scholarly journals An accidental finding of FAHR’s syndrome during computed tomography of skull – A case report

Biomedicine ◽  
2021 ◽  
Vol 40 (4) ◽  
pp. 557-559
Author(s):  
Archana Rajasundaram ◽  
Nitya Waghray ◽  
W.M.S. Johnson
Keyword(s):  
Computed Tomography ◽  
Case Report ◽  
White Matter ◽  
Basal Ganglia ◽  
Dentate Nucleus ◽  
Subcortical White Matter ◽  
Radiology Department ◽  
Medical College ◽  
Fahr’S Syndrome ◽  
Middle Aged Adults

Calcification of basal ganglia is known as FAHR’s syndrome or FAHR’s disease.  It’s a rare phenomenon which generally affects the young to middle aged adults. A 39-year-old female was sent to the radiology department of Sree Balaji Medical College, Chennai for computed tomography (CT) as she had a fall and injured her forehead. An axial plain CT scan without contrast was advised and performed. The CT study revealed mild frontal peri cranial swelling and calcification of dentate nucleus, basal ganglia, central semiovale and frontoparietal subcortical white matter was observed. On examination, however patient did not present with any other movement disorders.

scholarly journals Findings of Fahr’s Syndrome in A Patient Suspected With Subarachnoid Hemorrhage: An Evidence-Based Case Report

2019 ◽  
Vol 1 (2) ◽  
pp. 8
Author(s):  
Gilbert Sterling Octavius ◽  
Theo Audi Yanto ◽  
Nicholas Gabriel H.R. ◽  
Eka Julianta Wahjoepramono ◽  
Harsan
Keyword(s):  
Computed Tomography ◽  
Differential Diagnosis ◽  
Case Report ◽  
Parathyroid Hormone ◽  
Basal Ganglia ◽  
Psychiatric Symptoms ◽  
Evidence Based ◽  
Head Ct ◽  
Fahr’S Syndrome ◽  
Chief Complaints

ABSTRACT Fahr’s syndrome is an entity where there is symmetrical bilateral calcification of basal ganglia with parkinsonian and psychiatric symptoms. However, due to its rarity, clinicians tend not to include Fahr’s syndrome in their differential diagnosis. This article aims to raise awareness about the diagnosis of Fahr’s Syndrome through its unique presentation in this patient. Data was obtained primarily and secondarily. In this case, a 32 years old male was referred to our clinic with chief complaints of headache since a week prior and a seizure 5 days ago. A head CT and a CT angiography (CTA) of the circulus willisi were done and a bilateral and symmetrical calcification of the corona radiata, basal ganglia, thalamus, and nucleus dentatus were found. There is also hypocalcemia and low Parathyroid Hormone (PTH). Fahr’s Syndrome may manifest unusually and hence clinicians have to be aware of diagnosing this entity. Keywords: Fahr’s Disease, Fahr’s Syndrome   ABSTRAK Sindroma Fahr’s merupakan sebuah penyakit yang ditandai dengan kalsifikasi bilateral sietris dari basal ganglia dengan gejala psikatri dan parkinsonisme. Namun, akibat jarangnya ditemukan penyakit ini, klinisi cenderung tidak memikirkan sindroma Fahr’s sebagai salah satu diagnosis banding. Artikel ini bertujuan untuk meningkatkan kewaspadaan terhadap diagnosis Sindroma Fahr’s melalui presentasi unik pada pasien ini. Data diambil secara primer dan sekunder. Pada kasus ini, seorang laki-laki berusia 32 tahun dirujuk dengan keluhan utama sakit kepala sejak satu minggu dan kejang 5 hari sebelum masuk rumah sakit. Computed Tomography (CT) scan kepala dan Computed Tomography Angiography (CTA) dari sirkulus willisi dilakukan dan ditemukan adanya kalsifikasi bilateral simetris pada korona radiata, basal ganglia, talamus dan nukleus dentatus. Pada pemeriksaan laboratorium ditemukan adanya hipokalsemia dan hormon paratiroid (PTH) yang rendah. Sindroma Fahr’s dapat datang dengan presentasi yang tidak khas sehingga para klinisi harus lebih tajam dalam mendiagnosis kasus ini. Kata kunci: Penyakit Fahr’s, Sindroma Fahr’s

scholarly journals Bilateral Striopallidodentate Calcinosis in Female: A case report

2021 ◽  
Vol 1 (1) ◽  
pp. 33-34
Author(s):  
Arun Kadel ◽  
Avinash Chandra ◽  
Reema Rajbhandari ◽  
Pranaya Shrestha ◽  
Pravesh Rajbhandari
Keyword(s):  
Differential Diagnosis ◽  
Case Report ◽  
White Matter ◽  
Basal Ganglia ◽  
Clinical Diagnosis ◽  
Clinical Features ◽  
Dentate Nucleus ◽  
Intracranial Calcification ◽  
Occipital Lobes

