J de Sèze

Publications (27)23.85 Total impact

• Article: Effect of natalizumab on clinical and radiological disease activity in a French cohort of patients with relapsing-remitting multiple sclerosis.

  A Melin, O Outteryck, N Collongues, H Zéphir, M C Fleury, F Blanc, A Lacour, J C Ongagna, A S Berteloot, P Vermersch, J de Sèze
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  ABSTRACT: "Disease activity free" in relapsing-remitting multiple sclerosis (RRMS) is a new concept introduced by the results of the AFFIRM study. Our objective was to analyze the clinical and radiological efficacy of natalizumab treatment in actual clinical practice and compare it with the post hoc analysis of the AFFIRM study. All patients with RRMS who began treatment with natalizumab at our two French MS centres between April 2007 and May 2008 were included and followed-up for at least 2 years. No measurable disease activity ("disease activity free") was defined as no activity on clinical measures (no relapses and no sustained disability progression) and radiological measures (no gadolinium-enhancing lesions and no new T2-hyperintense lesions on cerebral MRI). A total of 193 patients were included. Natalizumab was discontinued in 25.9% of cases before the completion of 2 years of treatment. In our cohort, we observed patients with more severe disease than in the AFFIRM study. The proportion of patients remaining free of clinical activity during 2 years of treatment was lower than in the AFFIRM study (37.8% vs. 64.3%). The proportion of patients remaining free of radiological activity during 2 years of treatment was higher than in the AFFIRM study (68.9% vs. 57.7%), while the proportion of patients remaining free of disease activity during 2 years of treatment was comparable to the AFFIRM study (33.3% vs. 36.7%). Natalizumab seems to be as effective in a real-life setting as in pivotal and post hoc studies. The confirmation of such benefits is important because of the progressive multifocal leukoencephalopathy risk.
  Journal of Neurology 12/2011; 259(6):1215-21. · 3.47 Impact Factor
• Article: [Recurrent inflammatory optic neuritis and neuromyelitis optica].

  J de Sèze, C Arndt
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  ABSTRACT: Inflammatory optic neuritis (ON) represents a frequent clinical situation in neurology and ophthalmology. When MRI and CSF analysis are normal, ON is considered idiopathic with a suspected viral etiology. However, in several cases either a recurrence or a myelitis may occur. In the first case, it is relapsing inflammatory optic neuritis (RION) and in the second case it is neuromyelitis optica (NMO). Nevertheless, predictive criteria of a recurrence or an extension of the disease to spinal cord remains unknown, excepted for anti-NMO IgG antibodies which are probably highly specific for a future evolution to NMO. In the present paper, the authors successively present the two clinical situations (RION and NMO) and attempt to summarize diagnostic and prognostic criteria.
  Revue Neurologique 12/2010; 166(12):966-9. · 0.49 Impact Factor
• Article: [Acute transverse myelitis].

  J de Sèze
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  ABSTRACT: Acute transverse myelopathy (ATM) is a large group from various etiologies including multiple sclerosis, systemic diseases, post-infectious myelopathy, spinal cord infarct and neuromyelitis optica. In about 20% of cases, the etiology remains unknown even after a long-term follow-up. We call these cases "idiopathic ATM". The poor prognosis of ATM makes necessary an early diagnosis to avoid therapeutic delay. The aim of this review is to describe clinical and laboratory data concerning ATM allowing to improve diagnostic and therapeutic approaches.
  La Revue de Médecine Interne 10/2009; 30(12):1030-7. · 0.61 Impact Factor
• Article: Demographic and clinic characteristics of French patients treated with natalizumab in clinical practice.

