Neurosarcoidosis: the main role of MRI

Transcription

1 Neurosarcoidosis: the main role of MRI Poster No.: C-2087 Congress: ECR 2014 Type: Educational Exhibit Authors: A. B. Marin Quiles, B. RODRIGUEZ FISAC, M. J. Picado, L. Pallares, A. Mas Bonet, A. moll ; Palma De Mallorca/ES, 2 Mallorca/ES Keywords: Pathology, Perception image, MR, CNS DOI: /ecr2014/C-2087 Any information contained in this pdf file is automatically generated from digital material submitted to EPOS by third parties in the form of scientific presentations. References to any names, marks, products, or services of third parties or hypertext links to thirdparty sites or information are provided solely as a convenience to you and do not in any way constitute or imply ECR's endorsement, sponsorship or recommendation of the third party, information, product or service. ECR is not responsible for the content of these pages and does not make any representations regarding the content or accuracy of material in this file. As per copyright regulations, any unauthorised use of the material or parts thereof as well as commercial reproduction or multiple distribution by any traditional or electronically based reproduction/publication method ist strictly prohibited. You agree to defend, indemnify, and hold ECR harmless from and against any and all claims, damages, costs, and expenses, including attorneys' fees, arising from or related to your use of these pages. Please note: Links to movies, ppt slideshows and any other multimedia files are not available in the pdf version of presentations. Page 1 of 27

2 Learning objectives To review the central nervous system (CNS) MRI findings of patients affected by sarcoidosis. Background Sarcoidosis can affect patients of any ethnic group and age, but it is more common in African-Americans and North European caucasians. Young and middle aged women are most often affected. Sarcoidosis is a systemic disease of unknown cause, histologically characterized by noncaseating granulomas with proliferation of epithelioid cells that affect various organs and tissues. Symptomatic CNS involvement has been observed in 3-5% of patients with sarcoidosis, although up to 10% of patients with systemic disease have radiological evidence of CNS involvement. In about 50% of patients no other organ is affected, complicating the diagnostic process 1, 2, 3. Neurosarcoidosis may have different clinical presentations: - Cranial neuropathies (the most frequent): facial nerve paralysis and optic nerve involvement are common. - Headache. - Aseptic meningitis. - Visual disturbances. - Neuropsychiatric manifestations. - Symptoms due to spinal cord, brainstem, cerebellar or pituitary gland dysfunction. Definitive diagnosis of neurosarcoidosis is only given by histology. Depending on the affected area it may not be possible to perform a biopsy, therefore in most cases the diagnosis will be made by the patient's clinical history together with the MRI findings. The main MRI findings that point us to neurosarcosidosis are: Page 2 of 27

3 INTRACRANIAL LESIONS: Extra-axial lesions: Leptomeningeal involvement is the most typical manifestation of neurosarcoidosis (40% of the patients). It is common to find a leptomemingeal thickening an enhancement, either diffuse or nodular, on contrast-enhanced T1-weighted images. Infiltration typically involves the suprasellar and frontal basal meninges but it may occur anywhere and is more pronounced in the depths of the sulci (Fig. 1 on page 15). Dural involvement may appear as focal dural masses or a diffuse thickening. Masses tend to be isointense to gray matter on T1-weighted MR images and hypointense on T2-weighted 4 images, and they uniformly enhance after contrast administration. Fig. 1: 40-year-old woman with a one month history of headache, nausea and vomiting. Post-contrast axial T1-weighted images show leptomeningeal enhancement along the perivascular supra and infratentorial spaces. References: - Palma De Mallorca/ES Cranial nerve involvement: Neurosarcoidosis can affect any cranial nerve. The MRI findings are enlargement and contrast enhancement of the cranial nerves on fatsuppressed T1W sequences, even though there is a poor correlation between the evidence of cranial nerve involvement on MRI and clinical neuropathy. Facial palsy is the most common neurological symptom, whereas radiologically the optic nerves are the most commonly affected. The optic nerve can be involved in any location (intra or extraorbital), both uni or bilaterally, and it may even mimick a meningioma. In some cases when the orbital fat, muscles, lacrimal glands and globe are affected, we may see a diffuse contrast-enhanced infiltrative mass on T1WI that is sometimes indistinguishable 5 from an orbital pseudotumor (Fig. 2 on page 15, Fig. 3 on page 16). Page 3 of 27

