http://informahealthcare.com/mor ISSN 1439-7595 (print), 1439-7609 (online) Mod Rheumatol, 2014; Early Online: 1–2 © 2014 Japan College of Rheumatology DOI: 10.3109/14397595.2014.924469
LETTER
Spontaneous intracranial hypotension in a case of systemic lupus erythematosus Daisuke Ando1, Izaya Nakaya1, Shuji Watanabe1, Hiroyuki Osawa1, Naohiko Kubo2, and Jun Soma1 1Department of Nephrology, Iwate Prefectural Central Hospital, Morioka, Iwate, Japan, and 2Department of Neurosurgery, Morioka Red Cross Hospital,
Mod Rheumatol Downloaded from informahealthcare.com by University of Otago on 12/30/14 For personal use only.
Morioka, Iwate, Japan
Keywords Cerebrospinal fluid leakage, Plasma adsorption, Spontaneous intracranial hypotension, Systemic lupus erythematosus History Received 26 November 2013 Accepted 12 May 2014 Published online 18 June 2014
To the Editor, Spontaneous intracranial hypotension (SIH) is typically characterized by traction headaches. The most common pathological mechanism is cerebrospinal fluid (CSF) leakage after lumbar puncture, spine/spinal cord trauma, or an unidentified cause [1]. We present a case of SIH with systemic lupus erythematosus (SLE), where SIH symptoms synchronized with SLE activity. A 32-year-old woman was admitted to a hospital with gait disturbance and cognitive dysfunction in September 2012. In October 2004, she was diagnosed with SLE because of facial erythema, photosensitivity, oral ulcers, positive serum antinuclear antibody, elevated anti-double-stranded DNA antibody, polyarthritis, and leukocytopenia. Her symptoms receded after prednisolone treatment. However, proteinuria and hematuria first appeared in March 2009 after she became pregnant. Artificial abortion and renal biopsy were performed in February 2009. The histological diagnosis revealed lupus nephritis Class III ⫹ V (WHO classification). She was treated with prednisolone, immunosuppressants, and immunoabsorption; however, she presented with orthostatic headache and nausea in October 2011, and SLE exacerbation, such as increase of proteinuria, positive serum antinuclear antibody, elevation of anti-double-stranded DNA antibody, and leukocytopenia, was found to be associated with her poor treatment adherence. Magnetic resonance imaging (MRI) revealed dural thickening and subdural hygroma, suggesting SIH. However, she had no previous history of lumbar puncture or trauma, and MR myelography did not reveal the location of CSF leakage. After 1 year of follow-up, gait disturbance and cognitive dysfunction appeared in August 2012. MRI re-examination revealed worsening subdural hygroma
Correspondence to: Daisuke Ando, Department of Nephrology, Iwate Prefectural Central Hospital, Ueda 1-4-1, Morioka, Iwate 020-0066, Japan. Tel: ⫹ 81-19-653-1151. Fax: ⫹ 81-19-653-8919. E-mail: daisukeandojp@ gmail.com
and lateral ventricle narrowing (Figure 1a). MR myelography suggested CSF leakage at C1–3 levels (Figure 1b). After admission, prednisolone was increased from 20 to 30 mg/day, combined with cyclosporine 100 mg/day and mizoribine 150 mg/day following one course of methylprednisolone pulse therapy (500 mg/day) for three consecutive days. Immunoabsorption was performed weekly. These therapies attenuated serological SLE indicators. Although her headache and cognitive dysfunction improved, she transferred to another hospital’s neurosurgical department to determine the requirement of autologous blood injection into the epidural space in October 2012. Her CSF pressure decreased to 10 mm H2O, fulfilling the criteria for intracranial hypotension. However, CSF leakage was not confirmed using CT myelography or radioisotope cisternography. Then, she did not undergo autologous blood injection into the epidural space. CSF leakage possibly stopped before she was transferred. CSF leakage was not confirmed using MR myelography in December 2012 (Figure 1c), and MRI in July 2013 revealed improvements of subdural hygroma and lateral ventricle narrowing (Figure 1d). SLE patients manifest various central nervous system abnormalities. Intracranial hypertension has been reported to be found in 17% CNS lupus cases with intractable headaches [2]. In contrast, only one SIH case with SLE has been reported [3]. Intracranial hypertension in SLE was believed to occur because of an arachnoid occlusion by granulation or immune complex deposition in the choroid plexus [4]. In addition, hypertrophic pachymeningitis, which shows dural thickening and neurological symptoms, should be considered to be a differential diagnosis in intractable headache in SLE [5]. However, in this case, low CSF pressure and subdural hygoma were incompatible with hypertrophic pachymeningitis. Furthermore, the possibility of coincidence of SIH due to CSF leakage could not be denied in this case, but clinical symptoms such as headache, gait disturbance, and cognitive dysfunction and imaging results improved in correlation with SLE serological disease activity, suggesting an
2
D. Ando et al.
Mod Rheumatol, 2014; Early Online: 1–2
Mod Rheumatol Downloaded from informahealthcare.com by University of Otago on 12/30/14 For personal use only.
