Alpha-fodrin autoantibodies in the differential diagnosis of MS and Sjögren syndrome.
Explore the source record for details and available documents.
Adult↗
Biomedical subjects
D Ferriby
Publications and source records attributed to D Ferriby.
Devic's neuromyelitis optica: clinical, laboratory, MRI and outcome profile.
Devic's neuromyelitis optica (NMO) associates optic neuritis and myelitis without any other neurological signs. Many patients with NMO may be diagnosed as having multiple sclerosis (MS), optic neuritis and myelitis being the inaugural symptom in 20% and 5% of MS cases, respectively. The aim of our study was to compare a new NMO cohort with recent studies and to try to determine the place of NMO in the spectrum of MS. We retrospectively studied 13 patients with a complete diagnostic workup for NMO. We compared our data with the most recent studies on NMO and with the criteria proposed by Wingerchuck et al. [Neurology 53 (1999) 1107]. We also determined whether these patients fulfilled the diagnostic criteria for MS. Thirteen patients (10 women and three men, with a mean age of 37.4 years) were included in the study. We found similar results to previously published data, except for an association with vasculitis in 38% of our cases. All but three of the patients fulfilled the clinical criteria for MS and two patients fulfilled both clinical and MRI criteria for MS. However, if we applied more restrictive criteria concerning spinal cord and brain MRI and CSF, none of our NMO patients fulfilled the MS diagnostic criteria. NMO might therefore be differentiated from MS by the application of more stringent criteria. Furthermore, all NMO patients should be investigated for vasculitis, even those with no history of systemic disease.
Adrenal Cortex Hormones↗
[Interferons and neurologic diseases].
PURPOSES: Therapeutic strategies using interferons in neurology are limited to multiple sclerosis. In this disease, only beta interferons are used and now considered as a first line treatment in the relapsing-remitting forms of the disease. MAIN POINTS: Beta interferons have reduced the relapse rate by approximately 3% and also reduced the risk of disability progression. They have also favorably influenced the brain lesions on MRI. Numerous data have stressed the importance of starting treatment as early as possible. In the secondary form of the disease, the impact of beta interferons are still discussed. In some clinical endpoints, a dose-response has been demonstrated, probably related to the frequency of injections. Side effects are acceptable despite the high frequency of flu-like syndrome. PERSPECTIVES: Recent data argue for beta interferon treatment after the first demyelinating episode in subjects at risk for early new relapses. In some patients, in cases of treatment failure, combination strategies, including beta interferons, should be addressed in the near future. Interferons were also tested with disappointing results in other neuro-inflammatory disorders, such as chronic inflammatory demyelinating neuropathies.
Adjuvants, Immunologic↗
[Treatment of progressive multiple sclerosis with cyclophosphamide].
Intravenous (IV) cyclophosphamide is currently used in secondary progressive (SP) and Primary progressive (PP) Multiple Sclerosis (MS) but its efficacy remains uncertain. Furthermore, it is necessary to determine which MS should be successfully treated with IV cyclophosphamide. We retrospectively investigated 111 consecutive patients with progressive MS (21 PPMS and 90 SPMS) treated in an open label fashion with IV cyclophosphamide. We analysed clinical data (gender, age, duration of progression, primary versus secondary MS). The treatment response was assessed by EDSS change after 6 months and 1 year of treatment. The annual relapse average decreased from 1.92 before treatment to 0.39 during the treatment. Age and gender did not influence response to therapy. We did not find any difference of response between PPMS and SPMS. Duration of the progressive phase in SPMS was not a predictive factor of efficacy. A better response was noted in SPMS patients with surimposed relapses than in patients without relapses during the year before treatment (p<0.05). Furthermore, the better response in SPMS patients with relapses before treatment suggests that it is necessary to treat when MS is still in an inflammatory stage.
Adult↗
[Acute rhombencephalitis with fever and bilateral hearing loss secondary to Gougerot-Sjögren's syndrome].
