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Website: www.neurologyindia.com PMID: *** DOI: 10.4103/0028-3886.149411
Received: 28-11-2014 Review completed: 02-12-2014 Accepted: 05-12-2014
Systemic lupus erythematosus and subarachnoid hemorrhage Sir, Systemic lupus erythematosus (SLE) is an autoimmune disorder characterized by exacerbation and remission periods, triggered in genetically susceptible subjects by a set of hormonal and environmental factors. It is an inflammatory disorder that prominently affects the kidneys; although, any organ systems, including the skin, joints, or central nervous system, can be involved. We report a patient of SLE with subarachnoid hemorrhage (SAH). A 43‑year‑old woman with no history of diabetes mellitus, hyperlipidemia, headaches, and smoking admitted to emergency department for sudden onset of severe headache and pre‑syncope. She has been under follow‑up for SLE since 10 years and on low‑dose steroids. Physical examination revealed normal vital signs. Neurologic examination revealed patient in somnolent state with mild nucal rigidity and no fical deficit. Cranial computed tomography (CT) revealed SAH in quadrigeminal system. Magnetic resonance angiography and four vessel angiography studies were normal. Laboratory work‑up was normal except for elevated urea (61,8 mg/dL), and creatinine (2,48 mg/dL) levels. A year before this admission, she was detected to have high blood pressure was secondary to renal involvement of SLE for which she was started on amlodipine and cyclophosphamide. However, drug compliance was poor and she had missed her routine follow‑up visits. The working diagnosis was SAH probably due hypertension due to SLE kidney. Her general condition and neurological findings progressively got better, and control CT showed a total resorption. The reported frequency of cerebral vascular events during the course of the disease in patients with SLE varied between 3% and 15%, a frequency higher than that in the general population, especially in young women. [1] The reported rates of SAH in patients with SLE varied from 1% to 3.9%.[2] The pathogenic mechanisms underlying SAH in SLE remain poorly Neurology India | Nov-Dec 2014 | Vol 62 | Issue 6
understood. In SLE patients, SAH may be related to the primary disease, complications related to SLE, and treatment. Histopathologic studies in patients with SLE and SAH showed inflammatory response in the cerebral arterial and arteriolar walls, features that can go with vasculitis.[3] These patients were thought to have high disease activity. However, there have also been reports of SAH in patients with little or no disease activity. In these cases with low disease activity, other possible contributors to SAH include hemodynamic changes, coagulopathy, angiopathy due to premature atherosclerosis, and anticoagulant therapy in addition to other recognized factors.[2,4,5] In a series of 57 patients with SLE and SAH, 24 (42%) patients had variable degree of renal involvement.[6] In the study by Baizabal et al., nine patients had hypertension on medication at the time of SAH.[2] The importance of the kidneys in the long‑term control of blood pressure and the pathogenesis of hypertension is well‑documented.[7] Therefore, it is not surprising that impaired renal function is a primary culprit in the progression of SLE hypertension. Our patient suggests that early diagnosis and treatment of SLE exacerbations, as well as monitorization of severity of renal involvement, hypertension, and thrombotic and hemorrhagic events are critical for the optimal management of SLE patients. Otherwise, the sequels and death due to complications will bring about irreversible consequences and costs.
Hasan Hüseyin Kozak, Muazzez Betigül Yürüten Çorbacıoğlu, Süleyman Ömer Anlıaçık Department of Neurology, Meram Faculty of Medicine, Necmettin Erbakan University, Meram, Konya, Turkey E-mail: [email protected]
References 1.
Jennekenns FG, Kater L. The central nervous system in systemic lupus erythematosus. Part 2. Pathogenetic mechanisms of clinical syndromes: A literature investigation. Rheumatology 2002;41:619‑30. 2. Baizabal Carvallo JF, Cantú Brito C, Estañol B, García Ramos GS. Subarachnoid hemorrhage as a complication of systemic lupus erythematosus. Cerebrovasc Dis 2007;24:301‑4. 3. Kawamata T, Kagawa M, Kubo O, Takeshita M, Ujiie H, Sato K, et al. Clinicopathological studies of three cases of cerebral aneurysms associated with systemic lupus erythematosus. No Shinkei Geka 1991;19:633‑9. 4. Oshiro S, Motomura K, Fukushima T. Systemic lupus erythematosus manifesting as subarachnoid hemorrhage induced by cortical venous thrombosis and followed by medial medullary infarction. No To Shinkei 2003;55:791‑5. 5. van Gijn J, Rinkel GJ. Subarachnoid haemorrhage: Diagnosis, causes and management. Brain 2001;124:249‑78. 6. Owada T, Takahashi K, Kita Y. Subarachnoid hemorrhage in systemic lupus erythematosus in Japan: Two case reports and a review of the literature. Mod Rheumatol 2009;19:573‑80. 689
Letters to Editor
7. Guyton AC, Manning RD Jr, Hall JE, Norman RA Jr, Young DB, Pan YJ, et al. The pathogenic role of the kidney. J Cardiovasc Pharmacol 1984;6(Suppl 1):151‑61. Access this article online Quick Response Code:
infarct [Figure 4]. After 2 weeks, patient shows marked improvement with steroids, supportive measures, and active physiotherapy. At the time of discharge, his hearing had improved, his abdominal distension was relieved and
Website: www.neurologyindia.com PMID: *** DOI: 10.4103/0028-3886.149412
Received: 02-12-2014 Review completed: 02-12-2014 Accepted: 05-12-2014
