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Images in Neurology |

Density of the Brain, Decline of the Mind:  An Atypical Case of Fahr Disease

David C. Weisman, MD; Roy Yaari, MD; Lawrence A. Hansen, MD; Leon J. Thal, MD
Arch Neurol. 2007;64(5):756-757. doi:10.1001/archneur.64.5.756.
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The wife of a 66-year-old right-handed man reported that he had exhibited an insidious and slowly progressive memory problem of 5 years' duration. Initially, he forgot where he placed objects; within 2 years, he had further difficulty with writing, word finding, and maintaining his golf game. His personality changed, and he became hyperactive with angry outbursts.

His initial Mini-Mental State Examination score was 30 (of a maximum 30 points). He displayed rare paraphasic errors, mild anomic aphasia, 3 correct on a 5-item delayed recall test, and poor visual memory. Results of a neurological examination were otherwise unremarkable. Computed tomography of the head showed diffuse calcification (Figure, A). Blood chemistry results were normal, including those for calcium level and relating to the parathyroid. Neuropsychologic testing showed mild impairments on frontal lobe tests and language tests, suggestive of early frontal dementia. A lumbar puncture revealed a tau protein level of 526 pg/mL (high) and β-amyloid 42 of 1413 pg/mL (high), a nondiagnostic profile. A single-photon emission computed tomographic scan was nondiagnostic, demonstrating hypometabolism in bilateral parietal regions.

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Figure.

Computed tomography and histological analysis results in a 66-year-old man. A, Computed tomography of the head shows extensive white matter calcifications. B, Histological analysis of the left pons tegmentum shows circular calcifications within blood vessel walls. Parenchymal calcifications are also seen (von Kossa stain, original magnification ×10).

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