Fahr’s syndrome and clinical correlation: a case series and literature review

Michele Pistacchi; Manuela Gioulis; Flavio Sanson; Sandro Zambito Marsala

doi:10.5114/fn.2016.62538

eISSN: 1509-572x
ISSN: 1641-4640

Folia Neuropathologica

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3/2016
vol. 54

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abstract:

Case report

Fahr’s syndrome and clinical correlation: a case series and literature review

Michele Pistacchi

¹

,

Manuela Gioulis

²

,

Flavio Sanson

¹

,

Sandro Zambito Marsala

²

Neurology Service, Santorso Hospital, Santorso, Belluno, Italy
Department of Neurology, San Martino Hospital, Belluno, Italy

Folia Neuropathol 2016; 54 (3): 282-294

DOI: https://doi.org/10.5114/fn.2016.62538

Online publish date: 2016/10/03

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Introduction: Fahr’s disease is characterized by bilateral calcium deposition within the basal ganglia, cerebellar dentate nucleus and subcortical brain white matter. The main clinical manifestations are rigid or hyperkinetic syndrome, mood disorders and cognitive impairment. The correlation between neurological impairment and symmetrical basal ganglia calcification is not so frequent. Aim of the study was to report the results of neurological assessment of three sporadic cases of Fahr’s disease highlighting a correlation between the clinical syndrome and neuroimaging.

Case reports: Three adults of aged 32, 55 and 70, were studied. They all showed a heterogeneous clinical spectrum. One case developed neuropsychiatric symptoms, whereas the others complained of the tremorigen syndrome. Brain computed tomography scans revealed several calcifications in basal ganglia, cerebellar white matter and dentate nuclei.

Conclusions: The pathogenesis of Fahr’s disease is probably secondary to the dysfunction of cortico-basal connections and their interhemispheric relations. No significant correlation between calcifications and neurological symptoms is proved.

keywords:

Fahr’s syndrome and clinical correlation: a case series and literature review

Michele Pistacchi 1 , Manuela Gioulis 2 , Flavio Sanson 1 , Sandro Zambito Marsala 2

basal ganglia calcification, hypokinetic syndrome, hypoparathyroidism, hypocalcaemia

Michele Pistacchi

¹

,

Manuela Gioulis

²

,

Flavio Sanson

¹

,

Sandro Zambito Marsala

²