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Case Reports / Journal of Clinical Neuroscience 22 (2015) 1532–1536
Epidural spinal involvement of Erdheim–Chester disease causing myelopathy Brian Y. Hwang b, Ann Liu b, Jason Kern c, C. Rory Goodwin b, Jean Paul Wolinsky b, Atman Desai a,⇑ a b c
Department of Neurosurgery, Stanford University Medical Center, 300 Pasteur Drive Room 201, Edwards Building MC 5327, Stanford, CA 94305, USA Department of Neurosurgery, Johns Hopkins Hospital, Baltimore, MD, USA Department of Pathology, Johns Hopkins Hospital, Baltimore, MD, USA
a r t i c l e
i n f o
Article history: Received 2 April 2015 Accepted 8 April 2015
Keywords: Epidural Erdheim–Chester disease Non-Langerhans histiocytosis Spine
a b s t r a c t We present a 25-year-old woman with Erdheim–Chester disease (ECD) presenting with progressive myelopathy from multiple compressive spinal epidural lesions who required cervicothoracic decompression and fusion, and summarize the literature on epidural spinal involvement of ECD. ECD is a rare non-Langerhans histiocytosis affecting multiple organ systems through infiltration and characteristically causing multifocal osteosclerosis. Central nervous system involvement, particularly of the spine, is rare. Ó 2015 Elsevier Ltd. All rights reserved.
1. Introduction Erdheim–Chester disease (ECD) is a rare form of non-Langerhans histiocytosis with multisystem manifestations. Spinal involvement is uncommon, with even fewer reports of epidural involvement. We describe an unusual ECD patient who presented with progressive lower extremity numbness and gait difficulty from multiple compressive spinal epidural lesions requiring cervicothoracic decompression and instrumented fusion. 2. Case report 2.1. History A 25-year-old Caucasian woman with a history of ECD presented with 3 weeks of worsening bilateral lower extremity numbness and gait difficulty. She was diagnosed with ECD 2 years prior after presenting with longstanding idiopathic diabetes insipidus and new shoulder pain. Her CT scans showed involvement of the spleen and kidneys and surrounding the aorta (Fig. 1). A biopsy of the peri-aortic lesion showed dense fibrous tissue with minimal chronic inflammation. She was started on prednisone and methotrexate and a significant reduction was seen in the masses on a repeat CT scan 3 month later. 2.2. Examination She had full strength in her upper extremities, 4+/5 strength in her lower extremities, a T5 sensory level with approximately 50% decreased sensation to light touch, and intact proprioception. She had hyperreflexia of the bilateral biceps/triceps/brachioradialis/ patella/ankle reflexes. She had 3 to 4 beats of clonus and a positive Babinski’s sign bilaterally. Her gait was unsteady, needing some assistance to ambulate. A spine MRI with contrast showed an enhancing T1/T2weighted hypointense ventral epidural mass from C5–T1 compressing the spinal cord with T2-weighted cord signal change (Fig. 2). The lesion extended into the right C5–6, bilateral C6–C7, C7–T1 and T1–T2 neural foramina. An enhancing epidural mass ⇑ Corresponding author. Tel.: +1 650 495 6971. E-mail address: [email protected] (A. Desai).
