Atlas of Basal Ganglia
Calcification
Khaled M Sebawih
Gillian lieberman, MD May 2014
Khaled M Sebawih, Misr University for Science and Technology, Egypt
Gillian Lieberman, MD
Our patient Clinical history
Anatomy of the Basal Ganglia
Pathophysiology
Differential diagnosis
Imaging of diseases causing BGC
Summary
Khaled M Sebawih
Gillian lieberman, MD
Agenda
Our Patient Clinical history
Khaled M Sebawih
Gillian lieberman, MD
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.
Source: “PACS, BIDMC”
Khaled M Sebawih
Gillian lieberman, MD
Findings:
Bilateral high attenuation areas in the Basal Ganglia representing calcified areas.
Our Patient Axial CT non-contrast
Other findings: Choroid plexus calcification
Source: “PACS, BIDMC”
Khaled M Sebawih
Gillian lieberman, MD
Findings:
Low attenuation areas in both Globus pallidus
Our Patient Axial CT non-contrast
Other Findings:
Choroid plexus calcification
Source: “PACS, BIDMC”
Khaled M Sebawih
Gillian lieberman, MD
Findings:
High intensity of Globus Pallidus
Our Patient Axial T2 MRI
Khaled M Sebawih
Gillian lieberman, MD
Anatomy:
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
Illustration of the basal ganglia structures
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.
Courtesy of Dr Rafael Rojas
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
Fahr Disease: Companion patient 1: Axial CT non contrast:
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:
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
CO poisoning:
Sourcehttp: www.radiopaedia.org/cases/carbon-monoxide-poisoning, Dr Ruslan Esedov
Companion patient 2: Axial CT non contrast:
Findings: Bilateral Globus pallidus low attenuation.
Khaled M Sebawih
Gillian lieberman, MD
CO poisoning:
Source: http://radiopaedia.org/cases/carbon-monoxide-poisoning-1 ,Dr Muhammed Essam
Companion patient 3: Axial MRI T1
Findings:
Bilateral globus pallidus oval shaped areas of altered signals eliciting low T1
Khaled M Sebawih
Gillian lieberman, MD
Differential Diagnosis:
Toxic: • Carbon monoxide • Lead • Mineralizing microangiopathy • Anticonvulsant therapy
Source: Fluri F et al. Neurology 2007;69:929-930
Khaled M Sebawih
Gillian lieberman, MD
Lead toxicity: Companion patient 4: Axial MRI T2 with contrast
Findings:
Hyperintense signal alterations of the basal ganglia
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
Source: www.radiopaedia.org/cases/mineralising-microangiopathy Dr Ayush Goel
Khaled M Sebawih
Gillian lieberman, MD
Mineralizing
Angiopathy:
Companion patient 5: Axial CT post contrast
Findings: Hyperdense areas noted in the basal ganglia and sub-cortical white matter
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
Source:Indian J Radiol Imaging. Nov 2009; 19(4): 256–265.
Khaled M Sebawih
Gillian lieberman, MD
CNS Tuberculosis:
Companion patient 6 : Axial T1 MRI post contrast
Findings:
Multiple lesions involving the cerebral hemisphere including the basal ganglia.
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.
http://www.bipai.org/Educational-Resources/Pediatric-AIDS-Pictorial-Atlas/Bilateral-calcifications-of-the-basal-ganglia.aspx
Khaled M Sebawih
Gillian lieberman, MD
AIDS:
Companion patient 7 : Axial CT
Findings:
Bilateral basal ganglia calcification.
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
Neurocysticercosis:
Source: Clinical Neurology and Neurosurgery 104 (2002) 57–60
Companion patient 8: Axial T2 MRI
Findings:
Hyper intense lesion affecting the right putamen and left caudate.
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.
Source: M Mejdoubi, J Neurol Neurosurg Psychiatry. Dec 2006; 77(12): 1328.
Khaled M Sebawih
Gillian lieberman, MD
Hypoparathyroidism
Findings:
Bilateral Lentiform high attenuation.
Companion patient 9: Axial CT non contrast
Other Findings: Bilateral thalamus, and multiple subcortical lesions. Choroid plexus calcifications.
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
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
Pseudohypoparathyroidism:
Findings: Extensive basal ganglia and cerebral calcification
Companion patient 10: Axial Non-contrast CT head
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:
Source: Sheng-Horng Chung, Shyr-Chyr Chen, Wen-Jone Chen, et al.Neurology 2005;65;E19
Companion patient 11: Axial non Contrast CT
Findings: Bilateral Lentiform nucleus hyperintensity
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.
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Acknowledgement: Dr Rafael Rojas MD
Dr Gillian Lieberman
Megan Garber
Khaled M Sebawih
Gillian lieberman, MD
References:
Khaled M Sebawih
Gillian lieberman, MD
• 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
References:
Khaled M Sebawih
Gillian lieberman, MD
• 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