  Olivier Outteryck, J-C Ongagna, H Zéphir, M-C Fleury, A Lacour, F Blanc, P Vermersch, J de Sèze
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  ABSTRACT: Natalizumab is the first selective adhesion molecule inhibitor indicated for treatment of active relapsing-remitting multiple sclerosis (RRMS). Natalizumab has been available in France since April 2007. The aims of this study are to analyze demographic, clinical, and tolerance data from French patients with RRMS treated with natalizumab in actual clinical practice and to draw comparisons with patients in the pivotal AFFIRM study. All patients with RRMS in the Nord-Pas de Calais and Alsace regions of France treated with natalizumab at any time since April 2007 were included. Variables analyzed included previous treatments; disability status [Expanded Disability Status Scale (EDSS) score]; annualized relapse rate (ARR) at baseline and after 12 months of treatment; and adverse events. Data from 384 patients (72% female) were evaluated. Mean baseline EDSS score was 3.53 and mean baseline ARR was 2.19, both significantly greater than in AFFIRM. One hundred twenty-seven patients completed 12 months of treatment; mean EDSS score in this group was 3.02 (14% reduction) and mean ARR was 0.59 (73% reduction). Although these patients had significantly different baseline characteristics and greater disability compared with patients receiving natalizumab in AFFIRM, average disability remained stable and ARR declined by 73%. Tolerability was similar to that observed in AFFIRM.
  Journal of Neurology 09/2009; 257(2):207-11. · 3.47 Impact Factor
• Article: [Acute myelitis associated with systemic diseases].

  J de Sèze, E Hachulla
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  ABSTRACT: Myelitis secondary to systemic disease is a rare condition but clinical involvement is frequently severe. In case of a well-known systemic disease before myelitis diagnosis is relatively easy to perform. On contrary, when the systemic disease is not known previously the management is more difficult. This is of importance for therapeutical considerations. In the present paper we review the topic of acute myelitis secondary to systemic diseases.
  Revue Neurologique 05/2009; 165 Suppl 3:S66-9. · 0.49 Impact Factor
• Article: [Optic neuritis in multiple sclerosis: diagnosis and prognosis data].

  A Beddiaf, J de Sèze
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  ABSTRACT: The most frequent ophthalmologic sign in multiple sclerosis (MS) is optic neuritis (ON), but internuclear ophthalmoplegia, nystagmus, and ocular motor nerve palsies are also observed. This paper describes the most important signs suggesting an inflammatory origin of ON, most particularly MS. We detail new laboratory exams such as optic coherence tomography (OCT), which could be of potential interest for MS patients. Finally, we summarize the prognosis factors for disability in MS after a first episode of ON.
  Revue Neurologique 03/2009; 165 Suppl 4:S145-7. · 0.49 Impact Factor
• Article: Long-term outcome of acute and subacute myelopathies.

  S Debette, J de Sèze, J-P Pruvo, H Zephir, F Pasquier, D Leys, P Vermersch
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  ABSTRACT: We aimed to evaluate the long-term (>2 years) outcome of acute and subacute myelopathies (ASM). We systematically followed-up consecutive patients presenting with a first episode of ASM, defined by spinal cord symptoms with an onset <3 weeks and duration >or=48 h. Patients with compressive or traumatic spinal cord lesions are excluded from this report. Our cohort consisted of 170 patients (median age 39.0 years, median duration of follow-up 73.2 months). The death rate was 8.8%, Lipton and Teasdall's functional score was bad or fair in 38.2%, and 37.1% of the survivors who worked when the ASM occurred were unable to carry on with the same profession. Unfavorable functional outcome was more frequent when (1) symptoms were initially severe, (2) the lesion was located centrally on spinal cord MRI and (3) the etiology was neuromyelitis optica (NMO) or systemic disease (SD). In one-third of patients the etiology at the end of follow-up differed from the etiology suspected after the initial diagnostic workup. Over half of patients initially diagnosed with myelopathy of undetermined cause subsequently developed multiple sclerosis, NMO or SD. ASM is a severe condition with a bad or fair functional outcome and a major impact on professional activity in one-third of the patients. Central lesions on spinal cord MRI and etiologies such as NMO or SD are associated with a worse functional outcome. Finally, a long-term follow-up is important given the large number of causes that are identified at a distance from the initial event.
  Journal of Neurology 02/2009; 256(6):980-8. · 3.47 Impact Factor
• Article: [Autosomal recessive spastic ataxia of Charlevoix-Saguenay: study of a family and review of the literature].

