1/55. Can immunoadsorption plasmapheresis be used as the first choice therapy for neuroimmunological disorders?The subjects were 31 patients in whom immunoadsorption plasmapheresis (IAPP) was performed as the first choice therapy for primary or recurrent neuroimmunological disorders. The clinical manifestations before and after IAPP and the use of corticosteroids were investigated in the present study. IAPP was clinically effective in all patients. The corticosteroids (CSs) administration was begun or CSs were increased after completion of IAPP in 11 patients. IAPP was performed as the first choice therapy, and favorable results were obtained in patients with guillain-barre syndrome and Miller-Fisher syndrome. IAPP alone was also effective in a patient with lupoid sclerosis. When IAPP was used alone in 2 patients with chronic inflammatory demyelinating polyradiculoneuropathy, it completely eliminated the clinical manifestations, but the symptoms recurred about 2 months later. Therefore, although IAPP could be performed as the first choice therapy for many neuroimmunological disorders, subsequent therapies should be carefully investigated.- - - - - - - - - - ranking = 1keywords = sclerosis (Clic here for more details about this article) |
2/55. Can neurologic manifestations of Hughes (antiphospholipid) syndrome be distinguished from multiple sclerosis? Analysis of 27 patients and review of the literature.Hughes (antiphospholipid) syndrome (APS) can mimic multiple sclerosis (MS). We analyzed the clinical, laboratory, and imaging findings of MS-like expression in a cohort of patients with APS in an attempt to identify parameters that might differentiate the 2 entities. We studied 27 patients who were referred to our unit with the diagnosis of probable or definite MS made by a neurologist. All patients were referred to our lupus clinic because of symptoms suggesting an underlying connective tissue disease, uncommon findings for MS on magnetic resonance imaging (MRI), atypical evolution of MS, or antiphospholipid antibody (aPL) positivity. aPL, antinuclear antibody (ANA), anti-dsDNA, and anti-extractable nuclear antigen (ENA) antibodies were measured by standard methods. MRI was performed in every patient and compared with MRI of 25 definite MS patients who did not have aPL. An index severity score was calculated based on the size and number of increased signal intensity areas in MRI. In the past medical history, 8 patients with primary APS and 6 with APS secondary to systemic lupus erythematosus (SLE) had had symptoms related to these conditions. Neurologic symptoms and physical examination of the patients were not different from those common in MS patients. Laboratory findings were not a useful tool to distinguish APS from MS. When MRI from APS patients was compared globally with MRI from MS patients, MS patients had significantly increased severity score in white matter (p < 0.001), cerebellum (p = 0.035), pons (p < 0.015), and when all areas were taken together (p < 0.001). patients with APS had significantly increased scores in the putamen (p < 0.01). No differences were noticed in the degree of atrophy. When taken individually, MRI from APS patients could not be distinguished from MRI from MS patients. Most of the patients with primary APS showed a good response to oral anticoagulant treatment. In patients with secondary APS, the outcome was poorer. Hughes syndrome (APS) and MS can be difficult to distinguish. A careful medical history, a previous history of thrombosis and/or fetal loss, an abnormal localization of the lesions in MRI, and the response to anticoagulant therapy might be helpful in the differential diagnosis. We believe that testing for aPL should become routine in all patients with MS.- - - - - - - - - - ranking = 101.50057514175keywords = multiple sclerosis, sclerosis (Clic here for more details about this article) |
3/55. Bullous pemphigoid in a leg affected with hemiparesia: a possible relation of neurological diseases with bullous pemphigoid?We report a typical case of bullous pemphigoid (BP) associated with a neurological disorder and study a possible link between neurological disorders and BP. An 84-year-old hemiplegic woman presented with unilateral BP on the hemiparetic side. BP was confirmed by histological and immunofluorescence data. The medical records of the previous 46 consecutive patients with BP were retrospectively analyzed (average age: 79; median age: 85). Thirty of the 46 patients with BP had neurological disorders. These disorders included dementia, epilepsy, multiple sclerosis, cerebral stroke, Parkinson's disease, gonadotropic adenoma, trembling, dyskinesia, lumbar spinal stenosis. In a control group of the 46 consecutive oldest patients (older than 71; average age: 82,5; median age: 80) with another skin disease referred during the previous two-year-period to our one-day-unit only, 13 patients had a neurological disorder. This study demonstrates that there is a high prevalence of neurological disorders in patients with BP (p = 0.0004). A prospective case control study with neurological examination and psychometrical evaluation is warranted to confirm these data. We speculate that neuroautoimmunity associated with the aging process or neurological disorders may be involved in pemphigoid development via an autoimmune response against dystonin which shares homology with bullous pemphigoid antigen 1. Bullous pemphigoid could be considered to be a marker of neurological disorder.- - - - - - - - - - ranking = 20.30011502835keywords = multiple sclerosis, sclerosis (Clic here for more details about this article) |
4/55. Mitochondrial cytopathy presenting with focal segmental glomerulosclerosis, hypoparathyroidism, sensorineural deafness, and progressive neurological disease.A 6 year old boy who presented with steroid unresponsive nephrotic syndrome is reported. He was found to have focal segmental glomerulosclerosis and associated hypoparathyroidism and sensorineural deafness. The child progressed to end stage renal failure and was successfully managed by dialysis and cadaveric renal transplantation. He later developed progressive neurological deterioration and mitochondrial myopathy and neuropathy was diagnosed.- - - - - - - - - - ranking = 5keywords = sclerosis (Clic here for more details about this article) |
