Neurological symptoms and syndromes occurring in the course of collagenoses are discussed in the article. Special attention is paid to the relationship between the type of the nervous system lesion and the diagnosis, the course of the disease and prognosis in particular collagenoses.
Neurological disorders in systemic connective tissue diseases occur most frequently in the course of systemic lupus erythematosus and polyarteritis nodosa. Both the peripheral as well as central nervous system is affected. The disorders in question are caused by various autoimmunological processes, e.g. local vasculitis may be a reaction to deposits of antigen/antibody complexes, while disseminated neuronal lesions may be associated with the presence of these specific antibodies.
Prevalence rates of neurological complications in SLE patients as estimated by various authors range form under 20 up to 80%, most frequently reaching about 50%. These discrepancies result not only from the lack of a generally accepted classification of neurological symptoms typical of SLE, but also from difficulties in the differential diagnosis of organic and functional symptoms in this group of patients. Moreover, complications due to side effects of SLE treatment should be differentiated. The aim of the study was firstly, to estimate the frequency and character of neurological symptoms SLE patients, and secondly, to check if the presence of neurological symptoms is correlated with selected immunological parameter s (a-CL, p-ANCA, ANA, C3 and C4 fractions of complement). Participants in the study were 63 patients with SLE (58 women and 5 man), aged 17-63 (mean age 40.1 years). All the patients underwent a full rheumatological and neurological examination, supplemented with immunological assays and electrophysiological tests (EEG, EMG, BAEP, VEP). If necessary, in some cases CT and MR scans were performed. Neurological symptoms noted in 71.4% of the SLE patients turned out to be significantly more frequent in those with a high titre of anticardiolipin antibodies. No correlation was found between neurological symptoms frequency and a high titre of antinuclear antibodies, the presence of anticytoplasmatic antibodies of the perinuclear type, or the level of C3 and C4 fractions of complement.
The aim of the study was to analyse early manifestations of the nervous system involvement so that cerebral SLE can be early diagnosed and successfully treated before the onset of irreversible changes. Special attention was paid to the presence of risk factors (such as hypertension, diabetes mellitus, heart and renal failure, dyslipidemia, and large doses of steroid therapy), that may contribute to the nervous system lesions. 44 patients with SLE were reviewed retrospectively. Their mean age was 43.8± 11.5 years (range 22-68), while the mean duration of SLE evolution was 11.8±9.0 years (range 1-30). In 28 cases clinical evidence of the nervous system involvement was found. In 9 patients neurological abnormalities heralded other SLE signs and symptoms, in other 7 cases neurological symptoms occurred in the first year following the diagnosis of SLE, while in 12 patients – later in the course of the disease. Neurological disorders that appear either prior to other manifestations of SLE or in the first year after the diagnosis, included: headache and vertigo (in 7 and 4 cases, respectively), organic brain syndrome (4 cases), seizures (3 cases), focal CNS lesions (3 cases). Renal diseases were found in 62.5% patients with early neurological disorders, hypertension in 40%, heart diseases in 33.3% of the patients, and haematological disorders in 27.7% of cases. Conclusions: (l) Symptoms of CNS lesions in SLE may precede other SLE manifestations, so in young patients with neurological disorders of unknown origin a diagnostic examination for SLE should be performed. (2) Early neurological disorders are usually transient and unspecific: headache, vertigo, organic brain syndrome. (3) Special care should be provided to SLE patients suffering from hypertension and renal diseases, since these two factors predispose to the development of neurological disorders.
Neurological and psychiatric symptoms of SLE (NP-SLE) include a wide spectrum of the nervous system events that may lead to the patient's disability or even death. The aims of the study were: to determine the prevalence of NP manifestations in SLE patients with and without APS, and to analyze the usefulness of MR and CT techniques in the diagnosis of CNS lupus. 58 consecutive SLE patients were evaluated (54 F, 4 M, mean age: 40.4±15.5 years). In 50 patients the diagnosis of SLE and in 8 patients – SLE+APS was made. NP-SLE manifestations were diagnosed in 28 cases, i.e. 48% of patients. Cerebrovascular events (TIA, complete stroke, intracerebral hemorrhage) were present in 12 patients, migraine – in 11, peripheral neuropathies – in 7, psychiatric disorders – in 6, emotional distress – in 4, and motor disorders – in 3 patients. Other manifestations (epilepsy, dementia, cognitive impairment, sleeplessness) were observed in single cases. In 82% of patients NP symptoms were present from the onset of SLE. In the NP-SLE group the mean age was similar to that of other patients, i.e. 41.8 vs. 39.2 years, whereas the mean duration of the disease was much longer, i.e. 95.9 vs. 40.2 months. All 4 male patients were in the NP-SLE group. In the SLE+APS group NP symptoms were found in 7 (88%) patients, but only in 3 of them (43%) - from the beginning of SLE. In 20 patients MR and CT examinations were performed. In CT – 2 patients had multiple ischemic infarcts, 1 – intracerebral hemorrhage, 7 – slight or moderate cerebral atrophy. MR showed focal lesions in 10 patients (multiple ischemic changes – in 7 cases, single – in 2, multiple i.ch. with intracerebral hemorrhage – in l case, and atrophy in 5 patients). In this study a wide spectrum of NP manifestations was observed. The concurrent APS increased the risk of NP manifestations in SLE patients. MR turned out to be more useful than CT in diagnosing CNS lupus, regardless of the presence or absence of APS.