  M Anheim, D Chaigne, M Fleury, F M Santorelli, J De Sèze, A Durr, A Brice, M Koenig, C Tranchant
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  ABSTRACT: The autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS) is a cerebellar ataxia autosomal recessively inherited characterized by an ataxic and pyramidal syndrome usually occurring near two years of age. The spastic paraparesis progressively worsens and becomes the prominent sign. Sensorial and motor axonal peripheral neuropathy is frequently reported as well as prominent retinal myelinated fibers on fundus examination. Brain magnetic resonance imaging reveals a predominantly vermian cerebellar atrophy. Mutations in SACS gene on 13q11 are responsible for ARSACS. We report two patients from a non-consanguineous French family, affected with ARSACS, due to the compound heterozygous mutations p.Ala2558Val and p.Pro536Leu. The clinical presentation is in accordance with the previously described ARSACS cases, despite the presence of mental retardation, the predominantly demyelinating peripheral neuropathy, and the evidence of asymptomatic generalized spikes and waves on electroencephalography. We described the clinical heterogeneity in this family including the age at onset of the disease and the first signs as well as the rapidity of the disease progression. We present a review of the literature on this rare disease mostly described in Quebec.
  Revue Neurologique 05/2008; 164(4):363-8. · 0.49 Impact Factor
• Article: [Recurrent inflammatory optic neuropathy].

  C Arndt, P Labauge, C Speeg-Schatz, L Jeanjean, M Fleury, G Castelnovo, L Ballonzolli, F Blanc, B Carlander, J De Sèze
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  ABSTRACT: To analyze clinical and paraclinical characteristics of recurrent isolated optic neuropathy. and method: In three university hospitals (Montpellier, Nimes, and Strasbourg), between October 2005 and September 2006, the charts of patients with corticosensitive recurrent isolated optic neuropathy and normal cerebral magnetic resonance imaging included prospectively were reviewed. The following parameters were analyzed: date of the first relapse, age at onset, duration at the time of inclusion, recurrence after steroid withdrawal, unilateral or bilateral involvement, number of relapses, visual acuity, retinal nerve fiber layer thickness, diagnostic workup, and long-term treatment with immunosuppressive or immunomodulatory drugs. During the predefined period, 13 patients (11 women, 2 men; age, 17-54 years at onset) matched the inclusion criteria. Between two and six relapses of optic neuropathy were observed. The median duration was 4 years. In untreated patients (n=7), a significant (Spearman p=0.0156) inverse correlation was observed between visual acuity and duration of the disease; this correlation was not found in the group of patients (n=6) with long-term treatment (Spearman p=0.1032). The progressive loss of vision over time in this retrospective study of recurrent isolated optic neuropathy could be related to axonal loss. A prospective cohort study is necessary to confirm this hypothesis and to evaluate the benefit of long-term treatment on this progression.
  Journal francais d'ophtalmologie 05/2008; 31(4):363-7. · 0.51 Impact Factor
• Article: [Cognitive impact of mitoxantrone and methylprednisolone in multiple sclerosis: an open label study].

  H Zéphir, J de Sèze, K Dujardin, G Dubois, M Cabaret, S Bouillaguet, D Ferriby, T Stojkovic, P Vermersch
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  ABSTRACT: Interferons beta have shown some positive effects on cognitive function in multiple sclerosis (MS). The potential immunosuppressive impact of mitoxantrone on cognitive dysfunction in MS has never been evaluated. We assessed changes in cognitive dysfunction in patients with very active MS treated with mitoxantrone combined with methylprednisolone. We assessed a non randomized controlled trial including successively 15 consecutive MS patients. Very active MS was defined by a progression of at least two EDSS points or more than two relapses during the previous year and at least one enhanced lesion after gadolinium infusion on MRI. All patients received a monthly intravenous pulse of mitoxantrone (20mg) for six months with methylprednisolone (1g). Global cognitive efficiency, memory and executive function were assessed before treatment (M0) and after six months (M6) and 12 months (M12) of treatment. To evaluate the learning effect, 15 healthy subjects also participated. A significant improvement in global cognitive efficiency was observed at M6 and was sustained at M12, as a few parameters on memory and executive functions. We suggest that mitoxantrone combined with methylprednisolone has a potential positive effect on cognitive functions.
  Revue Neurologique 02/2008; 164(1):47-52. · 0.49 Impact Factor
• Article: [Devic disease associated with isolated spinal cord atrophy].

