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Vol. 4. Núm. 4.
(Octubre - Diciembre 2021)
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Vol. 4. Núm. 4.
(Octubre - Diciembre 2021)
Image in medicine
Open Access
Fahr's disease: An unexpected discovery
Enfermedad de Fahr, un hallazgo inesperado
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Ana Rita Parente
Autor para correspondencia
ritapp8@gmail.com

Corresponding author.
, Nídia Calado, André Gonçalves Mendes, Jerina Nogueira
Serviço de Medicina Interna da Unidade Local de Saúde do Norte Alentejano – Hospital Dr. José Maria Grande, Portalegre, Portugal
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An 81-year-old woman with a past medical history of depression, dyslipidaemia and osteoarthritis, was brought to the emergency department after having been discovered lying on the floor at home with sphincter incontinence. The patient was alert and had aphasia, right hemiparesis and a cutaneous-plantar reflex in extension on the right side.

The Head CT did not show acute ischemia or signs of bleeding, however it did reveal exuberant degenerative calcifications in the globus pallidus (Figs. 1 and 2).

Fig. 1
(0,06MB).
Fig. 2
(0,06MB).

A repeat Head CT after 48h showed a hypodense image in the left Centrum Semiovale, confirming the diagnosis of an Ischemic Stroke.

There weren’t relevant laboratory parameters changes, namely in the calcium and phosphate metabolism, thus suggesting Fahr's Disease of probable degenerative etiology.

Fahr's Disease is a rare neurodegenerative disease, most commonly characterised by symmetrical calcium deposition in the basal ganglia or in other brain areas.1,2

It may be asymptomatic or present with psychiatric2,4 or neurological symptoms,1–3 these typically appear after the 4th decade of life.

It is not associated with other disorders of the calcium and phosphate metabolism or parathyroid hormone, unlike what occurs in Fahr's Syndrome.3–5 Cerebral calcification may be an accidental finding in approximately 1% of all head CT.6

References
[1]
J. Salomão, P. Bandeira, M. Esteves.
Síndroma de Fahr – a propósito de um caso clínico.
Medicina Interna, 18 (2011), pp. 18-21
[2]
B.V. Manyam.
What is and what is not “Fahr's disease”.
Parkinson Relat Disord, 11 (2005), pp. 73-80
[3]
A. Malheiro, N. Pestana, S. Granito, A.I. Costa, M.L. Brazão.
Doença de Fahr: Um Caso Clínico Inesperado.
Med Intern, 24 (2017), pp. 33-35
[4]
E. Savino, C. Soavi, E. Capatti, M. Borrelli, G.B. Vigna, A. Passaro, et al.
Bilateral strio-pallido-dentate calcinosis (Fahr's disease): report of seven cases and revision on literature.
BMC Neurol, 16 (2016), pp. 165
[5]
G. Donzuso, G. Mostile, A. Nicoletti, M. Zappia.
Basal ganglia calcifications (Fahr's syndrome): related conditions and clinical features.
Neurol Sci, 40 (2019), pp. 2251-2263
[6]
M.H. Daghighi, V. Rezaei, S. Zarrintan, H. Pourfathi.
Intracranial physiological calcifications in adults on computed tomography in Tabriz, Iran.
Folia Morphol (Warsz), 66 (2007), pp. 115-119
Copyright © 2021. The Authors
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