortical. In most cases, the bands are symmetric, which means they occur in the same places on the right and left sides of the brain.
The abnormal brain development causes neurological problems in people with subcortical band heterotopia. The signs and symptoms of the condition depend on the size of the bands and the lack of development of the cerebral cortex. The signs and symptoms can vary from severe intellectual disability and seizures that begin early in life and affect both sides of the brain (generalized seizures) to normal intelligence with seizures occurring later in life and affecting only one side of the brain (focal seizures). Some affected individuals also have weak muscle tone (hypotonia), loss of fine motor skills such as using utensils, or behavioural problems. Subcortical band heterotopia is typically found when brain imaging is done following the onset of seizures, usually in adolescence or early adulthood.
Inheritance patterns
The source lists the following possible inheritance patterns: Not inherited; X-linked. More than one pattern can apply in different cases.
Related genes
Other names
DC syndrome
Double cortex syndrome
Heterotopia, subcortical band
SBH
SCLH
Subcortical laminar heterotopia
Related patient information and medicines
Patient information leaflet
Gedächtnisverlust und Demenz
Vascular dementia is caused by reduced blood flow ... This can happen for a variety of reasons. One type of vascular dementia, genannt subcortical vascular dementia, occurs due to damage to small blood vessels supplying the brain. Another type, multi-infarct dementia, occurs as a result of several different small strokes affecting different areas.
Patient information leaflet
Uveitis
Herpes zoster . Cytomegalovirus. Gonorrhoea . Tuberculosis. Lyme disease. HIV and syphilis are rare infectious causes. Injury to the eye Uveitis can occur after injury to your eye. Iatrogenic causes 'Iatrogenic' refers either to an unforeseen or to an inevitable side-effect from a medical treatment. In this case, iatrogenic uveitis is usually.
Patient information leaflet
Hämorrhoiden
Grade 3 piles hang out. You may feel one or more as small, soft lumps that hang from the bottom. However, you can push them back inside the bottom with a finger. Grade 4 piles permanently hang down from within the bottom (prolapse), and you cannot push them back inside. They sometimes become quite large. Types of piles Piles can be divided into.
Patient information leaflet
Peyronie-Krankheit
Peyronie's disease causes a curved or bent penis. It is caused by scar tissue forming along the shaft of the penis. This causes painful erections and a bend or curve, usually about halfway along the penis. The change in shape is usually only obvious when the penis is erect. Peyronie's disease symptoms Initial symptoms of Peyronie's disease.
Patient information leaflet
Barrett-Ösophagus
In Barrett's oesophagus the cells that line the lower gullet (oesophagus) sind abnormal. The main cause is long-standing reflux of acid from the stomach into the oesophagus. People with Barrett's oesophagus have an increased risk of developing cancer of the oesophagus. The risk is small. However, you may be advised to have regular endoscopies to.
Patient information leaflet
Bruststraffung
This leaflet is provided by the British Association of Aesthetic Plastic Surgeons , the professional body responsible for the advancement of education and safety in aesthetic plastic surgery. Mastopexy (up-lifting of droopy breasts) Droopiness of the breast is a common legacy of motherhood, nursing and the force of gravity taking their toll, and.
Weiterführende Lektüre und Referenzen
- Subcortical band heterotopia; MedlinePlus, National Library of Medicine.
- Bahi-Buisson N, Souville I, Fourniol FJ, Toussaint A, Moores CA, Houdusse A, Lemaitre JY, Poirier K, Khalaf-Nazzal R, Hully M, Leger PL, Elie C, Boddaert N, Beldjord C, Chelly J, Francis F; SBH-LIS European Consortium. New insights into genotype-phenotype correlations for the doublecortin-related lissencephaly spectrum. Brain. 2013 Jan;136(Pt 1):223-44. doi: 10.1093/brain/aws323.
- Fry AE, Cushion TD, Pilz DT. The genetics of lissencephaly. Am J Med Genet C Semin Med Genet. 2014 Jun;166C(2):198-210. doi: 10.1002/ajmg.c.31402. Epub 2014 May 23.
- Gonzalez-Moron D, Vishnopolska S, Consalvo D, Medina N, Marti M, Cordoba M, Vazquez-Dusefante C, Claverie S, Rodriguez-Quiroga SA, Vega P, Silva W, Kochen S, Kauffman MA. Germline and somatic mutations in cortical malformations: Molecular defects in Argentinean patients with neuronal migration disorders. PLoS One. 2017 Sep 27;12(9):e0185103. doi: 10.1371/journal.pone.0185103. eCollection 2017.
- Ito K, Nakata Y, Matsuda H, Sugai K, Watanabe M, Kamiya K, Kimura Y, Shigemoto Y, Okazaki M, Sasaki M, Sato N. Evaluation of FDG-PET and ECD-SPECT in patients with subcortical band heterotopia. Brain Dev. 2014 Aug;36(7):578-84. doi: 10.1016/j.braindev.2013.07.017. Epub 2013 Aug 16.
- Parrini E, Conti V, Dobyns WB, Guerrini R. Genetic Basis of Brain Malformations. Mol Syndromol. 2016 Sep;7(4):220-233. doi: 10.1159/000448639. Epub 2016 Aug 27.
Artikelverlauf
Artikel auch verfügbar in Englisch, Deutsch, Spanisch, Französisch, Italienisch, Portugiesisch, Hindi, Hebräisch, Arabisch, und Schwedisch.
18. Aug 2020 | Neueste Version
1 Aug 2018 | Ursprünglich veröffentlicht
Nehmen Sie an der Diskussion teil
Teilen Sie Ihre Gedanken oder Erfahrungen mit anderen in unserem Patientengemeinschaften.
Fragen, teilen, verbinden.
Durchsuchen Sie Diskussionen, stellen Sie Fragen und teilen Sie Erfahrungen zu Hunderten von Gesundheitsthemen.
Abonnieren Sie den Patienten-Newsletter
Ihre wöchentliche Dosis klarer, vertrauenswürdiger Gesundheitsberatung - geschrieben, um Ihnen zu helfen, sich informiert, selbstbewusst und in Kontrolle zu fühlen.
Durch das Abonnieren akzeptieren Sie unsere Datenschutzrichtlinie. Sie können sich jederzeit abmelden. Wir verkaufen Ihre Daten niemals.