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Table of Contents    
Year : 2018  |  Volume : 66  |  Issue : 5  |  Page : 1481-1482

Idiopathic retroperitoneal fibrosis presenting as spastic paraparesis

1 Department of Neurology, Global Hospitals, Hyderabad, Telangana, India
2 Department of Radiodiagnosis, Global Hospitals, Hyderabad, Telangana, India

Date of Web Publication17-Sep-2018

Correspondence Address:
Dr. Gopal Poduval
Department of Neurology, Global Hospitals, Lakdikapul, Hyderabad, Telangana
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0028-3886.241348

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How to cite this article:
Poduval G, Nathani P. Idiopathic retroperitoneal fibrosis presenting as spastic paraparesis. Neurol India 2018;66:1481-2

How to cite this URL:
Poduval G, Nathani P. Idiopathic retroperitoneal fibrosis presenting as spastic paraparesis. Neurol India [serial online] 2018 [cited 2019 Feb 16];66:1481-2. Available from:


Idiopathic retroperitoneal fibrosis (IRF) includes a spectrum of disorders where a fibro- inflammatory lesion surrounds the abdominal aorta and extends further into the retro- peritoneum to involve the ureters. Thus, the most common symptoms are of vascular origin and/or related to the renal outflow obstruction.[1] We report here a case of IRF whose initial presentation was of thoracic myelopathy.

A 54-year old male patient, non-diabetic, non-hypertensive, and non-smoker, presented with the complaints of progressive difficulty in walking of 2-month duration. The weakness was associated with decreased sensation below the waist level, stiffness of both legs, flexor spasms, and mild edema in both feet. He denied any history of upper limb weakness, leg claudication, bladder symptoms, back or abdominal pain, fever, weight loss, fatigue, or of any drug intake. On examination, he was conscious and oriented, his higher mental functions, speech, and cranial nerves were normal. Motor and sensory examination and reflexes in both upper limbs were normal. His both lower limbs were spastic with grade 3/5 power, the knee and ankle jerks were exaggerated with ankle clonus and the plantar response was bilateral extensor. A sensory level to pin prick was obtained just above the umbilicus. He had bilateral pitting pedal edema. The peripheral pulses were normally felt. The patient underwent magnetic resonance imaging (MRI) of the dorsal spine which showed an enhancing prevertebral mass extending from the eleventh thoracic to the first lumbar vertebrae [Figure 1]. The spinal cord and the exiting roots appeared normal [Figure 2]. His blood was negative for antinuclear antibodies and his other hematological and metabolic parameters were within normal limits. Histopathology of a laparoscopic biopsy of the lesion revealed a fibro-inflammatory infiltrate consistent with retroperitoneal fibrosis. The patient was managed with corticosteroids and tamoxifen and he showed significant improvement over the next 2 months. Over the next 1 week, he developed a large bilateral pleural effusion, which was successfully drained. Two days later, he expired due to sudden cardiac arrest.
Figure 1: Magnetic resonance imaging, T2 -. weighted sagittal image showing a normal spinal cord

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Figure 2: Magnetic resonance imaging spine, sagittal view, showing a prevertebral mass in the thoracolumbar region

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The case highlights a rare presentation of IRF as spastic paraparesis and the notable absence of any urinary or abdominal symptoms despite the lesion having reached such a large size.[2] The spinal cord appeared intrinsically normal, though a diffusion weighted magnetic resonance image would have possibly shown the spinal cord infarction. Previous case reports of spinal cord infarction are mostly related to aortic aneurysms, vascular occlusion of anterior spinal artery, and epidural hematoma.[3] Two earlier case reports of spinal cord compression by retroperitoneal fibrosis [4],[5] have shown direct compression of the cord following epidural invasion by the mass. In this case, the mass was confined to the prevertebral region and would have compromised the vascular supply of the spinal cord. Hence, it is important to consider spinal cord ischemia in all cases with an abdominal mass and paraplegia where no compression of the spinal cord has been detectable on imaging.

  References Top

Vaglio A, Maritati F. Idiopathic retroperitoneal fibrosis. J Am Soc Nephrol 2016;27:1880-9.  Back to cited text no. 1
Kermani TA, Crowson CS, Achenbach SJ, Luthra HS. Idiopathic retroperitoneal fibrosis: A retrospective review of clinical presentation, treatment, and outcomes. Mayo Clin Proc 2011;86:297-303.  Back to cited text no. 2
Cheshire WP, Santos CC, Massey EW, Howard JF Jr. Spinal cord infarction: Etiology and outcome. Neurology 1996;47:321-30.  Back to cited text no. 3
de Sá J, Pimentel J, Carvalho M, Evangelista P, Martins P. Spinal cord compression secondary to idiopathic retroperitoneal fibrosis. Neurosurgery 1990;26:678-81.  Back to cited text no. 4
Dorado-Bouix L, Millán-Torné M, Capellades-Font J, Gomà-Gállego M, Olivé A. Spinal cord compression: An unusual clinical manifestation of retroperitoneal fibrosis. Rev Neurol 2004;39:1143-5.  Back to cited text no. 5


  [Figure 1], [Figure 2]


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