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ESSENTIAL INFORMATION

Key Differential Diagnosis Issues
- MR ± contrast is best tool to evaluate intramedullary processes of cord
- Discovery of intramedullary cord lesion typically followed by imaging of remainder of neuraxis
- Infiltrative cord lesion: Image brain to potentially identify characteristic white matter lesion(s) of multiple sclerosis (MS)
- Discovery of intramedullary tumor typically accompanied by insidious onset of myelopathic symptoms (months or years)
- Patient with syrinx should be imaged at least once with contrast-enhanced MR to exclude cord neoplasm
- Hemorrhagic lesion: Think ependymoma, cavernous malformation, contusion
Helpful Clues for Common Diagnoses
- Multiple Sclerosis, Spinal Cord - Typically located eccentrically, not involving entire cord on axial imaging; relatively short in length (< 2 vertebral bodies) - Enlargement of cord is unusual (6-14%) - Dissemination in time: Some enhance, some do not - 5-24% of patients with MS cord plaques may not have supratentorial disease at presentation
- Neuromyelitis Optica Spectrum Disorder - Autoimmune astrocytopathic disease targeting water channel proteins (AQP4) with involvement of optic nerves and spinal cord with limited brain parenchymal involvement - Longitudinally extensive transverse myelitis (LETM) (> 3 vertebral segments) T2 hyperintensity within cord - Enhancement of optic nerves - Brain lesions not typical for MS
- Acute Disseminated Encephalitis, Spinal Cord - Clinical: Self-limited, monophasic demyelinating illness 5-14 days following viral infection or vaccination - Usually indistinguishable from MS on imaging at single time point
- Acute Transverse Myelitis, Idiopathic - Clinical: Acute-onset myelopathy, ascending or static loss of sensory and motor function in bilateral and symmetric distribution - Infiltrative signal abnormality may extend above level of deficit; variable enhancement - Mild fusiform enlargement may be present and simulate appearance of primary cord neoplasm
- Ependymoma, Spinal Cord - Circumscribed, enhancing intramedullary mass; located centrally within cord - Can show signs of necrosis (heterogeneity, cyst formation) and hemorrhage (hyperintense T1, susceptibility artifact, hemosiderin cap sign) - Most common intramedullary neoplasm in adults - 10% may show very little enhancement
- Astrocytoma, Spinal Cord - Fusiform enlargement, infiltrative margins, long segment of involvement; no or variable enhancement - Most commonly located in cervical and upper thoracic cord - Uncommon/rare imaging features: Hemorrhage, necrosis, caudal location, exophytic growth, holocord involvement - Cannot be reliably differentiated from ependymoma by imaging - 2nd most common cord neoplasm in adults; most common cord neoplasm in children (60%)
- Syringomyelia - Abnormal cystic cord lesion with surrounding gliosis; variable expansion of cord; focal or extensive; typically longitudinal - Secondary to chronic insult/injury (cavitation) or to altered CSF dynamics in central canal of cord (technically termed hydromyelia, such as seen in Chiari 1 malformation)
Helpful Clues for Less Common Diagnoses
- Hemangioblastoma, Spinal Cord - Intensely enhancing, hypervascular tumor(s); usually located dorsally within cord - Multiple lesions common (check posterior fossa) - May or may not have associated syrinx, which can be disproportionately large relative to size of actual enhancing tumor - Often with prominent serpiginous subarachnoid flow voids due to enlarged draining veins - 70-90%notassociated with von Hippel-Lindau
- Intramedullary Arteriovenous Malformation - Hyperintense T2 signal in cord - Tortuous vessels/flow voids on MR, hypervascularity on CT angiography
- Infarction, Spinal Cord - Hyperintensity on T2WI, possibly with mild expansion - Conus and variable thoracic cord involvement, cervical ischemia is atypical - Most often associated with aortic pathology (dissection, thoracoabdominal aortic surgery), rarely with atherosclerotic disease or embolism - Look for associated vertebral body infarction with geographic areas of abnormal increased T2 and decreased enhancement
- Cavernous Malformation, Spinal Cord - Variable hyperintensity on T1, heterogeneously hyperintense on T2 with surrounding rim of susceptibility due to prior episodes of hemorrhage that blooms on gradient-echo sequences - Rare enhancement; may have some surrounding edema if recent bleed
- Metastases, Spinal Cord - Most common primary is lung, followed by breast - Melanoma often shows intrinsic T1 hyperintensity
- Lymphoma - Relatively low signal on T2WI - Homogenous enhancement (like brain) - May be extradural, intradural extramedullary or intramedullary
Helpful Clues for Rare Diagnoses
- Sarcoidosis - Mimic of other diseases; diagnosis usually preceded by known pulmonary or systemic involvement
Other Essential Information
- Tumor associated syrinx (a.k.a., polar or satellite cysts): Intramedullary fluid collections rostral &/or caudal to tumor with nonenhancing margins
- More rostral tumor associated with higher likelihood of syrinx formation

References

Selected References

Images

Selected Images

Multiple Sclerosis, Spinal Cord Sagittal T2 MR shows focal areas of increased signal from the thoracic cord in this patient with multiple sclerosis. Cord expansion may occur in the acute phase but is uncommon.

Neuromyelitis Optica Spectrum Disorder Sagittal T2 MR shows a longitudinally extensive region of increased signal intensity within the expanded cervical cord. Neuromyelitis optica lesions typically are > 3 vertebral body segments in length.

Ependymoma, Spinal Cord Sagittal T1 MR shows expansion of the cervical cord with an isointense mass at the C3-C5 level with a large linear area of low-signal hemorrhage extending inferiorly within the cord .