Bilateral striopallidodentate calcinosis (BSPDC) is associated with many neurological and psychiatric abnormalities and most commonly present with extra pyramidal symptoms and can be idiopathic or associated with endocrinopathy, frequently with parathyroid disorders. Here we describe a case who presented with generalized seizure. During workup, the cause of seizure was found to be bilateral and symmetric, extensive, irregular, intraparenchymal calcifications involving the basal ganglia, thalamus and dentate nucleus, white matter in the frontal, parietal, and occipital lobes and dentate nuclei of cerebellum. On the basis of clinical features, investigations, and exclusion of other causes of intracranial calcification a clinical diagnosis of BSPDC was made. BSPDC should be considered in the differential diagnosis of endocrinopathy particularly parathyroid disorders, when associated with neurological and psychiatric abnormalities.

scholarly journals Risk Factors for Silent Cerebral Infarcts in Subcortical White Matter and Basal Ganglia

Stroke ◽  
1999 ◽  
Vol 30 (2) ◽  
pp. 378-382 ◽  
Author(s):  
Toshiyuki Uehara ◽  
Masayasu Tabuchi ◽  
Etsuro Mori
Keyword(s):  
Risk Factors ◽  
White Matter ◽  
Basal Ganglia ◽  
Subcortical White Matter ◽  
Cerebral Infarcts

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.

MR Imaging and Proton MR Spectroscopy of the Brain in Behçet's Disease

2003 ◽  
Vol 16 (2) ◽  
pp. 267-273 ◽  
Author(s):  
M. Cellerini ◽  
M. Bartolucci ◽  
M. Mortilla ◽  
M. Mascalchi ◽  
F. Li Gobbi ◽  
...  
Keyword(s):  
White Matter ◽  
Basal Ganglia ◽  
Mr Imaging ◽  
Mr Spectroscopy ◽  
Subcortical White Matter ◽  
Neurological Involvement ◽  
Medium Size ◽  
Proton Mr Spectroscopy ◽  
Group A ◽  
Group B

Cerebral MR imaging (MR) and proton-MR spectroscopy (H-MRS) data are lacking in neurologically asymptomatic patients with Behçet's disease (BD). Aim of the following work was to assess MR and H-MRS characteristics of brain involvement over time in patients with BD without clinical neurological involvement. Forty cerebral MR and 12 H-MRS examinations obtained over a one to nine year follow-up in 17 patients with (group A n=9) or without (group B n=8) neurological involvement were retrospectively reviewed. Four group-B patients had a normal first MR examination whereas all group-A and four group-B patients showed single (n?3) or multiple (n=10) subcortical white matter foci of signal change. Large midbrain, basal ganglia or subcortical white matter lesions were depicted in six group-A patients. Comparison between initial and last MR examinations revealed 28 new small-to-medium size lesions. Over the course of the study, lesion enhancement was seen in five patients all belonging to group A. NAA/Cr and Cho/Cr ratios of supraventricular white matter did not show significant differences between patients and healthy controls or between group-A and group-B patients. Clinically silent cerebral involvement in the form of small-to-medium size subcortical lesions may be present at MR examination in BD patients. Large brainstem and basal ganglia lesions associated with overt neurological symptoms are characteristic of the disease. H-MRS of cerebral normal-appearing white matter does not show any significant metabolic change in BD.

scholarly journals Jaw Dystonia and Reversible Basal Ganglia Changes as an Initial Presentation of Systemic Lupus Erythematosus

2017 ◽  
Vol 8 (1) ◽  
pp. 31-34 ◽  
Author(s):  
Meghan Romba ◽  
Yujie Wang ◽  
Shu-Ching Hu ◽  
Sandeep Khot
Keyword(s):  
Systemic Lupus Erythematosus ◽  
White Matter ◽  
Basal Ganglia ◽  
Brain Imaging ◽  
Lupus Erythematosus ◽  
Subcortical White Matter ◽  
High Dose ◽  
Neuropsychiatric Lupus ◽  
Systemic Lupus ◽  
Traditional Treatment

Dystonia as a manifestation of neuropsychiatric lupus erythematosus (NPSLE) is uncommon. We report a 25-year-old woman who experienced progressive confusion, reduced speech, and difficulty opening her mouth approximately 2 weeks after development of a facial rash. Brain imaging showed bilateral, symmetric signal abnormalities within the basal ganglia and subcortical white matter. Despite treatment with high-dose steroids, she continued to have difficulty speaking with evidence of jaw dystonia on examination. Jaw dystonia rapidly improved with the initiation of levodopa. Repeat evaluation 3 months later exhibited the absence of jaw dystonia and near resolution of the imaging abnormalities. Our patient demonstrated a unique presentation with jaw dystonia refractory to traditional treatment for NPSLE. Such a presentation likely represents a severe variant of NPSLE requiring both immunosuppressive and symptomatic therapies.

scholarly journals Hemiballism-hemichorea Induced by Subcortical Ischemia

1993 ◽  
Vol 20 (4) ◽  
pp. 324-328 ◽  
Author(s):  
Fukhi Toshiya ◽  
Hasegawa YuKihiro ◽  
Seriyama Shinya ◽  
Takeuchi Toru ◽  
Sugita Koujiro ◽  
...  
Keyword(s):  
White Matter ◽  
Cerebral Ischemia ◽  
Basal Ganglia ◽  
Subthalamic Nucleus ◽  
Clinical Manifestation ◽  
Cerebral Hemisphere ◽  
Subcortical White Matter ◽  
Pathological Changes ◽  
Limb Weakness ◽  
Involuntary Movements

