Idiopathic calcification of the basal ganglia

Abstract

Summary:Idiopathic calcification of the basal ganglia is a rare disorder characterized by neuropsychiatric abnormalities, a movement disturbance of parkinsonian and/or choreoathetoid type and dense calcification of the basal ganglia. We report a 60 year old patient diagnosed as having delusional disorder and tardive dyskinesia who was subsequently found to be suffering from idiopathic calcification of the basal ganglia.

Clinical Image: Fahr’s Syndrome

“…Fahrs syndrome has already been published in Bangladesh 3 where seizure and other movement disorder are common presentation on background of autosomal dominant inheritance. The idiopathic calcification should be supported by absence of other aetiological factor of calcification 4 .as was also proved in this index case.…”

FAHR SYNDROME: Three cases presenting with psychiatric signs

“…In a research it was reported that there is a close relationship between the depressive disorder and the basal ganglion calcification (18). Chiu and colleagues reported a case with a delusionel disorder and tardive diskinesia related to basal ganglia calcification (19).…”

Familial idiopathic basal ganglia calcification (Fahr's disease) without neurological, cognitive and psychiatric symptoms is not linked to the IBGC1 locus on chromosome 14q

“…Frontal subcortical dementia is thought to be the most usual type of dementia observed in IBGC. Reported psychiatric disturbances have been mainly schizophrenia-like psychoses (Cummings et al 1983;Chiu et al 1993;Hall 1972) or mood disturbances with either depression or mania (Trautner et al 1988). Associations have been reported between basal ganglia calcification and organic mood syndromes (Forstl et al 1991;Lopez-Villegas et al 1996), affective disorder (Forstl et al 1991), obsessive compulsive disorder (Lopez-Villegas et al 1996) and neurological and psychiatric symptoms generally (König 1989).…”

“…Neurological disturbances have been well described in IBGC and include seizures, speech difficulties (notably dysarthria and palilalia), extrapyramidal signs (bradykinesia and cogwheel rigidity), cerebellar signs (ataxia, spasticity, progressive choreoathetosis and choreic movements), bucco-linguo-masticatory dyskinesia and pyramidal signs (Ellie et al 1989;Cummings et al 1983;Francis 1979;Chiu et al 1993). Cognitive abnormalities described include slow mentation, poor concentration and attention and mild to moderate impairment of verbal and non-verbal memory with normal language, abstraction, construction and praxis (Cummings and Benson 1992;Chiu et al 1993).…”

“…Cognitive abnormalities described include slow mentation, poor concentration and attention and mild to moderate impairment of verbal and non-verbal memory with normal language, abstraction, construction and praxis (Cummings and Benson 1992;Chiu et al 1993). Aphasia does not occur but palilalia and dysarthria may be prominent (Boller et al 1973).…”