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|Year : 2019 | Volume
| Issue : 4 | Page : 1087-1089
Longitudinally Extensive Transverse Myelitis with Aquaporin-4 Antibody Positivity in Renal Cell Carcinoma: Rare Occurrence
Rohan R Mahale, Anish Mehta, Kiran Buddaraju, Mahendra Javali, Abhinandan K Shankar, Aju Abhraham John, Rangasetty Srinivasa
Department of Neurology, MS Ramaiah Medical College and Hospital, Bangalore, Karnataka, India
|Date of Web Publication||10-Sep-2019|
Dr. Rohan R Mahale
Department of Neurology, MS Ramaiah Medical College and Hospital, Bangalore - 560 054, Karnataka
Source of Support: None, Conflict of Interest: None
The occurrence of longitudinally extensive transverse myelitis (LETM) in an elderly patient should evoke search for underlying systemic malignancy. Intramedullary spinal cord metastases and paraneoplastic myelopathy are the most common etiology for LETM in patients with systemic malignancy. The occurrence of LETM in association with renal cell carcinoma with aquaporin-4 (AQP4) antibody positivity has not been reported. We report an elderly woman who presented with acute paraplegia and was diagnosed as having LETM with AQP4 positivity and renal cell carcinoma.
Keywords: Antibody, aquaporin-4, renal cell carcinoma, transverse myelitis
Key Message: Serum aquaporin 4 antibodies positivity can occur in paraneoplastic long segment myelitis.
|How to cite this article:|
Mahale RR, Mehta A, Buddaraju K, Javali M, Shankar AK, John AA, Srinivasa R. Longitudinally Extensive Transverse Myelitis with Aquaporin-4 Antibody Positivity in Renal Cell Carcinoma: Rare Occurrence. Neurol India 2019;67:1087-9
|How to cite this URL:|
Mahale RR, Mehta A, Buddaraju K, Javali M, Shankar AK, John AA, Srinivasa R. Longitudinally Extensive Transverse Myelitis with Aquaporin-4 Antibody Positivity in Renal Cell Carcinoma: Rare Occurrence. Neurol India [serial online] 2019 [cited 2020 Jun 5];67:1087-9. Available from: http://www.neurologyindia.com/text.asp?2019/67/4/1087/266293
The etiologies of paraplegia in patients with systemic malignancy include myelitis as a part of paraneoplastic syndrome and spinal cord metastasis. Myelitis is a rare paraneoplastic manifestation of malignancy. It is commonly seen with lung carcinoma and lymphoproliferative malignancies. Other less frequent malignancies include sarcoma; carcinoma of the breast, prostate, skin, stomach, esophagus, thyroid, and ovary; renal cell carcinoma; and hepatocellular carcinoma. The cord inflammatory changes span three or more vertebral segments vertically in longitudinally extensive transverse myelitis (LETM). LETM is frequently reported with neuromyelitis optica (NMO). Other less frequent causes include tuberculosis, systemic lupus erythematosus, and lung cancer. The occurrence of LETM in association with renal cell carcinoma with aquaporin-4 (AQP4) antibody positivity has not been reported. We report an elderly woman who presented with acute paraplegia and was diagnosed as having LETM with AQP4 positivity and renal cell carcinoma.
| » Case Report|| |
A 67-year-old woman presented with a history of acute-onset weakness of both lower limbs of a three-day duration. Weakness was noticed in the morning after sleep when she was unable to move her lower limbs. This weakness was associated with retention of urine, which was painless. There was loss of sensation to all modalities below epigastric level. There was no radicular pain in the limbs, preceding fever, diarrhea, trauma, or back pain. She was diabetic and was on oral hypoglycemics with fair glycemic control. Her pulse rate was 88 bpm, and blood pressure was 126/84 mmHg. Her higher mental functions and speech were normal. Cranial nerves, including fundus examination, were normal. Motor examination showed hypotonia of both lower limbs, with power of 0/5 (Medical Research Council grading). Deep tendon reflexes were not elicitable in lower limbs. Plantar responses were mute. There was complete loss of sensation below T6 (epigastric) level. Complete hemogram, renal, hepatic and thyroid functions were normal. Urine examination showed presence of red blood cells. Serum electrolytes were normal. Whole-spine magnetic resonance imaging (MRI) showed a longitudinal hyperintense signal on T2-weighted image involving the central spinal cord from D1 to D10 vertebral levels with contrast enhancement [Figure 1]. Incidentally, there was a mass lesion in the right kidney on coronal MRI images. Computed tomography (CT) of the abdomen showed a mass lesion in the anterior region of the right kidney [Figure 2]. CT-guided biopsy was suggestive of renal cell carcinoma. Cerebrospinal fluid examination protein of 84 mg/dl, glucose of 76 mg/dl and no pleocytosis. There were no malignant cells. Serum AQP4 antibody titer was positive. Serum antinuclear antibody was negative. Serological testing for human immunodeficiency virus and venereal disease research laboratory was non-reactive. She was treated with intravenous methylprednisolone for 5 days, with no improvement. Five cycles of plasma exchange was given, with mild improvement. She was referred to an urologist for further management of her renal cell carcinoma.
