Fahrs Disease

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Fahr's disease is a condition associated with calcifications within the brain. An alternate name for this condition is idiopathic bilateral basal ganglia (or striatopallidodentate) calcinosis. Many of the described cases have been associated with disturbances of calcium and phosphorus homeostasis, such as hyperparathyroidism. Familial forms have also been described. The nosology of Fahr's disease is complicated by the lack of clinical-pathological correlations and speculation on the role of calcification in the etiology of the observed syndrome.

Diagnostic criteria have been proposed based on a series of 17 patients described by Shibayama et al., although their accuracy and reliability has not been examined. Additionally, the criteria do not mention the Parkinsonism that has been associated with Fahr's disease.

Table 6

Proposed Diagnostic Criteria for Fahr's Disease

1. Insidious onset.

2. Progressive impairment of memory.

3. Early personality change (loss of social and ethical awareness).

4. Disinhibition.

5. Early loss of insight.

6. Late in the course.

a. Stereotyped and perseverative behavior and speech.

b. Mutism.

7. Brain computer tomography shows bilateral frontal and temporal atrophy and calcification of the basal ganglia.

Adapted with permission from Shibayana H, Iwai K, Takeuchi T. Clinical diagnostic criteria for non-Alzheimer nonPick dementia with Fahr's disease [NANPDF]. Neurobiol Aging 1996;17:S27, and Elsevier.

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