was seen from T2–T5 and at T9 with T2 cord signal change at T3–4, and extended into the right T2–T5 neural foramina. In the lumbosacral region, the epidural mass extended ventrally from L5–S3 with almost complete filling of the canal at S1–S3 and cauda equina compression. There was no osseous involvement. 2.3. Operation C4–T5 posterior decompression, C4–T6 instrumented fusion, and right-sided T3–T4 transpedicular decompression, and epidural tumor debulking were performed. Lateral mass screws were placed at C4–C6, pedicle screws at T1–T2, and T6 using standard anatomic landmarks. Bilateral laminectomies were performed from C4–T5. The right T4 pedicle was drilled down to the level of the vertebral body. The epidural lesion was well-visualized, pale, firm, rubbery, readily dissected from the dura and removed without difficulty. 2.4. Pathology Grossly, the epidural lesion was pale, fibrous and avascular. Histology showed a diffuse histiocytic infiltrate with scattered mononuclear inflammatory cells in a background of fibrosis (Fig. 3). The histiocytes, confirmed by a CD68 immunostain, showed abundant, foamy cytoplasm and focal giant cell formation. An S100 immunostain showed focal positivity, and a CD1a immunostain was negative (Fig. 4). 2.5. Postoperative The patient had an uneventful postoperative course. She was discharged to home in a stable condition on postoperative day 7, with improved lower extremity sensation and strength and the ability to ambulate independently. At 3 months, she regained her baseline neurologic function and returned to work. 3. Discussion ECD is a rare xanthogranulomatous non-Langerhans histiocytosis with less than 400 reported patients since its first description. Central nervous system involvement has been reported in 45– 51% of patients, accounting for 29% of all deaths [3]. The most
Case Reports / Journal of Clinical Neuroscience 22 (2015) 1532–1536
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Fig. 1. Thoraco-abdominal CT scan with contrast demonstrating diffuse soft tissue infiltration surrounding the aorta from the great vessels to the distal thoracic/upper abdominal regions (A). Soft tissue infiltration surrounds both kidneys with relative atrophy and chronic hydronephrosis of the left kidney (B).
Fig. 2. Preoperative sagittal MRI of the spine demonstrating multiple T1-weighted iso/hypointense (A), enhancing (B), T2-weighted hypointense (C) epidural lesions at C5–T1 causing spinal cord compression. Axial T1-weighted MRI with contrast showed that the lesion extended into the right C5–6 (D) and bilateral C6–C7, C7–T1 and T1–T2 neural foramina, and that the mass extended into the right T2–T3, T3–T4 (E) and T4–T5 neural foramina. An enhancing epidural mass is seen from the T2–T5 levels and at T9 (A, B, C). In the lumbosacral region, the epidural mass extended ventrally from L5–S3 with almost complete filling of the canal at S1–S3 (A, B, C).
common neurological manifestations include diabetes insipidus, exophthalmos, cerebellar ataxia, panhypopituitarism and papilledema [7]. Other reports include involvement of the superior sagittal sinus and choroid plexus and lesions mimicking glial
tumors, demyelinating diseases, or meningiomas. The involvement of the spine and spinal cord is rare with only nine reports in the literature (Table 1). Furthermore, few reports of epidural lesions exist, and only two describe the characteristic MRI findings [1].
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Case Reports / Journal of Clinical Neuroscience 22 (2015) 1532–1536
Fig. 3. Epidural tumor with diffuse histiocytic infiltrate with focal giant cell formation (arrowhead), fibrosis and scattered lymphocytes (A; 100; hematoxylin and eosin). Xanthogranulomatous tissue with foamy histiocytes, giant cell formation, and scattered lymphocytes (B; 400; hematoxylin and eosin). This figure is available in colour at www.sciencedirect.com.
Fig. 4. Immunostained sections of the epidural tumor (200): positive for CD68 stain (A), negative for CD1a immunostain (B), and S100 staining showed scattered positivity (C). This figure is available in colour at www.sciencedirect.com.
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Case Reports / Journal of Clinical Neuroscience 22 (2015) 1532–1536 Table 1 Summary of reported patients with epidural spinal involvements of Erdheim–Chester disease Author
Year
Presentation
Physical exam
Radiographic findings
T1-weighted MRI
T2-weighted MRI
Treatment
Outcome
Albayram et al. [1]
2002
43-year-old man with 2 weeks leg weakness and 2 months upper back pain.
Gait spasticity and hyperreflexia both legs. Diminished sensation below T1 level.
MRI: Epidural and subdural soft tissue masses causing cord compression T1–T6.
Epidural lesion hypointense to cord without enhancement. Subdural lesion isointense to cord.
Epidural lesion hypointense to cord without enhancement. Subdural lesion hypointense cord.
T1–T6 decompressive laminectomy.
