Peripheral neuropathy in systemic lupus erythematosus.

The prevalence of clinically apparent peripheral neuropathy in systemic lupus erythematosus is reported to be between 2% to 18%. The purpose of this prospective case-control study was to determine the prevalence of peripheral neuropathy (PN) using electrodiagnostic criteria. Subgroup analysis was performed to determine whether PN correlated with disease activity, renal involvement, or serum immune markers. Fifty-four systemic lupus erythematosus patients and 30 controls were recruited in the study. The right median, ulnar, peroneal, tibial, and sural sensory and motor nerve conduction studies were obtained. PN in our study was defined as any abnormal values in motor and sensory distal latency, sensory action potential, motor action potential, or conduction velocity affecting 2 or more nerves. Of the 54 patients studied, PN was present in 15 patients (27.8%) of which 4 were symptomatic. There was a significant correlation between PN and anti-SM antibody, and there was a trend showing decreased motor and sensory action potential amplitudes in our systemic lupus erythematosus group compared to the controls. This observation was also seen in an active disease group when compared to those with inactive disease. The amplitude of the action potential was more often affected than the distal latency, and sensory nerves were more susceptible than motor nerves. The sural sensory action potential amplitude appears to be the most sensitive indicator of PN which may be used as an index to monitor disease activity.     

Peripheral neuropathy in systemic lupus erythematosus.

We studied 33 consecutive patients with systemic lupus erythematosus (SLE) for neuropathy by employing the Neuropathy Symptom Score (NSS), Neurological Disability Score (NDS), EMG and nerve conduction velocity (NCV) studies, and determinations of vibration thresholds (VT). Polyneuropathy defined as NCV abnormalities of two or more nerves occurred in seven patients (21%). Neuropathic symptoms showed a poor correlation with NCV and VT, while clinical neuropathic signs, VT, and NCV correlated with each other in most instances. When reporting frequencies of neuropathy in SLE, NCV studies should be used as a basis. NSS, NDS, and VT give additional quantifiable information and can be useful in the follow-up of patients and for evaluating the response to therapy.     

Peripheral neuropathy in pediatric systemic lupus erythematosus

Peripheral neuropathy is an uncommon manifestation of systemic lupus erythematosus and has not yet been characterized in pediatric patients. We report the clinical and electrophysiologic features of peripheral neuropathy in one child and three adolescents with lupus erythematosus. There were three females and one male. The peripheral neuropathy followed the onset of lupus erythematosus by a mean of 3 years. The onset of the neuropathy correlated with lupus erythematosus activity and presented with either severe pain or dropfoot. Nerve conduction studies revealed sensory and motor polyneuropathy in all patients and mononeuritis multiplex in two patients. Only one patient had associated central nervous system involvement at that time. All patients were positive for IgM and IgG anticardiolipin antibodies. Patients were treated with steroids, gabapentin, carbamazepine, azathioprine, and cyclophosphamide. Response to treatment was variable: two patients recovered and two had a partial response. Although an unusual manifestation, peripheral neuropathy should be kept in mind as part of the neurologic spectrum in lupus erythematosus. It is not necessarily associated with central nervous system disease. A role for antiphospholipid antibodies in the pathogenesis is suggested.     

