Diffuse Intracranial Calcinosis

IMAGES IN NEUROLOGY

Diffuse Intracranial Calcinosis Fahr Disease

F

AHR DISEASE FEATURES

sporadic or familial calcifications of the basal ganglia leading to neurological and psychiatric disturbances. Here we describe a case of Fahr disease with striking computed tomography scan images. REPORT OF A CASE A 50-year-old man with no relevant medical history (started in 2000) suddenly experienced dysarthria and complained about having lockjaw and a deviation of the right corner of his mouth for a period of 6 hours. Although other symptoms disappeared, his dysarthria persisted. On the basis of the findings observed in a brain computed tomography scan performed at that time (Figure 1, Figure 2, and Figure 3), he was referred to the endocrinology unit. The results of his laboratory tests were normal and he was diagnosed with Fahr disease. The man was stable for the next 2 years, when his dysarthria progressively worsened, his handwriting became illegible, and he developed gait instability. One year ago, a wide-based gait became evident and he developed dysphagia for both liquid and solid food, as well as emotional lability, which partially improved with 20 mg/d of citalopram. He came to our department in August 2005 for a second opinion. He was alert and cooperative and his visual fields were nor-

mal. His Mini-Mental State Examination score was 28/28, because he was unable to write and exhibited pathological laughter. Cranial nerve testing disclosed supranuclear gaze paresis and bilateral Collier sign. A decreased bilateral gag reflex was observed. Strength was normal in all 4 limbs, but left-side Babinski, Hoffmann, and Tro¨mner signs could be elicited. Dysmetria and right bilateral dysdiadochokinesia were evident, as well as dystonic posturing of the left-upper extremity. COMMENT Here we present the case of a patient with Fahr disease featuring movement disorders, pyramidal signs, frontal lobe and cerebellar dysfunction, and most likely cranial nerve involvement. Slow progression of symptoms has been reported previously in similar cases.1,2 A thorough laboratory workup ruled out other conditions associated with brain calcifications, including hypoparathyroidism, systemic lupus erythematosus, and other genetic and metabolic diseases.3 Computed tomography scans are more sensitive to the detection of intracranial calcifications, which, in the particular case of Fahr disease, are often located in the globus pallidus. However, they can also be found in the putamen, caudate nucleus, dentate nucleus of cerebellum, thalamus, and white matter. Other locations are rare, as in the case of our patient who had diffuse intracranial

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calcifications in all of these locations as well as in the brainstem. The widespread location of the calcifications in our patient was extremely unusual. Jacobo Lester, MD Carlos Zu´n˜iga, MD Sergio Dı´az, MD Carlos Rugilo, MD Federico Micheli, MD Correspondence: Dr Micheli, Juncal 1695- P 5, Departamento J, 1062 Buenos Aires, Argentina (fmicheli @fibertel.com.ar). Author Contributions: Study concept and design: Lester, Zu´n˜iga, Dı´az, Rugilo, and Micheli. Acquisition of data: Lester, Zu´n˜iga, Rugilo, and Micheli. Analysis and interpretation of data: Lester, Dı´ az, Rugilo, and Micheli. Drafting of the manuscript: Lester, Zu´n˜iga, Dı´az, Rugilo, and Micheli. Critical revision of the manuscript for important intellectual content: Lester, Zu´ n˜ iga, Dı´ az, and Micheli. Statistical analysis: Dı´az, Rugilo, and Micheli. Administrative, technical, and material support: Zu´n˜iga, Dı´az, and Rugilo. Study supervision: Lester, Zu´n˜iga, and Micheli. Financial Disclosure: None reported. 1. Manyam BV, Walters AS, Narla KR. Bilateral striopallidodentate calcinosis: clinical characteristics of patients seen in a registry. Mov Disord. 2001;16: 258-264. 2. Warren JD, Mummery CJ, Al-Din AS, Brown P, Wood NW. Corticobasal degeneration syndrome with basal ganglia calcification: Fahr’s disease as a corticobasal look-alike? Mov Disord. 2002;17:563-567. 3. Manyam BV. What is and what is not ‘Fahr’s disease.’ Parkinsonism Relat Disord. 2005;11:73-80.

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Figure 1. Brain computed tomography scan showing bilateral and relatively symmetrical hyperdense images consistent with calcifications in the subcortical white matter of the temporal lobes, pontine tegmentum, and cerebellar hemispheres (white matter and dentate nuclei).

Figure 2. Brain computed tomography scan showing marked bilateral and symmetrical calcifications in basal ganglia predominantly in the putamens, rostral region of the mesencephalon, and subcortical white matter.

Figure 3. Brain computed tomography scan showing diffuse subcortical and paraventricular calcifications involving the U-shaped fibers and a slight ventricular dilation for the patient’s age.

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