The usual polymorphic central nervous system involvement observed in primary Sjögren's syndrome is classified as monofocal, multifocal or diffuse involvement. We present a case of acute rhombencephalitis associated with hearing loss that could correspond to an intermediate form between diffuse and monofocal involvement. The acute onset with fever and not clinical sign of xerostomia or xerophthalmia suggest the possibility of vascular-like or infectious-like forms. We emphasize the importance of obtaining a minor salivary gland biopsy for diagnosis of Sjögren's syndrome and suggest that Sjögren's syndrome could be frequently misdiagnosed.
Abducens Nerve Diseases↗
[Primary progressive forms of multiple sclerosis: application of the new diagnostic criteria in French].
Approximately 15 p.100 of all cases of multiple sclerosis (MS) are primary progressive multiple sclerosis (PPMS). Diagnosis is however frequently delayed due to the lack of relapse. The commonly used Poser criteria established in 1983 are not directly applicable to this subgroup of MS patients. In 2000, Thompson et al proposed new diagnostic criteria for PPMS. The aim of our study was to apply retrospectively these new criteria to a cohort of patients from northern France (G-SEP cohort). The cohort included 184 patients (94 women and 90 men, sex ratio=1.04). Mean age at disease onset was 40.3 years (18-67 years) and mean follow-up was 9.9 (1-39 years). Only one patient was older than 65 years but 13 patients (7.1 p.100) were younger than 25 years. Patients were classed as having definite, probable or possible PPMS on the basis of clinical, MRI, cerebrospinal fluid and visual evoked potential (VEP) data. Most of the patients (61.4 p.100) had one symptom at onset; spastic paraparesis was frequent (79 p.100). Mean delay to diagnosis was 4.4 years. Ten patients (5.4 p.100) were surgically treated for cervic arthrosic myelopathy. Mean EDSS score at the end of follow-up was 5.8 (3-10). MRI was positive in 87.3 p.100 of the patients. Oligoclonal bands were found in 78.2 p.100. VEPs ware abnormal in 79.9 p.100 of the patients. Applying the Thompson criteria, 57.7 p.100 of the patients had definite PPMS, 38.7 p.100 probable PPMS. Our results are very similar to previous studies and confirm the usefulness of the new proposed criteria, which however should be prospectively tested to determine sensitivity and specificity in a new cohort of patients.
Adolescent↗
[Chronic polyradiculoneuritis disclosing sarcoidosis].
Peripheral Nervous System (PNS) involvement occurs in 10 to 40 percent of patients presenting with sarcoidosis. Axonal polyneuropathies and mononeuropathies are the most frequently reported whereas acute or chronic polyradiculoneuritis are rarely described. We report the case of a 54 year-old-woman admitted for a progressive sensorimotor deficit of the lower limbs. Bilateral ocular redness corresponding to an uveitis was also observed. Neurological examination and electrophysiological data were consistent with a chronic polyradiculoneuritis. The neurological status of the patient improved after six months of treatment with oral corticotherapy associated with immunosuppressive treatment. Our case allows a review of the literature concerning PNS involvements and treatments in sarcoidosis.
Brain↗
[Epstein-Barr meningoencephaloradiculitis in a immunocompetent woman].
Neurological manifestations of viral diseases are predominantly secondary to herpes viridae infections. Among them Epstein-Barr (EBV) infections are rare particularly in immunocompetent adults. A 52 year old right-handed woman was admitted for progressive walking difficulties. She presented a proximal limb weakness with proprioceptive ataxia and pain because of lumbosacral radiculopathy confirmed by electromyography. Drowsiness and mnesic troubles were explained by encephalitis on electroencephalography. Brain MRI showed an enhancement of the sub-arachnoïd spaces. The diagnosis of meningoencephaloradiculitis due to primary EBV infection was supported by a mononucleosis syndrome, an increased antibody level and positive EBV PCR in the CSF. Clinical features spontaneously improved without antiviral treatment. Primary EBV infections are rare in adults, as more than 95p.cent of them are positive for EBV serology after 20 years of age. EBV PCR is CSF in an important diagnostic tool allowing to stop antiviral therapies in these cases having a good prognosis.
