Cerebral Aqueduct/Periaqueductal Lesion
Karen L. Salzman, MD
DIFFERENTIAL DIAGNOSIS
Common
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Aqueductal Stenosis
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Tectal Glioma
Less Common
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Diffuse Axonal Injury (DAI)
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Neurocysticercosis
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Multiple Sclerosis
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Enlarged Perivascular Spaces
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Diffuse Astrocytoma, Low Grade
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Encephalitis (Miscellaneous)
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Intraventricular Hemorrhage
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Wilson Disease
Rare but Important
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Metastasis, Parenchymal
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Wernicke Encephalopathy
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Behçet Disease
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Gliomatosis Cerebri (GC)
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Leigh Syndrome
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Alexander Disease
ESSENTIAL INFORMATION
Key Differential Diagnosis Issues
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Cerebral aqueduct/periaqueductal lesions may be separated by lesion type
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Masses & pseudomasses
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Infectious/inflammatory processes versus metabolic disorders
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Helpful Clues for Common Diagnoses
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Aqueductal Stenosis
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Focal reduction in aqueduct size, congenital or benign acquired
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Funnel-shaped aqueduct with “ballooned” lateral & 3rd ventricles & foramen of Monro proximal to obstruction
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Normal 4th ventricle & foramina distal
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All patients with suspected AS should be scrutinized for an obstructing mass!
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Tectal Glioma
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↑ T2 signal mass; ± enhancement
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Expands tectum, obstructs aqueduct
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Indolent, most only need CSF diversion
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Helpful Clues for Less Common Diagnoses
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Diffuse Axonal Injury (DAI)
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Multifocal punctate hemorrhages at corticomedullary junction, corpus callosum, deep gray matter (GM) & upper brainstem (dorsolateral midbrain & pons)
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Multifocal hypointense T2*/GRE foci related to blood product susceptibility
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Neurocysticercosis
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Cisterns > parenchyma > ventricles
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Basal cistern cysts may be racemose (grape-like), causing an aqueduct lesion
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Multiple Sclerosis
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Multiple T2 hyperintensities in periventricular white matter (WM) & callososeptal interface; 10% infratentorial
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Internuclear ophthalmoplegia (INO): Characteristic clinical finding related to brainstem lesion involving medial longitudinal fasciculus, present within periaqueductal region
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Enlarged Perivascular Spaces
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Benign fluid-filled structures, accompany penetrating arteries
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PVS usually 5 mm or less; may expand
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Most common location for expanded “giant” PVS is midbrain; may cause hydrocephalus
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Single or multiple well-delineated cysts isointense with CSF; no enhancement
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Diffuse Astrocytoma, Low Grade
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Nonenhancing T2 hyperintense mass; supratentorial 2/3, infratentorial 1/3
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50% of brainstem “gliomas” are low grade astrocytoma
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Occur in pons & medulla of children, may involve midbrain
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Usually no enhancement, if C+ worry about malignant progression
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Encephalitis (Miscellaneous)
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Location dependent on etiology
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Diffuse brain parenchymal inflammation caused by a variety of pathogens, most commonly viruses
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Abnormal T2 hyperintensity of GM ± WM or deep gray nuclei
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Epstein-Barr virus: Symmetric BG, thalami, cortex, or brainstem
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Varicella-zoster virus: Brainstem/cortical GM, cranial nerves
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Japanese encephalitis: Bilateral thalami, brainstem, cerebellum, spinal cord, cerebral cortex
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Listeria rhombencephalitis: Brainstem & cerebellum
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West Nile virus: Brainstem, substantia nigra, BG, thalami, anterior horn (cord), cerebellum
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Enteroviral encephalomyelitis: Brainstem, spinal cord, & cerebellum
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Intraventricular Hemorrhage
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Associated with significant trauma
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May occur within cerebral aqueduct
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Wilson Disease
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Symmetric T2 hyperintensity or mixed signal in putamen, globus pallidus (GP), caudate, & thalami
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Characteristic “face of the giant panda” sign at midbrain
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Helpful Clues for Rare Diagnoses
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Metastasis, Parenchymal
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May involve brainstem; typically multiple lesions
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Wernicke Encephalopathy
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Curable neurologic disease caused by thiamine deficiency
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Triad of neuro-ophthalmologic manifestations, ataxia, & global confusion
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Symmetric increased T2 signal surrounding aqueduct & 3rd ventricle, floor of 4th ventricle & medial thalami
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May affect only periaqueductal grey matter
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Behçet Disease
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Multisystem vasculitis of unknown origin, CNS involvement 5-10%
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Classic triad of oral & genital ulcerations with uveitis
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T2 hyperintense lesions in brainstem, BG /or deep WM
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