N/A
Allmendinger et al. [2]
2010
87-year-old man with low back pain.
N/A
MRI: Epidural lesion of T10, L3, and S1 vertebrae.
Hypointense.
Isointense.
Radiotherapy and interferona therapy.
Died 2 months after diagnosis.
Babu et al. [4]
1997
22-year-old man with 4 months feet numbness and gait difficulty.
Spastic paraparesis (2/5) with sensory level T6.
MRI: Epidural mass extending from T5–T11.
N/A
N/A
T5–T12 laminectomy with radiation (8 Gy).
Neurologically intact at 4 years.
CaparrosLefebvre et al. [5]
1995
74-year-old woman with paraplegia, ataxia and bladder incontinence.
N/A
CT scan: Large lesion in epidural space at L1.
N/A
N/A
N/A
Died of aspiration pneumopathy.
Curgunlu et al. [6]
2003
43-year-old man with several months progressive weakness, feet numbness, spasticity and muscle wasting lower limbs.
Weakness of hip, knee flexors, and knee extensors. Increased reflexes in both extremities. Normal sensory exam.
MRI: Multiple epidural masses between T1–T9, T11–L1.
N/A
N/A
T1–T5 laminectomy and decompression, palliative therapy.
Recurrence and systemic progression.
Pego-Reigosa et al. [8]
2000
50-year-old woman with 5 months paresthesia and weakness of the lower extremities.
Spastic paraparesis (4/5) and bilateral Babinski responses.
CT myelogram: Extramedullary intradural mass at T3 and T9.
N/A
N/A
Surgical resection.
N/A
Shimada et al. [9]
2007
47-year-old man with lesions of the spinal dura on autopsy.
N/A
N/A
N/A
N/A
N/A
Died 3.5 years after initial presentation of disease.
Takeuchi et al. [10]
2012
56-year-old man with weakness of the left side and paresis.
N/A
MRI: Cervical intramedullary lesion.
Hyperintense.
Hypointense.
Surgical resection.
N/A
Tzoulis et al. [11]
2012
31-year-old woman 10 years of slowly progressive gait difficulty.
Spastic paraparesis with extensor plantar responses and normal sensory function.
MRI: Diffuse, noncontrast enhancing lesion cervical and thoracic vertebrae with intramedullary lesion T4–T6.
Inhomogeneously hyperintense.
N/A
Interferon-a.
At 1.5 years after diagnosis, no signs of disease progress.
This case report
2014
25-year-old woman with 3 weeks worsening bilateral lower extremity numbness and paresthesias and difficulty with ambulation.
4+/5 strength lower extremities. T5 sensory level with 50% decreased sensation to light touch.
MRI: enhancing T1/T2-weighted hypointense ventral epidural mass at C5–T1 compressing cord.
Hypointense.
Hypointense.
Surgical resection.
At 3 months, regained baseline neurologic function and returned to work.
N/A = not applicable.
Epidural lesions have been found to be hypointense on T1-weighted MRI and either hypo- or isointense on T2-weighted sequences [1]. In our patient, multiple spinal epidural ECD lesions were found. Consistent with previous reports [1], the epidural lesions in our patients were hypointense on both T1- and T2-weighted sequences. The epidural lesions easily peeled off the dura with minimal risk of cerebrospinal fluid leak. Therefore, decompressive
surgery, with or without fusion, for epidural lesions can effectively and safely improve neurological symptoms in ECD patients. 4. Conclusion ECD can involve the spinal cord through epidural extension, and thorough evaluation is warranted in patients with ECD who present with back pain or neurologic symptoms. Surgery is indicated
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Case Reports / Journal of Clinical Neuroscience 22 (2015) 1532–1536
in the setting of new/progressive symptoms and good surgical outcomes can be expected. Conflicts of Interest/Disclosures
[4] [5] [6]
The authors declare that they have no financial or other conflicts of interest in relation to this research and its publication.
