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Familial idiopathic basal ganglia calcification (Fahr’s disease)

Amir A. Mufaddel and Ghanem A. Al-Hassani
Neurosciences Journal July 2014, 19 (3) 171-177;

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  • Table 1

    Clinical presentations of Fahr’s disease as reported in the literature.

    Psychiatric features:
     ● Cognitive deterioration: dementia, delirium, confusion2,3,9,11,17,20-22,25
     ● Psychotic symptoms:hallucinations, delusions5,20,22,23,25,27
     ● Catatonia18
     ● Mood disorders: depression, manic symptoms11,20,25,26
     ● Anxiety, panic attacks, and obsessive behaviors5,11,20,23
     ● Irritability11,26
     ● Aggression11,23
     ● Personality disorder and personality changes11,20
    Somatic symptoms:
     ● Parkinsonism and movement disorders2,3,5,7,9,20,21,31,32
     ● Seizures2,20,21,23
     ● Headache18,25
     ● Vertigo18,25
     ● Paresis18,25
     ● Stroke20
     ● Syncope18,25
     ● Ataxia3,5,9,10
     ● Dysarthria10
     ● Tremor26,31 Orthostatic hypotension30
    Radiologic findings:1,8,23,24
     ● Bilateral symmetrical calcifications of basal ganglia and dentate nucleus
     ● Other sites of calcifications: thalamus, centrum semi-ovale, cerebellum, and cerebral white matter
  • Table 2

    Differential diagnosis of brain calcifications.

    Extra-axial calcificationsIntra-axial calcifications
    Structures involved:Structures involved:
     ● Falx cerebri ● Basal ganglia
     ● The pineal gland ● Cerebellum
     ● Choroid plexusCauses:
     ● HabenulaNeoplastic:
     ● Dura and arachnoid ● Oligodendrogliomas
     ● Tentorium cerebelli ● Astrocytomas
     ● Superior sagittal sinus ● Medulloblastomas
     ● Petroclinoid and interclinoid ligaments ● Other primary brain tumours
     ● Arachnoid granulations ● Metastatic tumors
    Causes:Vascular:
     ● Meningiomas ● Angiomatous malformations
     ● Dural osteomas ● Arteriovenous malformations
     ● Calcifying tumours ● Dystrophic calcification in chronic infarction
     ● Exaggerated physiological calcifications ● Chronic vasculitis
     ● Aneurysms
    Infectious:
     ● Congenital childhood infections, particularly TORCH
     ● Tuberculosis
     ● Parasitic infections such as neurocysticercosis and cerebral hydatid cyst disease
    Congenital:
     ● Sturge-Weber syndrome
     ● Tuberous sclerosis
     ● Lipomas
     ● Neurofibromatosis
    Endocrine/metabolic:
     ● Diabetes mellitus
     ● Hypoparathyroidism
     ● Pseudohypoparathyroidism
     ● Hyperparathyroidism
    Idiopathic/genetic:
     ● Familial idiopathic basal ganglia calcification.
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