Paraneoplastic Miller-Fisher syndrome associated with splenic lymphoma

Victoria Ros-Castello; Arantxa Sanchez-Sanchez; Ana Gomez-Lopez; Elena Natera-Villalba; Nuria Garcia-Barragan; Javier Buisan-Catevilla; Iñigo Corral-Corral

doi:10.4103/IJNO.IJNO_6_19

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CASE REPORT

Year : 2019 | Volume : 2 | Issue : 2 | Page : 135-136

Paraneoplastic Miller-Fisher syndrome associated with splenic lymphoma

Victoria Ros-Castello, Arantxa Sanchez-Sanchez, Ana Gomez-Lopez, Elena Natera-Villalba, Nuria Garcia-Barragan, Javier Buisan-Catevilla, Iñigo Corral-Corral
Department of Neurology, Ramón Y Cajal University Hospital, Madrid, Spain

Date of Submission	25-Apr-2019
Date of Acceptance	03-Nov-2019
Date of Web Publication	10-Jan-2020

Correspondence Address:
Dr. Victoria Ros-Castello
Ctra. De Colmenar Viejo, Km. 9100, 28034 Madrid
Spain

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/IJNO.IJNO_6_19

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 2022 Jun 27];2:135-6. Available from: https://www.Internationaljneurooncology.com/text.asp?2019/2/2/135/275538

Introduction

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

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

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.

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