|Figure 1: Sagittal T2-weighted (a) and (b) and postcontrast MR images (c) of the spine showing hyperintense signal in spinal cord from D1 to D10 vertebral level (a and b) with abnormal contrast enhancement. (c) Axial T2-weighted images (d-f) showing central cord hyperintensities (red arrow)|
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|Figure 2: Coronal postcontrast MR images (a and b) showing a mass lesion in the upper pole of the right kidney; axial (c and d) and coronal (e and f) CT images of the abdomen showing a mass lesion in the upper pole of the right kidney (red arrow)|
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| » Discussion|| |
The most common cause of LETM is an inflammatory demyelinating disease in the form of NMO. Spinal intramedullary neoplasm can present with long-segment spinal lesions. Intramedullary ependymoma, astrocytoma, and hemangioblastoma are the common spinal neoplasm presenting as long-segment spinal lesions. Intramedullary spinal cord metastases (ISCM) can produce long-segment spinal lesions and is observed in approximately 0.1%–0.4% of patients with malignancy. It is a relatively rare sequelae of systemic malignancy and carries a worse prognosis. Breast and lung carcinoma are the most frequent source of ISCM. Other less frequent source include, malignant melanoma, renal cell carcinoma, colon carcinoma, etc. Usually, patients with ISCM have a known primary cancer along with cerebral and systemic metastases. Cervical, thoracic, and lumbar spinal segments are equally affected. MRI shows isointense lesion on T1-weighted imaging, hyperintense lesion on T2-weighted imaging, with extensive surrounding edema and ring or homogenous contrast enhancement. Paraneoplastic myelopathy can present with long-segment myelitis. They are very rare and can occur before a malignancy is detected. Patients present with progressive myelopathy over weeks to months. Tumoral antigens, also called as onconeural antigens, are considered foreign by the immune system. Autoantibodies produced against onconeural antigens cause cytotoxic T-cell destruction of neurons or glia. Paraneoplastic myelopathy can present as massive cord necrosis or multifocal cord necrosis involving the white matter. The hallmark MRI finding is longitudinally extensive, symmetrical, tract-specific signal changes within the spinal cord with symmetric contrast enhancement. Patchy contrast enhancement has also been seen in necrotizing paraneoplastic myelopathy. There are numerous neural antibodies associated with paraneoplastic myelopathy. Anti-amphiphysin and collapsin response-mediator protein-5 antibodies are the most common neural antibodies associated with paraneoplastic myelopathy.
There are reports of systemic malignancy associated with paraneoplastic neuromyelitis optica spectrum disorder (NMOSD) in the literature. Invasive thymoma, stomach carcinoid, prostrate adenocarcinoma, and lung carcinoma  are the reported systemic malignancy is associated with paraneoplastic NMOSD. Our patient was an elderly woman who presented with acute-onset paraplegia. On evaluation, she had LETM as a cause for her paraplegia. Incidentally, there was a mass lesion in the upper pole of the right kidney, which was confirmed to be a renal cell carcinoma on histopathological study. Possibilities of ISCM, paraneoplastic myelopathy, or demyelinating disease like NMO were considered. ISCM was ruled out because there were no systemic metastases. Serum AQP4 antibodies were positive, which is more commonly associated with NMO and NMO spectrum disorders. AQP4 antibodies have been reported to be associated with paraneoplastic myelopathy. The occurrence of demyelinating disease in elderly patients is rare. In view of the associated renal malignancy, paraneoplastic myelopathy with AQP4 antibody positivity as a cause for LETM was considered in our patient.
| » Conclusion|| |
A thorough search for underlying systemic malignancy should be carried out in elderly patients presenting with LETM. Serum AQP4 antibodies can be positive in paraneoplastic myelopathy. The occurrence of LETM in association with renal cell carcinoma with aquaporin 4 (AQP4) antibody positivity is rare.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Authors thank the Department of Urology, MS Ramaiah Medical College and Hospital, for aiding in the management of patient.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2]