4 Fig. 2: 35-year-old woman with right exoftalmus. MRI images show an intraconal lesion surrounding the optic nerve. A. Axial T1-weighted and T2-weighted images. B. Contrast-enhanced axial and coronal T1-weighted images. References: - Palma De Mallorca/ES Page 4 of 27

5 Fig. 3: 41-year-old woman with a history of headache, right proptosis and visual disturbances. A. CT shows the presence of an orbital pseudotumor. B. One month after steroid treatment MRI images show a total resolution of the process. References: - Palma De Mallorca/ES Intraparenchymal lesions: The most common MRI findings are multiple periventricular 6 high signal white matter lesions on non-enhanced T2WI (Fig. 4 on page 17). Enhancing lesions after contrast administration on T1W sequences may be seen on T2W sequences as hypointense masses, either with or without T2-hyperintense vasogenic 7 edema (Fig. 5 on page 18). These lesions are frequently associated with surrounding leptomeningeal affectation. Page 5 of 27

6 Fig. 4: 32-year-old woman with a known ganglionar sarcoidosis and a 3 week history of ataxia, paresthesias, nausea, vomiting and dizziness. A. Multiple periventricular white matter lesions with high signal on T2/FLAIR images. B. Axial T1-weighted image shows the absence of enhancement after contrast administration. References: - Palma De Mallorca/ES Page 6 of 27

7 Fig. 5: Same patient as in Fig. 4. A. Periventricular lesion with contrast enhancement on T1-weighted images. B. The MRI performed after the steroid treatment shows complete resolution of the lesion. Page 7 of 27

8 References: - Palma De Mallorca/ES Hypothalamus and pituitary involvement: Hypothalamic, infundibular and pituitary involvement may be seen on MRI as an isolated enlargement and contrast enhancement of these structures on T1WI. We may also see basal leptomeningeal involvement 8,9. Hydrocephalus: Hydrocephalus appears in 5% of patients with neurosarcoidosis. We may find enlargement and contrast enhancement on T1WI of the ventricular walls although they can also enhance even without hydrocephalus.(fig. 6 on page 20). 2,10 Page 8 of 27,

9 Fig. 6: Same patient as in Fig. 1. T1-weighted image shows enlargement and contrast enhancement of the ventricular walls. References: - Palma De Mallorca/ES Page 9 of 27

10 Vascular lesions: Vasculitis is the most typical vascular lesion in neurosarcoidosis. It has been described as the presence of high T2 signal intensity non-enhancing white 6 matter lesions on MRI. Stroke is a rare form of presentation for neurosarcoidosis, and only a few cases have been reported. The cause of stroke in neurosarcoidosis is not completely understood but is thought to result from small-vessel vasculitis, embolus, or 11 large-vessel inflammation. Skull: The incidence of osseous sarcoidosis has been shown in various studies to range from 1 to 13%, generally involving the centrifugal skeleton. Sarcoidosis of the axial skeleton has only occasionally been reported. Skull affectation is uncommon, usually presenting with well circumscribed lytic lesions and less frequently with sclerotic or mixed 12,13 lytic/sclerotic lesions. They often enhance after contrast administration on T1WI. SPINAL LESIONS: Intramedullary spinal lesions: They usually appear as hyperintense on T2WI and hypointense on T1WI fusiform enlargements of the spinal cord, with nodular (Fig. 7 on page 20) or micronodular (Fig. 8 on page 21) enhancement after contrast 14 administration. There is a certain predilection for the cervical or upper thoracic level. Page 10 of 27

11 Fig. 7: Same patient as in Fig. 4. A. Multiple intramedullary lesions with fusiform medullary enlargement in T2W sequences. Contrast-enhanced fat-saturated sagittal T1-weighted image shows leptomeningeal and nodular intramedullary lesions. B. Lesion improvement after the steroid treatment. References: - Palma De Mallorca/ES Page 11 of 27

12 Fig. 8: 45-year-old woman, in 1996 she presented with seizures and she was diagnosed of neurosarcoidosis. The patient came with a 2 day history of headache, fever and weakness. A. Intramedullary diffuse hyperintensity on T2W images. B. Postcontrast T1-weighted image shows intramedullary diffuse micronodular enhancement. References: - Palma De Mallorca/ES Page 12 of 27

