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Table 2 Principal clinical features of Fahr’s disease

From: Bilateral strio-pallido-dentate calcinosis (Fahr’s disease): report of seven cases and revision of literature


Fahr’s disease


Early onset

Late onset

Cognitive impairment/dementia

A cortical dementia may develop in advanced stages

Usually starts with a subcortical dementia

Psychotic disorders

Organic psychosis (basal ganglia calcification); delusion has syndromic pervasiveness behavioral, is moderately structured and organized, has low emotional participation, varied content. Abnormalities of perception are rare.

Organic psychosis (dementia): delusion is poorly structured and organized, override the contents of jealousy, poisoning, and persecution. Abnormalities of perception are frequent.

Mood disorders

May be associated to psychotic symptoms. Depressive disorders are prevalent towards maniacal ones.

Always associated with cognitive impairment, may precede symptoms as prodromes. Prevailing depression, irritability, hyper-emotionality, apathy

Anxiety disorders

Possible association between Fahr’s disease and obsessive-compulsive disorder.

Possible association between Fahr’s disease and obsessive-compulsive disorder. They can also be associated to cognitive impairment

Other neuro psychiatric/cognitive disorders

Possible attention’s disorders.

Progressive alteration of cognitive functions (attention, language, memory, constructive abilities, etc.)

Extrapyramidal movement disorders

In advanced stages of the disease.

May be present since the onset.

Response to therapy

Poor sensibility to neuroleptic treatment; high susceptibility to side effects.

Poor response to any type of symptomatic therapy. Frequent side effects.