Transcranial Sonography of Basal Ganglia Calcifications in Fahr Disease

Toscano, Massimiliano; Canevelli, Marco; Giacomelli, Elena; Zuco, Carmela; Fiorelli, Marco; Piero, Vittorio Di; Lenzi, Gian Luigi; Vicenzini, Edoardo

doi:10.7863/jum.2011.30.7.1032

Cited by 9 publications

(5 citation statements)

References 6 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Compared with PD, hyperechogenicity of the LN has been reported more frequently in atypical parkinsonism, including the parkinsonian phenotype of a multiple system atrophy or progressive supranuclear palsy [ 44 , 45 ]. Interestingly, LN hyperechogenicity has also been described in patients with Wilson’s disease [ 46 ], Creutzfeldt–Jakob disease [ 47 ], primary focal dystonia [ 48 ], Fahr’s disease [ 49 ], and pantothenate kinase-associated neurodegeneration [ 50 ]. This would suggest a common, or at least a similar, pathomechanism underlying LN hyperechogenicity, which might lie in an iron–copper–calcium accumulation and the subsequent gliosis [ 43 , 46 ].…”

Section: Discussionmentioning

confidence: 99%

Diabetic Striatopathy: Parenchymal Transcranial Sonography as a Supplement to Diagnosis at the Emergency Department

Godani¹,

Lanza

2022

Diagnostics

View full text Add to dashboard Cite

Background: Diabetic striatopathy (DS) is a rare condition with a debated pathophysiology; a local metabolic dysfunction is the most likely hypothesis. We present a case of DS mimicking an acute stroke, outline a few uncommon/atypical features, and report for the first time the parenchymal transcranial sonography (pTCS) findings. Case Report: An 86-year-old man, treated for insulin-dependent diabetes, presented at an emergency department because of the occurrence of isolated choreo-athetotic movements in his left limbs with fluctuations in the location, frequency, and duration. The blood glucose level was 569 mg/dL. Both urgent and follow-up brain computed tomography (CT) were negative for recent lesions whereas pTCS revealed hyperechogenicity in the right lenticular nucleus. Subsequent magnetic resonance imaging (MRI) showed T1-weighted hyperintensity in the right putamen with negative diffusion-weighted imaging. The symptoms were responsive to glucose control and haloperidol administration, although they persisted during sleep. Conclusions: Unlike previously described cases characterized by hemichorea and/or hemiballism, our patient presented with a stroke-like onset of unilateral irregular choreo-athetotic movements. Notably, based on CT alone, it would not have been possible to distinguish DS from a stroke. In this scenario, the pTCS hyperechogenicity of the right lenticular nucleus helped to hypothesize a metabolic disorder, which was subsequently confirmed by MRI.

show abstract

Section: Discussionmentioning

confidence: 99%

Diabetic Striatopathy: Parenchymal Transcranial Sonography as a Supplement to Diagnosis at the Emergency Department

Godani¹,

Lanza

2022

Diagnostics

View full text Add to dashboard Cite

show abstract

“…Fahr's Syndrome (FS) is a rare neurological disorder of abnormal symmetric calcium deposition, primarily in the basal ganglia, cerebellum dentate nuclei, thalami, cerebellum, hippocampus, and cerebral hemisphere white matter [1]. The calcifications are calcium carbonate and calcium phosphate [2].…”

Section: Discussionmentioning

confidence: 99%

“…First described in 1930 by the German pathologist, Karl Theodor Fahr, Fahr's Syndrome (FS) is a rare neurological disorder of abnormal calcium deposition. FS is characterized by deterioration of motor function, neuropsychiatric impairment and seizures [1]. These symptoms are due to calcifications of the basal ganglia and cerebral cortex.…”

Section: Introductionmentioning

confidence: 99%

Idiopathic hypoparathyroidism induced fahr’s syndrome

Stuart¹,

Duarte²,

Gilbert³

et al. 2021

J Clin Images Med Case Rep

View full text Add to dashboard Cite

Fahr’s Syndrome (FS) is a rare neurological disorder of abnormal calcium deposition. FS is most notably characterized by deterioration of motor function, neuropsychiatric impairment and seizures. These symptoms are primarily due to calcification of the basal ganglia and cerebral cortex. Although there are currently no curative therapies, treatments are available which can significantly improve quality of life, making early diagnosis and identification of etiology essential. Hypoparathyroidism with intracranial calcification is extremely rare clinically. Here we present a case of idiopathic hypoparathyroidism induced Fahr’s syndrome with classic symptomatology, imaging of examination findings and extensive cerebral calcifications. Keywords: Fahr’s Syndrome; idiopathic hypoparathyroidism.

show abstract

“…Calcification of the basal ganglia and the cerebellum are obvious in approximately 40% to 72% of autopsies . Fahr disease (or BSCP) is characterized by symmetrical calcifications of the basal ganglia and the dentate nuclei of the cerebellum and the thalami . Calcifications can be detected using transcranial sonography .…”

Section: Discussionmentioning

confidence: 99%

Atypical Occipital Calcinosis in a Caucasian Individual with Probable Diffuse Neurofibrillary Tangles with Calcification

Faissner

Hoepner

Ellrichmann

et al. 2014

J American Geriatrics Society

View full text Add to dashboard Cite

subsequent transformation to PLL or whether the weight loss could be attributed to his initial indolent PLL.Therapy is indicated only for individuals with symptomatic and progressive disease, such as other indolent lymphoid malignancies, but given the rarity of PLL, only retrospective studies have been done. 2 Rituximab, fludarabine, and anthracycline monotherapy or combination therapy are some of the treatments for PLL. 2 Other options include alemtuzumab or hematopoietic stem cell transplantation. 2 Regardless, PLL has a poorer prognosis than CLL, and median survival has been found to be 65 months. 4 The man described herein developed rapid refractory hypercalcemia, which limited the consideration of any chemotherapy, and he died shortly. In elderly adults with a Karnofsky Performance Status score of 70 or greater (good status; range 0-100), aggressive treatment strategies may lead to good outcomes, as a study in primary central nervous system lymphoma showed. 7 B-cell PLL is rare, but older men are at risk. Geriatricians often evaluate and treat delirium and fatigue. Hypercalcemia in B-PLL should be considered in the appropriate clinical setting. B-PLL in elderly adults would be a fruitful area for collaborative research between geriatricians and oncologists.

show abstract

Transcranial Sonography of Basal Ganglia Calcifications in Fahr Disease

Cited by 9 publications

References 6 publications

Diabetic Striatopathy: Parenchymal Transcranial Sonography as a Supplement to Diagnosis at the Emergency Department

Diabetic Striatopathy: Parenchymal Transcranial Sonography as a Supplement to Diagnosis at the Emergency Department

Idiopathic hypoparathyroidism induced fahr’s syndrome

Atypical Occipital Calcinosis in a Caucasian Individual with Probable Diffuse Neurofibrillary Tangles with Calcification

Contact Info

Product

Resources

About