[MIM 213 600, 114 100]

(Bilateral striopallidodental calcinosis, FIBGC, Fahr syndrome, PFBC)

Very rare. Autosomal dominant transmission or sporadic cases. Mutation of the IBGC1 gene on 14q. Massive calcifications of the basal ganglia without abnormalities of calcium metabolism nor infectious or toxic causes. The first signs usually appear in the 3rd decade but onset in children have been reported. 

These clinical signs are:

-         parkinsonism: inexpressive facies, dysarthria, walking disorders, shivering

-         choreo-athetosis

-         ataxia.

Slow evolution towards behavioural disorders and dementia. Sometimes epilepsy.

Fahr syndrome is referred to when bilateral calcifications of the basal ganglia are associated with a phosphocalcic metabolism disorder. All dysparathyroidisms have already been described in association with this syndrome: mostly hyperparathyroidism; more rarely, hypoparathyroidism or pseudohypoparathyroidism. The treatment of dysparathyroidism can stabilize the central lesions.

Anesthetic implications: 

management of a  parkinsonian or insane patient; check calcemia

References : 

-        Kahloul N, Chaari W, Boughamoura L, Charfeddine L, Khammeri S, Amri F. 
Un syndrome de Fahr révélateur d’une pseudohypoparathyroïdie. 
Arch Pédiatr 2009 ; 16 :444-8.

Updated: November 2018