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LETTER TO EDITOR
Year : 2016  |  Volume : 64  |  Issue : 4  |  Page : 805-807

Pathologically proven peripheral neurolymphomatosis


1 Department of Neurology, Navy General Hospital, Beijing, China
2 Department of Neurology, The Affiliated Hospital of Weifang Medical University, Weifang, China

Date of Web Publication5-Jul-2016

Correspondence Address:
Xiaokun Qi
Department of Neurology, Navy General Hospital, Beijing
China
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0028-3886.185354

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How to cite this article:
Yu Y, Ren M, Qi X. Pathologically proven peripheral neurolymphomatosis. Neurol India 2016;64:805-7

How to cite this URL:
Yu Y, Ren M, Qi X. Pathologically proven peripheral neurolymphomatosis. Neurol India [serial online] 2016 [cited 2019 Aug 25];64:805-7. Available from: http://www.neurologyindia.com/text.asp?2016/64/4/805/185354


Sir,

The term “neurolymphomatosis” (NL) refers to lymphomatous infiltration of peripheral nerves, cranial nerves, nerve roots, and plexus.[1] NL may occur in the peripheral nervous system initially or as a part of systemic lymphoma.[1] Non-Hodgkin B-cell lymphoma is the most common entity.[2] We report one case of diffuse large B-cell non-Hodgkin lymphoma with lymphomatous infiltration of the peripheral nerves, cranial nerves, nerve roots, and plexus, which was confirmed by using nerve biopsy.

A 43-year-old right-handed man with significant history of traumatic brain injury was admitted to our hospital on January 15, 2015 due to 8 years of progressive numbness and pain and 1 month of weakness in both lower extremities. Due to the initial diagnosis of lumbar disc protrusion at a local hospital, he received mecobalamin injection and intravenous hydrocortisone but without any improvement. A lumbar puncture revealed that white cell count was 900/mm 3, level of protein was 3 g/L, glucose was 1.11 mmol/L, and chloride was 101 mmol/L in the cerebrospinal fluid (CSF). The patient was transferred to our hospital. Occasional mild fever at about 37.5°C was reported from the onset of the disease with dysuria for the recent 2 weeks. The neurologic examination demonstrated mild memory and attention impairment. His bilateral lower extremities had Grade II muscle strength and the muscle volume was reduced. His tendon reflexes were weak. Neck stiffness and Kernig's sign was present. His cremasteric reflex was not elicited. The nerve conduction study revealed decreased compound motor action potential amplitudes and reduced sensory conduction velocity in bilateral median nerves. F-wave and H-reflex were not elicited. The brain and spinal cord magnetic resonance imaging (MRI) are shown in [Figure 1] and [Figure 2]. Positron emission tomography-computed tomography (PET-CT) of the whole body showed no evidence of systemic lymphoma. PET-CT of the brain and spinal are shown in [Figure 3]. Biopsy of the coccygeal nerves was performed, and the pathological study is shown in [Figure 4]. NL of the diffuse large B-cell non-Hodgkin lymphoma type was considered. His symptoms were relieved after treatment with methotrexate for 7 months, and the repeat brain and spinal cord MRI at that time showed resolution of the previous abnormal signals.
Figure 1: Cranial nerve enlargement and enhancement were identified on axial brain magnetic resonance imaging in the patient with neurolymphomatosis. Medullary level magnetic resonance imaging showed that the glossopharyngeal nerve was enlarged [white arrow] (a) with enhancement [white arrow] (e). Pontine level magnetic resonance imaging showed that the trigeminal nerve was enlarged [white arrow] (b) with enhancement [white arrow] (f). Brian magnetic resonance imaging at mesencephalon level showed oculomotor nerve was enlarged [white arrow] (c) and was enhancing [white arrow] (g). Tentorium of the cerebellum was enhanced (g). Magnetic resonance imaging at the mesencephalon level showed that the optic nerve was enlarged [white arrow] (d) and was enhancing [white arrow] (h)

