scholarly journals A Case of Fahr's Disease Presenting as Chorea Successfully Treated by the Use of Quetiapine

2009 ◽  
Vol 2 ◽  
pp. CCRep.S3423
Author(s):  
Syoichiro Kono ◽  
Yasuhiro Manabe ◽  
Tomotaka Tanaka ◽  
Daiki Fujii ◽  
Yasuko Sakai ◽  
...  
Keyword(s):  
Computed Tomography ◽  
Cerebrospinal Fluid ◽  
Lactic Acid ◽  
White Matter ◽  
Calcium Phosphate ◽  
Subcortical White Matter ◽  
Laboratory Studies ◽  
Brain Computed Tomography ◽  
Fahr’S Disease ◽  
Fahr's Disease

We report a case of 30-year-old man presenting chorea in his legs. A brain computed tomography (CT) scan showed bilateral symmetric calcifications in the basal ganglia, thalamus, cerebellum and subcortical white matter. Laboratory studies showed no abnormalities of serum calcium, phosphate, PTH, lactic acid, pyruvic acid and cerebrospinal fluid. Under the diagnosis of Fahr's disease (FD), we treated with quetiapine (75 mg/day), which completely abolished his symptoms and he showed no other side effect. Our experience suggests that quetiapine is well tolerated in FD patients and effectively treats chorea without extrapyramidal movement.

scholarly journals Fahr’s Disease: A Rare Neurodegenerative Disorder in Children

1970 ◽  
Vol 20 (1) ◽  
pp. 86-88 ◽  
Author(s):  
AKMM Rahman ◽  
RS Begum ◽  
MZ Hossain ◽  
MR Ali ◽  
M Rahman
Keyword(s):  
White Matter ◽  
Basal Ganglia ◽  
Ct Scan ◽  
Rare Case ◽  
Neurodegenerative Disorder ◽  
Subcortical White Matter ◽  
Basal Ganglia Calcification ◽  
Fahr’S Disease ◽  
Unusual Condition ◽  
Fahr's Disease

A case of Fahr’s disease in reported in a 2 year 9 month old male child presented with developmental delay and seizure. 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 calcification was found. This rare case of Fahr’s disease in a child, which has never been reported in Bangladeshi literature, has been brought out to highlight this unusual condition. Key words: Fahr’s disease; basal ganglia; calcification. DOI: http://dx.doi.org/10.3329/jdmc.v20i1.8589 J Dhaka Med Coll. 2011; 20(1) :86-88

Fahr’s disease with an initial presentation of crescendo TIA

2021 ◽  
Vol 14 (6) ◽  
pp. e242837
Author(s):  
Paul Smith ◽  
Kalun Ng ◽  
Kailash Krishnan
Keyword(s):  
White Matter ◽  
Psychiatric Symptoms ◽  
Symptomatic Relief ◽  
Management Strategies ◽  
Initial Presentation ◽  
Fahr’S Disease ◽  
Fahr's Disease ◽  
Personality Changes ◽  
Brain Calcification ◽  
Ischaemic Attack

A 51-year-old man presented with vertigo, slurred speech and left facial droop. He had been previously diagnosed with transient ischaemic attack (TIA) and had a prior lacunar infarct. Imaging showed heavy symmetrical calcification in the globus pallidus, frontal white matter and cerebellar dentate nuclei/deep white matter. The imaging was pathognomonic for Fahr’s disease and diagnosis was confirmed when other secondary causes of hypercalcemia were excluded. Fahr’s disease is a rare, autosomal dominant, neurological condition characterised by primary brain calcification. Patients present with progressive neurological and psychiatric symptoms; commonly, Parkinsonian movement disorders, seizures, headaches, dysarthria, cognitive decline, psychosis and personality changes. There is an association with intracerebral ischaemic events. This case supports a growing body of anecdotal evidence of this association and is the first in which crescendo TIA may be the initial presentation of Fahr’s disease. Referral for genetic counselling and symptomatic relief for neurological symptoms are the main management strategies.

scholarly journals The prognostic value of gray–white matter ratio on brain computed tomography in adult comatose cardiac arrest survivors

2018 ◽  
Vol 81 (7) ◽  
pp. 599-604 ◽  
Author(s):  
Gan-Nan Wang ◽  
Xu-Feng Chen ◽  
Jin-Ru Lv ◽  
Na-Na Sun ◽  
Xiao-Quan Xu ◽  
...  
Keyword(s):  
Computed Tomography ◽  
Cardiac Arrest ◽  
White Matter ◽  
Prognostic Value ◽  
Brain Computed Tomography

scholarly journals TINJAUAN ASPEK RADIOLOGIS FAHR'S DISEASE

2019 ◽  
Vol 2 (2) ◽  
pp. 49-51
Author(s):  
Jimmy Indarto Gunawan ◽  
Rizaldy Taslim Pinzon
Keyword(s):  
Computed Tomography ◽  
Ct Scan ◽  
Fahr’S Disease ◽  
Fahr's Disease ◽  
Diabetes Melitus

