The cerebellar hemisphere

Rare disorders of the cerebellum

Some congenital defects (defects present at birth), are inherited from the parents, while others are caused by environmental factors in the womb, such as nutritional deficiencies or some viral infections. For many, the cause is unknown.

Puppies are born with a nervous system that is not fully developed, and birth defects may not become apparent until they begin to walk. In some cases, evidence of an inherited disorder may not be seen until the dog has reached adulthood, even though the defect has been present since birth.

Birth defects of the nervous system are categorized according to the primary region of the nervous system affected: forebrain, cerebellum, spinal cord, peripheral nerve and muscle disorders, or multifocal disorders that include signs of more than one area. Many of these inherited disorders are rare or breed-specific, or both. A few of the more common disorders of each area are described below.

Forebrain Disorders

Forebrain disorders (defects in the cerebrum) often result in vision problems, changes in awareness or behavior, abnormal movements or postures, and seizures.

Hydrocephalus, commonly known as “water on the brain, ” is an excess of fluid that puts pressure on the brain and may damage the cerebrum. This condition is not uncommon in puppies, especially in toy and brachycephalic breeds, such as Pugs, Bulldogs, and Bull Mastiffs. Hydrocephalus usually results in signs similar to those of a cerebral injury, and may worsen over time. However, some animals may not show any obvious signs. Blindness or impaired vision can also develop. Ultrasonography or magnetic resonance imaging (MRI) can confirm the diagnosis. This condition may be treated with corticosteroids, but surgery may be necessary in severe cases.

Idiopathic epilepsy refers to epileptic seizures of unknown cause. It may be inherited in certain breeds, including Beagles, Keeshonden, Irish Setters, Belgian Tervurens, Siberian Huskies, Springer Spaniels, Labrador Retrievers, Golden Retrievers, and German Shepherds. A diagnosis of idiopathic epilepsy depends on eliminating other causes of seizures, particularly structural brain abnormalities (such as hydrocephalus), encephalitis, or metabolic disorders such as hepatic encephalopathy.

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