Bilateral basal ganglia & thalamic abnormalities

Dr / Hytham Nafady

Bilateral globus pallidus abnormal signal.

Bilateral corpus striatum abnormal signal.

Bilateral thalamic abnormal signal.

Basal ganglia

Globus pallidus

Globus pallidus interna

Globus pallidus externa

Striatum

Caudate Putamen

Subthalamus

Substantia nigra

Globus = sphere.Pallidus = pale relative to the

surrounding brain substance.Caudate = (head, body and tail).Lentiform = lens shaped.Striatum = stripped. Substantia nigra = pigmented

nerve cells.

Substantia nigra

What are the basal ganglia?

What are the basal ganglia?Basal ganglia Vs basal nuclei.

Subthalamus

Corpus striatum

Bilateral globus pallidus normal variants

Bilateral globus pallidus abnormalities

Acute: CO poisoning. Ischemia (heroin abuse). Kernictreus Hemolytic uremic syndromeChronic: Chronic hepatic encephalopathy. Chronic renal dialysis. Non ketotic hyperglycemia. Kernictreus. Neurofibromatosis. Hallervorden Spatz syndrome. Methyl malonic aciduria. Fucosidosis.

Bilateral globus pallidus

abnormalities

Acute

Adult

CO

Ischemia (heroin)

Pediatric Kernicterus

chronic

Adult

Chronic hepatic encephalopathy

Chronic renal dialysis

Non ketotic hyperglycemia

Pediatric

Kernicterus

NF1

Hallorverden Spatz syndrome

Wilson disease

Fucosidosis

T1

T1

T1

T2

T2

T2

T2

T2

T2

T1

T1

T1

T2

CO poisoning

Monoxide mania

CO delayed leukoencephalopathy

2 month

Areas involved with CO

Globus pallidus. Deep white matter. Hippocampus.Pathogenesis:CO is colourless, odorless,

tastless gas generated through incomplete consumption of carbon containing products.

Mechanism of brain injury:

Hypoxia.

Non ketotic hyperglycemia

Non ketotic hyperglycemia

Pathogenesis: Old diabetic patient. Unknown mechanism.Location: Corpus striatum unilaterally. Globus pallidus bilaterally.C.P: Chorea. Hemiballisums.

Non ketotic hyperglycemia

Non ketotic hyperglyemia

Non ketotic hyperglycemia

Chronic hepatic encephalopathy

Chronic hepatic encephalopathyPathogenesis:Manganese toxicity.Location: Globus pallidus. Subthalamus Substantia nigra.

Chronic renal dialysis

Pathogenesis:Manganese toxicity.Location: Globus pallidus. Subthalamus Substantia nigra.

Heroin abuse(bilateral globus pallidus ischemia)

Kernicterus

(Yellow nuclear region of the brain)Acute: Increased T1 signal intensity in globus

pallidus, substantia nigra & dentate nucleus.Chronic: Increased T2 signal intensity in globus

pallidus.C.P (triad):Choreoathetosis.Deafness.Upward gaze palsy.

Chronic kernicterus

Hallervorden Spatz syndrome

Hallervorden Spatz syndrome

Fucosidosis

Pathogenesis: Lysosomal storage disease

characterized by deficiency of enzyme fucosidase.

Location: White matter hypomyelination. Bilateral globus pallidus T2 low

signal. Bilateral substantia nigra T2 low

signal.

Methyl malonic aciduria

Hepatic Wilson disease

Hepatic Wilson disease

Wilson disease:

Pathogenesis:Defective incorporation of copper into

ceruloplasmin.Location:Putamina.Caudate nuclei.Globus pallidus (hepatic patients).Midbrain.Dentate nucleus.

Neuropsychatric Wilson disease

3 years

Neuropsychatric Wilson disease

3 years

Wilson disease

NF1

Neurofibromatosis type 1

Pathogenesis: Hamartomas. Myeline vacuolation.Location: Globus pallidus. Brain stem. Cerebellum.

Bilateral corpus striatum abnormalities

Acute: Acute hepatic encephalopathy. Hypoxia. Hypoglycemia. PRES. Encephalitis. Osmotic myelinolysis.Chronic: Leigh disease. Wilson disease. Huntigton disease. Glutaric aciduria. Gangliosidosis. Urea cycle disorders.

