Presentation
Fahr's syndrome presenting with pure and progressive presenile dementia. Neurol Sci. 2005;26:367–9. [PubMed: 16388376] Morita M, Tsuge I, Matsuoka H, Ito Y, Itosu T, Yamamoto M, Morishima T. [ncbi.nlm.nih.gov]
Entire Body System
- Inflammation
Human intracellular ISG15 prevents interferon-α/β over-amplification and auto-inflammation. Nature. 2015;517:89–93. [PMC free article: PMC4303590] [PubMed: 25307056] [ncbi.nlm.nih.gov]
Psychiatrical
- Suggestibility
Mapping of the minimal inorganic phosphate transporting unit of human PiT2 suggests a structure universal to PiT-related proteins from all kingdoms of life. BMC Biochemistry. 2011;12:21. [ncbi.nlm.nih.gov]
If no other primary cause for brain calcification is detected or if the family history is suggestive of autosomal dominant inheritance, molecular genetic testing should be considered.[1]Sequencing of SLC20A2 should be pursued first. [rarediseases.info.nih.gov]
- Delusion
A young woman with visual hallucinations, delusions of persecution and a history of performing arson with possible three-generation Fahr disease. Acta Psychiatr Scand. 2010;121:75–7. [ncbi.nlm.nih.gov]
- Visual Hallucination
A young woman with visual hallucinations, delusions of persecution and a history of performing arson with possible three-generation Fahr disease. Acta Psychiatr Scand. 2010;121:75–7. [ncbi.nlm.nih.gov]
Neurologic
- Dystonia
Dystonia and calcification of the basal ganglia. Neurology. 1985;35:533–7. [ncbi.nlm.nih.gov]
Medications may be used to improve anxiety, depression, obsessive-compulsive behaviors, and dystonia. Antiepileptic drugs (AEDs) can be prescribed for seizures. Oxybutynin may be prescribed for urinary incontinence (loss of bladder control). [rarediseases.info.nih.gov]
Workup
Other Pathologies
- Neurofibrillary Tangle
Diffuse neurofibrillary tangles with calcification (Kosaka-Shibayama disease) in Japan. Psychiatry Clin Neurosci. 2016;70:131–40. [ncbi.nlm.nih.gov]
Treatment
Treatment Treatment There is no standard course of treatment for PFBC. Treatment typically addresses symptoms on an individual basis. Medications may be used to improve anxiety, depression, obsessive-compulsive behaviors, and dystonia. [rarediseases.info.nih.gov]
Prognosis
Surveillance typically includes yearly neurologic and neuropsychiatric assessments.[1] Last updated: 9/4/2015 Prognosis Prognosis The prognosis for any individual with PFBC is variable and hard to predict. [rarediseases.info.nih.gov]
Prevention
Human intracellular ISG15 prevents interferon-α/β over-amplification and auto-inflammation. Nature. 2015;517:89–93. [PMC free article: PMC4303590] [PubMed: 25307056] [ncbi.nlm.nih.gov]