“Pulvinar Sign”
Karen L. Salzman, MD
DIFFERENTIAL DIAGNOSIS
Common
Creutzfeldt-Jakob Disease (CJD)
Creutzfeldt-Jakob Disease, Variant (vCJD)
Less Common
Fabry Disease
Thalamic Infarct (Mimic)
Neoplasms (Mimic)
ADEM (Mimic)
Rare but Important
Periventricular Leukomalacia
Status Epilepticus
ESSENTIAL INFORMATION
Key Differential Diagnosis Issues
“Pulvinar sign”: T2 hyperintensity in bilateral pulvinar, most sensitive for vCJD
T1 hyperintensity in pulvinar also called “pulvinar sign” (Fabry disease)
Helpful Clues for Common Diagnoses
Creutzfeldt-Jakob Disease (CJD)
Rapidly progressive, fatal neurodegenerative disease
Prion protein accumulates in neurons
85% of cases sporadic; 15% genetic or familial
Infectious/iatrogenic cases, including vCJD, < 1%
↑ T2 in basal ganglia (BG), thalamus, cortex
FLAIR & DWI MR most sensitive
Creutzfeldt-Jakob Disease, Variant (vCJD)
Bilateral T2 pulvinar hyperintensity
± ↑ T2 dorsomedial thalami, periaqueductal gray, caudate nuclei
Helpful Clues for Less Common Diagnoses
Fabry Disease
Multisystem X-linked disorder with renal & cardiac dysfunction and stroke
T1 hyperintensity in bilateral pulvinar
CT may show mineralization in pulvinar
May see ischemia, white matter (WM) lesions, & vertebrobasilar dolichoectasia
Thalamic Infarct (Mimic)
Artery of Percheron infarct & internal vein thrombosis: Bilateral T2 hyperintensity
HIE may affect only deep gray nuclei
DWI bright in acute setting
Neoplasms (Mimic)
Lymphoma or astrocytoma may cause bilateral thalamic T2 hyperintensity
ADEM (Mimic)
T2 hyperintensity in bilateral thalami
WM lesions typically also present
Helpful Clues for Rare Diagnoses
Periventricular Leukomalacia
Pulvinar hyperintensity may be seen in association with PVL
Thalamic involvement suggests more severe motor & mental disabilities
Status EpilepticusRelated posts:
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