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CASE REPORT
Year : 2013  |  Volume : 7  |  Issue : 2  |  Page : 80-82

Recurrent transient ischemic attacks due to vascular steal syndrome secondary to the Paget's disease of bone: A rare presentation


1 Department of General Medicine, Aarupadai Veedu Medical College and Hospital, Kirumampakkam, Puducherry, India
2 Department of Radiology, Aarupadai Veedu Medical College and Hospital, Kirumampakkam, Puducherry, India

Date of Web Publication23-May-2014

Correspondence Address:
Anand Pai
Department of General Medicine, Aarupadai Veedu Medical College and Hospital, Kirumampakkam, Puducherry
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0331-3131.133102

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   Abstract 

Paget's disease of bone (osteitis deformans) is a condition of unknown etiology, characterized by rapid bone remodeling and formation of new bone that is structurally abnormal. A wide range of clinical manifestations are reported among patients with Paget's disease including bone pain, fractures, secondary osteoarthritis, hearing loss, and syndrome of neurological compression secondary to vascular steal phenomenon. Here we report a rare case of recurrent transient ischemic attacks due to vascular steal syndrome secondary to Paget's disease of bone.

Keywords: Paget′s disease, transient ischemic attacks, vascular steal syndrome


How to cite this article:
Pai A, Balakumar K, Shankar S, Umadevi V, Manash B, Narayanasamy S. Recurrent transient ischemic attacks due to vascular steal syndrome secondary to the Paget's disease of bone: A rare presentation. Ann Nigerian Med 2013;7:80-2

How to cite this URL:
Pai A, Balakumar K, Shankar S, Umadevi V, Manash B, Narayanasamy S. Recurrent transient ischemic attacks due to vascular steal syndrome secondary to the Paget's disease of bone: A rare presentation. Ann Nigerian Med [serial online] 2013 [cited 2021 May 6];7:80-2. Available from: https://www.anmjournal.com/text.asp?2013/7/2/80/133102


   Introduction Top


Paget's disease of bone (osteitis deformans) is a condition of unknown etiology, characterized by rapid bone remodeling and formation of new bone that is structurally abnormal. A wide range of clinical manifestations are reported among patients with Paget's disease including bone pain, fractures, secondary osteoarthritis, hearing loss, and syndrome of neurological compression secondary to vascular steal phenomenon.


   Case Report Top


A 51-year-old female patient admitted to the hospital with history of sudden onset of giddiness followed by right hemiparesis and double vision. There were h/o recurrent similar episodes for past 1 month with transient hemiparesis, diplopia, and giddiness reported on 10 occasions, which recovered spontaneously within few minutes to hours. Patient was not on any chronic medications. Patient was conscious, oriented, BP: 140/100 mmHg, pulse rate: 70/min, regular rhythm, carotid pulse palpable, and no bruit. Neurological examination revealed right hemiparesis and right sided sensorineural deafness. Patient recovered spontaneously after an hour without any neurological deficit except for deafness. Biochemical parameters such as blood routine, blood sugar levels, serum electrolytes, renal, and liver function tests were normal except for increased alkaline phosphatase −2185 units/L. Urinary hydroxyproline test was positive. Serum calcium and phosphorus was normal. Thyroid function test and parathyroid hormone levels were normal. Peripheral smear and bone marrow aspiration were normal.

In posteroanterior view chest X-ray, multiple lytic lesions were noted on both the scapula involving the acromion process, coracoid process glenoid process, body of scapula, both distal third of the clavicle, and ribs bilaterally. X-ray pelvis anterior-posterior view including hip joint showed multiple lytic lesions in the medullary cavity and cortex of bilateral ilium, ischium, head, and neck of the femur with mild expansion of the bones [Figure 1]. Nonenhanced computerized tomography of the brain showed expansion of most of the calvarial bones with obliteration of the medullary cavity of diploid space due to sclerosis and multiple lytic lesions noted in bilateral occipital bones, petrous and squamous temporal bones; and bilateral parietal bones [Figure 2].
Figure 1: X-ray pelvis anterior-posterior view including hip joint shows multiple lytic lesions in the medullary cavity and cortex of bilateral ilium, ischium, head, and neck of the femur with mild expansion of the bones

