Mitochondrial fission encephalopathy(MFE)
Also known as: Mitochondriale Fissions-Enzephalopathie · Encephalopathy due to defective mitochondrial fission · MFF-related encephalopathy · Familial cerebellar ataxia with hydrocephalus
Overview
What it is
Mitochondrial fission encephalopathy, also known as MFF-related encephalopathy, is a severe inherited brain disorder where the normal division of mitochondria (the energy-producing parts of a cell) is impaired. Without this division, nerve cells in the brain suffer a severe energy deficit and progressively die. This condition is historically referred to as familial cerebellar ataxia with hydrocephalus.
How it presents
Symptoms typically become noticeable during puppyhood and include severe neurological issues such as seizures, blindness, difficulty moving or coordinating, and sudden behavioral changes.
Treatment
There is no cure for this disease, and treatment is limited to palliative care to manage pain and symptoms. Supportive care typically costs between 500 and 2000 EUR, and euthanasia is eventually required to prevent suffering.
How it's tested
Mitochondriale Fissions-Enzephalopathie (MFE) Gentest(MFE)
Primary testDNA testDer DNA-Test identifiziert die ursächliche Mutation im DNM1L-Gen, welche für die mitochondriale Fissions-Enzephalopathie verantwortlich ist, und ermöglicht den Nachweis von Trägern und betroffenen Hunden.
Issuing body: other
Result scheme: Clear/Carrier/Affected
Affected breeds
Treatment cost
Estimated range of typical treatment cost. Actual cost depends on severity, clinic and region.
Frequently asked
What is Mitochondrial fission encephalopathy?
Mitochondrial fission encephalopathy, also known as MFF-related encephalopathy, is a severe inherited brain disorder where the normal division of mitochondria (the energy-producing parts of a cell) is impaired. Without this division, nerve cells in the brain suffer a severe energy deficit and progressively die. This condition is historically referred to as familial cerebellar ataxia with hydrocephalus.
How is Mitochondrial fission encephalopathy tested?
Tests currently in our database: Mitochondriale Fissions-Enzephalopathie (MFE) Gentest.
Which breeds are most affected?
This condition isn't tied to a specific breed in our database.