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|LETTERS TO EDITOR
|Year : 2018 | Volume
| Issue : 5 | Page : 1481-1482
Idiopathic retroperitoneal fibrosis presenting as spastic paraparesis
Gopal Poduval1, Priya Nathani2
1 Department of Neurology, Global Hospitals, Hyderabad, Telangana, India
2 Department of Radiodiagnosis, Global Hospitals, Hyderabad, Telangana, India
|Date of Web Publication||17-Sep-2018|
Dr. Gopal Poduval
Department of Neurology, Global Hospitals, Lakdikapul, Hyderabad, Telangana
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Poduval G, Nathani P. Idiopathic retroperitoneal fibrosis presenting as spastic paraparesis. Neurol India 2018;66:1481-2
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. 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. 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. Two earlier case reports of spinal cord compression by retroperitoneal fibrosis , 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.
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[Figure 1], [Figure 2]