Figure 1. (a) Axial and sagittal views of gadolinium-enhanced T1-weighted MRI revealed dural thickening, subdural hygroma (arrow heads), and lateral ventricle narrowing. (b) MR myelography suggested CSF leakage at the C1–3 levels (arrow head). (c) CSF leakage was not confirmed by MR myelography. (d) Note that dural thickening, subdural hygroma (arrow heads), and lateral ventricle narrowing were ameliorated in axial and sagittal views of gadolinium-enhanced T1-weighted MRI.
etiological relationship between SLE and SIH. The mechanism of SIH development remains unclear, but SLE-related intracranial inflammation may weaken and perforate dura mater at the time of SLE exacerbation, and strict medical management may eliminate such perforating lesions.
Conflict of interest None.
References 1. Schievink WI. Spontaneous spinal cerebrospinal fluid leaks and intracranial hypotension. JAMA. 2006;295(19):2286–96.
2. Dave S, Longmuir R, Shah VA, Wall M, Lee AG. Intracranial hypertension in systemic lupus erythematosus. Semin Ophthalmol. 2008;23(2):127–33. 3. Arai H, Yamamoto Y, Maeda Y, Aga F, Dobashi H, Nishiyama Y. SPET/ CT imaging in radionuclide cisternography to detect cerebrospinal fluid leakage in spontaneous intracranial hypotension associated with SLE. Eur J Nucl Med Mol Imaging. 2012;39(7):1225–6. 4. Green L, Vinker S, Amital H, Amir T, Bar-Dayan Y, Levi Y, Schoenfeld Y. Pseudotumor cerebri in systemic lupus erythematosus. Semin Arthritis Rheum. 1995;25(2):103–8. 5. Ochi S, Nanki T, Komano Y, Suzuki F, Ogawa J, Sugihara T, et al. A case report of hypertrophic pachymeningitis associated with systemic lupus erythematosus, showing a headache and hearing loss resembling intracranial hypotension. Nihon Rinsho Meneki Gakkai Kaishi. 2007;30(1):55–60.
LETTER
Spontaneous intracranial hypotension in a case of systemic lupus erythematosus Daisuke Ando1, Izaya Nakaya1, Shuji Watanabe1, Hiroyuki Osawa1, Naohiko Kubo2, and Jun Soma1 1Department of Nephrology, Iwate Prefectural Central Hospital, Morioka, Iwate, Japan, and 2Department of Neurosurgery, Morioka Red Cross Hospital,
Mod Rheumatol Downloaded from informahealthcare.com by University of Otago on 12/30/14 For personal use only.
Morioka, Iwate, Japan
Keywords Cerebrospinal fluid leakage, Plasma adsorption, Spontaneous intracranial hypotension, Systemic lupus erythematosus History Received 26 November 2013 Accepted 12 May 2014 Published online 18 June 2014
To the Editor, Spontaneous intracranial hypotension (SIH) is typically characterized by traction headaches. The most common pathological mechanism is cerebrospinal fluid (CSF) leakage after lumbar puncture, spine/spinal cord trauma, or an unidentified cause [1]. We present a case of SIH with systemic lupus erythematosus (SLE), where SIH symptoms synchronized with SLE activity. A 32-year-old woman was admitted to a hospital with gait disturbance and cognitive dysfunction in September 2012. In October 2004, she was diagnosed with SLE because of facial erythema, photosensitivity, oral ulcers, positive serum antinuclear antibody, elevated anti-double-stranded DNA antibody, polyarthritis, and leukocytopenia. Her symptoms receded after prednisolone treatment. However, proteinuria and hematuria first appeared in March 2009 after she became pregnant. Artificial abortion and renal biopsy were performed in February 2009. The histological diagnosis revealed lupus nephritis Class III ⫹ V (WHO classification). She was treated with prednisolone, immunosuppressants, and immunoabsorption; however, she presented with orthostatic headache and nausea in October 2011, and SLE exacerbation, such as increase of proteinuria, positive serum antinuclear antibody, elevation of anti-double-stranded DNA antibody, and leukocytopenia, was found to be associated with her poor treatment adherence. Magnetic resonance imaging (MRI) revealed dural thickening and subdural hygroma, suggesting SIH. However, she had no previous history of lumbar puncture or trauma, and MR myelography did not reveal the location of CSF leakage. After 1 year of follow-up, gait disturbance and cognitive dysfunction appeared in August 2012. MRI re-examination revealed worsening subdural hygroma
