a family history consistent with autosomal dominant inheritance The calcification is usually identified on CT scan but may be visible on plain films of the skull. Differential diagnosis Basal ganglia calcification may occur as a consequence of several other known genetic conditions and these have to be excluded before a diagnosis can be made. Management There is currently no cure for PFBC nor a standard course of treatment. The available treatment is directed symptomatic control. If parkinsonian features develop, there is generally poor response to levodopa therapy. Case reports have suggested that haloperidol or lithium carbonate may help with psychotic symptoms. One case report described an improvement with the use of a bisphosphonate. Prognosis The prognosis for any individual with PFBC is variable and hard to predict. There is no reliable correlation between age, extent of calcium deposits in the brain, and neurological deficit. Since the appearance of calcification is age-dependent, a CT scan could be negative in a gene carrier who is younger than the age of 55. Progressive neurological deterioration generally results in disability and death. History The disease was first noted by German pathologist Karl Theodor Fahr in 1930. A less common name for the condition is Chavany-Brunhes syndrome and Fritsche's syndrome, the former named after Jacques Brunhes, Jean Alfred Émile Chavany, while the later named after R. Fritsche. Fewer than 20 families had been reported in the literature up to 1997. In literature Fahr's syndrome features in Norwegian Jo Nesbø's crime fiction novel "The Snowman" (the seventh novel in the Harry Hole detective series). See also Primrose syndrome References External links Fahr Syndrome Images (MedPix) National Organization for Rare Disorders (NORD) National Institute on Aging (NIA) National Institute of Mental Health (NIMH) Neurological disorders Genetic disorders with OMIM but no gene Rare syndromes Syndromes affecting the nervous system