|Year : 2014 | Volume
| Issue : 1 | Page : 54-56
Deafness and blindness: A rare presentation of meningeal carcinomatosis
Navneet Kumar1, Chanchal Gera2, Alok Gupta3
1 Department of Ear, Nose and Throat, Christian Medical College, Ludhiana, India
2 Department of Medicine, Christian Medical College, Ludhiana, India
3 Department of Consultant Pathologist, Chandigarh, India
|Date of Web Publication||11-Feb-2014|
60 A, Rishi Nagar, Ludhiana 141 008, Punjab
Source of Support: None, Conflict of Interest: None
A 60-year-old woman presented with progressive loss of vision and deafness with recurrent simple partial seizures. This patient was treated for carcinoma breast 13 years before this presentation. Magnetic resonance imaging brain and magnetic resonance angiography was normal. Cerebrospinal fluid analysis was inconclusive. Meningeal biopsy revealed metastatic deposits and malignant cells were positive for estrogen, progesterone, and HER2nu receptor. This case has some unique features in the form of involvement of optic and vestibulocochlear nerves together, primary site of malignancy was hidden, and diagnosis was made with meningeal biopsy.
Keywords: Blindness, breast carcinoma, cranial nerve palsies, deafness, meningeal carcinomatosis
|How to cite this article:|
Kumar N, Gera C, Gupta A. Deafness and blindness: A rare presentation of meningeal carcinomatosis. CHRISMED J Health Res 2014;1:54-6
|How to cite this URL:|
Kumar N, Gera C, Gupta A. Deafness and blindness: A rare presentation of meningeal carcinomatosis. CHRISMED J Health Res [serial online] 2014 [cited 2017 May 25];1:54-6. Available from: http://www.cjhr.org/text.asp?2014/1/1/54/126798
| Introduction|| |
Meningeal carcinomatosis is characterized by diffuse or multifocal infiltration of leptomeninges by metastatic cells. This complication was first reported by Eberth in 1870. It is associated with myriad of presentations, which often make diagnosis difficult. Underlying malignancies are usually adenocarcinoma of the breast, lung or malignant melanoma.  Most commonly involved cranial nerves are occulomotor, abducens, and trigeminal nerves in solid tumors and facial nerve in leukemic meningitis.  There are only a few reports of deafness or blindness as presenting complaint of meningeal carcinomatosis. Our patient suffered both blindness and deafness and we could not find this type of presentation in literature.
| Case Report|| |
A 60-year-old woman presented to a tertiary care hospital with history of involuntary movements of right leg for 14 months. She suffered multiple episodes per day lasting for 1-2 min. After 1 month of onset of involuntary movements, she developed loss of vision in left eye which slowly progressed to right eye. She was investigated elsewhere and was prescribed steroids with oxcarbazepine. Her vision did not show any improvement but it did not deteriorate either. She took steroids for 4 months and stopped as advised. One month after stopping steroids, she developed progressive hearing loss in both the ears. She was hypertensive and taking amlodipine for last 15 years. Patient also had history of right-sided carcinoma breast in 1994 which was treated with mastectomy and six cycles of chemotherapy but exact details were not available. Patient was in regular follow-up with her oncologist till 2 years back.
On admission, she was conscious, oriented with normal higher mental functions. Visual acuity was restricted to finger counting at less than half meter. Bilateral pupils were normal in size and reacting to light. Fundus examination showed bilateral optic atrophy. Pure tone audiometry showed profound hearing loss on right side and moderate sensorineural hearing loss on left side.
Speech discrimination scores were very low. A healthy scar of mastectomy was present on right side of chest and there was no swelling or induration. Rest of the physical examination was unremarkable. Our presumptive diagnosis was temporal arteritis, but temporal artery biopsy was already done outside and did not show any evidence of vasculitis.