5/55. Another autosomal recessive form of focal glomerulosclerosis with neurological findings.We report four patients in a consanguineous family with focal segmental glomerulosclerosis (FSGS), early onset nephrotic syndrome, eventual end-stage renal failure, psychomotor retardation, seizures and microcephaly or brain atrophy without hiatus hernia. Other characteristic dysmorphic features were convergent strabismus and narrow forehead. One patient had enamel hypoplasia of the upper incisors and deviation of bilateral thumbs to palm side. We could not detect an NPHS2 mutation in this family. We propose that this may be another autosomal recessive syndrome with FSGS and neurological findings.- - - - - - - - - - ranking = 5keywords = sclerosis (Clic here for more details about this article) |
6/55. MR findings of erdheim-chester disease.Lipoid granulomatosis (erdheim-chester disease) is a rare but distinct form of histiocytosis. This disease has characteristic radiologic findings involving the musculoskeletal system that are critical to the diagnosis: symmetric sclerosis of the metaphysis and diaphysis of long bones with relative sparing of the epiphysis as depicted on conventional radiography. However, it is a systemic disease that involves multiple organ systems. This pictorial essay is of a single patient imaged over multiple years, using various pulse sequences with both low and high field strength MR scanners. It depicts many of the characteristic findings encountered in this rare systemic disorder.- - - - - - - - - - ranking = 1keywords = sclerosis (Clic here for more details about this article) |
7/55. Neurological complications of acute and persistent Epstein-Barr virus infection in paediatric patients.Neurological complications of Epstein-Barr virus (EBV) have been reported almost exclusively in the course of acute primary infections. The role of EBV in paediatric neurological disease was investigated prospectively over a 2-year period, searching for acute primary, chronic, and reactivated EBV infections. Active EBV infections were diagnosed in 10/48 patients, including two with acute primary EBV infections (cranial neuritis and cerebellitis), one with chronic active infection (T/NK cell lymphoma with cranial neuritis), and seven with reactivated infections. Among these seven patients, three showed "Alice in Wonderland" syndrome, one facial nerve palsy, one progressive macrocephaly, and two prolonged encephalitic illness. The prognosis was good except for the patient with lethal T/NK cell lymphoma and the two girls with encephalitic illness. Despite steroid treatment, these girls suffered prolonged cognitive impairment and epileptic seizures. Both developed left-sided hippocampal atrophy, and one of them hippocampal sclerosis. Like primary infections, reactivated EBV infections cause neurological complications in a considerable number of paediatric patients, lead to serious long-term complications, and may contribute to the pathogenesis of hippocampal lesions.- - - - - - - - - - ranking = 1keywords = sclerosis (Clic here for more details about this article) |
8/55. Partial and significant reversal of progressive visual and neurological deficits in multiple sclerosis: a possible therapeutic effect.A 24-year-old woman developed over 18 months fluctuating and progressively increasing visual and cerebellar deficits, due to multiple sclerosis. The minimum visual acuities were 3/19 on the right and the perception of finger movement at 1 m on the left. She became able to walk only with a wide base and with support. Her condition deteriorated despite the use of methylprednisolone, prednisone and interferon beta-1b, so these were ceased. azathioprine, 25 mg daily increasing to 100 mg daily, reducing following abnormal liver function tests to 50 mg daily, and glatiramer acetate 20 mg daily were commenced. The visual acuities two months later were 3/7.5 on the right and 3/6 on the left and after 4 months 3/4.5 on the right and 3/3 on the left. She was able after 2 months to walk unaided on a narrow base and after 4 months to jog half a kilometre without difficulty. Progressive multiple sclerosis has been held to be irreversible. The response in the present case therefore raises the possibility of a drug effect. It indicates the need for trials of combined immunomodulatory and immunosuppressive therapies in progressive multiple sclerosis.- - - - - - - - - - ranking = 142.10080519845keywords = multiple sclerosis, sclerosis (Clic here for more details about this article) |
9/55. Polyneuropathy and anasarca: evidence for a new connective-tissue syndrome and vasculopathic contribution.A 41-year-old woman manifested a polyneuropathy, anasarca, pseudotumor cerebri, hyperhidrosis and hyperpigmentation of the skin, generalized lymphadenopathy, distal esophageal dysphagia, pleuritis, platelike pulmonary atelectasis, fluctuating renal insufficiency, hepatosplenomegaly, amenorrhea, and slight fever suggesting a connective-tissue disorder. Extensive clinical and laboratory evaluation did not support the initial impression of progressive systemic sclerosis or systemic lupus erythematosus but did show nonnecrotizing vascular changes, mild polyclonal gammopathy, and low thyroxin levels similar to the syndrome of polyneuropathy and endocrine disturbances recently reported from japan. The impressive response to moderate-dose corticosteroids and exacerbation on withdrawal require diagnostic awareness of this insidiously progressive multisystem disorder. A bland vasculopathic process resulting from metabolic or immunologic disturbances appears to be the best explanation for this new syndrome, which has previously been recognized only in japan.- - - - - - - - - - ranking = 1keywords = sclerosis (Clic here for more details about this article) |
10/55. Migraine, memory loss, and "multiple sclerosis ". Neurological features of the antiphospholipid (Hughes') syndrome.The antiphospholipid syndrome (APS, Hughes' syndrome), first described in 1983, is a prothrombotic disease in which neurological events feature prominently. Strokes, transient ischaemic attacks, and headaches (including migraine) are important complications. However, it is clear that other neurological symptoms, including diplopia, memory loss, ataxia, and "multiple sclerosis-like" features are common. A notable feature of Hughes' syndrome is the clinical response to anticoagulants; features such as headache and memory loss often improving dramatically with appropriate warfarin dosage. APS may well become recognised as an important (and potentially treatable) cause of neurological disease.- - - - - - - - - - ranking = 101.50057514175keywords = multiple sclerosis, sclerosis (Clic here for more details about this article) |
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