Annals of Indian Academy of Neurology
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Year : 2006  |  Volume : 9  |  Issue : 2  |  Page : 116-118

Neuropathy similar to Miller Fisher syndrome associated with primary Sjogren's syndrome: Response to intravenous immunoglobulins

1 Departments of Neurology, Selcuk University, Meram Medical Faculty, Turkey
2 Departments of Pathology, Selcuk University, Meram Medical Faculty, Turkey

Correspondence Address:
Emine Genc
Selcuk University, Meram Medical Faculty, Department of Neurology, Konya - 42080
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0972-2327.25985

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There is a diverse clinical spectrum of peripheral nervous system involvement in Sjogren's syndrome (SS). Most of the peripheral neuropathies in SS are typically sensory. In this report, we present a case who showed signs similar to those of the Miller Fisher variant of Guillain Barre syndrome, associated with SS. The patient demonstrated a rapid improvement both clinically and electrophysiologically, after high dose intravenous immunoglobulin therapy.

Keywords: Guillain Barre syndrome, acute demyelination, connective tissue disease

How to cite this article:
Genc E, Genc BO, Avunduk MC, Kozak HH, Ilhan N. Neuropathy similar to Miller Fisher syndrome associated with primary Sjogren's syndrome: Response to intravenous immunoglobulins. Ann Indian Acad Neurol 2006;9:116-8

How to cite this URL:
Genc E, Genc BO, Avunduk MC, Kozak HH, Ilhan N. Neuropathy similar to Miller Fisher syndrome associated with primary Sjogren's syndrome: Response to intravenous immunoglobulins. Ann Indian Acad Neurol [serial online] 2006 [cited 2022 Dec 2];9:116-8. Available from:

  Introduction Top

A distinctive connection between sensory neuropathy and connective tissue disease is the association of sensory neuropathy with SS.[1],[2],[3] Estimates of the incidence of the peripheral nervous system involvement in patients with SS range from less than 10 to 50%.[1] Among the diverse spectrum of peripheral nervous system involvement in SS, distal sensorimotor and distal pure sensory patterns are the most common.[1],[4] Miller fisher syndrome (MFS) which is characterized by acute onset of ataxia, external ophthalmoplegia and areflexia, is considered a variant form of Guillain Barre syndrome (GBS).[5] GBS is linked to SS by case reports only.[6],[7]

  Case Report Top

A 48-year-old woman suffered from tingling and burning sensations of the toes and fingers one week before admission. They ascended the next day to the knees and forearms in a symmetric pattern, after which she developed double vision. The day before admission she had numbness of the four limbs, poor balance, unsteady gait, inability to use the arms instead of normal muscle strength and was unable to make any eye movements. Neurologic examination was remarkable for a total external ophthalmoplegia, with bilateral ptosis prominent on the left eye. The pupils were equal and pupillary reflexes were preserved. She had no muscular weakness. The tendon reflexes were hypoactive, with flexor plantar responses present bilaterally. She had decreased sensation to all modalities in the four limbs. The joint position and vibration sensations were decreased and Romberg's sign was positive. Her gait was ataxic and she could not walk without assistance. Analysis of CSF showed a protein level of 80 mg/dl with normal chloride and sugar levels and no cells. Electrophysiologic study showed that median, ulnar and sural nerve sensory action potentials were bilaterally absent or attenuated [Table - 1]. Motor nerve conduction studies were bilaterally normal. A needle EMG study demonstrated no abnormality of the muscles. The patient was at first diagnosed with MFS. Further questioning revealed that she complained of itching eyes and a dry mouth which had bothered her for several years. Schirmer's test was positive (right 1 mm/ 5 min, left 1 mm / 5 min). A minor salivary gland biopsy showed sialadenitis with mononuclear inflammatory infiltrates, interstitial fibrosis and acinar atrophy of the gland [Figure - 1]. Her right sural nerve biopsy revealed moderate diffuse loss of myelinated fibers and no evidence of arteriolar vasculitis. Anti Ro (SS-A) was positive at a titer of 4.3 U/ml and anti La (SS-B) at 2.6 U/ml. Routine hematological and blood chemical tests were normal. Erythrocyte sedimentation rate was 30 mm/h, CRP and rheumatoid factor were negative, antinuclear antibodies and extractable nuclear antigens were negative and serum immunoglobulin and complement levels were normal. Serum concentrations of folic acid, vitamin B12, vitamin E and thyroid hormones were normal. Cranial and orbital MRI were normal. The patient was diagnosed with SS according to the European community criteria.[8]

She was started with 0.4 g/kg immunoglobulin daily, for 5 days. Two weeks later, vertical movement of both eyes was at first achieved, followed by improvement of horizontal eye movements. She became less ataxic and could walk without support six weeks after admission. She had no sensory symptoms at the follow-up, 3 months later. Improvement was demonstrated on EMG at the follow-up six weeks and approximately one year later [Table - 1].

