Fahr's disease: bilateral symmetrical striopallidodentate calcification

Hecser, L; Croitorescu, Lucian; Biris, Dacian; Siklodi, Katalin Palfi; Jung, Harald

doi:10.4323/rjlm.2010.25

Cited by 4 publications

(5 citation statements)

References 11 publications

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“…According to Hecser, the majority of Fahr's patients present between the fourth and sixth decades of life; however, the onset of symptoms in younger patients (as in our patient) and children is not rare (10). Although the parents admitted to noticing the abnormal behavior and mentality of their daughter at the age of 19 years, careful questioning and history taking from her siblings uncovered poor social interaction, non-participation in family events, avoiding everyday house and farm activities, and insomnia starting around the age of 11 years.…”

Section: Case Presentationmentioning

confidence: 54%

Fahr and #8217;s disease and frontal lobe-like cognitive dysfunction

Amin¹

2012

JMOOD

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Fahr hastalığı ve frontol lob benzeri bilişsel işlev bozukluğuGiriş: İdiyopatik, bilateral ve simetrik striatopallidodentate kalsinozis (veya Fahr hastalığı) genellikle anormal hareketler, parkinsonizm, nöbetler ve bilişsel işlev bozukluğun gibi belirtilerin değişken birliktelikleri ile yaşamın dördüncü, altıncı ve onuncu yılları arasında nadir görülen bir hastalıktır. Beyin BT görüntülemesinin yaygın kullanımı sebebi ile belirtisiz olguların teşhisi giderek artmaktadır. Olgu sunumu: Biz yaygın beyin içi kalsifikasyon ve normal kalsiyum metabolizmasına sahip; bellek bozukluğu, zihinsel yavaşlık, irritabilite, apati, kötü gece uykusu ve sosyal izolasyon gibi belirtilerin eşlik ettiği 6 yıl içinde giderek artan frontal-lob benzeri bilişsel yıkımla seyreden 25 yaşında bir kadın hastayı sunuyoruz. Hastanın genel bir rijiditesi vardı fakat titremesi yoktu. Bir buçuk yıldan fazla bir süredir devam eden takipleri sırasında hastada nöbet gelişmedi. Sonuç: Fahr hastalığı karşımıza sadece frontal lob benzeri bilişsel işlev bozukluğu olarak ve beynin radyolojik bulgularının klinik belirtileri ve hastalığın gidişatını öngöremediği bir klinik tablo ile ortaya çıkabilir.

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Section: Case Presentationmentioning

confidence: 54%

Fahr and #8217;s disease and frontal lobe-like cognitive dysfunction

Amin¹

2012

JMOOD

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“…Cases presenting emotional lability or progressive changes in personality and behavior, without parkinsonian features, have been described (Lam et al, 2007). Other neu-rological features are seizures, syncope or stroke-like events, and frontal-subcortical patterns of behavior (Hecser, Croitorescu, Siklodi, & Jung, 2010). Importantly, psychiatric symptoms such as psychosis, mood disorders, personality changes, anxiety and apathy are common (López-Villegas et al, 1996), and they were present at onset in several cases (Calabrò, Spadaro, Marra, & Bramanti, 2014;Gülsün, Baykiz, Kabatas ¸, & Belli, 2006).…”

mentioning

confidence: 99%

Imaging and neuropsychological profile of four patients with Fahr’s disease.

Zangrandi¹,

Gasparini²,

Marti³

et al. 2018

Psychology & Neuroscience

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Idiopathic basal ganglia calcification, also known as Fahr’s disease, is a rare neurological disorder characterized by abnormal calcium deposition in basal ganglia and cerebellum, usually associated with neuropsychiatric symptoms and movement disorders. The same diagnostic label probably encompasses different diseases with variable characteristics. Here we report 4 patients who meet criteria for Fahr’s disease, presenting different radiological, neuropsychological, psychiatric, and systemic features, to assess the role of cerebral calcifications in the clinical picture. All patients underwent complete neuropsychological evaluations at our unit. Cerebral computed tomography scans were obtained for all patients, and magnetic resonance images were also available for patients 1, 2, and 4. Patients presented heterogeneous radiological and neuropsychological features; overall clinical pictures varied from completely asymptomatic to severe cognitive impairment. In our patients, the site and extent of brain calcification appear only partially indicative of clinical severity because the worsening of brain calcifications does not lead to a corresponding clinical worsening.

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“…[12] FD is a clinically heterogeneous condition and may manifest in the form of a broad range of psychiatric, cognitive impairments, and neurological features. [3] FD needs to be differentiated from Fahr's syndrome, where similar clinical features are produced by underlying preexisting diseases.…”

mentioning

confidence: 99%

“…Fahr's disease (FD) is commonly called as idiopathic basal ganglia calcification and it was found that lateral thalamus, dentate nucleus of cerebellum as well as the frontal cortex also get affected as the disease progresses. [ 1 2 ] FD is a clinically heterogeneous condition and may manifest in the form of a broad range of psychiatric, cognitive impairments, and neurological features. [ 3 ] FD needs to be differentiated from Fahr's syndrome, where similar clinical features are produced by underlying preexisting diseases.…”

mentioning

confidence: 99%