Primary familial brain calcification, also called familial idiopathic basal ganglia calcification or Fahr's disease, is a rare inherited neurological disorder, passed on in an autosomal dominant or recessive pattern, marked by abnormal calcium deposits in the brain regions that control movement, chiefly the basal ganglia and cerebral cortex, visible on CT scans. It typically emerges in the third to fifth decade of life, though onset can be earlier or later, often beginning with clumsiness, fatigue, an unsteady gait, slurred speech, swallowing difficulty and involuntary muscle contractions. It can progress to seizures, headaches, vision problems and dementia, along with neuropsychiatric changes such as impaired concentration and memory, behavioral change and psychosis. The disorder was first described in 1930 by Karl Theodor Fahr. No cure exists; treatment is limited to managing individual symptoms as they arise.
Facts
Classification
ICD-10 ChapterVI: Diseases of the Nervous System 2 Communicable or Non-communicable Causative Agent
Causative Agent (category) First Described Sources
1. Primary familial brain calcification (Wikipedia)
lead sentence 1
Primary familial brain calcification (PFBC), also known as familial idiopathic basal ganglia calcification (FIBGC) and Fahr's disease, is a rare, genetically dominant or recessive, inherited neurological disorder characterized by abnormal deposits of calcium in areas of the brain that control movement.
Lead: disease category
Primary familial brain calcification (PFBC), also known as familial idiopathic basal ganglia calcification (FIBGC) and Fahr's disease, is a rare, genetically dominant or recessive, inherited neurological disorder characterized by abnormal deposits of calcium in areas of the brain that control movement.
Article text
The disease was first noted by German pathologist Karl Theodor Fahr in 1930.
Lead - body-system
Primary familial brain calcification (PFBC), also known as familial idiopathic basal ganglia calcification (FIBGC) and Fahr's disease, is a rare, genetically dominant or recessive, inherited neurological disorder characterized by abnormal deposits of calcium in areas of the brain that control movement.
View the Source2. World Health Organization: ICD-10 Version:2019, Chapter VI, G20-G26
World Health OrganizationCategory G23.8Quote, Category G23.8
G23.8 Other specified degenerative diseases of basal
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