Three cases of cerebral stroke in the course of the antiphospholipid syndrome in systemic lupus erythematosus (SLE) are presented. In alt the three female patients SLE was diagnosed after the stroke, even though signs suggestive of a systemic disease had been present much earlier. In the first case the symptoms included glomerulonephritis and myocardial infarction, in the second one – thrombocytopenia (due to which splenectomy was performed) and myocardial infarction, while in the third case – arterial thrombosis of lower limbs, spontaneous abortions, recurring pneumonia, and leucopenia. A possibility of a systemic disease had been taken into account in alt the three cases, due to the patients' young age and past history. This was confirmed by laboratory tests results, especially by elevated anticardiolipin and antinuclear antibodies levels. In all the cases the course of SLE was severe. However, in two cases the administered treatment (with antibiotics, piracetam, prednisone, cyclophosphamide) resulted in an amelioration of symptoms of neurological deficit, while in one case the disease was fatal. Our cases evidence that alt stroke patients (especially young ones) should be thoroughly analyzed for systemic diseases involving the risk of cerebral stroke.
Two patients with numerous pronounced neurological symptoms are presented in the article. By clinical and immunological criteria they were diagnosed with cerebrovascular lesions and a co-existing antiphospholipid syndrome. Case 1: 39-year-old man, treated a year before for the right – sided hemiplegia resulting from an infarct to the left cerebral hemisphere; readmitted due to a severe pseudobulbar syndrome, with signs suggestive of the fifth nerve impairment, and bilateral peripheral damage of the seventh cranial nerve. Case 2: 28-year-old man, with right hemiplegia, sensorimotor dysphasia, and sensory impairment of the right leg.
The paper presents spinal pathologies that occur in the course of same systemic connective tissue diseases and may lead to neurological complications. A special emphasis is laid on the cervical spine condition in patients with rheumatoid arthritis (RA). Moreover, the type and character of changes in this respect are discussed in terms of risks resulting to the nervous system structures. Research findings obtained in the Rheumatological Institute in Warsaw indicate that the most severe neurological complications occurred in patients with the dens of the epistropheus invagination into the great foramen of the occipital bane, and a simultaneous horizontal subluxation of C1/C2 vertebrae (6.25% of RA cases). The problems of severe neurological complications prevention are also outlined.
A case of a 58-year-old woman with pseudo-SLA myelopathy associated with the onset of atypical rheumatoid arthritis is reported. No compressive lesions were seen in the spinal canal in spine X-ray, CT, and MRI scans. The treatment with predonisolone and metotrexate was followed by an improvement of the patient's neurological state. This may suggest that her spinal myelopathy was caused by an inflammatory-autoimmunological process.
Limitation of the vertebral column mobility and elasticity in ankylosing spondylitis results in an increased risk of fractures. Cervical spine fractures in ankylosing spondylitis (3.5 times more frequent than in the general population) are usually caused by minor traumas and traffic accidents. The most frequent mechanism underlying such fractures is that of extension. Fractures of the dens of epistropheus are reported very rarely. They lead to symptoms of massive spinal cord lesions. Two cases o f a dens of epistropheus fracture are described: one with tetraparesis and the other with the Brown-Sequard syndrome. They were very difficult to diagnose because of the fracture localisation. In both cases conservative treatment was applied with good results (a partial recovery from paresis).
The most characteristic types of microscopic vascular lesions in patients with various inflammatory systemic connective tissue diseases are described on the basis of the literature as well as biopsy and autopsy studies of the Pathoanatomy Department of the Institute of Rheumatology. Special emphasis is laid on diseases in which the nervous system involvement is not infrequent. Irrespective of the character of the observed vascular lesions, they result in occlusion of the lumen or a vessel wall rupture. In both the peripheral and central nervous system involvement these vascular lesions are accompanied by a variety of neurological manifestations. In rheumatoid arthritis necrotizing arteritis and intimal proliferation may result in neurological symptoms mostly peripheral, while CNS disorders are extremely rare. In polyarteritis nodosa in as many as over 50% of patients symptoms of peripheral or CNS involvement are observed. Their ischemic or hemorrhagic lesions result from occlusive arteritis nacroticans or from a rupture of altered vessel walls, respectively. In systemic lupus erythematosus CNS involvement (noted in 25-75% of patients) is due to dispersed thrombotic or inflammatory vascular lesions associated with immune complexes deposition or circulating antiphospholipid antibodies. The latter are probably related to the emergence of thrombosis or intimal proliferation of vessels.
Both central and peripheral nervous system damage is rather frequent in systemic connective tissue diseases. CNS damage is mainly due to changes in vessel walls, resulting in ischaemic foci in the brain. Peripheral neuron damage is the cause of neuropathies, usually of axonal or axonal--demyelinating type: mononeuropathy, multifocal mononeuropathy and polyneuropathy. The neuropathies may be caused also by changes in vascular walls, including these in nerve-nutrient vessels. Electroneurography is the main diagnostic method in this group of disorders. It permits to assess whether a given nerve is damaged, whether the damage is local or generalised, whether motor and sensory fibers are equally affected. Besides, the pathological process intensity and dynamics may be evaluated, as well as the character of the lesion (axonal or demyelinating). Electromyography is a complementary technique in the diagnosis of, but the main diagnostic method in primarily myogenic pathology (such as e.g. polymyositis, dermatomyositis, scleromyositis, the Sharp syndrome, etc.), frequently present in the course of collagenoses. Electromyography allows to differentiate between the primarily myogenic and neurogenic muscle changes. On finding primarily myogenic changes in EMG recording, not only the lesion intensity can be evaluated, but sometimes also its dynamics, i.e. whether it is of acute or chronic character. Electroneurography and electromyography may be also helpful in the prognosis and in treatment monitoring.