Khaled M Sebawih Gillian lieberman, MD
May 2014
Atlas of Basal Ganglia Calcification Khaled M Sebawih, Misr University for Science and Technology, Egypt Gillian Lieberman, MD
Khaled M Sebawih Gillian lieberman, MD
Agenda
Our patient Clinical history
Anatomy of the Basal Ganglia
Pathophysiology
Differential diagnosis
Imaging of diseases causing BGC
Summary
Khaled M Sebawih Gillian lieberman, MD
Our Patient Clinical history A Female patient, HTN, was found down in her bed unresponsive. History notable for 4L coffee ground emesis at OSH and a fever as well as elevated CK and transaminases. Neurologically she is grossly nonfocal but does have significant cognitive slowing and difficulty with more complex commands. The patient has had a prodrome of personality changes, specifically apathy and seeming depression four month ago.
Khaled M Sebawih
Gillian lieberman, MD
Our Patient Axial CT non-contrast
Findings: Bilateral high attenuation areas in the Basal Ganglia representing calcified areas. Other findings: Choroid plexus calcification
Source: “PACS, BIDMC”
Khaled M Sebawih Gillian lieberman, MD
Our Patient Axial CT non-contrast
Findings: Low attenuation areas in both Globus pallidus
Other Findings: Choroid plexus calcification
Source: “PACS, BIDMC”
Khaled M Sebawih Gillian lieberman, MD
Our Patient Axial T2 MRI
Findings: High intensity of Globus Pallidus
Source: “PACS, BIDMC”
Khaled M Sebawih Gillian lieberman, MD
Anatomy:
Illustration of the basal ganglia structures
Basal Ganglia: • Caudate nucleus • Putamen • Globus Pallidus
Source: Clinical Motor and Cognitive Neurobehavioral Relationships in the Basal Ganglia By Gerry Leisman, Robert Melillo and Frederick R. Carrick
Khaled M Sebawih Gillian lieberman, MD
Anatomy: Basal Ganglia nuclei Grouped together: Striatum: Caudate + Putamen Lentiform: Globus pallidus + Putamen Corpus Striatum: Lentiform + Caudate
Khaled M Sebawih Gillian lieberman, MD
Pathophysiology: • Calcium interaction with fatty acids • Rupture of Blood Brain Barrier • Iron may play a role as it catalyzes reactive oxygen radicals • Elevated intracellular Calcium
Khaled M Sebawih Gillian lieberman, MD
Differential Diagnosis: Idiopathic: • Ageing • Fahr disease
Toxic: • • • •
Carbon monoxide Lead Mineralizing microangiopathy Anticonvulsant therapy
Infectious: • CNS Tuberculosis • AIDS • Neurocysticercosis
Khaled M Sebawih Gillian lieberman, MD
Differential Diagnosis: Metabolic: • Hypoparathyroidism • Pseudohypoparathyroidism
Inherited: • Mitochondrial disease as MELAS
Khaled M Sebawih Gillian lieberman, MD
Differential Diagnosis: Idiopathic: • Ageing • Idiopathic Fahr disease
Khaled M Sebawih Gillian lieberman, MD
Ageing: • Usually idiopathic, with an incidence rate of 1%
• Age of presentation seems to regulate the type of symptoms expressed by affected patients. • Incidence of neuropsychiatric findings is most dependent on amount of mineralization. • 50% of patients with extensive brain mineralization exhibited mental disorders.
Khaled M Sebawih Gillian lieberman, MD
Differential Diagnosis: Idiopathic: • Ageing • Idiopathic Fahr disease
Khaled M Sebawih Gillian lieberman, MD
Fahr Disease: • Idiopathic Basal Ganglia Calcification • Presents in 4th and 5th decade. Symmetric, bilateral involvement of : • Globus pallidus • Caudate • Lentiform nucleus • Thalamus • Dentate nucleus • MRI T1 show high signal. • PET scan may show decrease FDG uptake.
Khaled M Sebawih Gillian lieberman, MD
Fahr Disease:
Companion patient 1: Axial CT non contrast:
Findings: Bilateral Dentate nucleus Calcification
Other findings: Pineal gland calcification
Courtesy of Dr Rafael Rojas
Khaled M Sebawih Gillian lieberman, MD
Companion patient 1: Axial CT non contrast:
Fahr Disease: Findings: Bilateral Basal ganglia and Dentate nucleus calcification
Courtesy of Dr Rafael Rojas
Khaled M Sebawih Gillian lieberman, MD
Fahr Disease:
Companion patient 1: Axial CT non contrast:
Findings: Bilateral Corpus Striatum & subcortical calcification
Courtesy of Dr Rafael Rojas
Khaled M Sebawih Gillian lieberman, MD
Fahr Disease: Companion patient 1: MRI T1 and T2:
Courtesy of Dr Rafael Rojas
Khaled M Sebawih Gillian lieberman, MD
Fahr Disease: Companion patient 1: MRI Flair and T1:
Courtesy of Dr Rafael Rojas
Khaled M Sebawih Gillian lieberman, MD
Differential Diagnosis: Toxic: • • • •
Carbon monoxide Lead Mineralizing microangiopathy Anticonvulsant therapy
Khaled M Sebawih Gillian lieberman, MD
CO poisoning: • Carbon monoxide binds to hemoglobin approximately 200 times more tightly than oxygen. • The neurotoxicity of CO could be acute or chronic. • Globus pallidus is the most affected area.
