Shaking Puppy Syndrome, X-linked Generalized Tremor Syndrome

When axonal disease is present, affected animals show signs that are consistent with a delay between the brain and the body: sometimes they walk with an uncoordinated gait, or don't seem to know where their limbs are relative to the ground, leading to scuffing or dragging of the paws.

Signs develop in puppies.

Unless a genetic basis is suspected due to the age, breed, or history of the dog, diagnostics must be performed to rule out infectious, inflammatory, or neoplastic causes.

There is no treatment for this disorder. Certain medications may be prescribed to control symptoms.

Talk to your vet about your dog’s shaking puppy syndrome result so you can plan appropriate monitoring and care.

Signs such as body tremors usually appear in very young puppies and may improve as they mature, though some dogs continue to show mild tremors as adults.

Keep your puppy’s environment calm and free from slippery surfaces to help them move safely and avoid injury.

Gentle handling, supportive care, and patience during development can help your puppy stay comfortable and adapt as they grow.

In English Springer Spaniels, X-linked generalized tremor has been mapped to the PLP gene. PLP has a crucial role in oligodendrocyte development and myelin sheath compaction: in its absence, myelination will be inappropriately established, leading to tremors beginning as early as 12 days of age and culminating in severe seizures and death by 3-4 months of age. Currently there is no treatment for shaking puppy syndrome.

Penetrance: This mutation has a sex-linked recessive inheritance pattern, which means that the gene is on the X chromosome. Females have two X chromosomes and must inherit two abnormal copies to be affected with this disease. Males have only one X chromosome and either have a normal copy of the gene or the mutation.

Gene

PLP1

Inheritance type

x-linked recessive

Clinical category

Neurologic

Citations

1. Nadon et al 1990

• English Springer Spaniel