First case report of caissons disease with pneumobilia Sir, A 35‑year‑old male went for deep sea diving using self‑contained underwater breathing apparatus (SCUBA) diving apparatus for collecting sea shells. At the end of two‑and‑half hours, he was rapidly pulled up to surface. He immediately developed severe back pain and neck pain, followed by severe myalgia. Within few minutes, he also had giddiness, abdominal pain, and vomiting. On arrival, he complained of bilateral hearing loss and weakness of both lower limbs. On examination, patient was irritable and agitated. Vitals at admission were: Pulse 120/min regular, blood pressure 100/70 mm Hg, respiratory rate 30/min, and oxygen saturation 80%. On neurologic examination, he had hypotonia of all the limbs, motor power 4/5 in upper limbs and 3/5 lower limbs which further worsened to 0/5, areflexia, and plantar responses were mute. Fundus showed few petechial hemorrhages. Abdomen examination showed guarding and right hypochondriac tenderness with distended bladder. The clinical diagnosis was acute decompression sickness (DCS). Patient was intubated and given injection methyl prednisolone for 3 days. On day 5, he was extubated and started on nasogastric feeds. Blood biochemistry and complete blood picture were normal except for mild elevation of alkaline phosphatase (ALP) of 189 IU/L. Chest X‑ray revealed bilateral hilar shadows [Figure 1]. X‑ray abdomen erect was normal but ultrasonogram showed pneumobilia [Figure 2]. Magnetic resonance imaging (MRI) of brain showed T2 and fluid attenuated inversion recovery (FLAIR) hyperintensities in periventricular and parieto occipital grey matter [Figure 3]. MRI spine showed hyperintense signal involving central portion of spinal cord extending from C2 to C5, possibility of spinal cord 690
Figure 1: CXR shows bilateral perihilar shadows
Figure 2: USG abdomen shows pneumobilia
Figure 3: MRI brain shows FLAIR hyperintensities in periventricular and parieto occipital grey matter
Neurology India | Nov-Dec 2014 | Vol 62 | Issue 6
Copyright of Neurology India is the property of Medknow Publications & Media Pvt. Ltd. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.
Access this article online Quick Response Code:
Website: www.neurologyindia.com PMID: *** DOI: 10.4103/0028-3886.149411
Received: 28-11-2014 Review completed: 02-12-2014 Accepted: 05-12-2014
Systemic lupus erythematosus and subarachnoid hemorrhage Sir, Systemic lupus erythematosus (SLE) is an autoimmune disorder characterized by exacerbation and remission periods, triggered in genetically susceptible subjects by a set of hormonal and environmental factors. It is an inflammatory disorder that prominently affects the kidneys; although, any organ systems, including the skin, joints, or central nervous system, can be involved. We report a patient of SLE with subarachnoid hemorrhage (SAH). A 43‑year‑old woman with no history of diabetes mellitus, hyperlipidemia, headaches, and smoking admitted to emergency department for sudden onset of severe headache and pre‑syncope. She has been under follow‑up for SLE since 10 years and on low‑dose steroids. Physical examination revealed normal vital signs. Neurologic examination revealed patient in somnolent state with mild nucal rigidity and no fical deficit. Cranial computed tomography (CT) revealed SAH in quadrigeminal system. Magnetic resonance angiography and four vessel angiography studies were normal. Laboratory work‑up was normal except for elevated urea (61,8 mg/dL), and creatinine (2,48 mg/dL) levels. A year before this admission, she was detected to have high blood pressure was secondary to renal involvement of SLE for which she was started on amlodipine and cyclophosphamide. However, drug compliance was poor and she had missed her routine follow‑up visits. The working diagnosis was SAH probably due hypertension due to SLE kidney. Her general condition and neurological findings progressively got better, and control CT showed a total resorption. The reported frequency of cerebral vascular events during the course of the disease in patients with SLE varied between 3% and 15%, a frequency higher than that in the general population, especially in young women. [1] The reported rates of SAH in patients with SLE varied from 1% to 3.9%.[2] The pathogenic mechanisms underlying SAH in SLE remain poorly Neurology India | Nov-Dec 2014 | Vol 62 | Issue 6
understood. In SLE patients, SAH may be related to the primary disease, complications related to SLE, and treatment. Histopathologic studies in patients with SLE and SAH showed inflammatory response in the cerebral arterial and arteriolar walls, features that can go with vasculitis.[3] These patients were thought to have high disease activity. However, there have also been reports of SAH in patients with little or no disease activity. In these cases with low disease activity, other possible contributors to SAH include hemodynamic changes, coagulopathy, angiopathy due to premature atherosclerosis, and anticoagulant therapy in addition to other recognized factors.[2,4,5] In a series of 57 patients with SLE and SAH, 24 (42%) patients had variable degree of renal involvement.[6] In the study by Baizabal et al., nine patients had hypertension on medication at the time of SAH.[2] The importance of the kidneys in the long‑term control of blood pressure and the pathogenesis of hypertension is well‑documented.[7] Therefore, it is not surprising that impaired renal function is a primary culprit in the progression of SLE hypertension. Our patient suggests that early diagnosis and treatment of SLE exacerbations, as well as monitorization of severity of renal involvement, hypertension, and thrombotic and hemorrhagic events are critical for the optimal management of SLE patients. Otherwise, the sequels and death due to complications will bring about irreversible consequences and costs.