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Figure 2: Spinal nerve roots wereenlarged and enhancing on magnetic resonance imaging in the patient with neurolymphomatosis. Sagittal thoracic spinal cord images showed multiple nerve root enlargement [white arrow] (a) and enhancement [white arrow] (b). Axial thoracic spinal cord magnetic resonance imaging of the segments showed nerve root enlargement [white arrow] (c) and enhancement [white arrow] (d). Sagittal lumbar spinal cord magnetic resonance imaging showed multiple nerve root enlargement [white arrow] (e) and enhancement [white arrow] (f). Axial lumbar spinal cord magnetic resonance imaging showed nerve root nerve enlargement [white arrow] (g) and enhancement [white arrow] (h). Sagittal thoracic spinal cord magnetic resonance imaging showed spinal enhancement of pia mater (b and f)

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Figure 3: Positron emission tomography-computed tomography showed significantly elevated glucose metabolism in the tentorium cerebelli and trigeminal nerves [white arrow] (a). Coronal and sagittal spinal cord images on positron emission tomography-computed tomography showed significantly increased levels of glucose metabolism in the area of T12-L5 spinal cord and coccygeal nerves [white arrow] (c and d). Axial spinal cord on positron emission tomography-computed tomography showed elevated glucose metabolism in the area of spinal cord [white arrow] (b)

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Figure 4: Diffuse allotypic lymphocytes infiltrating the nerve fibers were demonstrated with hematoxylin and eosin staining of coccygeal nerves after their biopsy (a). Sparse positive CD3 was found in coccygeal nerves [white arrow] (b); positive CD20 was detected in coccygeal nerves (c); Ki67 index was 80% [white arrow] (d)

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In this patient, a cause for the diffuse development of NL such as a viral infection and hypoimmunity could not be identified. NL involving of oculomotor, trigeminal, vestibulocochlear and glossopharyngeal nerves, lumbosacral plexus, and coccygeal nerves simultaneously without presenting with manifestations of systemic lymphoma, as was seen in this case, is extremely rare. All previous reports have been summarized in [Table 1].[1] Clinical presentations of NL include painful infiltration of nerves or roots, cranial neuropathy, or painless involvement of a single peripheral nerve. Painful neuropathy developed in 76% of NL patients.[1] The differential diagnosis of NL patients includes inflammatory and drug-induced neuropathy, tumor compression, and paraneoplastic syndrome.[3] Electromyography and elevated protein in CSF are nonspecific investigations for determining the presence of NL.[4] Brain MRI is a very useful tool for its diagnosis. PET/CT positivity rate is as high as 87.5–91% for NL.[5] Systemic chemotherapy alone or in combination with radiotherapy is critical to address the multiple sites of involvement.
Table 1: Review of previously published cases of neurolymphomatosis with cranial nerves involvement

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Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
  References Top

1.
Grisariu S, Avni B, Batchelor TT, van den Bent MJ, Bokstein F, Schiff D, et al. Neurolymphomatosis: An International Primary CNS Lymphoma Collaborative Group report. Blood 2010;115:5005-11.  Back to cited text no. 1
    
2.
Liu Z, Jiang T, Hou N, Jia Y. Peripheral neurolymphomatosis with tracheal asphyxia: A case report and literature review. BMC Neurol 2015;15:149.  Back to cited text no. 2
    
3.
Hughes RA, Britton T, Richards M. Effects of lymphoma on the peripheral nervous system. J R Soc Med 1994;87:526-30.  Back to cited text no. 3
    
4.
Ramirez-Zamora A, Morales-Vidal S, Chawla J, Biller J. Autopsy proven peripheral nervous system neurolymphomatosis despite negative bilateral sural nerve biopsy. Front Neurol 2013;4:197.  Back to cited text no. 4
    
5.
Talanow R, Shrikanthan S. Value of FDG PET in the evaluation of therapy response in nerve root neurolymphomatosis. Clin Nucl Med 2011;36:389-91.  Back to cited text no. 5
    


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