Case of the Week
Section Editors: Matylda Machnowska1 and Anvita Pauranik2
1University of Toronto, Toronto, Ontario, Canada
2BC Children's Hospital, University of British Columbia, Vancouver, British Columbia, Canada
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February 9, 2017
Intramedullary Neurosarcoidosis
- Background:
- Central nervous system involvement of sarcoidosis is very common and can manifest in a variety of ways. However, intramedullary neurosarcoidosis is extremely rare, occurring in less than 1% of patients with sarcoidosis.
- In this case, the diagnosis of sarcoidosis was confirmed by bronchoscopic biopsy of an enlarged lymph node.
- Clinical Presentation:
- Clinical presentation is variable depending on lesion location and size.
- Common symptoms include facial nerve palsy, vision loss, headache, seizure, and signs of meningeal irritation.
- Additional symptoms such as paresis, paresthesia, weakness, diplopia, and dysarthria can easily be confused with those of multiple sclerosis.
- Key Diagnostic Features:
- Sarcoid spinal lesions are often located in the cervical or upper thoracic levels and appear as fusiform, expansile intramedullary lesions.
- On MRI, the spinal cord is expanded with high signal intensity on T2-weighted images, low signal intensity on T1-weighted images, and focal homogenous enhancement of the granulomatous lesion(s).
- Differential Diagnoses:
- Ependymoma: Well-circumscribed intramedullary lesions with T2 hyperintensity.; peritumoral cysts may be seen in a majority of cases. There is often associated hemorrhage, which may lead to peripheral hemosiderin deposition, with a resultant hypointense rim of T2-weighted images.
- Multiple sclerosis: Combination of both well-defined and ill-defined T2 hyperintense lesions within the cord.; although incomplete peripheral enhancement may be seen in the setting of active demyelination, this is less common than with lesions in the brain. Careful examination of the entire central nervous system should be performed to search for other lesions.
- Transverse myelitis: Imaging findings are highly variable. Lesions are usually isointense to hypointense on T1-weighted images, and hyperintense on T2 (though not well-delineated). Contrast enhancement patterns are likewise variable.
- Treatment:
- High-dose corticosteroids are often effective and are the first-line treatment.
- Methotrexate and cyclophosphamide may be required in cases of refractory or aggressive disease, or with frequent recurrence.
- Furthermore, in some patients, neurologic compromise or complications may require surgical intervention.
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