Download Atlas of Basal Ganglia Calcification

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
{
Dr Gillian Lieberman
Megan Garber
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