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Several neurological syndromes have been described in Cavalier King Charles spaniels and many of the conditions have similar clinical signs. The current knowledge of these syndromes is reviewed in this article, with the aim of enabling the general practitioner to formulate a differential diagnosis and plan for diagnostic tests and treatment. Specifically, the article discusses and contrasts the most common conditions seen, including occipital hypoplasia/syringomyelia, episodic collapse, epilepsy and vestibular disorders.