[7] [8]
References [1] Albayram S, Kizilkic O, Zulfikar Z, et al. Spinal dural involvement in Erdheim– Chester disease: MRI findings. Neuroradiology 2002;44:1004–7. [2] Allmendinger AM, Krauthamer AV, Spektor V, et al. Atypical spine involvement of Erdheim–Chester disease in an elderly male. J Neurosurg Spine 2010;12: 257–60. [3] Arnaud L, Hervier B, Neel A, et al. CNS involvement and treatment with interferon-alpha are independent prognostic factors in Erdheim–Chester http://dx.doi.org/10.1016/j.jocn.2015.04.004
[9] [10] [11]
disease: a multicenter survival analysis of 53 patients. Blood 2011;117: 2778–82. Babu RP, Lansen TA, Chadburn A, et al. Erdheim–Chester disease of the central nervous system report of two cases. J Neurosurg 1997;86:888–92. Caparros-Lefebvre D, Pruvo JP, Remy M, et al. Neuroradiologic aspects of Chester–Erdheim disease. AJNR Am J Neuroradiol 1995;16:735–40. Curgunlu A, Karter Y, Ozturk A, et al. Erdheim–Chester disease: a rare cause of paraplegia. Eur J Intern Med 2003;14:53–5. Mazor RD, Manevich-Mazor M, Shoenfeld Y, et al. Erdheim–Chester disease: a comprehensive review of the literature. Orphanet J Rare Dis 2013;8:137. Pego-Reigosa R, Branas-Fernandez F, Martinez-Vazquez F, et al. Erdheim– Chester disease with spinal cord manifestations. Eur Neurol 2000;43:242–4. Shimada S, Ono K, Hashizume Y, et al. Intracranial lesion of Erdheim–Chester disease. Hum Pathol 2007;38:950–1. Takeuchi T, Sato M, Sonomura T, et al. Erdheim–Chester disease associated with intramedullary spinal cord lesion. Br J Radiol 2012;85:e62–4. Tzoulis C, Gjerde IO, Softeland E, et al. Erdheim–Chester disease presenting with an intramedullary spinal cord lesion. J Neurol 2012;259:2240–2.
Case Reports / Journal of Clinical Neuroscience 22 (2015) 1532–1536
Epidural spinal involvement of Erdheim–Chester disease causing myelopathy Brian Y. Hwang b, Ann Liu b, Jason Kern c, C. Rory Goodwin b, Jean Paul Wolinsky b, Atman Desai a,⇑ a b c
Department of Neurosurgery, Stanford University Medical Center, 300 Pasteur Drive Room 201, Edwards Building MC 5327, Stanford, CA 94305, USA Department of Neurosurgery, Johns Hopkins Hospital, Baltimore, MD, USA Department of Pathology, Johns Hopkins Hospital, Baltimore, MD, USA
a r t i c l e
i n f o
Article history: Received 2 April 2015 Accepted 8 April 2015
Keywords: Epidural Erdheim–Chester disease Non-Langerhans histiocytosis Spine
a b s t r a c t We present a 25-year-old woman with Erdheim–Chester disease (ECD) presenting with progressive myelopathy from multiple compressive spinal epidural lesions who required cervicothoracic decompression and fusion, and summarize the literature on epidural spinal involvement of ECD. ECD is a rare non-Langerhans histiocytosis affecting multiple organ systems through infiltration and characteristically causing multifocal osteosclerosis. Central nervous system involvement, particularly of the spine, is rare. Ó 2015 Elsevier Ltd. All rights reserved.
