- Email: [email protected]
An unusual cause of chronic headaches: answer
Journal of Clinical Neuroscience 21 (2014) 2032
Contents lists available at ScienceDirect
Journal of Clinical Neuroscience journal homepage: www.elsevier.com/locate/jocn
Images in Neuroscience: Answer
An unusual cause of chronic headaches: answer José Rafael P. Zuzuárregui a, Jessica Lin b,⇑, James A. Otis a a b
Boston University School of Medicine, Department of Neurology, Boston, MA, USA Boston University School of Medicine, 72 E. Concord Street, C3, Boston, MA 02118, USA
1. Answer D. Rheumatoid pachymeningitis. 2. Discussion In the setting of a negative infectious and autoimmune workup as well as a history of rheumatoid arthritis, the biopsy results supported the diagnosis of rheumatoid pachymeningitis in this patient. Rheumatoid pachymeningitis is a rare complication of rheumatoid arthritis, and few case reports exist in the literature. Rheumatoid pachymeningitis can occur without concurrent symptoms of arthritis and may present early or late in the course of rheumatoid disease [1,2]. Other neurologic complications of rheumatoid arthritis include cerebral hemorrhage, infarction, vasculitis and spinal cord compression secondary to vertebral subluxation [3,4]. There are no defined diagnostic criteria for rheumatoid meningitis. Manifestations include seizure, headache, confusion, cranial nerve palsies, cortical blindness, and sensorineural hearing loss [2,4]. MRI typically demonstrates focal or diffuse pachymeningeal and/or leptomeningeal thickening with contrast enhancement, while cerebrospinal fluid studies show mild pleocytosis, elevated protein and hypoglycorrhachia, as was seen in our patient [4]. Typically, a biopsy is performed to support the diagnosis or to rule out other etiologies. Histopathologic findings may include rheumatoid nodules, inflammatory changes in the pachymeninges and/or leptomeninges, and vasculitis [5]. Rheumatoid nodules in the dura have typically been found only on autopsy as tissue sampling is limited during biopsy, requiring that other etiologies of pachymeningitis be ruled out [2]. The differential diagnosis for
DOI of question: http://dx.doi.org/10.1016/j.jocn.2014.04.023
⇑ Corresponding author. Tel.: +1 626 808 2881. E-mail address: [email protected] (J. Lin). http://dx.doi.org/10.1016/j.jocn.2014.06.011 0967-5868/Ó 2014 Published by Elsevier Ltd.
pachymeningitis includes tuberculosis, sarcoidosis, fungal infections, lymphoma, meningeal carcinomatosis, vasculitis, and intracranial hypotension [3,4]. Rheumatoid meningitis is usually treated with immunosuppression, although optimal studies are lacking. Methotrexate, cyclophosphamide, rituximab and oral prednisone have all been used with anecdotal success [1–5]. Conflicts of Interest/Disclosures The authors declare that they have no financial or other conflicts of interest in relation to this research and its publication. Acknowledgements We would like to acknowledge and thank Dr. Ivana Delalle, a neuropathologist at Boston Medical Center, for providing the biopsy images and the biopsy report. References [1] Schmid L, Müller M, Treumann T, et al. Induction of complete and sustained remission of rheumatoid pachymeningitis by rituximab. Arthritis Rheum 2009;60:1632–4. [2] Starosta MA, Brandwein SR. Clinical manifestations and treatment of rheumatoid pachymeningitis. Neurology 2007;68:1079–80. [3] Cellerini M, Gabbrielli S, Maddali Bongi S, et al. MRI of cerebral rheumatoid pachymeningitis: report of two cases with follow-up. Neuroradiology 2001;43:147–50. [4] Tan HJ, Raymond AA, Phadke PP, et al. Rheumatoid pachymeningitis. Singapore Med J 2004;45:337–9. [5] Bathon JM, Moreland LW, DiBartolomeo AG. Inflammatory central nervous system involvement in rheumatoid arthritis. Semin Arthritis Rheum 1989;18:258–66.