  Discussion Top

The clinical spectrum of peripheral nervous system involvement in SS is diverse, including distal sensorimotor polyneuropathy, trigeminal sensory neuropathy, mononeuropathy multiplex, pure sensory or sensory ataxic neuropathy, autonomic neuropathy and various combinations of these disorders.[8] Rare cases of SS with features similar to GBS and chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) were also reported.[6],[7] The clinical course of this case was completed in approximately one week. Clinical manifestations, together with CSF and EMG examinations, were identical to classic Miller-Fisher variant of GBS. Careful questioning of the patient later, revealed that symptoms of xerophthalmia and xerostomia preceded the diagnosis of primary SS by three years. Indeed, sicca complex was previously unrecognized in most cases and neuropathic symptoms were the main reason for seeking medical attention.[2] CSF findings showed an albuminocytologic dissociation. Unlike carcinomatous sensory neuropathy where the CSF analysis shows elevated protein and pleocytosis, CSF in sensory neuronopathy associated with SS is reported to be normal.[3] Occasionally, the spinal fluid may show normal cell count and elevated protein, the so called albuminocytologic dissociation, indistinguishable from classic GBS.[1]

Nerve ischemia from arteritis, dorsal root ganglionitis and demyelination are possible underlying mechanisms of the peripheral neuropathy seen in primary SS . The most common cause for the mild distal axonal polyneuropathy in SS, may be vasculitis. This has been supported by vasculitic changes found in sural nerve biopsies from such patients.[4] However, some nerve biopsied reports of SS neuropathy failed to reveal either vasculitis or lymphocytic infiltration, though remyelination was observed.[2] In previously reported cases of ascending motor polyneuropathy, biopsies of the patients revealed no vasculitic findings with loss of myelinated fibers, which is compatible with the pathology of CIDP.[6] Pure sensory neuropathies associated with SS are usually without evidence of vasculitis.[9]

With regard to therapy, intravenous immunoglobulins were reported to be successful in patients with SS and sensory neuropathy.[6],[10] These therapeutic findings suggest that the exact immune mechanism in sensory neuropathy patterns observed in SS may not be identical. The existence of different types of neuropathies in association with SS, are also suggestive of different underlying pathogenetic mechanisms. There is a spectrum of neuropathic patterns associated with SS and even if all are immunologically mediated, it cannot be assumed that the exact immune mechanism and the optimal treatment is identical in all.[2] SS complicated with both vasculitic and sensory neuropathies with two years interval was reported in the same patient, whose vasculitic neuropathies responded to methylprednisolone pulse therapy, whereas her sensory neuropathies responded to IVIG.[10] After laboratory and clinical data supported the diagnosis of primary SS, we concluded that the patient's neuropathies were associated with SS rather than with an MFS. The co-occurrence of both primary SS and a neuropathy mimicking MFS in our patient, might have resulted by one or more immunologically-mediated pathogenetic mechanisms. Our patient indicate that there is clinical rationale for searching for occult Sjogren's syndrome in patients who present with symptoms and signs of classic MFS and that high dose intravenous immunoglobulins can be a valuable treatment option.

  References Top

1.Rosenbaum R. Neuromuscular complications of connective tissue diseases. Muscle Nerve 2001;24:154-69.   Back to cited text no. 1  [PUBMED]  [FULLTEXT]
2.Grant IA, Hunder GG, Homburger HA, Dyck PJ. Peripheral neuropathy associated with sicca complex. Neurology 1997;48:855-62.   Back to cited text no. 2  [PUBMED]  
3.Griffin JW, Cornblath DR, Alexander E. Ataxic sensory neuropathy and dorsal root ganglionitis associated with Sjogren's syndrome. Ann Neurol 1990;27:304-15.  Back to cited text no. 3    
4.Mellgren SI, Conn DL, Stevens JC, Dyck PJ. Peripheral neuropathy in primary Sjogren's syndrome. Neurology 1989;39:390-4.  Back to cited text no. 4  [PUBMED]  
5.Fisher M. An unusual variant of acute idiopathic polyneuritis (syndrome of ophthalmoplegia, ataxia and areflexia). N Engl J Med 1956;255:57-65.  Back to cited text no. 5    
6.Mochizuki H, Kamakura K, Masaki T, Hirata A, Nakamura R, Motoyoshi K. Motor dominant neuropathy in Sjogren's syndrome: Report of two cases. Int Med 2002;41:142-6.  Back to cited text no. 6    
7.Pryszmont M, Sierakowski S, Poplawska T, Domyslawska I, Pryszmont J, Pawlak-Tumiel B. Guillain-Barre syndrome in a patient with primary sicca syndrome. Neurol Neurochir Pol 2000;34:1235-41.   Back to cited text no. 7    
8.Lafitte C. Neuroradiological manifestations of primary Sjogren's syndrome. Rev Neurol 2002;158:959-65.  Back to cited text no. 8    
9.Kaplan JG, Rosenberg R, Reinitz E, Buchbinder S, Schaumburg H. Peripheral neuropathy in Sjogren's syndrome. Muscle Nerve 1990;13:570-9.   Back to cited text no. 9    
10.Noguchi Y, Tsuchiyama T, Matsumoto T, Fujigasaki H, Inaba A, Yokota T, et al . Two distinct types of neuropathy associated with Sjogren's syndrome developed in one patient. The importance of selection of an appropriate therapeutic regimen. Rinsho Shinkeigaku 2003;43:539-43.  Back to cited text no. 10    


[Figure - 1]


[Table - 1]


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