1. Introduction Erdheim–Chester disease (ECD) is a rare form of non-Langerhans histiocytosis with multisystem manifestations. Spinal involvement is uncommon, with even fewer reports of epidural involvement. We describe an unusual ECD patient who presented with progressive lower extremity numbness and gait difficulty from multiple compressive spinal epidural lesions requiring cervicothoracic decompression and instrumented fusion. 2. Case report 2.1. History A 25-year-old Caucasian woman with a history of ECD presented with 3 weeks of worsening bilateral lower extremity numbness and gait difficulty. She was diagnosed with ECD 2 years prior after presenting with longstanding idiopathic diabetes insipidus and new shoulder pain. Her CT scans showed involvement of the spleen and kidneys and surrounding the aorta (Fig. 1). A biopsy of the peri-aortic lesion showed dense fibrous tissue with minimal chronic inflammation. She was started on prednisone and methotrexate and a significant reduction was seen in the masses on a repeat CT scan 3 month later. 2.2. Examination She had full strength in her upper extremities, 4+/5 strength in her lower extremities, a T5 sensory level with approximately 50% decreased sensation to light touch, and intact proprioception. She had hyperreflexia of the bilateral biceps/triceps/brachioradialis/ patella/ankle reflexes. She had 3 to 4 beats of clonus and a positive Babinski’s sign bilaterally. Her gait was unsteady, needing some assistance to ambulate. A spine MRI with contrast showed an enhancing T1/T2weighted hypointense ventral epidural mass from C5–T1 compressing the spinal cord with T2-weighted cord signal change (Fig. 2). The lesion extended into the right C5–6, bilateral C6–C7, C7–T1 and T1–T2 neural foramina. An enhancing epidural mass ⇑ Corresponding author. Tel.: +1 650 495 6971. E-mail address: [email protected] (A. Desai).
was seen from T2–T5 and at T9 with T2 cord signal change at T3–4, and extended into the right T2–T5 neural foramina. In the lumbosacral region, the epidural mass extended ventrally from L5–S3 with almost complete filling of the canal at S1–S3 and cauda equina compression. There was no osseous involvement. 2.3. Operation C4–T5 posterior decompression, C4–T6 instrumented fusion, and right-sided T3–T4 transpedicular decompression, and epidural tumor debulking were performed. Lateral mass screws were placed at C4–C6, pedicle screws at T1–T2, and T6 using standard anatomic landmarks. Bilateral laminectomies were performed from C4–T5. The right T4 pedicle was drilled down to the level of the vertebral body. The epidural lesion was well-visualized, pale, firm, rubbery, readily dissected from the dura and removed without difficulty. 2.4. Pathology Grossly, the epidural lesion was pale, fibrous and avascular. Histology showed a diffuse histiocytic infiltrate with scattered mononuclear inflammatory cells in a background of fibrosis (Fig. 3). The histiocytes, confirmed by a CD68 immunostain, showed abundant, foamy cytoplasm and focal giant cell formation. An S100 immunostain showed focal positivity, and a CD1a immunostain was negative (Fig. 4). 2.5. Postoperative The patient had an uneventful postoperative course. She was discharged to home in a stable condition on postoperative day 7, with improved lower extremity sensation and strength and the ability to ambulate independently. At 3 months, she regained her baseline neurologic function and returned to work. 3. Discussion ECD is a rare xanthogranulomatous non-Langerhans histiocytosis with less than 400 reported patients since its first description. Central nervous system involvement has been reported in 45– 51% of patients, accounting for 29% of all deaths [3]. The most
Case Reports / Journal of Clinical Neuroscience 22 (2015) 1532–1536
1533
Fig. 1. Thoraco-abdominal CT scan with contrast demonstrating diffuse soft tissue infiltration surrounding the aorta from the great vessels to the distal thoracic/upper abdominal regions (A). Soft tissue infiltration surrounds both kidneys with relative atrophy and chronic hydronephrosis of the left kidney (B).
Fig. 2. Preoperative sagittal MRI of the spine demonstrating multiple T1-weighted iso/hypointense (A), enhancing (B), T2-weighted hypointense (C) epidural lesions at C5–T1 causing spinal cord compression. Axial T1-weighted MRI with contrast showed that the lesion extended into the right C5–6 (D) and bilateral C6–C7, C7–T1 and T1–T2 neural foramina, and that the mass extended into the right T2–T3, T3–T4 (E) and T4–T5 neural foramina. An enhancing epidural mass is seen from the T2–T5 levels and at T9 (A, B, C). In the lumbosacral region, the epidural mass extended ventrally from L5–S3 with almost complete filling of the canal at S1–S3 (A, B, C).
common neurological manifestations include diabetes insipidus, exophthalmos, cerebellar ataxia, panhypopituitarism and papilledema [7]. Other reports include involvement of the superior sagittal sinus and choroid plexus and lesions mimicking glial
tumors, demyelinating diseases, or meningiomas. The involvement of the spine and spinal cord is rare with only nine reports in the literature (Table 1). Furthermore, few reports of epidural lesions exist, and only two describe the characteristic MRI findings [1].