Investigations showed hemoglobin of 14.2 gm/dL, total leukocyte count of 13,300/μL, platelet count of 540000/μL, and erythrocyte sedimentation rate of 90. Liver and renal function tests were essentially normal. ANA, anti-double stranded deoxyribonucleic acid, complement, human immunodeficiency virus, HBsAg, and antihepatitis C virus were negative. Her chest radiograph, electrocardiogram, and echocardiography all were normal. CA15.3 was mildly elevated 49.7 U/mL (0-25 U/mL). Nerve conduction study of all four limbs was normal. Electroencephalography revealed background activity of 8-9 Hz with frequent sharp wave discharges (epileptiform abnormalities) arising from left temporal region. Magnetic resonance imaging (MRI) brain showed confluent hyperintense signals on T2/T2 fluid attenuated inversion recovery images surrounding the both lateral ventricles, most prominent in the posterior peritrigonal region (age-related changes). Cerebrospinal fluid (CSF) revealed red blood cells 50/mm 3 , white blood cells 25/mm 3 with 100% lymphocytes, elevated protein (580 mg/dL), normal sugar (208 mg/dL). CSF gram staining was negative and culture did not show any growth. Tuberculosis polymerase chain reaction and oligoclonal band were negative.
CSF cytology revealed scattered cells which showed moderate amount of cytoplasm with fraying of margins and irregular hyperchromatic nuclei with inconspicuous nucleoli (suggestive of histiocytes or neoplastic cells). Meningial biopsy [Figure 1] showed the meningeal surface layered with malignant cells having pleomorphic nuclei, prominent nucleoli, and abundant cytoplasm (arrow). The tumor cells were strongly positive for estrogen, (inset of Figure), progesterone, and HER2nu. Last, when computed tomography chest and abdomen was performed to see metastasis to other organs it showed mild irregular septal thickening suggestive lymphangitis carcinomatosis with skeletal metastasis with left pleural effusion.
|Figure 1: The meningeal surface layered with malignant cells having pleomorphic nuclei, prominent nucleoli, and abundant cytoplasm (arrow). (H and E, ×40). Inset the tumor cells are strongly positive for estrogen receptor staining (Immuno histocompatibility complex, ×10)|
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After final diagnosis of leptomeningeal carcinomatosis, patient wanted to follow-up with her previous oncologist and she was referred there.
| Discussion|| |
Incidence of meningeal carcinomatosis is approximately 5% of patients with cancer,  but it is increasing recently because of prolonged survival of carcinoma patients. Our patient was treated for carcinoma breast many years ago and at this presentation, clinically there was no evidence of tumor in breast. Meningeal carcinomatosis presents in numerous ways, for example, cranial nerve palsies, cerebral signs, headache, spinal nerves involvement, mental changes, limb weakness, ataxia, meningism, sensory abnormalities, nausea-vomiting, cerebellar signs, seizures, dizziness, and autonomic dysfunction.  Metastatic cells can infiltrate cerebrum, meninges, cranial nerves, or spinal cord and produce clinical features accordingly.
Deafness due to meningeal carcinomatosis was first described by Saenger in 1900; Since then less than 20 cases have been published. 
Blindness in these patients is thought to be due to meningeal tumor cuffing in the subarachnoid space around the optic nerves and rarely because of direct infiltration of the nerve by tumor cells.  Meningeal carcinomatosis is not a common differential in a patient who presents with deafness and blindness.
MRI of our patient showed only age-related changes and there was no evidence of metastasis. MRI has a sensitivity of about 70% which may demonstrate thickening of nerve roots, subependymal or pachymeningeal enhancement, or multiple enhancing nodular deposits.  A normal MRI does not exclude meningeal carcinomatosis. Our patient's CSF examination gave some clue in the form of rare scattered cells with moderate amount of cytoplasm with fraying of margins and irregular hyperchromatic nuclei. CSF usually represents inflammatory meningitis, consisting of lymphocytic pleocytosis elevated protein level and normal or low glucose. CSF cytology results are positive in 50% of cases after a single lumbar tap and in 85%-90% of cases after multiple taps.  In present case, diagnosis could be established only after meningeal biopsy.
There are no standard treatment guidelines available for this complication. Intravenous administration of conventional doses of several drugs cannot adequately penetrate into CSF; therefore, high doses of systemically administered methotrexate, cytarabine, or thiotepa are required to yield therapeutic concentration in spinal fluid. Intrathecal chemotherapy can be administered either by lumbar puncture or intraventricularly through an Ommaya implanted subcutaneous reservoir. 
To conclude, meningeal carcinomatosis can present with any combination of cranial nerve paralysis. One should consider leptomeningeal biopsy and histopathological examination if cause of chronic meningitis is not clear.
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