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Figure 2: Nonenhanced computerized tomography of the brain shows multiple lytic lesions noted in bilateral occipital bones, petrous and squamous temporal bones and bilateral parietal bones

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   Discussion Top


Paget's disease is a chronic skeletal disorder causing enlarged and deformed bones; characterized by abnormal bone turnover, structure, architecture, skeletal remodeling due to increased osteoclastic activity, and bone resorption followed by excessive bone formation resulting in abnormal highly vascularized bone that is structurally disorganized. [1],[2] Paget's disease commonly involves skull, humerus, spine, and pelvic bone. Patient may present with bone pain and pathological fractures. Neurological complications result from expansion of diseased bone causing compression of brainstem, cerebellum, and lower cranial nerves; hydrocephalus, myelopathy, cauda equina syndrome, radiculopathy of the spine most commonly resulting from hypertrophy of the spine with direct compression or secondary to vascular steal syndrome. [3],[4] Circulatory "steal" condition is one in which blood is shunted to diseased bone thereby leaving neighboring parts of the body deprived of its blood supply. This has been postulated in cognitive decline and similar reports of neurological dysfunction progressing to paraparesis secondary to steal phenomenon. [5]


   Conclusion Top


A transient ischemic attack is classically defined as an episode of focal cerebral dysfunction with symptoms lasting for <24 h and leaving no residual damage after the attack. Most ischemic cerebral disease results from narrowing or occlusion of cerebral vessels. With regard to our present case, another cause of transient ischemic attack (i.e.,) subclavian steal syndrome deserves further attention. Development of highly vascular skull lesions and peripheral bone lesions lead to subsequent decrease in blood flow to the carotid artery, low flow hypothesis further supported the characteristic attacks (i.e.,) short lived and repetitive. In addition, all transient ischemic attacks disappeared after treatment with subcutaneous salmon calcitonin injection, bisphosphonate-pamidronate for the follow-up period of 6 months. [6],[7] On continuation of calcitonin treatment, there was a decrease in levels of serum alkaline phosphatase and it can also be concluded that calcitonin treatment reduces the abnormal high metabolic activity of diseased bone and its vascular perfusion, thus allowing more blood to the brain.

 
   References Top

1.Poncelet A. The neurologic complications of Paget's disease. J Bone Miner Res 1999;14 Suppl 2:88-91.  Back to cited text no. 1
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2.Gumà M, Rotés D, Holgado S, Monfort J, Olivé A, Carbonell J, et al. Paget's disease of bone: Study of 314 patients. Med Clin (Barc) 2002;119:537-40.  Back to cited text no. 2
    
3.Sadar ES, Walton RJ, Gossman HH. Neurological dysfunction in Paget's disease of the vertebral column. J Neurosurg 1972;37:661-5.  Back to cited text no. 3
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4.Wang WC, Cheng YS, Chen CH, Lin YJ, Chen YK, Lin LM. Paget's disease of bone in a Chinese patient: A case report and review of the literature. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2005;99:727-33.  Back to cited text no. 4
    
5.Yost JH, Spencer-Green G, Krant JD. Vascular steal mimicking compression myelopathy in Paget's disease of bone: Rapid reversal with calcitonin and systemic steroids. J Rheumatol 1993;20:1064-5.  Back to cited text no. 5
    
6.Chen JR, Rhee RS, Wallach S, Avramides A, Flores A. Neurologic disturbances in Paget disease of bone: Response to calcitonin. Neurology 1979;29:448-57.  Back to cited text no. 6
    
7.Ziegler DK, Hassanein RS. Prognosis in patients with transient ischemic attacks. Stroke 1973;4:666-73.  Back to cited text no. 7
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    Figures

  [Figure 1], [Figure 2]



 

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