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Case Reports / Journal of Clinical Neuroscience 22 (2015) 1532–1536
Fig. 3. Epidural tumor with diffuse histiocytic infiltrate with focal giant cell formation (arrowhead), fibrosis and scattered lymphocytes (A; 100; hematoxylin and eosin). Xanthogranulomatous tissue with foamy histiocytes, giant cell formation, and scattered lymphocytes (B; 400; hematoxylin and eosin). This figure is available in colour at www.sciencedirect.com.
Fig. 4. Immunostained sections of the epidural tumor (200): positive for CD68 stain (A), negative for CD1a immunostain (B), and S100 staining showed scattered positivity (C). This figure is available in colour at www.sciencedirect.com.
1535
Case Reports / Journal of Clinical Neuroscience 22 (2015) 1532–1536 Table 1 Summary of reported patients with epidural spinal involvements of Erdheim–Chester disease Author
Year
Presentation
Physical exam
Radiographic findings
T1-weighted MRI
T2-weighted MRI
Treatment
Outcome
Albayram et al. [1]
2002
43-year-old man with 2 weeks leg weakness and 2 months upper back pain.
Gait spasticity and hyperreflexia both legs. Diminished sensation below T1 level.
MRI: Epidural and subdural soft tissue masses causing cord compression T1–T6.
Epidural lesion hypointense to cord without enhancement. Subdural lesion isointense to cord.
Epidural lesion hypointense to cord without enhancement. Subdural lesion hypointense cord.
T1–T6 decompressive laminectomy.
N/A
Allmendinger et al. [2]
2010
87-year-old man with low back pain.
N/A
MRI: Epidural lesion of T10, L3, and S1 vertebrae.
Hypointense.
Isointense.
Radiotherapy and interferona therapy.
Died 2 months after diagnosis.
Babu et al. [4]
1997
22-year-old man with 4 months feet numbness and gait difficulty.
Spastic paraparesis (2/5) with sensory level T6.
MRI: Epidural mass extending from T5–T11.
N/A
N/A
T5–T12 laminectomy with radiation (8 Gy).
Neurologically intact at 4 years.
CaparrosLefebvre et al. [5]
1995
74-year-old woman with paraplegia, ataxia and bladder incontinence.
N/A
CT scan: Large lesion in epidural space at L1.
N/A
N/A
N/A
Died of aspiration pneumopathy.
Curgunlu et al. [6]
2003
43-year-old man with several months progressive weakness, feet numbness, spasticity and muscle wasting lower limbs.
Weakness of hip, knee flexors, and knee extensors. Increased reflexes in both extremities. Normal sensory exam.
MRI: Multiple epidural masses between T1–T9, T11–L1.
N/A
N/A
T1–T5 laminectomy and decompression, palliative therapy.
Recurrence and systemic progression.
Pego-Reigosa et al. [8]
2000
50-year-old woman with 5 months paresthesia and weakness of the lower extremities.
Spastic paraparesis (4/5) and bilateral Babinski responses.
CT myelogram: Extramedullary intradural mass at T3 and T9.
N/A
N/A
Surgical resection.
N/A
Shimada et al. [9]
2007
47-year-old man with lesions of the spinal dura on autopsy.
N/A
N/A
N/A
N/A
N/A
Died 3.5 years after initial presentation of disease.
Takeuchi et al. [10]
2012
56-year-old man with weakness of the left side and paresis.
N/A
MRI: Cervical intramedullary lesion.
Hyperintense.
Hypointense.
Surgical resection.
N/A
Tzoulis et al. [11]
2012
31-year-old woman 10 years of slowly progressive gait difficulty.
