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