Plasmapheresis in the treatment of ataxic sensory neuropathy associated with Sjögren's syndrome

Sj?gren's syndrome (SS) is an important but poorly recognized cause of peripheral neuropathy. Several forms of peripheral nerve dysfunction occur, including trigeminal sensory neuropathy, mononeuropathy multiplex, distal sensorimotor polyneuropathy and pure sensory neuronopathy. The pathological findings vary and the definite treatment is not known. Here we present 4 cases of acute ataxic sensory polyneuropathy with SS, and the experience of treatment with plasmapheresis (PP). The 4 patients were all females; ages ranged from 30 to 58 years. All had prominent loss of kinesthetic and proprioceptive sensation. The course ranged from acute to subacute onset. Patients were treated with 5-9 sessions of PP. Two patients with initiation of treatment within 2 weeks of onset showed dramatic and sustained responses after PP, while the other 2 had no detectable effects. Our experience showed that PP should be considered in patients who present with sensory neuropathy associated with SS, and the treatment should be given as early as possible.     

Primary Sjögren's syndrome associated neuropathy

Primary Sj?gren's syndrome (PSS) mainly affects exocrine glands and is clinically characterized by keratoconjunctivitis sicca and xerostomia. Among several possible extraglandular manifestations, involvement of the peripheral nervous system may occur with reported frequencies from 10% to 60%. Peripheral nerve manifestations constitute sensory neuropathy, including sensory ganglioneuronopathy, sensorimotor, including polyradiculoneuropathy and demyelinating neuropathy, motor neuropathy, multiple mononeuropathy, trigeminal and other cranial neuropathies, autonomic neuropathy, and mixed patterns of neuropathy. Knowledge of the neurological manifestations of PSS is hampered by evolving classification criteria of PSS over the years, and by use of highly selected patient populations on the basis of a primary neurological diagnosis. Sural nerve biopsy may show vascular or perivascular inflammation of small epineurial vessels (both arterioles and venules) and in some cases necrotizing vasculitis. Loss of myelinated nerve fibers is common and loss of small diameter nerve fibers occurs. Pathology in cases of sensory ganglioneuronopathy consists of loss of neuronal cell bodies and infiltration of T cells. Peripheral neuropathy in PSS often is refractory to treatment although newer biological agents may provide more effective treatment options. Current treatment strategies used in autoimmune neuropathies may be tried depending upon characteristics of the neuropathy and results obtained by a thorough clinical and laboratory investigation.     

Antiganglion neuron antibodies correlate with neuropathy in Sjögren's syndrome

To investigate the possible implication of antibodies against dorsal root ganglion neuron in the pathogenesis of sensory neuropathy with Sj?gren's syndrome, we examined the pathogenic role of antiganglion neuron antibodies by immunoblotting, immunohistochemistry and immunoreactive assay. Sj?gren's syndrome patients without neuropathy, patients with vasculitic neuropathy and normal volunteers were evaluated as controls. Antiganglion neuron antibodies recognizing certain proteins of several different molecular weights were detected only in patients of sensory neuropathy with Sj?gren's syndrome. Those antibodies labeled specific-sized neurons in the fixed ganglion and isolated ganglion neurons under the culture condition, each of which corresponded well to clinical manifestations. These results suggest that antiganglion neuron antibodies may contribute to the pathogenesis of sensory neuropathy with Sj?gren's syndrome.     

Bilateral optic neuropathy revealing Sjögren's syndrome

IntroductionThe central nervous system involvement has been reported in 20% of cases of primary Sjogrën's syndrome (SS), a chronic autoimmune disease characterized by a disorder of the exocrine glands secondary to progressive lymphocyte infiltration. Classically described neurological manifestations include sensorimotor deficits, aseptic meningitis or meningoencephalitis, multiple sclerosis-like syndromes and myeolopathies.ObservationWe report here the case of a 53-year-old woman who exhibited rapidly progressive visual loss, disclosing bilateral optic neuropathy, as an uncommon initial symptom of primary SS. Examination of CSF revealed associated aseptic meningitis. Because of the lack of efficacy of the first treatment by intravenous corticosteroids, monthly intravenous cyclophosphamide was quickly introduced. After six months, significant visual recovery was observed.ConclusionOptic neuropathies have been rarely reported as the initial symptom revealing primary Sjogrën syndrome, and bilateral simultaneous lesions remain exceptional.     