Correspondence to: Daisuke Ando, Department of Nephrology, Iwate Prefectural Central Hospital, Ueda 1-4-1, Morioka, Iwate 020-0066, Japan. Tel: ⫹ 81-19-653-1151. Fax: ⫹ 81-19-653-8919. E-mail: daisukeandojp@ gmail.com
and lateral ventricle narrowing (Figure 1a). MR myelography suggested CSF leakage at C1–3 levels (Figure 1b). After admission, prednisolone was increased from 20 to 30 mg/day, combined with cyclosporine 100 mg/day and mizoribine 150 mg/day following one course of methylprednisolone pulse therapy (500 mg/day) for three consecutive days. Immunoabsorption was performed weekly. These therapies attenuated serological SLE indicators. Although her headache and cognitive dysfunction improved, she transferred to another hospital’s neurosurgical department to determine the requirement of autologous blood injection into the epidural space in October 2012. Her CSF pressure decreased to 10 mm H2O, fulfilling the criteria for intracranial hypotension. However, CSF leakage was not confirmed using CT myelography or radioisotope cisternography. Then, she did not undergo autologous blood injection into the epidural space. CSF leakage possibly stopped before she was transferred. CSF leakage was not confirmed using MR myelography in December 2012 (Figure 1c), and MRI in July 2013 revealed improvements of subdural hygroma and lateral ventricle narrowing (Figure 1d). SLE patients manifest various central nervous system abnormalities. Intracranial hypertension has been reported to be found in 17% CNS lupus cases with intractable headaches [2]. In contrast, only one SIH case with SLE has been reported [3]. Intracranial hypertension in SLE was believed to occur because of an arachnoid occlusion by granulation or immune complex deposition in the choroid plexus [4]. In addition, hypertrophic pachymeningitis, which shows dural thickening and neurological symptoms, should be considered to be a differential diagnosis in intractable headache in SLE [5]. However, in this case, low CSF pressure and subdural hygoma were incompatible with hypertrophic pachymeningitis. Furthermore, the possibility of coincidence of SIH due to CSF leakage could not be denied in this case, but clinical symptoms such as headache, gait disturbance, and cognitive dysfunction and imaging results improved in correlation with SLE serological disease activity, suggesting an
2
D. Ando et al.
Mod Rheumatol, 2014; Early Online: 1–2
Mod Rheumatol Downloaded from informahealthcare.com by University of Otago on 12/30/14 For personal use only.
Figure 1. (a) Axial and sagittal views of gadolinium-enhanced T1-weighted MRI revealed dural thickening, subdural hygroma (arrow heads), and lateral ventricle narrowing. (b) MR myelography suggested CSF leakage at the C1–3 levels (arrow head). (c) CSF leakage was not confirmed by MR myelography. (d) Note that dural thickening, subdural hygroma (arrow heads), and lateral ventricle narrowing were ameliorated in axial and sagittal views of gadolinium-enhanced T1-weighted MRI.
etiological relationship between SLE and SIH. The mechanism of SIH development remains unclear, but SLE-related intracranial inflammation may weaken and perforate dura mater at the time of SLE exacerbation, and strict medical management may eliminate such perforating lesions.
Conflict of interest None.
References 1. Schievink WI. Spontaneous spinal cerebrospinal fluid leaks and intracranial hypotension. JAMA. 2006;295(19):2286–96.
2. Dave S, Longmuir R, Shah VA, Wall M, Lee AG. Intracranial hypertension in systemic lupus erythematosus. Semin Ophthalmol. 2008;23(2):127–33. 3. Arai H, Yamamoto Y, Maeda Y, Aga F, Dobashi H, Nishiyama Y. SPET/ CT imaging in radionuclide cisternography to detect cerebrospinal fluid leakage in spontaneous intracranial hypotension associated with SLE. Eur J Nucl Med Mol Imaging. 2012;39(7):1225–6. 4. Green L, Vinker S, Amital H, Amir T, Bar-Dayan Y, Levi Y, Schoenfeld Y. Pseudotumor cerebri in systemic lupus erythematosus. Semin Arthritis Rheum. 1995;25(2):103–8. 5. Ochi S, Nanki T, Komano Y, Suzuki F, Ogawa J, Sugihara T, et al. A case report of hypertrophic pachymeningitis associated with systemic lupus erythematosus, showing a headache and hearing loss resembling intracranial hypotension. Nihon Rinsho Meneki Gakkai Kaishi. 2007;30(1):55–60.