ABSTRACT:Four patients presented with hemiballism-hemichorea as a clinical manifestation of white matter ischemia. These patients illustrate “positive” motor phenomena rather than limb weakness as a consequence of cerebral ischemia. In each patient, the involuntary movements disappeared following worsening of paresis. Subcortical white matter infarction in three patients and hemodynamic hypo-perfusion in the cerebral hemisphere contralateral to dyskinetic movements were possible causes. Neuroradiologically, none had pathological changes in the vicinity of the subthalamic nucleus. We presume from these observations that ischemia of the subcortical white matter, without involvement of the basal ganglia or the subthalamic nucleus, may cause hemiballism-hemichorea

Hemorrhagic Venous Infarction after Excision of an Arteriovenous Malformation: Case Report

Neurosurgery ◽  
1991 ◽  
Vol 29 (2) ◽  
pp. 265-268 ◽  
Author(s):  
Yoshio Miyasaka ◽  
Kenzoh Yada ◽  
Takashi Ohwada ◽  
Akira Kurata ◽  
Kaiti Tokiwa ◽  
...  
Keyword(s):  
High Pressure ◽  
Case Report ◽  
White Matter ◽  
Arteriovenous Malformation ◽  
Time Course ◽  
Subcortical White Matter ◽  
Initial Operation ◽  
Venous Infarction ◽  
Hemorrhagic Infarction ◽  
Delayed Onset

Abstract A case of arteriovenous malformation (AVM) in which postoperative hemorrhagic infarction developed, probably because of occlusion of the former draining veins, is reported. The hemorrhage developed in the temporal lobe 3 days after the initial operation and was located in the immediate vicinity of the site of the AVM. The following findings suggest that the postsurgical hemorrhage probably resulted from a venous thrombosis: 1) no evidence of residual AVM; 2) delayed onset of the hemorrhage, inconsistent with the time course of a hemorrhage developing according to the breakthrough theory or with insufficient hemostasis with a high-pressure afterload; 3) good correlation between the location of the hemorrhage and the occlusion of the draining veins; and 4) multifocal hemorrhage affecting both the gray matter and the subcortical white matter. Postoperative hemorrhagic infarction caused by thrombosis in the draining veins is rare, but it should be considered as a distinct postoperative complication after removal of an AVM.

scholarly journals Pelizaeus-Merzbacher Disease: A Case Report

2020 ◽  
Author(s):  
Ghazaleh Jamalipour Soufi ◽  
Siavash Iravan
Keyword(s):  
Case Report ◽  
White Matter ◽  
Brain Mri ◽  
Brain Magnetic Resonance Imaging ◽  
Proteolipid Protein ◽  
Radiology Department ◽  
Magnetic Resonance Imaging Mri ◽  
Normal White Matter ◽  
Pelizaeus Merzbacher Disease ◽  
The Brain

Pelizaeus-Merzbacher Disease (PMD), as a rare genetically x-linked leukodystrophy, is a disorder of proteolipid protein expression in myelin formation. This disorder is clinically presented by neurodevelopmental delay and abnormal pendular eye movements. The responsible gene for this disorder is the proteolipid protein gene (PLP1). Our case was a oneyear-old boy referred to the radiology department for evaluating the Central Nervous System (CNS) development by brain Magnetic Resonance Imaging (MRI). Clinically, he demonstrated neuro-developmental delay symptoms. The brain MRI results indicated a diffuse lack of normal white matter myelination. This case report should be considered about the possibilityof PMD in the brain MRI of patients who present a diffuse arrest of normal white matter myelination.

scholarly journals A rare variation of hydranencephaly: case report

F1000Research ◽  
2012 ◽  
Vol 1 ◽  
pp. 22
Author(s):  
Buddhika TB Wijerathne ◽  
Geetha K Rathnayake ◽  
Sisira K Ranaraja
Keyword(s):  
Computed Tomography ◽  
Cerebral Cortex ◽  
Case Report ◽  
Basal Ganglia ◽  
Posterior Fossa ◽  
Cerebral Hemisphere ◽  
Optimal Conditions ◽  
Third Trimester ◽  
Ultrasonographic Diagnosis ◽  
Obstetric Management

Hydranencephaly is a rare severe abnormality characterized by replacement of cerebral hemisphere with fluid covered by leptomeninges such that absent cerebral cortex. We present the Ultrasonographic diagnosis of a case of fetal Hydranencephaly at 38 weeks of gestation. Sonography revealed the absence of cerebral cortex, thalami and basal ganglia with disrupted falx and preserved posterior fossa structures. This was the first reported case of Hydranencephaly with absence thalami and basal ganglia along with midbrain. Confirmation of diagnosis was made with postnatal computed tomography. Even though it was diagnosed in the late third trimester, it allowed prompt and finest obstetric management which reduced the risk for both fetus and mother. Furthermore it allowed arrangement of optimal conditions for birth at a unit with accessible specialized pediatric facilities.

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