Benefit of IVIG for long-standing ataxic sensory neuronopathy with Sjögren's syndrome. IV immunoglobulin

Ataxic sensory neuronopathy with Sj?gren's syndrome is a devastating neurologic complication for which there is no established treatment. IV immunoglobulin (IVIG) was given to five patients with severe disabilities for an average of 12 years. Four patients showed remarkable improvement, two of whom responded after the first course. The authors conclude that IVIG is safe and effective to treat even chronically debilitated patients who have the disease, presumably because it ameliorates smoldering inflammation.     

Spontaneous improvement of subacute exacerbation in a case of sensory ataxic neuropathy associated with Sj?gren's syndrome]

We reported a 66-year-old man with Sj?gren's syndrome (sicca syndrome) presenting a sensory ataxic neuropathy, which showed spontaneous remission. He developed difficulty in standing and walking during recent several months. Neurological examinations showed sensory ataxia with areflexia in all extremities and mild distal-dominant decrease in the superficial sensation. Laboratory examinations of blood, urine, and cerebrospinal fluid were all unremarkable; the blood levels of vitamin B1, B2, B6 and B12 were low normal. Sensory nerve action potentials and somatosensory evoked potentials were absent. Cervical cord MRI revealed no abnormal signals. Severe loss of myelinated fibers and scattered myelin ovoids were seen in sural nerve biopsy. Tentative diagnosis at admission was subacute sensory neuropathy associated with malignancy. Screening examinations for malignancy were undertaken and all revealed negative. Because of coexisting sicca symptoms and positive Shirmer test (0 mm), a lip biopsy was performed and a diagnosis of Sj?gren's syndrome was confirmed. Sensory ataxia improved gradually. Two years later, blood B1 levels were low but he remained able to walk. This case is an uncommon example of spontaneous symptomatic remission in sensory ataxic neuropathy associated with Sj?gren's syndrome.     

Peripheral neuropathy in an outpatient cohort of patients with Sjögren's syndrome

Peripheral neuropathy is common in patients with Sj?gren's syndrome (SS), but its precise prevalence is unknown. Most prior studies were conducted at neurology or rheumatology specialty clinics and likely selected for a more severely affected population. We evaluated 22 SS patients and 10 controls for evidence of neuropathy in an outpatient setting at a regional meeting of the Sj?gren's Syndrome Foundation. We performed neurological examinations and nerve conduction studies (NCSs) and measured serum antinuclear antibody (ANA) and SS-A and SS-B antibody levels. Participants filled out a questionnaire pertaining to symptoms, diagnosis, and treatment. We found that signs and symptoms related to small axons were more common in patients with SS than in controls. Complaints of painful distal paresthesias in the feet were noted in 59% of patients but in only 10% of controls, and of abnormal sweating in 41% and 0%, respectively. Examination revealed decreased pinprick sensation in 64% of patients with SS, but in only 30% of controls. Overall, 45% of the patients but none of the controls were thought to have an isolated small-fiber neuropathy. Large-fiber dysfunction (as measured by testing vibration, deep tendon reflexes, and NCSs) was similar between the two groups. We conclude that small-fiber neuropathy is common in patients with SS.     

Dermatomyositis associated with Sjögren's syndrome: VEGF involvement in vasculitis

Two patients with dermatomyositis complicated with Sj?gren's syndrome (SjS), are reported. Both patients exhibited sensory-dominant polyneuropathy, compatible with neurologic involvement in SjS. Vascular endothelial growth factor (VEGF) levels were increased in their plasma. Histological examination demonstrated vasculitic changes in biopsied specimens of muscle and salivary glands from the patients, and VEGF was overexpressed in the vasculitic lesions. These findings suggest that VEGF overexpression was associated with the development of vasculopathy in skeletal muscle and salivary glands and possibly in the peripheral nervous system.     

Differential, size-dependent sensory neuron involvement in the painful and ataxic forms of primary Sjögren's syndrome-associated neuropathy

Primary Sj?gren's syndrome (pSS)-associated neuropathy manifests a wide variety of peripheral neuropathies that may show overlap among the neuropathic forms. In this report, we describe histopathological findings of two autopsy cases with pSS-associated neuropathy; one of them manifested the painful form and another showed ataxic form. The population of dorsal root ganglion (DRG) neurons and the density of myelinated fibers in the dorsal spinal root were variably reduced among spinal segments in both forms. In the painful form, there was a prominent reduction of small neurons, while in the ataxic form, large neurons were predominately lost. In accordance with the degree of the DRG cell loss, the modality of nerve fiber loss in the dorsal spinal roots and sural nerve correlated well with the corresponding DRG neuron loss. Prominent CD8+ T lymphocyte infiltration was present in the DRG, sympathetic ganglion, epineurial and perineurial space throughout the peripheral nerve trunks, and visceral organs, including the submandibular gland of both forms. Although the size of affected DRG neurons is different, these two forms share a similar causal mechanism, namely, cytotoxic autoimmunity to ganglion neurons, which may be one of a continuum of etiological factors. This hypothesis may have an impact on therapeutic approach.     

