Lissencephaly
Also known as: Lissenzephalie · Smooth brain syndrome · Agyria · Agyrie · Cerebral lissencephaly · …
Overview
What it is
Lissencephaly is an inherited brain malformation where the cerebral cortex, which is the outer layer of the brain, fails to develop its normal folds and remains abnormally smooth. This genetic defect disrupts how nerve cells migrate during development, severely impairing the central nervous system's ability to process information.
How it presents
Symptoms typically appear in puppies between two and four months of age. Owners usually notice severe learning difficulties, behavioral changes, blindness, and frequent, difficult-to-control seizures.
Treatment
This condition is incurable, and treatment is limited to lifelong supportive care to manage symptoms like seizures. Managing this care typically costs between 1,000 and 3,500 EUR, though the prognosis is poor and often leads to early euthanasia.
How it's tested
Hüftgelenksdysplasie-Auswertung (HD-Röntgen) nach FCI(HD-FCI)
Primary testRadiographRöntgenaufnahme der Hüftgelenke unter Sedation, ausgewertet nach FCI-Standard (A-E). Standardpflicht bei den meisten VDH-Zuchtvereinen.
Issuing body: FCI
Result scheme: HD-FCI
Embark Breed + Health Panel(Embark)
DNA testUS-Anbieter; 250+ genetische Erkrankungen + Abstammung. Anerkennung in DE/AT variiert je nach VDH-Verein.
Issuing body: Embark
Result scheme: Clear/Carrier/Affected
MyDogDNA Panel(MyDogDNA)
DNA testGenoscoper-basiertes Panel (Mars Petcare). 200+ Erkrankungen + Rassenkomposition.
Issuing body: MyDogDNA
Result scheme: Clear/Carrier/Affected
Feragen Komplettpaket (rassespezifisch)(Feragen-Panel)
DNA testRassespezifisches DNA-Panel (Feragen, Österreich) — gleiches Konzept wie Labogen.
Issuing body: Feragen
Result scheme: Clear/Carrier/Affected
Labogen Komplettpaket (rassespezifisch)(Labogen-Panel)
DNA testRassespezifisches DNA-Panel (Labogen / Laboklin) — bündelt alle relevanten Erbkrankheiten der Rasse in einem Test.
Issuing body: Labogen
Result scheme: Clear/Carrier/Affected
Affected breeds
2 of 2 breeds
Treatment cost
Estimated range of typical treatment cost. Actual cost depends on severity, clinic and region.
Frequently asked
What is Lissencephaly?
Lissencephaly is an inherited brain malformation where the cerebral cortex, which is the outer layer of the brain, fails to develop its normal folds and remains abnormally smooth. This genetic defect disrupts how nerve cells migrate during development, severely impairing the central nervous system's ability to process information.
How is Lissencephaly tested?
Tests currently in our database: Hüftgelenksdysplasie-Auswertung (HD-Röntgen) nach FCI, Embark Breed + Health Panel, MyDogDNA Panel, Feragen Komplettpaket (rassespezifisch), Labogen Komplettpaket (rassespezifisch).
Which breeds are most affected?
Most commonly affected: Irish Red Setter, Lhasa Apso.