Idiopathic basal ganglia calcification, also known as Fahr disease , is a rare, genetically dominant, inherited neurological disorder characterized by abnormal deposits of calcium in areas of the brain that control movement. Through the use of CT scans, calcifications are seen primarily in the basal ganglia and in other areas . Bufret Oversett denne siden It has been argue however, that the term Fahr disease should be reserved for – an in fact, perhaps replaced by – primary familial brain calcification, whereas Fahr syndrome should be used only for secondary causes 6. This distinction has merit as it serves to clarify an otherwise confusing topic, and is also in line with . Familial idiopathic basal ganglia calcification (FIBGC) is a rare neurological disorder characterized by the presence of abnormal calcium deposits ( calcifications) of unknown cause.
Calcified deposits are made up of calcium carbonate and calcium phosphate, and are commonly located in the Basal Ganglia, Thalamus, Hippocampus, . Symptoms of the disorder may include deterioration of motor function, dementia, seizures . In this case series, we describe two cases of basal ganglia calcification, one of whom presented with psychotic symptoms and the other with . FD is a clinically heterogeneous condition and may manifest in the form of a broad range of . It is a rare degenerative . Also known as Familial idiopathic Basal Ganglia Calcification. Classically, bilateral calcification of the basal ganglia is seen on CT. Endemic infections, metabolic, and toxic causes should be excluded.
A 40-year-old unmarried female presented with abnormal involuntary choreo- athetoid movements involving both upper limbs for years along with features, such as bouts of disorientation, anxiety, personality changes, reckless behaviour, inappropriate laughter and progressive decline in the neurological status. These presentations usually involve basal ganglia, manifested with multiple neuropsychiatric disorders. Secondary bilateral calcification is also reported in a variety of genetic, developmental, metabolic, infectious and other conditions. Primary familial brain calcification has recently become the preferred name for this condition because mutations in specific genes are now known to be the cause.
Previously, familial idiopathic basal ganglia calcification was the preferred name. A year-old man with a history of bipolar disorder presented to the neurology clinic with a two-year history of tremor affecting both hands that was worse on action. He also complained of being “slow moving” for many years.
There was no family history of tremor or other movement disorders. On examination, his verbal. Um caso atípico de doença de Fahr. I Resident, Department of Neurosurgery, Santa Casa de Belo . Parkinsonism (symptoms such as impaired speech, muscle stiffness), tremors, and chorea (jerky involuntary movements).
A 35-year-old white man was admitted with generalised tonic-clonic seizures. He had short stature, plethoric facies, shortened metatarsals (figure, left) and metacarpals. Blood tests showed hypocalcaemia, hypomagnesaemia, hyper- phosphataemia, high serum parathyroid hormone, and normal serum . We present a case of a 55-year-old male who presented with the psychotic feature, bilateral tremors .
The basal ganglia are most commonly affecte but calcium deposits also . Calcifications may also occur in other brain regions such as dentate nucleus, thalamus, and cerebral cortex. Both familial and non-familial cases of . The clinical characteristics are various and usually appears such as disfunction of the affected areas. Bilateral almost symmetric calcification involving striatum, pallidum with or without deposits in dentate nucleus, thalamus and white matter is reported from asymptomatic individuals to a variety of neurological conditions including autosomal dominant inheritance to pseudo-pseudohypoparathyroidism.
The CT scan of brain showed multiple symmetric calcifications in the basal ganglia, thalamus, and subcortical white matter of cerebral and cerebellar areas. No underlying cause for the bilateral .