Lissencephaly in Dogs

Some of the symptoms include difficulty housetraining, seizures, ataxia, mental and growth impairment and impaired motor skills. Lissencephaly will generally be diagnosed in young dogs but can go undiagnosed until your dog is older if at first the symptoms are not severe enough to cause alarm. 

There are several breeds where lissencephaly has been diagnosed. These breeds include Bernese Mountain Dog, Chow Chow, English Springer Spaniel, Goldendoodle, Golden Retriever, Samoyed, Springer Spaniel, Weimaraner, Welsh Springer Spaniel and Wire Fox Terrier. This will include any cross breeds that have these breeds in them.

Lissencephaly in dogs is a neurological disease that is extremely rare and is suspected to be a genetic defect. It is categorized as a cerebellar disease that will affect the part of the brain that controls your dog’s coordination and movement. It will cause a malformation in the gyri of the cerebral cortex. There will be an abnormal thickening of the cortical grey matter. An affected dog’s brain will be abnormally smooth.

Symptoms of lissencephaly in dogs will be detected within the first year of life. Dogs will have erratic behavior and coordination problems. If you notice your dog exhibiting any of these symptoms, contact your veterinarian for an appointment.

• Episodic aggression
• Growling at imaginary objects
• Confusion
• Depression
• Hyperactivity
• Vision problems, possible blindness
• Seizures
• Self-mutilation
• Incoordination or difficulty walking
• Mild to severe retardation 
• Delayed or stunted growth

It is difficult to understand the exact cause of lissencephaly in dogs. It is known to be a genetic disorder that usually becomes evident in the first year of your dog’s life. The actual genetic link is still unknown. Lissencephaly is rare and therefore clinical studies are also rare, making research for the cause and effective treatments slow. 

Behavioral, convulsive and visual disorders occur with this disease. Lissencephaly will affect the part of your dog’s brain that controls movement and coordination. The gyri within the cerebral cortex will be malformed and the affected dog will have an abnormally smooth brain.

In some cases you will know your dog’s parentage and if there are any genetic problems that are present. Be sure to tell your veterinarian if parents or siblings exhibited the same symptoms. This information should be included in your dog’s medical history. 

Your veterinarian will begin their assessment of your dog by asking you questions about their medical history and the symptoms that you have witnessed. A fully physical examination, including blood work, will be conducted. 

Your veterinarian will order a magnetic resonance imaging scan of your dog’s brain. Your dog’s brain will appear smooth because the gyri and sulci formation of the cerebrum have not properly formed. The cortical grey matter will appear thick. Based on the MRI findings, your dog will definitively be diagnosed with lissencephaly.

Once lissencephaly has been diagnosed in your dog, your veterinarian will discuss a treatment plan with you. There is no specified treatment for lissencephaly; therefore treatment consists of treating the symptoms as they present. 

Medications to control seizures and other neurological disorders that are caused by lissencephaly will be prescribed for your dog. During your dog’s treatment, you will want to remain in close contact with your veterinarian so any changes to your dog’s condition can be quickly assessed by your veterinarian and proper medications and/or therapies can be given.

Be sure to follow all instructions carefully when treating your dog for lissencephaly. Do not discontinue any medication without first consulting your veterinarian. If you have any questions regarding your dog’s treatment and medications, you should ask your veterinarian.

Your dog’s prognosis will be guarded. Some dogs are able to live longer lives by managing the symptoms associated with lissencephaly. Your veterinarian will be able to give you a more specific prognosis once they have fully assessed your dog’s condition and see how well they are responding to the symptomatic treatments. However, as symptoms progress it may become necessary to consider euthanasia if your dog’s quality of life is suffering. 

Dogs that have been diagnosed with lissencephaly should never be bred. Siblings and parents of dogs that are affected should also never be bred. This will help to eliminate dogs that are predisposed to developing lissencephaly from producing offspring that may also develop the disease. Researchers are working on the development of genetic testing to determine if a dog is a carrier prior to them exhibiting symptoms.