Hasan Hüseyin Kozak, Muazzez Betigül Yürüten Çorbacıoğlu, Süleyman Ömer Anlıaçık Department of Neurology, Meram Faculty of Medicine, Necmettin Erbakan University, Meram, Konya, Turkey E-mail: [email protected]
References 1.
Jennekenns FG, Kater L. The central nervous system in systemic lupus erythematosus. Part 2. Pathogenetic mechanisms of clinical syndromes: A literature investigation. Rheumatology 2002;41:619‑30. 2. Baizabal Carvallo JF, Cantú Brito C, Estañol B, García Ramos GS. Subarachnoid hemorrhage as a complication of systemic lupus erythematosus. Cerebrovasc Dis 2007;24:301‑4. 3. Kawamata T, Kagawa M, Kubo O, Takeshita M, Ujiie H, Sato K, et al. Clinicopathological studies of three cases of cerebral aneurysms associated with systemic lupus erythematosus. No Shinkei Geka 1991;19:633‑9. 4. Oshiro S, Motomura K, Fukushima T. Systemic lupus erythematosus manifesting as subarachnoid hemorrhage induced by cortical venous thrombosis and followed by medial medullary infarction. No To Shinkei 2003;55:791‑5. 5. van Gijn J, Rinkel GJ. Subarachnoid haemorrhage: Diagnosis, causes and management. Brain 2001;124:249‑78. 6. Owada T, Takahashi K, Kita Y. Subarachnoid hemorrhage in systemic lupus erythematosus in Japan: Two case reports and a review of the literature. Mod Rheumatol 2009;19:573‑80. 689
Letters to Editor
7. Guyton AC, Manning RD Jr, Hall JE, Norman RA Jr, Young DB, Pan YJ, et al. The pathogenic role of the kidney. J Cardiovasc Pharmacol 1984;6(Suppl 1):151‑61. Access this article online Quick Response Code:
infarct [Figure 4]. After 2 weeks, patient shows marked improvement with steroids, supportive measures, and active physiotherapy. At the time of discharge, his hearing had improved, his abdominal distension was relieved and
Website: www.neurologyindia.com PMID: *** DOI: 10.4103/0028-3886.149412
Received: 02-12-2014 Review completed: 02-12-2014 Accepted: 05-12-2014
First case report of caissons disease with pneumobilia Sir, A 35‑year‑old male went for deep sea diving using self‑contained underwater breathing apparatus (SCUBA) diving apparatus for collecting sea shells. At the end of two‑and‑half hours, he was rapidly pulled up to surface. He immediately developed severe back pain and neck pain, followed by severe myalgia. Within few minutes, he also had giddiness, abdominal pain, and vomiting. On arrival, he complained of bilateral hearing loss and weakness of both lower limbs. On examination, patient was irritable and agitated. Vitals at admission were: Pulse 120/min regular, blood pressure 100/70 mm Hg, respiratory rate 30/min, and oxygen saturation 80%. On neurologic examination, he had hypotonia of all the limbs, motor power 4/5 in upper limbs and 3/5 lower limbs which further worsened to 0/5, areflexia, and plantar responses were mute. Fundus showed few petechial hemorrhages. Abdomen examination showed guarding and right hypochondriac tenderness with distended bladder. The clinical diagnosis was acute decompression sickness (DCS). Patient was intubated and given injection methyl prednisolone for 3 days. On day 5, he was extubated and started on nasogastric feeds. Blood biochemistry and complete blood picture were normal except for mild elevation of alkaline phosphatase (ALP) of 189 IU/L. Chest X‑ray revealed bilateral hilar shadows [Figure 1]. X‑ray abdomen erect was normal but ultrasonogram showed pneumobilia [Figure 2]. Magnetic resonance imaging (MRI) of brain showed T2 and fluid attenuated inversion recovery (FLAIR) hyperintensities in periventricular and parieto occipital grey matter [Figure 3]. MRI spine showed hyperintense signal involving central portion of spinal cord extending from C2 to C5, possibility of spinal cord 690
Figure 1: CXR shows bilateral perihilar shadows
Figure 2: USG abdomen shows pneumobilia
Figure 3: MRI brain shows FLAIR hyperintensities in periventricular and parieto occipital grey matter
Neurology India | Nov-Dec 2014 | Vol 62 | Issue 6
Copyright of Neurology India is the property of Medknow Publications & Media Pvt. Ltd. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.