Bilateral corpus striatum abnormalities

Acute

Hypoxia

Hypoglycemia

PRES

Osmotic myelinolysis

Encephalitis

Acute hepatic encephalopathy

Chronic

Pediatric

Leigh disease

Wilson disease

Glutaric aciduria

Gangliosidosis

Urea cycle disorders

Adult

Huntington disease

Acute hepatic encephalopathy

Pathogenesis: Hyperammonemia.Location (Grey matter): Basal ganglia. Insular cortex. Cingulate gyrus.MRS:Elevated gluatamine/glutamate

complex.

Acute hepatic encephalopathy

MRS in a patient with hepatic failure & after hepatic transplantation

Hypoxia

Mild HIE involve water shed zones.Sever HIE involve grey matter Cerebral cortex. Basal ganglia. Thalami. Hippocampus.

Sever hypoxic ischemic encephalopathy

Hypoxia

Hypoglycemia

Mild hypoglycemia:Transient white matter abnormalities: Splenium of corpus callosum. Internal capsule. Corona radiata.Sever hypoglycemia:Diffuse grey matter abnormalities: Cortical grey matter Basal ganglia.

Mild hypoglycemia

Sever Hypoglycemia

Osmotic myelinolysis

Pathogenesis: Osmotic insult (change in osmotic gradient). Endothelial damage. Break BBB. Accumulation of Na in ECF Release of myelin toxins.Causes: Rapid correction of hyponatremia (classic). Hyperglycemia. Hypokalaemia. Ketoacidosis.Comorbid conditions: Hepatic, renal or paraneoplastic disease. Neutritional (alcohol, malnutrition, vomiting). Burn, transplantation, other surgical patient.

Osmotic myelinolysis

Location:50% basis pontis (central pontine

myelinolysis)50% basal ganglia (extra-pontine

myelinolysis)

Osmotic myelinolysis

Osmotic myelinolysis

Osmotic myelinolysis

Central pontine myelinolysis

Extrapontine myelinolysis

Herpes encephalitis

Herpes encephalitis

Leigh disease

Pathogenesis: Mitochondrial disorder characterized

by neurodegeneration.Location: Putamina. Caudate nuclei. Periaqueductal grey matter. Cerebral peduncles. Thalami.

Leigh disease

Leigh disease

Leigh disease

Leigh disease

Leigh disease

Leigh disease

Leigh disease

Leigh disease

Leigh disease

Leigh disease

Glutaric aciduria type 1

Glutaric aciduria type 1

Urea cycle disordershyperammonic encephalopathy

Bilateral lentiform nuclei T1 bright signal on T1.

Bilateral globus pallidus T2 low signal. Bilateral putaminal T2 bright signal. Bilateral insular & bilateral perirolandic

T1 bright signal.

MRS: Glutamine shoulder on the left of NAA.

Bilateral thalamic lesions

Acute:Ischemia (arterial).Ischemia (venous).Hypoxia.PRESEncephalitis.Wernick’s encephalopathyOsmotic myelinolysis.Chronic:Fabry disease.Fahr disease.Leigh disease.Wilson disease.Gangliosidosis.Krabbe’s disease.Bilateral thalamic glioma.

Artery of Percheron infarct

Deep venous thrombosis

Deep venous thrombosis

Deep venous thrombosis

Deep venous thrombosis

Deep venous thrombosis

Deep venous thrombosis

PRES

Wernicke’s encephalopathyPathogenesis:Thiamine (Vit. B1) deficiency. Alcoholism. Hyperemesis gravidarum.Location:Medial thalami.Mammillary bodies.Hypothalamus. Fornix.Periaqueductal grey.Tectal plate.

Wernicke’s encephalopathy

Wernicke’s encephalopathy

Wernicke’s encephalopathy

Wernicke’s encephalopathy

Wernicke’s encephalopathy

Wernicke’s encephalopathy

Wernicke’s encephalopathy

Wernicke’s encephalopathy

West Nile viral encephalitis

Streptococcal encephalitis

Viral encephalitis

Viral encephalitis

Limbic encephalitis

Fabry disease

Fabry disease

Pathogenesis: Inborn error of metabolism

characterized by deficiency of galactosidase enzyme.

Gangliosidosis

Gangliosidosis

Gangliosidosis

Gangliosidosis

Gangliosidosis

Decreased NAA/Cr.

Increased mI/Cr.

Normal choline/Cr.

Krabbe’s diseasegloboid cell leukodystrophy

Bilateral thalamic glioma