Small-diameter nerve fiber neuropathy in systemic lupus erythematosus

BACKGROUND: Systemic lupus erythematosus (SLE) is an inflammatory, autoimmune, multiorgan disease often involving the central and peripheral nervous systems. OBJECTIVE: To determine whether there is a selective small-diameter nerve fiber neuropathy in patients with SLE. DESIGN: Cross-sectional study. SETTING: Stavanger University Hospital, Stavanger, Norway.Patients Sixty patients with SLE, aged 43.2 +/- 13.5 years (mean +/- SD). INTERVENTIONS: Skin biopsies, nerve conduction studies, and clinical neurologic examinations. MAIN OUTCOME MEASURES: Density of intraepidermal small-diameter nerve fibers in skin biopsy specimens and large-diameter nerve fiber function as determined by nerve conduction studies and clinical examinations. RESULTS: The mean density of intraepidermal small-diameter nerve fibers in patients with SLE was 7.5 +/- 3.8/mm. Eight patients (13%) had densities below reference values, consistent with small-diameter nerve fiber neuropathy, and results of nerve conduction studies were normal in 6 of them. Eleven patients (18%) had abnormal results of nerve conduction studies, reflecting large-diameter nerve fiber neuropathy, and 4 patients (7%) were classified by an experienced neurologist as having polyneuropathy after the clinical examination. CONCLUSIONS: An abnormal reduction in intraepidermal small-diameter nerve fiber densities is evident in some patients despite normal function of their larger nerve fibers. This adds further support to the theory that a pure small-diameter nerve fiber neuropathy may occur in SLE.     

Optic neuropathy and chiasmopathy in the diagnosis of systemic lupus erythematosus.

PURPOSE: To report the clinical presentation of acute visual loss in six patients who were ultimately diagnosed with systemic lupus erythematosus (SLE). METHODS: Retrospective case series. RESULTS: All patients had a positive antinuclear antibody and elevated anti-double stranded DNA titers. Five of six patients demonstrated gadolinium enhancement of the optic nerve and/or chiasm on magnetic resonance imaging (MRI). Most patients showed initial improvement after treatment with high-dose systemic corticosteroids, but five experienced recrudescences during steroid taper, requiring further treatment with immunosuppressive or cytotoxic medications. CONCLUSIONS: Visual loss owing to optic neuropathy or chiasmopathy may be the presenting sign of SLE or the event that leads to this diagnosis. Gadolinium-enhanced MRI is useful for identifying anterior visual pathway lesions in these patients. Corticosteroids are effective in the treatment of this condition; however, relapses requiring further treatment are common.     

Sensorimotor and autonomic neuropathy associated with systemic lupus erythematosus

In this article, one described 7 patients with systemic lupus erythematosus (SLE) and the pathological findings of their sural nerves. In 4 of the 7 cases the criteria for diagnosis of SLE were satisfied and in 3 cases there was serological evidence of an undifferentiated connective tissue disease, most likely to be SLE. The peripheral neuropathy was of a chronic sensorimotor type with predominantly sensory features and gradual onset. In 2 cases the presentation was asymmetrical. One patient had autonomic dysfunction. There was ischemic neuropathy in 2 cases, segmental demyelination in 2 cases, and axonal degeneration with demyelination in 3 cases. In 6 cases there was increased expression of Ia antigen within the nerve fascicle, perineurium and endothelial cells.     

A controlled study of peripheral neuropathy in systemic lupus erythematosus

In 34 patients with systemic lupus erythematosus (SLE) and 34 age- and sex-matched healthy controls we performed standardized quantifiable neurological testing for neuropathic symptoms (Neuropathic Symptom Score) and deficits (Neurological Disability Score), as well as nerve conduction velocity studies (NCV) and determination of vibration thresholds (VT). SLE patients had more neuropathic symptoms and deficits than controls. Most quantitative and qualitative NCV attributes showed no difference between patients and controls. However, if categorized as NCV abnormalities of 2 or more nerves in each individual, a frequency of polyneuropathy of 21% was seen in SLE patients compared to 6% in controls. VT indicated a slight, but widespread, diffuse polyneuropathy in patients compared to controls.     

Peripheral neuropathy in systemic lupus erythematosus with epineural vasculitis and antiphospholipid antibodies

Neurological manifestations of systemic lupus erythematosus are frequent and polymorphic. Their frequency varies according to authors (24-75p.cent). Central nervous system complications predominate; peripheral features are rare, classically symmetrical polyneuropathy, multiple mononeuropathies or cranial nerve involvement. We report a case of a 48-year-old woman presenting a histologically documented sensitivo-motor polyneuropathy with severe motor involvement complicating lupus associated with antiphospholipides antibodies. Outcome was good after cyclophosphamid pulse. We discuss the frequency of peripheral involvement in systemic lupus erythematosus, pathogenic mechanisms, therapeutic possibilities and outcome of this complication.     