• Classically seen as low attenuation of globus pallidus on CT, low signal on MRI T1 weighted imaging and high signal on T2/FLAIR.
Khaled M Sebawih Gillian lieberman, MD
Companion patient 2: Axial CT non contrast:
CO poisoning: Findings: Bilateral Globus pallidus low attenuation.
Sourcehttp: www.radiopaedia.org/cases/carbon-monoxidepoisoning, Dr Ruslan Esedov
Khaled M Sebawih Gillian lieberman, MD
Companion patient 3: Axial MRI T1
CO poisoning: Findings: Bilateral globus pallidus oval shaped areas of altered signals eliciting low T1
Source: http://radiopaedia.org/cases/carbonmonoxide-poisoning-1 ,Dr Muhammed Essam
Khaled M Sebawih Gillian lieberman, MD
Differential Diagnosis: Toxic: • • • •
Carbon monoxide Lead Mineralizing microangiopathy Anticonvulsant therapy
Khaled M Sebawih Gillian lieberman, MD
Companion patient 4: Axial MRI T2 with contrast
Lead toxicity: Findings: Hyperintense signal alterations of the basal ganglia
Source: Fluri F et al. Neurology 2007;69:929-930
Khaled M Sebawih Gillian lieberman, MD
Differential Diagnosis: Toxic: • • • •
Carbon monoxide Lead Mineralizing microangiopathy Anticonvulsant therapy
Khaled M Sebawih Gillian lieberman, MD
Mineralizing Angiopathy: • Usually presents in children receiving Chemo or radiotherapy, but other causes are possible as trauma.
Areas mostly affected include: • Corticomedullary junction • Lentiform nucleus • Dentate nucleus of cerebellum
Khaled M Sebawih Gillian lieberman, MD
Companion patient 5: Axial CT post contrast
Mineralizing Angiopathy: Findings: Hyperdense areas noted in the basal ganglia and sub-cortical white matter
Source: www.radiopaedia.org/cases/mineralising-microangiopathy Dr Ayush Goel
Khaled M Sebawih Gillian lieberman, MD
Differential Diagnosis: Infectious: • CNS Tuberculosis • AIDS • Neurocysticercosis
Khaled M Sebawih Gillian lieberman, MD
CNS Tuberculosis: • Tuberculosis is caused by mycobacterium tuberculosis.
• The disease begins with the development of small tuberculous foci (Rich foci) in the brain, spinal cord, or meninges. • CT non-contrast scans may be normal • MRI T1 gadolinium enhanced shows hyperintensity
Khaled M Sebawih Gillian lieberman, MD
Companion patient 6 : Axial T1 MRI post contrast
CNS Tuberculosis: Findings: Multiple lesions involving the cerebral hemisphere including the basal ganglia.
Source:Indian J Radiol Imaging. Nov 2009; 19(4): 256–265.
Khaled M Sebawih Gillian lieberman, MD
Differential Diagnosis: Infectious: • CNS Tuberculosis • AIDS • Neurocysticercosis
Khaled M Sebawih Gillian lieberman, MD
AIDS: • AIDS is caused by infection of HIV, which affects CD4+ cells. • AIDS affects the basal ganglia early in the disease as evidenced by slow cognition and motor reaction times even in asymptomatic HIV positive patients.
Khaled M Sebawih Gillian lieberman, MD
Companion patient 7 : Axial CT
AIDS: Findings: Bilateral basal ganglia calcification.
http://www.bipai.org/Educational-Resources/Pediatric-AIDS-PictorialAtlas/Bilateral-calcifications-of-the-basal-ganglia.aspx
Khaled M Sebawih Gillian lieberman, MD
Differential Diagnosis: Infectious: • CNS Tuberculosis • AIDS • Neurocysticercosis
Khaled M Sebawih Gillian lieberman, MD
Neurocysticercosis: • Caused by ingestion of Tenia solium eggs. • Larval cysts commonly found in the central nervous system but they can also be found in the eye, muscle or other tissues. • Findings are variable on CT, but most prominent during the calcified stage. • MRI is the modality of choice to view Neurocysticercosis
Khaled M Sebawih Gillian lieberman, MD
Companion patient 8: Axial T2 MRI
Neurocysticercosis: Findings: Hyper intense lesion affecting the right putamen and left caudate.