Latar Belakang: Fahr’s disease merupakan penyakit langka saat deposit kalsium abnormal berada di area otak yang mengontrol pergerakan. Kasus: Wanita 54 tahun datang ke mengeluh badan lemah, sulit menelan, demam, dan sulit berbicara. Pasien memiliki riwayat diabetes melitus, hipertensi dan stroke. Pemeriksaan fisik menunjukkan tekanan darah tinggi dan tonus otot meningkat. Pemeriksaan laboratorium menunjukkan kondisi hiperglikemia. Pemeriksaan computed tomography (CT)-scan pasien menunjukkan gambaran kalsifikasi di ganglia basalis dan kedua hemisfer serebelum. Diskusi: Fahr’s disease merupakan penyakit yang memiliki satu atau lebih gejala gangguan neurologis, kognitif, dan psikiatri secara progresif atau dengan adanya kombinasi dari kalsifikasi ganglia basalis yang simeteris. Pasien pada kasus ini tergolong sebagai Fahr’s disease idiopatik. Penyakit ini bersifat progresif dan memerlukan CT-scan serial untuk mengevaluasi keberhasilan pengobatan. Saat ini belum ada terapi definitif Fahr’s disease, dan terapi masih simptomatik. Simpulan: Penemuan kasus Fahr’s disease memerlukan pemeriksaan yang runtut dan evaluasi CT-scan berkala sembari mencari faktor risiko pasien. Kata Kunci: Fahr’s disease, CT scan, radiologis, gambaran radiologis

Gray Matter–White Matter De-Differentiation on Brain Computed Tomography Predicts Brain Death Occurrence

2016 ◽  
Vol 48 (6) ◽  
pp. 1893-1897 ◽  
Author(s):  
C. Vigneron ◽  
V. Labeye ◽  
M. Cour ◽  
S. Hannoun ◽  
A. Grember ◽  
...  
Keyword(s):  
Computed Tomography ◽  
White Matter ◽  
Brain Death ◽  
Gray Matter ◽  
Brain Computed Tomography

scholarly journals High Levels of Copper, Zinc, Iron and Magnesium, but not Calcium, in the Cerebrospinal Fluid of Patients with Fahr’s Disease

2010 ◽  
Vol 2 (2) ◽  
pp. 46-51 ◽  
Author(s):  
Isao Hozumi ◽  
Akihiro Kohmura ◽  
Akio Kimura ◽  
Tatsuya Hasegawa ◽  
Akiko Honda ◽  
...  
Keyword(s):  
Cerebrospinal Fluid ◽  
Fahr’S Disease ◽  
Fahr's Disease ◽  
Copper Zinc

scholarly journals Multimodal Long-Term Predictors of Outcome in Out of Hospital Cardiac Arrest Patients Treated with Targeted Temperature Management at 36 °C

2021 ◽  
Vol 10 (6) ◽  
pp. 1331
Author(s):  
Erik Roman-Pognuz ◽  
Jonathan Elmer ◽  
Frank X. Guyette ◽  
Gabriele Poillucci ◽  
Umberto Lucangelo ◽  
...  
Keyword(s):  
Computed Tomography ◽  
Cardiac Arrest ◽  
White Matter ◽  
Temperature Management ◽  
Single Test ◽  
Brain Computed Tomography ◽  
Good Outcome ◽  
Eeg Reactivity ◽  
Single Predictor

Introduction: Early prediction of long-term outcomes in patients resuscitated after cardiac arrest (CA) is still challenging. Guidelines suggested a multimodal approach combining multiple predictors. We evaluated whether the combination of the electroencephalography (EEG) reactivity, somatosensory evoked potentials (SSEPs) cortical complex and Gray to White matter ratio (GWR) on brain computed tomography (CT) at different temperatures could predict survival and good outcome at hospital discharge and six months after the event. Methods: We performed a retrospective cohort study including consecutive adult, non-traumatic patients resuscitated from out-of-hospital CA who remained comatose on admission to our intensive care unit from 2013 to 2017. We acquired SSEPs and EEGs during the treatment at 36 °C and after rewarming at 37 °C, Gray to white matter ratio (GWR) was calculated on the brain computed tomography scan performed within six hours of the hospital admission. We primarily hypothesized that SSEP was associated with favor-able functional outcome at distance and secondarily that SSEP provides independent information from EEG and CT. Outcomes were evaluated using the Cerebral Performance Category (CPC) scale at six months from discharge. Results: Of 171 resuscitated patients, 75 were excluded due to missing data or uninterpretable neurophysiological findings. EEG reactivity at 37 °C has been shown the best single predictor of good out-come (AUC 0.803) while N20P25 was the best single predictor for survival at each time point. (AUC 0.775 at discharge and AUC 0.747 at six months follow up). The predictive value of a model including EEG reactivity, average GWR, and SSEP N20P25 amplitude was superior (AUC 0.841 for survival and 0.920 for good out-come) to any combination of two tests or any single test. Conclusions: Our study, in which life-sustaining treatments were never suspended, suggests SSEP cortical complex N20P25, after normothermia and off sedation, is a reliable predictor for survival at any time. When SSEP cortical complex N20P25 is added into a model with GWR average and EEG reactivity, the predictivity for good outcome and survival at distance is superior than each single test alone.

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