Peripheral neuropathy in systemic lupus erythematosus – a longitudinal study

OBJECTIVE: Peripheral neuropathy (PN) is reported to occur in 5-27% of patients with systemic lupus erythematosus (SLE) mostly as a length-dependent sensorimotor axonopathy. Studies over time have not been performed. Design - Longitudinal study. Subjects and Methods: Thirty-three Caucasian SLE patients consented to participate in the study and were subjected to clinical examination, laboratory tests, and nerve conduction velocity (NCV) studies. At the follow-up 7 years later, 7 patients (21%) were dead, 4 refused to participate, and 2 did not want to perform NCV studies. Twenty patients were thus available for longitudinal study. RESULTS: When all SLE patients were considered on a group basis at follow-up, 8 (33%) out of 24 NCV parameters showed significant deterioration despite correction for time, while 16 (67%) were unchanged. Analysis of change from baseline showed that, except for F-responses, several NCV changes were highly dependent (negative regression coefficients) on baseline levels at start of study. No demographic, laboratory, or disease associated quantitative factor was associated with these changes in NCV parameters over time. Nor was a consistent effect on NCV parameters from any qualitative demographic or disease associated factor confirmed by Repeated Measures ANOVA analyses. CONCLUSIONS: A modest progressive neuropathic process exists in patients with SLE. Important is also the finding that, over time, the abnormalities of NCV parameters fluctuate in the individual patients, and the impairments are not necessarily irreversible. This study also shows no association to medication, demographic-, or other disease associated factors.     

Brachial plexus neuropathy as the initial presentation of systemic lupus erythematosus

A patient presented with bilateral brachial plexus neuropathy which 2 weeks later evolved into a polyneuropathy involving all four extremities. On further investigation, the patient was found to have systemic lupus erythematosus. Treatment with steroids was accompanied by complete resolution of the neurologic findings.     

Reversible parkinsonism in systemic lupus erythematosus.

Parkinsonism as a manifestation of central nervous system (CNS) lupus is extremely rare. We report the first patient with systemic lupus erythematosus (SLE) who developed a reversible parkinsonian syndrome associated with enhancing subcortical lesions on magnetic resonance imaging (MRI). Following treatment with prednisolone and cyclophosphamide, her bradyphrenia, bradykinesia, hypophonia, rigidity, and abnormal gait progressively improved. Three months after she commenced treatment, repeat MRI scanning demonstrated resolution of the abnormal subcortical white matter enhancement. Our case illustrates unusual clinico-radiologic correlates of reversible parkinsonism in a SLE patient; these findings suggest that disruption of the subcortical frontal pathways may be a possible pathophysiologic mechanism for parkinsonism in cerebral lupus.     

Stroke in systemic lupus erythematosus

We investigated the clinical and pathologic characteristics of stroke in 234 patients with systemic lupus erythematosus. Thirteen patients (5.6%) developed cerebrovascular disease. Cerebral infarction was noted in eight, cerebral hemorrhage in two, and subarachnoid hemorrhage in three. In seven (54%) of these 13 patients, stroke occurred less than or equal to 5 years after systemic lupus erythematosus was diagnosed. Among the predisposing risk factors for stroke, hypertension was the most important. Lupus anticoagulant was detected in three (38%) and anticardiolipin antibody in three (43% of seven investigated) of the patients with infarction. Evaluation of the clinical manifestations and autoantibodies indicated that renal involvement and high titers of anti-deoxyribonucleic acid antibody were more frequent in the stroke group than in the non-stroke group. Autopsy studies on six of the patients with stroke revealed small infarcts and hemorrhages in all, but in no case was true angiitis observed. Libman-Sacks endocarditis was found in two of the three patients with infarction. In conclusion, the important contributory factor to the development of stroke in patients with systemic lupus erythematosus is considered to be hypertension mediated by immunologic abnormalities. Antiphospholipid antibodies and Libman-Sacks endocarditis are closely associated with occlusive cerebrovascular disease.