Progressive striopallidodentate calcinosis (Fahr disease)

Case contributed by Dr Mohammad A. ElBeialy

Presentation

History of seizures, gait disturbance and dysarthria.

Patient Data

Age: 25 years
Gender: Male

Bilateral almost symmetrical basal ganglia, thalamic and dentate nuclei calcifications.
Demyelinating foci are seen in the centrum semiovale, pons and bilateral brachium pontis.

They show high T2/FLAIR signal with most of these foci show restricted diffusion.

Case Discussion

Fahr's disease (familial cerebrovascular ferrocalcinosis, progressive strio-pallido-dentate calcinosis, familial idiopathic basal ganglia calcifications (FIBGC) is a chronic neurodegenerative disorder with bilateral symmetrical calcification / calcium deposition in the basal ganglia, thalami and dentate nuclei. Patients usually present in the third or fourth decade by parkinsonism-like disorders, seizures, cognitive impairment or chronic headache. Elderly patients present with progressive dementia and extrapyramidal symptoms.

The main differentials are hypoparathyroidism or pseudohypoparathyroidism or hyperparathyroidism. Other causes of bilateral BG calcification are TB, toxoplasmosis, cysticercosis, congenital HIV; yet not with that typical imaging criteria in Fahr's disease.

The centrum semiovale, pontine and brachium pontis lesions could be due to regional ischaemia related to cerebrovascular calcinosis. These lesions may calcify later.

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Case Information

rID: 23617
Case created: 28th Jun 2013
Last edited: 26th Feb 2016
Inclusion in quiz mode: Included

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