Antibodies, Viral↗
[Late onset multiple sclerosis].
Multiple sclerosis (MS) with clinical onset after 50 years old is unusual and frequently misdiagnosed. Clinical presentation and course seem also to be different that in MS occurring between 20 and 50 years old. The aim of this study was to evaluate the clinical and paraclinical characteristics of late onset MS. We respectively studied MS patients older than 50 years at the onset of the disease. We evaluated demographical data, clinical symptoms, cerebrospinal fluid (CSF), visual evoked potentials (VEPs) and MRI of these patients. We also studied clinical data during the follow-up with the occurrence of new symptoms and the evolution of the disease by the index of progression (EDSS unit per year). In a population of 1 417 MS, 3.4 p.cent had their first symptoms at 50 years old or older and 0.45 p.cent after 59 years old. At the time of the study patients had more frequently a progressive form: 37 p.cent had a primary progressive form and 35 p.cent a secondary progressive MS. None of the patients with onset after 60 years old had relapsing remittent MS. Motor symptoms were the most common neurologic presentation (54 p.cent of patients). Very few patients had a clinical optic nerve involvement during the follow-up. The mean progression index was 1 suggesting a most severe evolution in this subgroup of MS patients. 76 p.cent of patients had oligoclonal banding detected by CSF electrophoresis. The VEPs were abnormal in 81 p. cent of patients tested. 71 p.cent of the brain MRI were consistent with the diagnosis of MS. 60 p.cent of patients had spinal cord MRI abnormal. This study highlights the differences between the late onset MS and earlier onset. As previously reported, our study underlines the high frequency of progressive course, motor function involvement and poor prognosis.
Age of Onset↗
The prevalence of Sjögren syndrome in patients with primary progressive multiple sclerosis.
OBJECTIVE: To assess the prevalence of Sjögren syndrome (SS) in patients with primary progressive MS (PPMS). BACKGROUND: SS may be considered in the differential diagnosis of MS. Age at onset and clinical presentation are similar in SS and PPMS. However, occurrence of SS in definite cases of PPMS has been recently reported. METHODS: Proposed clinical and laboratory diagnostic criteria for SS were systematically assessed in 60 consecutive patients with PPMS. The authors questioned all patients about xerophthalmia and xerostomia, biopsied minor salivary glands, and performed a Schirmer test, a salivary gland scintigraphy, and anti-Ro (SSa) and anti-La (SSb) serologies. RESULTS: Ten patients (16.6%) met four or more criteria for SS. This prevalence is higher than in the general population (1 to 5%) and implies that SS can mimic PPMS. CONCLUSIONS: The authors propose that SS should be screened for systematically in patients with PPMS.
Adult↗
Long-term follow-up of neurosarcoidosis.
The authors evaluated the long-term clinical outcome of neurosarcoidosis and determined predictive factors of disease course. Twenty-seven patients with neurosarcoidosis were followed for at least 5 years from the onset of neurologic symptoms. Patients with CNS involvement during the course of the disease had a higher Modified Oxford Handicap Scale score than those with peripheral nervous system involvement (p < 0.02). CNS involvement may be a predictive factor for a less favorable disease course. Early and intensive treatment should be considered in such cases.
Adolescent↗
Clinical picture: bronchiolitis obliterans with organising pneumonia during interferon beta-1a treatment.
Explore the source record for details and available documents.
Cryptogenic Organizing Pneumonia↗
[Kjellin syndrome].