Source: Clinical Neurology and Neurosurgery 104 (2002) 57–60
Khaled M Sebawih Gillian lieberman, MD
Differential Diagnosis: Metabolic: • Hypoparathyroidism • Pseudohypoparathyroidism
Khaled M Sebawih Gillian lieberman, MD
Hypoparathyroidism • Decreased PTH levels causing ↓ Ca & ↑ P. • Increase P levels causes Ca to deposit in the brain tissue. • Bilateral, Symmetrical • Affects grey-white junction, Cerebellum • Non Contrast CT has highest sensitivity and specificity • MRI not useful as signal intensity of calcified lesion varies widely.
Khaled M Sebawih Gillian lieberman, MD
Companion patient 9: Axial CT non contrast
Hypoparathyroidism Findings: Bilateral Lentiform high attenuation.
Other Findings: Bilateral thalamus, and multiple subcortical lesions. Choroid plexus calcifications.
Source: M Mejdoubi, J Neurol Neurosurg Psychiatry. Dec 2006; 77(12): 1328.
Khaled M Sebawih Gillian lieberman, MD
Differential Diagnosis: Metabolic: • Hypoparathyroidism • Pseudohypoparathyroidism
Khaled M Sebawih Gillian lieberman, MD
Pseudohypoparathyroidism • Pseudohypoparathyroidism is a condition associated with resistance to parathyroid hormone. • Subtypes: type I : abnormal cAMP response to PTH stimulation type Ia : has characteristic phenotypical features type Ib : lacks phenotypical features type II : normal cAMP response to PTH stimulation • Affects deep white matter and basal ganglia
Khaled M Sebawih Gillian lieberman, MD
Pseudohypoparathyroidism:
Companion patient 10: Axial Non-contrast CT head
Findings: Extensive basal ganglia and cerebral calcification
Source: Bhadada SK, Bhansali A, Upreti V, Subbiah S, Khandelwal N. Spectrum of neurological manifestations of idiopathic hypoparathyroidism and pseudohypoparathyroidism. Neurol India 2011;59:586-9
Khaled M Sebawih Gillian lieberman, MD
Differential Diagnosis: Inherited: • MELAS
Khaled M Sebawih Gillian lieberman, MD
MELAS: • Mitochondrial encephalo- myopathy, lactic acidemia, and stroke like symptoms. • Mitochondrial disease of maternal inheritance. • Symmetric basal ganglia calcification
• Focal cerebral lesions not confined to the vascular territories in a young patient. • Muscle biopsy may show ragged fibers.
Khaled M Sebawih Gillian lieberman, MD
MELAS:
Companion patient 11: Axial non Contrast CT
Findings: Bilateral Lentiform nucleus hyperintensity
Source: Sheng-Horng Chung, Shyr-Chyr Chen, Wen-Jone Chen, et al.Neurology 2005;65;E19
Summary • The best modality is CT non Contrast. • Incidental findings are common with age.
• The most common area affected is Globus pallidus. • Most likely mechanism is disruption of Blood Brain Barrier.
• The most common cause is Fahr disease and metabolic disorders.
Khaled M Sebawih Gillian lieberman, MD
Acknowledgement: Dr Rafael Rojas MD
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Khaled M Sebawih Gillian lieberman, MD
References: • • • • • • • • •
M Mejdoubi, J Neurol Neurosurg Psychiatry. Dec 2006; 77(12): 1328 Fluri F et al. Neurology 2007;69:929-930 M.F. Casanova, J.M. Araque / Psychiatry Research 121 (2003) 59–87 Clinical Motor and Cognitive Neurobehavioral Relationships in the Basal Ganglia, By Gerry Leisman, Robert Melillo and Frederick R. Carrick Sheng-Horng Chung, Shyr-Chyr Chen, Wen-Jone Chen, et al.Neurology 2005;65;E19 Indian J Radiol Imaging. Nov 2009; 19(4): 256–265 AJNR Am J Neuroradiol 19:83–89, January 1998 http://www.bipai.org/Educational-Resources/Pediatric-AIDS-Pictorial-Atlas/ Bilateral-calcifications-of-the-basal-ganglia.aspx Bhadada SK, Bhansali A, Upreti V, Subbiah S, Khandelwal N. Spectrum of neurological manifestations of idiopathic hypoparathyroidism and pseudohypoparathyroidism. Neurol India 2011;59:586-9
Khaled M Sebawih Gillian lieberman, MD
References:
• www.radiopaedia.org/cases/carbon-monoxide-poisoning-1 , Dr Muhammed Essam • www.radiopaedia.org/cases/carbon-monoxide-poisoning, Dr Ruslan Esedov • www.radiopaedia.org/cases/mineralising-microangiopathy, Dr Ayush Goel