13 Extramedullary lesions: Leptomeningeal involvement may be seen as a lineal or a nodular enhancement of the meninges on gadolinium-enhanced T1WI (Fig. 9 on page 22). Dural lesions are uncommon, but they have been described as mass-like lesions with a dural base without predilection for any specific location. They can be iso or hypointense on T1 sequences and hyperintense on T2WI. They also enhance after 15 contrast administration and a dural tail may be seen sometimes. Page 13 of 27

14 Fig. 9: Same patient as in Fig. 4. Contrast-enhanced T1-weighted images show medullary leptomeningeal involvement with lineal enhancement and small nodules. Page 14 of 27

15 References: - Palma De Mallorca/ES Bone involvement: Vertebral lesions are uncommon and have predilection for the lower thoracic and upper lumbar spine. The MRI findings of vertebral involvement have been described as hypointense on T1W images and hyperintense on T2W images. Although there are cases described of vertebral sarcoidosis with low signal intensity on both T1W 12 and T2W images. Images for this section: Fig. 1: 40-year-old woman with a one month history of headache, nausea and vomiting. Post-contrast axial T1-weighted images show leptomeningeal enhancement along the perivascular supra and infratentorial spaces. Page 15 of 27

16 Fig. 2: 35-year-old woman with right exoftalmus. MRI images show an intraconal lesion surrounding the optic nerve. A. Axial T1-weighted and T2-weighted images. B. Contrastenhanced axial and coronal T1-weighted images. Page 16 of 27

17 Fig. 3: 41-year-old woman with a history of headache, right proptosis and visual disturbances. A. CT shows the presence of an orbital pseudotumor. B. One month after steroid treatment MRI images show a total resolution of the process. Page 17 of 27

18 Fig. 4: 32-year-old woman with a known ganglionar sarcoidosis and a 3 week history of ataxia, paresthesias, nausea, vomiting and dizziness. A. Multiple periventricular white matter lesions with high signal on T2/FLAIR images. B. Axial T1-weighted image shows the absence of enhancement after contrast administration. Page 18 of 27

19 Fig. 5: Same patient as in Fig. 4. A. Periventricular lesion with contrast enhancement on T1-weighted images. B. The MRI performed after the steroid treatment shows complete resolution of the lesion. Page 19 of 27

20 Fig. 6: Same patient as in Fig. 1. T1-weighted image shows enlargement and contrast enhancement of the ventricular walls. Page 20 of 27

21 Fig. 7: Same patient as in Fig. 4. A. Multiple intramedullary lesions with fusiform medullary enlargement in T2W sequences. Contrast-enhanced fat-saturated sagittal T1-weighted image shows leptomeningeal and nodular intramedullary lesions. B. Lesion improvement after the steroid treatment. Page 21 of 27

22 Fig. 8: 45-year-old woman, in 1996 she presented with seizures and she was diagnosed of neurosarcoidosis. The patient came with a 2 day history of headache, fever and weakness. A. Intramedullary diffuse hyperintensity on T2W images. B. Post-contrast T1weighted image shows intramedullary diffuse micronodular enhancement. Page 22 of 27

23 Fig. 9: Same patient as in Fig. 4. Contrast-enhanced T1-weighted images show medullary leptomeningeal involvement with lineal enhancement and small nodules. Page 23 of 27

24 Findings and procedure details MRI examinations were performed with a Siemens MAGNETOM Avanto 1.5T, General Electric Optima MR T and with a General Electric Signa HDxt 3.0T Optima Edition. CT examination was performed with a GE BrightSpeed 16 slice CT. We reviewed the MRI findings of 6 patients with a diagnosis of neurosarcoidosis followed up in a Systemic Autoimmune Diseases Unit in our hospital over a period of 11 years ( ). All patients were women with an age range from 32 to 45 years old. The MRI findings were (Table 1 on page 24): Table 1: Summary of findings in our patients. References: - Palma De Mallorca/ES Patient 1: Brain: Nonenhancing T2/FLAIR hyperintense parenchymal lesions and leptomeningeal enhancement. Spine: Intramedullary and leptomeningeal enhancing lesions. Patients 2 and 4: Isolated orbital pseudotumor. Patient 3: Brain: Nonenhancing T2/FLAIR hyperintense parenchymal lesions; contrast enhancing T1W parenchymal lesions and leptomeningeal enhancement. Spine: Intramedullary and leptomeningeal enhancing lesions. Patient 5: Brain: Leptomeningeal and ependymal enhancement. Patient 6: Isolated focal sellar lesion. Images for this section: Page 24 of 27