A previously healthy 30-year-old woman who had cognitive impairment since childhood suddenly developed progressive spastic paraparesis. Visual impairment and characteristic retinal macular spots supported the diagnosis of Kjellin syndrome. This disease, probably transmitted by autosomal recessive inheritance, is seldom observed in clinical practice. We describe the characteristics of Kjellin syndrome and the differential diagnosis, including other macular changes associated with spastic paraparesis.
Adult↗
[Cytomegalovirus rhombencephalomyelitis in an immunocompetent subject].
We report the clinical and MRI findings of a 31-year-old healthy immuno-competent patient who presented following 48 hours of diffuse headache with progressive and severe rhombencephalomyelitis signs. Cerebral and medullar MRI confirmed the central nervous system improvement. Serology showed relation with cytomegalovirus infection. Spontaneous improvement was observed and favoured by ganciclovir administration.
Adult↗
[Sarcoidosis and progressive multifocal leukoencephalopathy].
A 70-year-old woman treated for sarcoidosis complained of progressive cognitive impairment and gait disability. Magnetic resonance imaging of the brain revealed a nonenhancing lesion in T1-weighted imaging in the left parieto-occipital region and sarcoidosis of the central nervous system was evoked. However, she rapidly deteriorated with posterior and cerebellar extension of the lesions, suggesting of progressive multifocal leukoencephalopathy (PML). DNA of the JC virus (JCV) was detected in the cerebrospinal fluid (CSF) by a polymerase chain reaction. Despite antiviral therapy, she died nine months after the first neurological signs. This case illustrates the possible association between sarcoidosis and PML, and underlines the interest to detect the presence of JCV in the CSF when the diagnosis of neurosarcoidosis appeared uncertain.
Aged↗
Unusual MR findings of the brain stem in arterial hypertension.
MR imaging findings have been reported in only a few cases of severe arterial hypertension. We report two cases of severe paroxysmal arterial hypertension associated with unusual brain stem hyperintensity. The lesions improved dramatically after stabilization of blood pressure, suggesting that edema could be the main cause of the MR imaging-observed hyperintensity.
Adrenal Gland Neoplasms↗
[Clinical manifestations and therapeutic approach in neurosarcoidosis].
Neurological impairment is a frequent cause of morbidity and mortality in patients with sarcoidosis. The aim of this study was to evaluate the clinical manifestations of the disease, the response to corticosteroids and alternative treatments. During a 5 year period, diagnosis of neurosarcoidosis was performed in 40 patients. We retrospectively analyzed clinical, laboratory data and response to treatments. Mean age was 41.3 years (range 17-72). Mean time of follow-up was 46 months. Neurologic signs were the first symptom in 50 p. 100 of cases and an isolated manifestation in 12.5 p. 100. Central nervous system impairment was seen in 60.7 p. 100, meningitis in 27 p. 100. Other clinical manifestations were cranial nerve palsies (27 p. 100), peripheral neuropathy (33 p. 100), myopathy (16 p. 100). Eighty percent of the patients were treated by corticosteroids. Because of a lack of efficacy 40 p. 100 of patient required alternative treatment (including methotrexate, cyclophosphamide, azathioprin, cyclosporin). Complete recovery was observed in only 27.5 p. 100 of cases confirming the severity of neurosarcoidosis. Forty percent of patients were clinically stable and 10 p. 100 worsened. No patient died. This study confirms that intensive initial treatment is often necessary to prevent irreversible lesions. Alternative treatment should be rapidly initiated in resistant forms.
Adolescent↗
[Magnetic resonance imaging of the facial nerve in a case of Melkerson-Rosenthal syndrome].
A 35-year-old man treated for a Crohn's disease presented with a second facial palsy in a setting of recurrent labial edema known since childhood. The diagnosis of Melkerson-Rosenthal syndrome was established. MRI showed a small T1 gadolinium-enhanced lesion of the facial nerve suggesting an inflammatory process. Similarities of pathologic lesions found in Melkerson-Rosenthal syndrome, Crohn's disease and sarcoidosis raise the question of the relationships between these disorders.
Adult↗