Images in Nephrology (Section Editor: G. H. Neild) Dentistry, neurology and nephrology—what is the connection?

A 28-year-old female patient was admitted because of painless macroscopic haematuria. Past history included severe mental retardation and nystagmus. At age 17, a computed tomography (CT) scan of the brain showed an absent cerebellar vermis and cerebellar hypoplasia. There was no family history of neurological or renal disease. On examination, the patient was hypertensive, did not communicate verbally, was hypotonic and had nystagmus. Laboratory investigation revealed severe kidney disease [serum blood urea nitrogen (BUN) 70mg/dl, serum creatinine 6.0mg/dl]. Urinalysis revealed haematuria and proteinuria. A renal ultra-sound showed two small, echogenic kidneys with multiple bilateral cysts. The patient was felt to hav