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Fahr’s syndrome: literature review of current evidence

It comprises of developmental delay, hearing and visual loss, CNS and PNS disabilities along with Intracranial calcification of basal ganglia [ 1735 ]. The patient’s wife reported that until the day of presentation to the fncephalitis department, his cognition and gait had been completely normal. Anales de medicina interna. While hyponatremia is a common finding in VGKC encephalitis, asymptomatic, chronically low serum sodium preceding an acute delirium is unusual.

Archives of Medical science: Articles irrelevant to our study objectives enephalitis excluded. Korean J Fam Med. Neuroferritinopathy It is also a form of NBIA and it typically encepphalitis as adult onset chorea, dystonia and subtle cognitive defect along with excess iron storage or cystic degeneration in the Putamen [ 17 ]. Epidemiology To this date, very few studies have determined the frequency of basal ganglia calcification.

The plain skull radiograph has been shown to be the imaging modality of diagnostic value. Most cases present with extra pyramidal symptoms initially. Brain CT scan serves as a pre-symptomatic test in at risk individual but its not useful for predicting age of onset, severity, type of symptoms or rate of progression in an asymptomatic individual. Find articles by Julian Yaxley.


Calcifications in the putamen, thalami, caudate, and dentate nuclei are also common. Pub med, Google Scholar and Google. There is no single diagnostic test, although lumbar puncture and CSF analysis are central.

The presence of chronic hyponatremia without cognitive dysfunction, eventually culminating in an acute episode of encephalopathy and severe hyponatremia, is a pattern of natural history not previously documented in this condition.

Some exhibit subtle residual neurological deficits.

Treatment strategies involving the use of Carbamazepine, Benzipenes and Barbiturates may exacerbate underlying gait disorders [ 24 ]. Calcifications appear as clusters of punctate densities symmetrically distributed above Sella Turica and encephalitiw to the midline, while subcortical and cerebellar calcifications appear wavy [ 17 ].


Indian J of Radiology and Imaging. National Center for Biotechnology InformationU. It has been demonstrated that functional changes may precede cerebral atrophy in Fahr’s syndrome and may reflect deficits in functional circuits, which involve both the basal ganglia and the frontal, parietal, and temporal lobes [ 63 ].

These range from mild difficulty with concentration and memory to changes in personality or behaviors to psychosis and dementia [ 828 ].

It has been suggested that extent or side of the lesion have an effect on manifestation but when it comes to dementia and extrapyramidal symptoms, they become worse with more extensive calcification [ 53 ]. His spasms increased in frequency over the following months, but the patient did not seek further medical attention. No to hattatsu Brain and development.


There is also an indication to measure the levels of 1,25 hydroxy Vit D3 [ 2021 ].

Fahr’s syndrome: literature review of current evidence

The high rate of prevalence of CT-detected basal ganglia calcification in neuropsychiatric CNS brucellosis. This review was written with the aim to remark on the current substantial evidence surrounding this disease.

However, in another study, researchers found a sub-group with isolated calcification confined to Medial Pallidum [ 2326 ]. Predictive testing for at-risk asymptomatic adult family members may include brain CT scan to evaluate calcium deposits.

Extensive bilateral intracranial calcifications: These calcifications are generally confined to the Globus Pallidus and do not have associated clinical findings [ 4647 ]. Faciobrachial dystonic seizures precede Lgi1 antibody limbic encephalitis.

Infectious disease Intrauterine or perinatal Infections by Enncephalitis, rubella, CMV, herpes may result in calcifications of basal ganglia and dentate nucleus [ 17 ].

The pathophysiological mechanisms leading to Bilateral Calcification. Identification of a novel genetic locus on chromosome 8p