|Year : 2015 | Volume
| Issue : 3 | Page : 130-132
Bilateral sixth cranial nerve palsy as a presenting feature of multiple myeloma: A rare presentation
Lulup Kumar Sahoo, Nihar Ranjan Biswal, Ashok Kumar Mallik, Geeta Mohanty, Kali Prasanna Swain
Department of Neurology, S.C.B. Medical College, Cuttack, Odisha, India
|Date of Web Publication||18-Sep-2015|
Lulup Kumar Sahoo
Department of Neurology, S.C.B. Medical College, Cuttack - 753 007, Odisha
Source of Support: Nil., Conflict of Interest: There are no conflicts of interest.
Sixth cranial nerve palsy is the most common cause of acquired extraocular muscle paralysis. Neoplasm, trauma, raised intracranial pressure, and vascular disease are the most common etiologies of sixth nerve dysfunction. Multiple myeloma is a chronic, progressive, and fatal neoplastic proliferation of plasma cells and can produce tumors or plasmacytomas, and bone marrow lesions. We here present a case of a 53-year-old man who was presented with a holocranial headache and double vision. On examination, patient was found to have bilateral lateral rectus palsy. The patient was diagnosed as having IgA kappa type multiple myeloma based on immunological and pathological investigation. Contrast-enhanced computed tomography brain showed an enhancing lesion in clivus and sphenoid bone. Under chemotherapy, sixth cranial nerve palsy was resolved completely. We speculated that sixth nerve palsy might be caused by compression and stretching along the path of the sixth nerve at clivus.
Keywords: B/L sixth nerve palsy, clivus, multiple myeloma
|How to cite this article:|
Sahoo LK, Biswal NR, Mallik AK, Mohanty G, Swain KP. Bilateral sixth cranial nerve palsy as a presenting feature of multiple myeloma: A rare presentation. J Appl Hematol 2015;6:130-2
|How to cite this URL:|
Sahoo LK, Biswal NR, Mallik AK, Mohanty G, Swain KP. Bilateral sixth cranial nerve palsy as a presenting feature of multiple myeloma: A rare presentation. J Appl Hematol [serial online] 2015 [cited 2020 Apr 1];6:130-2. Available from: http://www.jahjournal.org/text.asp?2015/6/3/130/165652
| introduction|| |
Multiple myeloma is a disease characterized by the proliferation of malignant plasma cells and a subsequent overabundance of monoclonal protein. Sixth cranial nerve palsy is the most common cause of acquired extraocular muscle paralysis. Neoplasm, trauma, raised intracranial pressure, and vascular disease are the most common etiologies of sixth nerve dysfunction. In multiple myeloma, abducens nerve palsy may be usually caused by its invasion to the clivus , or intracranial plasmacytoma.,,,, Multiple myeloma is a rare cause of tumors at the base of the skull and cavernous sinus, therefore, cranial nerve palsies due to intracranial plasmacytoma are rarely found in this condition. If sixth nerve palsy occurs because of compression due to plasmacytoma, it is most likely affected due to its long, winding course from pons to the lateral rectus muscle.
This case is unique because bilateral sixth nerve palsy can be the initial presenting sign of multiple myeloma, and therefore, should be considered if systemic symptoms are present such as fatigue, weakness, bone pain, or weight loss in elderly patients. There are only 3 reported cases in world and none from India.
| Case Report|| |
A 53-year-old male was presented with a holocranial headache and vomiting for 20 days with double vision for 10 days. On examination, the patient was conscious, oriented, both pupils were normal in size, and reactive to light. Extraocular muscle testing showed B/L lateral rectus palsy. Optic discs were normal, bilaterally. All the other cranial nerves were intact. There was absence of meningeal signs. Laboratory tests showed normal hemoglobin, total leukocyte count, and thrombocyte particle concentration with raised erythrocyte sedimentation rate of 110 mm/h. Blood biochemical parameters showed normal urea and creatinine. On serum immunological examination, marked elevation of IgA level at 3080.30 mg/dl (normal = 70–400 mg/dl) was observed, despite decreased IgG level at 329 mg/dl (normal = 700–1600 mg/dl) and IgM level at<4.33 mg/dl (normal = 40–230 mg/dl). On the immunoglobulin free light chain estimation, serum free kappa type was at 390 mg/L (normal = 3.3–19.4 mg/L) and serum free lambda type at 8.54 mg/L (normal = 5.71–26.3 mg/L) was observed. Free kappa to free lambda light chain ratio was 45.67 (normal = 0.26–1.65). In serum protein electrophoresis, M-Band was seen. In the urinary study, Bence-Jones protein was detected. Bone marrow aspiration examination demonstrated plasma cells occupying for 10%. As a result, the patient was diagnosed as having IgA kappa type multiple myeloma.
Noncontrast computed tomography scan brain was done and the axial view revealed lenticular extra-axial lesion of size 17 mm × 28 mm in the right frontal convexity [Figure 1]. On contrast-enhanced computed tomography (CECT) brain, on axial and sagittal view, there was an enhancing lesion in clivus and sphenoid bone extending to the sphenoid sinus [Figure 2] and [Figure 3] and multiple lytic lesions in the skull. CECT brain finding suggests that B/L sixth cranial nerve palsy was attributed to compression and stretching along the path of sixth cranial nerve palsy at the clivus.
|Figure 1: Noncontrast computed tomography brain on axial view, there is a lenticular extra-axial lesion in the right frontal convexity|
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|Figure 2: Contrast-enhanced computed tomography brain on axial view, there is an enhancing lesion in the clivus and sphenoid bone|
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|Figure 3: Contrast-enhanced computed tomography brain on sagittal view, there is an enhancing lesion in the clivus and sphenoid bone extending into sphenoid sinus causing bilateral sixth cranial nerve palsy|
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Then the patient was treated with a chemotherapy regimen of combination bortezomib, cyclophosphamide, and dexamethasone once weekly regimen for four cycles. It took 2 cycles of chemotherapy for the patient for the full recovery of sixth cranial nerve palsy.
| Discussion|| |
The sixth cranial nerve palsy has a long pathway from its nucleus at the inferior border of the pons to the innervations of the lateral rectus muscle. It is vulnerable to insult from the brain stem and skull base, through the petrous portion of the temporal bone and cavernous sinus to the superior orbital fissure and orbit. Paralysis of the sixth cranial nerve palsy is the most common type of acquired extraocular muscle paresis. The nerve travels along the base of the brain and enters the cavernous sinus, after traversing the petrous part of the temporal bone and ascending the clivus. The clivus is a shallow depression formed by the sphenoid and occipital bones and is located just posterior and slightly inferior to the sella turcica behind the dorsum sellae. It is continuous with the basilar portion of the occipital bone and supports the upper part of the pons. Because of its small size, anatomic location, and long oblique pathway, the sixth cranial nerve palsy is particularly susceptible to insult.
Consequently, in multiple myeloma, abducens nerve palsy can be caused by its invasion to the clivus , or intracranial plasmacytoma.,,,, A plasmacytoma due to multiple myeloma is a rare cause of sixth nerve palsy, but the most common ophthalmic manifestations of multiple myeloma are neuro-ophthalmic and the most common cranial nerve affected is the sixth.
This case report highlights that bilateral sixth nerve palsy can be the initial presenting sign of multiple myeloma and therefore, should be considered if systemic symptoms are present such as fatigue, weakness, bone pain, or weight loss in elderly patients. This is thefirst patient from India being reported to have bilateral sixth cranial nerve palsy due to clivus involvement in multiple myeloma.
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[Figure 1], [Figure 2], [Figure 3]