Spastic paraparesis with extensor plantar responses and normal sensory function.
MRI: Diffuse, noncontrast enhancing lesion cervical and thoracic vertebrae with intramedullary lesion T4–T6.
Inhomogeneously hyperintense.
N/A
Interferon-a.
At 1.5 years after diagnosis, no signs of disease progress.
This case report
2014
25-year-old woman with 3 weeks worsening bilateral lower extremity numbness and paresthesias and difficulty with ambulation.
4+/5 strength lower extremities. T5 sensory level with 50% decreased sensation to light touch.
MRI: enhancing T1/T2-weighted hypointense ventral epidural mass at C5–T1 compressing cord.
Hypointense.
Hypointense.
Surgical resection.
At 3 months, regained baseline neurologic function and returned to work.
N/A = not applicable.
Epidural lesions have been found to be hypointense on T1-weighted MRI and either hypo- or isointense on T2-weighted sequences [1]. In our patient, multiple spinal epidural ECD lesions were found. Consistent with previous reports [1], the epidural lesions in our patients were hypointense on both T1- and T2-weighted sequences. The epidural lesions easily peeled off the dura with minimal risk of cerebrospinal fluid leak. Therefore, decompressive
surgery, with or without fusion, for epidural lesions can effectively and safely improve neurological symptoms in ECD patients. 4. Conclusion ECD can involve the spinal cord through epidural extension, and thorough evaluation is warranted in patients with ECD who present with back pain or neurologic symptoms. Surgery is indicated
1536
Case Reports / Journal of Clinical Neuroscience 22 (2015) 1532–1536
in the setting of new/progressive symptoms and good surgical outcomes can be expected. Conflicts of Interest/Disclosures
[4] [5] [6]
The authors declare that they have no financial or other conflicts of interest in relation to this research and its publication.
[7] [8]
References [1] Albayram S, Kizilkic O, Zulfikar Z, et al. Spinal dural involvement in Erdheim– Chester disease: MRI findings. Neuroradiology 2002;44:1004–7. [2] Allmendinger AM, Krauthamer AV, Spektor V, et al. Atypical spine involvement of Erdheim–Chester disease in an elderly male. J Neurosurg Spine 2010;12: 257–60. [3] Arnaud L, Hervier B, Neel A, et al. CNS involvement and treatment with interferon-alpha are independent prognostic factors in Erdheim–Chester http://dx.doi.org/10.1016/j.jocn.2015.04.004
[9] [10] [11]
disease: a multicenter survival analysis of 53 patients. Blood 2011;117: 2778–82. Babu RP, Lansen TA, Chadburn A, et al. Erdheim–Chester disease of the central nervous system report of two cases. J Neurosurg 1997;86:888–92. Caparros-Lefebvre D, Pruvo JP, Remy M, et al. Neuroradiologic aspects of Chester–Erdheim disease. AJNR Am J Neuroradiol 1995;16:735–40. Curgunlu A, Karter Y, Ozturk A, et al. Erdheim–Chester disease: a rare cause of paraplegia. Eur J Intern Med 2003;14:53–5. Mazor RD, Manevich-Mazor M, Shoenfeld Y, et al. Erdheim–Chester disease: a comprehensive review of the literature. Orphanet J Rare Dis 2013;8:137. Pego-Reigosa R, Branas-Fernandez F, Martinez-Vazquez F, et al. Erdheim– Chester disease with spinal cord manifestations. Eur Neurol 2000;43:242–4. Shimada S, Ono K, Hashizume Y, et al. Intracranial lesion of Erdheim–Chester disease. Hum Pathol 2007;38:950–1. Takeuchi T, Sato M, Sonomura T, et al. Erdheim–Chester disease associated with intramedullary spinal cord lesion. Br J Radiol 2012;85:e62–4. Tzoulis C, Gjerde IO, Softeland E, et al. Erdheim–Chester disease presenting with an intramedullary spinal cord lesion. J Neurol 2012;259:2240–2.
