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.     

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.     

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.     

Neurological complications of primary Sjögren's syndrome

Objective: To better delineate the spectrum of neurological complications of primary Sjögren's syndrome (PSS). Methods: A detailed neurological investigation was prospectively performed in a group of 25 consecutive patients with PSS followed in an internal medicine department between June 1996 and December 1997 (Internal Medicine group). In addition, eleven patients with neurological complications of PSS were identified in the Neurological Department of the same institution during the same period (Neurological group). Results: In the Internal Medicine group, neurological complications were discovered in 10/25 (40 %) patients. Peripheral nervous system involvement was present in 4/25 patients from the Internal Medicine group and in 10/11 patients from the Neurological group and consisted mainly of axonal sensorimotor/sensory polyneuropathy. A motor neuron syndrome was identified in two patients. CNS involvement occurred in 7/25 patients from the Internal Medicine group and in 4/11 patients from the Neurological group. Three patients had spinal cord involvement. Cognitive dysfunction was the most frequent finding (5/25 in the Internal Medicine group, 3/11 in the Neurological group) characterized either by subcortical or corticosubcortical dysfunction. Cognitive impairment was not attributed to mood disturbance and was not associated with specific laboratory or radiological abnormalities. Conclusion: Neurological complications of PSS are frequent since they were present in 40 % (10/25) of patients in a consecutive series of patients from a department of Internal Medicine. Although PNS involvement predominates, complications of PSS affecting the brain or spinal cord are not rare, with subcortical dysfunction as the main finding.     

Cranial pachymeningitis and primary Sjögren's syndrome

Introduction

Central neurological manifestations of primary Sjogren's syndrome are very polymorphic, including pachymeningitis, one of the rarest manifestations, which is exceptionally inaugural.

Clinical case

A 50-year-old patient was admitted for an intracranial hypertension syndrome with paralysis of the III and VII cranial nerves. Brain MRI revealed pachymeningitis of the right cerebral hemisphere. History taking revealed the existence of xerostomy and xerophthalmos. The accessory salivary gland biopsy demonstrated Chisholm stage III sialadenitis. Search for anti-SSA antibodies and anti-SSB antibodies was positive. The diagnosis of primary Sjogren's syndrome was retained. The patient improved with corticosteroid therapy and cyclophosphamide.

Conclusion

This case illustrates the unusual observation of pachymeningitis in primary Sjogren's syndrome in the rare setting as an inaugural manifestation.     

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.     

Headache in primary Sjögren's syndrome: a prevalence study

Objectives –  To determine the prevalence of headache in patients with primary Sjögren's syndrome (pSS) and to examine the relationship between headache types and clinical, serologic features of the disease.
Methods –  The study enclosed 133 patients with the diagnoses of pSS and 97 healthy controls. A questionnaire designed to assess the presence of headache and if present to classify it according to the criteria of the International Headache Society was used.
Results –  In 133 of the pSS patients evaluated, 104 had headache. No association was present between types of headache and the clinical and laboratory manifestations of the disease. Both migraine and tension-type headache were more common in patients with pSS when compared with healthy controls ( P  < 0.001).
Conclusions –  The high prevalence of migraine in pSS patients might be explained by a vascular headache triggered by immuno-mediated disease activity without an obvious clinic or laboratory marker.     

Myelopathy in primary Sjögren's syndrome: diagnostic and therapeutic aspects

We report a patient with a myelopathy in primary Sjögren's syndrome, proven by salviary gland biopsy and specific antibodies. Under steroid medication, the patient had a remitting and relapsing clinical course. The severity of clinical symptoms correlated with a transient contrast uptake in spinal magnetic resonance imaging. Under a treatment with azathioprine and prednisone the patient has suffered no relapse within the last 20 months. Although this is only a case report, the combination of azathioprine and prednisone may be a valuable medication in chronic cases of Sjögren's syndrome with neurologic symptoms.     

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.     

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.     

Role of anti-calcium channel and anti-receptor autoantibodies in autonomic dysfunction in Sjögren's syndrome

Auto-antibodies cross-reacting with L-type voltage-gated calcium channels (VGCCs) have been described in primary Sj?gren's syndrome (pSS), and may mediate the cardiac defects in neonates born to mothers with pSS. L-type VGCCs are also present in autonomically innervated tissues. Therefore, the aim of this project was to investigate a role for anti-VGCC antibodies and antibodies to alpha(1)-adrenoceptors or P(2X)-purinoceptors in the autonomic dysfunction that occurs in pSS. Contraction of the sympathetically innervated vas deferens in response to stimulation of the muscle by an alpha(1)-adrenoceptor agonist (phenylephrine) or a P(2X)-purinoceptor agonist (alpha,beta-methylene ATP) was measured in the absence and presence of 2% serum. Contractions produced by phenylephrine and by alpha,beta-methylene ATP were abolished by nicardipine, demonstrating that they are coupled to calcium influx through L-type VGCCs. Serum from patients with pSS or from healthy controls did not significantly alter the L-type channel-dependent responses of smooth muscle to agonist stimulation. We therefore conclude that pSS serum does not contain autoantibodies that functionally inhibit L-type VGCCs, alpha(1)-adrenoceptors or P(2X)-purinoceptors in smooth muscle and that such autoantibodies cannot explain the autonomic dysfunction in pSS.     

Speech-language performance in Sjögren-Larsson syndrome

Objective: To describe speech-language pathology in patients with Sjögren-Larsson syndrome (SLS) in relation to their cognitive and motor impairment.

Design: Observational case series.

Methods: Cognitive functioning was assessed in 16 patients with SLS (nine males; seven females) using different neuropsychological tests. Speech-language pathology was studied focusing on dysarthria, oral motor functioning, speech intelligibility and language development. Potential correlations between speech-language pathology and other neurological symptoms (e.g. spasticity) were studied.

Results: The median cognitive developmental age was 5;8 (n = 13; range 3;5–8;0) years. A variable degree of mainly pseudobulbar dysarthria was found. Speech intelligibility was influenced by dysarthria, but was also related to language pathology. No correlation between motor functioning and dysarthria or cognitive development was observed.

Conclusion: Dysarthria and language problems are important factors in daily life functioning of patients with SLS. Based upon the clinical profile found, early speech-language therapy is recommended in order to optimize their speech-language development.