• Users Online: 221
  • Print this page
  • Email this page


 
 
Table of Contents
CASE REPORT
Year : 2019  |  Volume : 2  |  Issue : 2  |  Page : 135-136

Paraneoplastic Miller-Fisher syndrome associated with splenic lymphoma


Department of Neurology, Ramón Y Cajal University Hospital, Madrid, Spain

Date of Submission25-Apr-2019
Date of Acceptance03-Nov-2019
Date of Web Publication10-Jan-2020

Correspondence Address:
Dr. Victoria Ros-Castello
Ctra. De Colmenar Viejo, Km. 9100, 28034 Madrid
Spain
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/IJNO.IJNO_6_19

Rights and Permissions
  Abstract 


Paraneoplastic neurologic syndromes (PNSs) are infrequently associated with lymphoma. Manifestations of lymphoma-associated PNS include both central and peripheral nervous systems. We report the case of a patient with Miller-Fisher syndrome (MFS) and a final diagnosis of splenic lymphoma whose neurological symptoms improved only after surgery and chemotherapy. Our report outlines the importance of suspecting a paraneoplastic etiology in patients with MFS and no response to conventional therapies.

Keywords: Lymphoma, Miller-Fisher syndrome, paraneoplastic neurologic syndrome


How to cite this article:
Ros-Castello V, Sanchez-Sanchez A, Gomez-Lopez A, Natera-Villalba E, Garcia-Barragan N, Buisan-Catevilla J, Corral-Corral I. Paraneoplastic Miller-Fisher syndrome associated with splenic lymphoma. Int J Neurooncol 2019;2:135-6

How to cite this URL:
Ros-Castello V, Sanchez-Sanchez A, Gomez-Lopez A, Natera-Villalba E, Garcia-Barragan N, Buisan-Catevilla J, Corral-Corral I. Paraneoplastic Miller-Fisher syndrome associated with splenic lymphoma. Int J Neurooncol [serial online] 2019 [cited 2020 May 26];2:135-6. Available from: http://www.Internationaljneurooncology.com/text.asp?2019/2/2/135/275538




  Introduction Top


Paraneoplastic neurologic syndromes (PNSs) are infrequently associated with lymphoma. Manifestations of lymphoma-associated PNS include both central and peripheral nervous systems and have been associated with several antibodies.[1] Miller-Fisher syndrome (MFS) is an autoimmune disease characterized by a triad of ophthalmoplegia, ataxia, and areflexia.[2]


  Case Report Top


We report the case of a patient with MFS and a final diagnosis of splenic lymphoma whose neurological symptoms improved only after surgery and chemotherapy.

A 75-year-old woman was admitted to our hospital due to progressive onset of diplopia, dysarthria, dysphagia, unsteady gait, and paresthesias. She referred weight loss, asthenia, and anorexia for the past 6 months. Neurologic examination revealed bilateral limited upgaze, incomplete abduction of the right eye, bilateral peripheral facial palsy, severe dysarthria, dysphagia, bilateral hypoglossal paresis, lower limb somatosensory defects (proprioception, vibration sense, discriminative touch and pain, and temperature sense), areflexia, and ataxic gait.

The neurophysiological study revealed sensory axonal polyneuropathy, more severe in lower limbs, and facial motor mononeuropathy with absent H-reflex. Blood and cerebrospinal fluid (CSF) analyses were normal. CSF cytology was negative for malignant cells. Antiganglioside and onconeural antibodies were negative. Cranial magnetic resonance imaging (MRI) was normal. No response to a course of immunoglobulin (Ig, 0.4 g/kg/day for 5 days) was achieved.

A computed tomography (CT) body was performed and revealed a splenic lesion. To characterize the lesion, the study was completed with a positron emission tomography (PET)-CT that showed a splenic mass with increased uptake of 18-FDG (maximum standard uptake value of 23.94) [Figure 1]. The patient underwent surgery (splenectomy), and the histopathological findings were consistent with a diffuse large B-cell lymphoma with CD20, CD79a, and CD10 positivity.
Figure 1: Coronal positron emission tomography-computed tomography image showing a splenic mass with increased uptake of 18-FDG (maximum standard uptake value of 23.94) consistent with malignancy

Click here to view


After the surgery, she received four cycles of chemotherapy with rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone. Shortly after starting chemotherapy, neurological symptoms progressively improved. A PET-CT performed 6 months after the treatment showed no evidence of recurrence. Nine months after surgery, the neurological examination was normal. After 1 year follow-up, the patient was free of illness.


  Discussion Top


To the best of our knowledge, only four cases of paraneoplastic MFS associated with lymphoma have been previously reported.[3],[4],[5],[6] This patient presented neurological syndrome with clinical and neurophysiological characteristics of MFS, with additional involvement of lower cranial nerves. Antiganglioside antibodies are not detected in all patients with MFS.[2] In the previous reported four cases, anti-GQ1b IgG antibodies were positive only in one patient.[5] Although antiganglioside antibodies were negative in the present case, leptomeningeal involvement and peripheral nerve malignant infiltration were ruled out by CSF analysis, MRI studies, and PET-CT studies. In addition, the absence of response to Ig therapy and the improvement of neurological symptoms after surgery and chemotherapy suggest a paraneoplastic etiology for MFS.

Our report outlines the importance of suspecting a paraneoplastic etiology in patients with MFS and no response to conventional therapies. In these patients, specific treatment of the underlying malignancy is essential to improve the neurological symptoms.

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.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Briani C, Vitaliani R, Grisold W, Honnorat J, Graus F, Antoine JC, et al. Spectrum of paraneoplastic disease associated with lymphoma. Neurology 2011;76:705-10.  Back to cited text no. 1
    
2.
Wakerley BR, Uncini A, Yuki N; GBS Classification Group, GBS Classification Group. Guillain-Barré and Miller Fisher syndromes – New diagnostic classification. Nat Rev Neurol 2014;10:537-44.  Back to cited text no. 2
    
3.
Gentile S, Messina M, Rainero I, Lo Giudice R, De Martino P, Pinessi L, et al. Miller fisher syndrome associated with Burkitt's lymphoma. Eur J Neurol 2006;13:430.  Back to cited text no. 3
    
4.
Usmani N, Bhatia R, Ikpatt OF, Sharma KR. Diffuse large B-cell lymphoma presenting as Miller Fisher syndrome. Muscle Nerve 2012;45:138-43.  Back to cited text no. 4
    
5.
Rubio-Nazabal E, Marey-Lopez J, Torres-Carrete JP, Alvarez-Perez P, Rey Del Corral P. Miller-Fisher syndrome and Hodgkin's disease. J Neurol Neurosurg Psychiatry 2002;73:344.  Back to cited text no. 5
    
6.
Özdemir ZC, Kar YD, Yarar C, Þaylýsoy S, Bör Ö. Incomplete Miller-Fisher syndrome with advanced stage Burkitt lymphoma. Indian Pediatr 2017;54:413-5.  Back to cited text no. 6
    


    Figures

  [Figure 1]



 

Top
 
  Search
 
    Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
    Access Statistics
    Email Alert *
    Add to My List *
* Registration required (free)  

 
  In this article
Abstract
Introduction
Case Report
Discussion
References
Article Figures

 Article Access Statistics
    Viewed299    
    Printed20    
    Emailed0    
    PDF Downloaded54    
    Comments [Add]    

Recommend this journal