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A four-year-old neutered male bichon frise was presented for the evaluation of chronic, progressive yet episodic neurological dysfunction that was predominantly cerebellar in nature. Diagnostic testing including haematology, serum chemistry, magnetic resonance imaging, cerebrospinal fluid analysis and urine organic acid screening was normal. Trial therapies with phenobarbital, prednisone and acetazolamide were unsuccessful. Treatment with 4-aminopyridine led to complete resolution of the signs.