25 Table 1: Summary of findings in our patients. Page 25 of 27

26 Conclusion Sarcoidosis can involve CNS, producing a wide variety of symptoms and MRI findings that can mimick other neurological diseases. The most typical imaging feature is a leptomeningeal enhancement but it can also affect the dura mater. The most common parenchymal affectation is the presence of multiple non-enhancing T2 hyperintense white matter lesions. Sarcoidosis may also involve hypothalamus and the pituitary gland, the cranial nerves, the skull and vertebrae, the spinal cord and the intracranial vasculature. The Diagnosis of Neurosarcoidosis remains a challenge, especially in the absence of extraneural involvement. MRI is a useful tool that can be used in the diagnosis and followup of neurosarcoidosis. Personal information References 1. Koyama T, Ueda H, Togashi K, Umeoka S, Kataoka M, Nagai S. Radiologic manifestations of sarcoidosis in various organs. RadioGraphics 2004;24(1): Smith JK, Matheus MG, Castillo M. Imaging manifestations of neurosarcoidosis. AJR Am J Roentgenol. 2004; 182: Johns CJ, Michele TM. The clinical management of sarcoidosis: a 50-year experience at the Johns Hopkins Hospital. Medicine 1999;78: Christofordis GA, Spickler EM, Reccio MV, Mehta BM. MR of CNS sarcoidosis: correlation of imaging features to clinical symptoms and response to treatment. AJNR 1999;20: Peterson EA, Hymas DC, Pratt DV, Mortenson SW, Anderson RL, Mamalis N. Sarcoidosis with orbital tumor outside the lacrimal gland: initial manifestation in 2 elderly white women. Arch Ophthalmol 1998;116: Dumas JL, Valeyre D, Belin C, et al. Central nervous system sarcoidosis: followup at MRI during steroid therapy. Radiology 2000;214: Page 26 of 27

27 7. R. Shah, G.H. Roberson, J.K. Cure. Correlation of MR Imaging Findings and Clinical Manifestations in Neurosarcoidosis. AJNR Am J Neuroradiol 2009; 30: Langrand C, Bihan H, Raverot G, Varron L, Androdias G, Borson-Chazot F, Brue T, Cathebras P, Pinede L, Muller G, Broussolle C, Cotton F, Valeyre D, Seve P. Hypothalamo-pituitary sarcoidosis: a multicenter stud of 24 patients. Q J Med 2012; 105: Nowak DA, Widenka DC. Neurosarcoidosis: a review of its intracranial manifestation. J Neurol 2001;248: Brouwer MC, de Gans J, Willemse RB, van de Beek D. Neurological picture. Sarcoidosis presenting with hydrocephalus. J Neurol Neurosurg Psychiatry May;80(5): M.H. Hodge, R.L. Williams, M.B. Fukui. Neurosarcoidosis Presenting as Acute Infarction on Diffusion-Weighted MR Imaging: Summary of Radiologic Findings. AJNR Am J Neuroradiol 2007;28: Jelinek JS, Mark AS, Barth WF. Sclerotic lesions of the cervical spine in sarcoidosis. Skeletal Radiol 1998;27: Slart RM, de Jong JW, Haeck PW, Hoogenberg K. Lytic skull lesions and symptomatic hypercalcaemia in bone marrow sarcoidosis. J Intern Med 1999;246: Bradley S. Duhon, Lubdha Shah, Meic H. Schmidt. Isolated intramedullary neurosarcoidosis of the thoracic spine: case report and review of the literature. Eur Spine J (2012) 21 (Suppl 4):S390-S Connor SEJ, Marshman L, Al-Sarraj S, et al. MRI of a spinal intradural extramedullary sarcoid mass. Neuroradiology 2001;43: Page 27 of 27