MRI imaging finding in Wilson disease

Dr Gulab Soni

MRI finding in Wilson disease: 5 pattern1. T2- Hyperintense lesion/T1-Hypointense2. T2-Hypointense center with peripheral hyperintense3. T 1- Hyperintense lesion/T2-Iso/Hypointense4. Generalised brain atrophy5. Specific abnormal sign-i. Face of giant panda sign ii. Face of miniature panda signiii. Bright claustrum sign

T2- Hyperintense lesion/T1-Hypointense:

• The hyperintensity in the putamen can actually be fairly suggestive if it has a lamellated pattern, with modest hyperintensity medially in the putamen and an outer strip of marked hyperintensity.

• All the lesions showed bilateral and symmetric involvement.• The high-signal-intensity lesions on T2-weighted images can be

caused by edema, gliosis, demyelination, neuronal necrosis, or cystic degeneration.• Some of the lesions showed reversible changes following copper

chelating treatment

Sequence of T2 hyperintense lesion:• Putamen, • Globus pallidus• Thalamus• Caudate nucleus• Midbrain tegmentum• Pontine tegmentum• Subcortical region (frontal, parietal, temporal)-10%• Cerebellar peduncle

T2-Hypointense center with peripheral hyperintense:• T2-weighted images show

hypointensity in the basal ganglia (globus pallidus) region may be as a result of deposition of iron in exchange for copper after chelation

• Sometimes, especially later in the disease, there is deposition of iron in the zones of gliosis, and the putamen and caudate become quite hypointense on T2. • However, the lateral putaminal margins will typically remain markedly

hyperintense, again reflecting the lamellated pattern . These findings are seen in only in minority of cases.

T 1- Hyperintense lesion/T2-Iso/Hypointense:

• High signal intensity abnormality on T1-weighted images showed bilateral and symmetric distribution.• In Wilson with hepatic involvement.• Sites of involvements were the globus pallidus , putamen, midbrain

and caudate nucleus .• Secondary to the accumulation of manganese in basal ganglia

because of portal-systemic shunting.

Gray matter lesion- Symmetric

White matter lesion-Asymmetric

• Wilson disease can be categorized into 2 groups on the basis of MRI imaging finding-

(1) High-signal intensity lesions in the basal ganglia on T1-weighted images reflected hepatic involvement of Wilson disease.(2) High-signal-intensity lesions on T2-weighted images reflected cerebral involvement of Wilson disease.

Kim T J et al.MR Imaging of the Brain in Wilson Disease of Childhood: Findings Before and After Treatment with Clinical Correlation. AJNR Am J Neuroradiol 27:1373–78 Jun-Jul 2006

Generalised brain atrophy

Generalised brain atrophy:

• Diffuse brain atrophy on MR imaging was evident in 68% of neurologically symptomatic patients. • Diffuse brain atrophy suggests a generalized susceptibility and

longstanding effect of the central nervous system to copper intoxication

Grimm G, Prayer L, Oder W, et al. Comparison of functional and structural brain disturbances in Wilson disease. Neurology 1991;41:272–76

Face of a giant panda sign:

• First described by Hitoshi et al. in 1991• ‘Face of giant panda’ in the midbrain

on T2W images-• high signal intensity in the tegmentum

except the red nucleus forming ‘eyes’,• preservation of signal intensity in

lateral portion of pars reticulata of substantia nigra forming ‘ears’ • and hypointensity in the superior

colliculus forming ‘mouth’

Face of the miniature panda/panda cub• The “face of the miniature panda” is

seen within the pontine tegmentum. • It is delineated by the relative

hypointensity of the medial longitudinal fasciculi and central tegmental tracts (“eyes of the panda”) in contrast with the hyperintensity of the aqueduct opening into the fourth ventricle (“nose and mouth of the panda”) bounded inferiorly by the superior medullary velum.

• The superior cerebellar peduncles form the panda’s “cheeks.”

Double panda sign:

Face of a giant panda sign + face of the miniature panda/panda cub

Bright claustrum sign:

• Abnormal signal intensity arises from the claustrum and not the putamen.• There is a thin rim of T2

hyperintensity in the claustrum known as the bright claustrum sign (yellow arrow).

Sener R.N.The claustrum on MRI: the bright claustrum as a new sign in wilson’s disease. PediatrRadiol 1993;23:597-596

Cerebellum in Wilson:• Cerebellum was affected in half

of all MR imagitig studies showing abnormalities involving the superior cerebellar peduncles and middle cerebellar peduncles.

• In a large study of MRI in 100 patients with WD, the salient findings included: Atrophy of the cerebrum (70%), brainstem (66%) and cerebellum (52%), • Signal abnormalities in putamen (72%), caudate (61%), thalami (58%),

midbrain (49%), pons (20%), cerebral white matter (25%), cortex (9%), medulla (12%) and cerebellum (10%). • The characteristic 'face of giant panda' sign was noted in 12% and feature of

central pontine myelinolysis was noted in 7% and bright claustral sign in 4% of patients.

Sinha S, Taly AB, Ravishankar S, Prashanth LK, Venugopal KS, Arunodaya GR, et al. Wilson's disease: Cranial MRI observations and clinical correlation. Neuroradiology 2006;48:613-21

DWI in Wilson:• Diffusion-weighted images may show areas of restricted diffusion

early in the disease process due to cytotoxic edema, or inflammation due to excessive copper deposition. • However, this restricted diffusion is not seen in chronic cases. which

are characterized by necrosis, spongiform degeneration, and demyelination.

Imaging D/D:• Japanese B encephalitis,• Leigh disease, • Hypoxic–ischemic encephalopathy, • CO poisioning, and • Extrapontine myelinolysis • CJD

THANK-U