Ependymoma, Spinal Cord Sagittal T2 MR shows heterogenous expansion of the cervical cord from the ependymoma nidus plus extension of hemorrhage inferiorly .

Syringomyelia Sagittal T1 MR shows well-defined fluid signal intensity from the upper cervical cord in this patient with a syrinx and prior posterior fossa decompression for Chiari 1 malformation.

Contusion-Hematoma, Spinal Cord Sagittal T2 MR shows a severe flexion injury at C6-C7 with a traumatic disc herniation and extensive contusion and hemorrhage within the cord . Note the edema extending superiorly and inferiorly from the injury site .

Hemangioblastoma, Spinal Cord Sagittal T1 C+ FS MR shows multiple enhancing masses along and within the thoracic cord in this patient with von Hippel-Lindau.

Intramedullary Arteriovenous Malformation Sagittal T2 MR shows multiple prominent round flow voids from nidal aneurysms and venous varices in this patient with a large conus-type arteriovenous malformation (AVM).

Intramedullary Arteriovenous Malformation Sagittal T1 MR shows a small, nonspecific focus of low signal intensity within the cervical cord at the C2-C3 level in this patient with a type II intramedullary AVM.

Intramedullary Arteriovenous Malformation Sagittal T2 MR in this patient with a type II AVM shows the site of the nidus as well as linear areas of hemosiderin deposition with the cord from prior hemorrhage.

Infarction, Spinal Cord Sagittal T2WI FSE MR shows ill-defined hyperintensity and mild enlargement of the conus medullaris in this patient with lower extremity paralysis following aortic aneurysm repair.

Cavernous Malformation, Spinal Cord Sagittal T2 MR shows slightly expansile intramedullary lesion at the C2-C3 level with a classic heterogeneous hyperintense (popcorn) lesion surrounded by a hypointense hemosiderin rim.

Metastases, Spinal Cord Sagittal T2WI STIR MR shows nonspecific expansion and hyperintensity within the cervical cord in this patient with metastatic disease.

Metastases, Spinal Cord Sagittal T1 C+ MR shows a focal mass enhancing within the cervical cord in this patient with cord metastases .

Lymphoma Sagittal T1 C+ MR shows nonspecific enlargement and enhancement of the cervical cord. The differential would include tumors (astrocytoma) and granulomatous disease. This patient has path-proven lymphoma.

Sarcoidosis Sagittal T1 C+ MR shows an ovoid focus of irregular enhancement within the cervical cord at the C6 and C7 levels . This lesion was eventually diagnosed as sarcoidosis.

Additional Images

Demyelinating Disease Sagittal T1 C+ MR shows 3 foci of pathologic enhancement due to acute disseminated encephalomyelitis within the cervical cord in this patient with an acute illness following viral prodrome.

Demyelinating Disease Sagittal T1 C+ MR shows patchy enhancement and mild enlargement of cervical cord due to multiple sclerosis (Devic subtype).

Ependymoma, Spinal Cord Sagittal T1 C+ MR shows the typical appearance of ependymoma. Note a circumscribed, enhancing mass in the central cord with tumor syrinx.

Ependymoma, Spinal Cord Sagittal T1 C+ MR shows an enhancing intraaxial mass with rostral and caudal syrinx and a satellite lesion enlarging the cervical cord. Note severe syringobulbia .

Ependymoma, Spinal Cord Sagittal T2 MR shows a complex cervical cord mass containing foci of susceptibility , demonstrating prior hemorrhage within an ependymoma.

Astrocytoma, Spinal Cord Sagittal T2 MR shows an infiltrative lesion expanding the midcervical cord. C3-C5 decompressive laminectomy has been performed.

Astrocytoma, Spinal Cord Sagittal T1 C+ MR shows an astrocytoma enlarging the cervical cord with patchy enhancement and infiltrative margins.

Syringomyelia Sagittal T2 MR shows haustrated CSF signal mass in the central cord in a patient with a Chiari 1 malformation. Hypointense foci are due to flow artifact.

Hemangioblastoma, Spinal Cord Sagittal T1 C+ MR shows holocord syrinx and 2 small dorsal enhancing lesions in this patient with multiple hemangioblastomas.

Hemangioblastoma, Spinal Cord Sagittal T2 MR shows a nodular mass dorsally at the C4 level with a peripheral cyst . Syrinx enlarges the cord. The ring of hypointensity suggests sequelae of hemorrhage.

Intramedullary Arteriovenous Malformation Sagittal T2 MR shows patchy hyperintensity within the cord due to intramedullary AVM. Note dorsal flow voids due to tortuous, dilatated vascularity .

$Sagittal T1 C+ FS MR shows focal intramedullary breast carcinoma metastasis enlarging the distal cord . Note vertebral metastasis with a mild compression fracture.$ Metastases, Spinal Cord Sagittal T1 C+ FS MR shows focal intramedullary breast carcinoma metastasis enlarging the distal cord . Note vertebral metastasis with a mild compression fracture.

Sarcoidosis Sagittal T1 C+ MR demonstrates extensive cord enlargement and intramedullary enhancement due to sarcoidosis.

Cysticercosis Sagittal T2WI MR shows cystic thoracic cord mass in a patient with cysticercosis.

Lipoma, Spinal Sagittal T1 MR shows a fusiform mass contacting the dorsal pial surface of the cord. The mass was isointense to subcutaneous fat on all pulse sequences.

Glioblastoma, Spinal Cord Sagittal T1 C+ MR shows a large enhancing mass involving the upper thoracic cord with fusiform cord expansion.

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ESSENTIAL INFORMATION

Key Differential Diagnosis Issues

Helpful Clues for Common Diagnoses

Helpful Clues for Less Common Diagnoses

Helpful Clues for Rare Diagnoses

Other Essential Information