Contents lists available at ScienceDirect
Journal of Clinical Neuroscience journal homepage: www.elsevier.com/locate/jocn
Images in Neuroscience: Answer
An unusual cause of chronic headaches: answer José Rafael P. Zuzuárregui a, Jessica Lin b,⇑, James A. Otis a a b
Boston University School of Medicine, Department of Neurology, Boston, MA, USA Boston University School of Medicine, 72 E. Concord Street, C3, Boston, MA 02118, USA
1. Answer D. Rheumatoid pachymeningitis. 2. Discussion In the setting of a negative infectious and autoimmune workup as well as a history of rheumatoid arthritis, the biopsy results supported the diagnosis of rheumatoid pachymeningitis in this patient. Rheumatoid pachymeningitis is a rare complication of rheumatoid arthritis, and few case reports exist in the literature. Rheumatoid pachymeningitis can occur without concurrent symptoms of arthritis and may present early or late in the course of rheumatoid disease [1,2]. Other neurologic complications of rheumatoid arthritis include cerebral hemorrhage, infarction, vasculitis and spinal cord compression secondary to vertebral subluxation [3,4]. There are no defined diagnostic criteria for rheumatoid meningitis. Manifestations include seizure, headache, confusion, cranial nerve palsies, cortical blindness, and sensorineural hearing loss [2,4]. MRI typically demonstrates focal or diffuse pachymeningeal and/or leptomeningeal thickening with contrast enhancement, while cerebrospinal fluid studies show mild pleocytosis, elevated protein and hypoglycorrhachia, as was seen in our patient [4]. Typically, a biopsy is performed to support the diagnosis or to rule out other etiologies. Histopathologic findings may include rheumatoid nodules, inflammatory changes in the pachymeninges and/or leptomeninges, and vasculitis [5]. Rheumatoid nodules in the dura have typically been found only on autopsy as tissue sampling is limited during biopsy, requiring that other etiologies of pachymeningitis be ruled out [2]. The differential diagnosis for
DOI of question: http://dx.doi.org/10.1016/j.jocn.2014.04.023
⇑ Corresponding author. Tel.: +1 626 808 2881. E-mail address: [email protected] (J. Lin). http://dx.doi.org/10.1016/j.jocn.2014.06.011 0967-5868/Ó 2014 Published by Elsevier Ltd.
pachymeningitis includes tuberculosis, sarcoidosis, fungal infections, lymphoma, meningeal carcinomatosis, vasculitis, and intracranial hypotension [3,4]. Rheumatoid meningitis is usually treated with immunosuppression, although optimal studies are lacking. Methotrexate, cyclophosphamide, rituximab and oral prednisone have all been used with anecdotal success [1–5]. Conflicts of Interest/Disclosures The authors declare that they have no financial or other conflicts of interest in relation to this research and its publication. Acknowledgements We would like to acknowledge and thank Dr. Ivana Delalle, a neuropathologist at Boston Medical Center, for providing the biopsy images and the biopsy report. References [1] Schmid L, Müller M, Treumann T, et al. Induction of complete and sustained remission of rheumatoid pachymeningitis by rituximab. Arthritis Rheum 2009;60:1632–4. [2] Starosta MA, Brandwein SR. Clinical manifestations and treatment of rheumatoid pachymeningitis. Neurology 2007;68:1079–80. [3] Cellerini M, Gabbrielli S, Maddali Bongi S, et al. MRI of cerebral rheumatoid pachymeningitis: report of two cases with follow-up. Neuroradiology 2001;43:147–50. [4] Tan HJ, Raymond AA, Phadke PP, et al. Rheumatoid pachymeningitis. Singapore Med J 2004;45:337–9. [5] Bathon JM, Moreland LW, DiBartolomeo AG. Inflammatory central nervous system involvement in rheumatoid arthritis. Semin Arthritis Rheum 1989;18:258–66.