Spinal cord magnetic resonance imaging demonstrates sensory neuronal involvement and clinical severity in neuronopathy associated with Sjögren's syndrome

OBJECTIVES—To determine spinal cord MRI findings in neuronopathy associated with Sjögren''s syndrome and their correlation with severity of sensory impairment.
METHODS—Clinical and electrophysiological features, pathological findings in the sural nerve, and hyperintensity on T2* weighted MRI in the spinal dorsal columns were evaluated in 14 patients with neuronopathy associated with Sjögren''s syndrome.
RESULTS—Of 14 patients, 12 showed high intensity by T2* weighted MRI in the posterior columns of the cervical cord. High intensity areas were seen in both the fasciculus cuneatus and gracilis in nine patients, who showed severe and widespread sensory deficits in the limbs and trunk; these patients also had a high frequency of autonomic symptoms. Somatosensory evoked potentials often could not be elicited. Hyperintensity restricted to the fasciculus gracilis was seen in three patients, who showed sensory deficits restricted to lower limbs without trunk involvement, or with only partial limb involvement; no autonomic symptoms were noted. The two patients who did not show high intensity areas in the dorsal columns showed restricted sensory involvement in the limbs. All patients showed axonal loss predominantly affecting large fibres, without axonal sprouting.
CONCLUSIONS—High intensity areas on T2* weighted MRI in the spinal dorsal columns reflect the degree of sensory neuronal involvement in neuronopathy associated with Sjögren''s syndrome; this finding could also be a helpful marker for estimating severity of this neuronopathy.

     

Intravenous immunoglobulin in children with Guillain-Barré syndrome

We report our experience with intravenous immunoglobulin (IVIG), plasmapheresis and supportive care in 13 patients with the Guillain-Barré syndrome. Seven of 13 patients received IVIG, 2 plasmapheresis and 4 supportive care. At 15th day after IVIG administration, all patients in this group had improved at least one disability grade. In the plasmapheresis group, 1 improved at 5th day after the procedure. Two of the 4 patients that received supportive care improved at 20th day of evaluation. In the IVIG group, the final scores were lower and had no relapses. These results suggest faster clinical improvement with IVIG when compared with supportive measures.     

Tonic pupils in Sjögren's syndrome

INTRODUCTION: Adie's syndrome is usually a disease of unknown origin. We report two cases secondary to Sj?gren syndrome. CASE REPORTS: A 26-year-old man developed in few months a sensitive neuropathy with a bilateral tonic pupil. A 50-year-old woman complained of sensitive signs probably related to a ganglionopathy and dysautonomic disorders affecting sudomotor and vasomotor functions. Adie syndrome had been diagnosed three years earlier. In both patients, the systemic signs and the results of the complementary tests led to the diagnosis of Sj?gren's syndrome. Corticosteroids had limited effects on the sensitive signs and no influence on the tonic pupils. CONCLUSION: Adie syndrome, isolated or accompanied by other dysautonomic disorders, may reveal or precede the diagnosis of Sj?gren's syndrome.     

Acute motor axonal neuropathy in association with Sjögren syndrome

Sjögren syndrome (SS) has been known to manifest with neurological complications, most frequently of the peripheral nervous system, and often in advance of xerostomia and xerophthalmia. There has been one case report of a patient with SS presenting with acute motor neuropathy similar to Guillain–Barré syndrome (GBS). We report the case of a patient who developed rapidly fulminant acute motor axonal neuropathy (AMAN) with positive anti‐GM1 antibody at high titers in association with serological and pathological evidence of SS without xerostomia or xerophthalmia. Muscle Nerve 42: 828–832, 2010     

Demyelinating neuropathy and Sjögren's syndrome: a diagnostic pitfall

INTRODUCTION: Neuropathies induced by Sj?gren's syndrome (SS) are usually axonal. Nevertheless some demyelinating neuropathies have been described in patients with SS. To date, the relationship between demyelinating neuropathies and SS remains imprecise. CASE REPORT: A 75 year-old man presented with a chronic history of sensory disturbances linked to demyelinating neuropathy. Electroneuromyography revealed a demyelinating neuropathy and complementary tests revealed both Sj?gren's syndrome (SS) and HMSN IA. CONCLUSION: We suggested that an inherited affection might be researched before considering that demyelinating neuropathy might be a form of peripheral nervous system involvement in SS.