  N Landragin, L Jeanjean, S Bouly, J Honnorat, J de Sèze, G Castelnovo, P Labauge
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  ABSTRACT: Devic disease is a rare entity characterized by bilateral optic neuritis and transverse myelitis. Recently, recognition of antibody activity (Anti NMO) led to broaden the clinical and MR phenotype spectrum of this disease. This report is about a patient with spinal cord atrophy and bilateral optic neuritis, occurring more than 8 years after symptom onset.
  Revue Neurologique 01/2008; 163(12):1236-8. · 0.49 Impact Factor
• Article: [Cortical blindness associated with Guillain-Barre syndrome: a complication of dysautonomia?].

  S Delalande, J De Sèze, J-P Hurtevent, T Stojkovic, J-F Hurtevent, P Vermersch
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  ABSTRACT: We report a case of a Guillain-Barre syndrome (GBS) with subarachnoid hemorrhage and regressive occipital white matter lesions. A 62-year-old woman developed ascendant progressive paresthesia and weakness of arms and legs, 48 hours after enteritis infection. Neurological examination showed tetraparesia with loss of deep tendon reflexes and alteration of proprioception tests. Nerve conduction studies revealed polyradiculoneuritis. Then she presented an acute blindness and hypertension. Brain magnetic resonance imaging showed bilateral occipital lesions and subarachnoid hemorrhage. Cerebrospinal fluid analysis revealed an elevated protein level (1.54 g/l) and red blood cells without meningitis. Brain arteriography was normal. Intravenous immunoglobulins improved neurological symptoms. Posterior localisation of reversible white matter lesions evoked a reversible posterior leukoencephalopathy. The implication of arterial hypertension caused by dysautonomia during GBS could be suspected.
  Revue Neurologique 05/2005; 161(4):465-7. · 0.49 Impact Factor
• Article: [Presentation of Niemann-Pick type C disease with psychiatric disturbance in an adult].

  L Tyvaert, T Stojkovic, J-M Cuisset, M-T Vanier, J-C Turpin, J De Sèze, P Vermersch
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  ABSTRACT: Niemann-Pick Type C disease (NPC) is an autosomal recessive neurovisceral lysosomal lipid storage disorder. A 31-year-old right-handed woman had suffered from schizophrenia for 13 years. At 25 years of age, she developed a gait disorder with a static and kinetic cerebellar syndrome, dysarthria, vertical supranuclear gaze palsy and cognitive impairment. Brain MRI was normal. Abdominal ultrasonography was performed because of hypercholesterolemia and elevated transaminases and revealed hepatosplenomegaly, which in conjunction with other signs and symptoms, suggested the diagnosis of NPC. The diagnosis was confirmed by demonstration of lysosomal storage of unesterified cholesterol (filipin staining) and of a reduced rate of LDL-induced cholesterol esterification. Implication of the NPC1 gene was assessed by genetic complementation analysis. The phenotypic presentation of NPC is remarkably variable. The rarer adult-onset form has a slowly progressive course. Psychotic manifestations are often prominent and may precede neurologic symptoms. Exposure to neuroleptics delays the diagnosis of NPC. Psychotic manifestations associated with cerebellar syndrome, vertical supranuclear gaze palsy, and splenomegaly are very suggestive of NPC disease which can be reliably diagnosed on cultured skin fibroblasts by filipin staining.
  Revue Neurologique 04/2005; 161(3):318-22. · 0.49 Impact Factor
• Article: [Guillain-Barré syndrome with hallucinations and onirism: an underestimated association].

  E Le Rhun, J de Sèze, T Stojkovic, D Ferriby, F Fourrier, P Vermersch
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  ABSTRACT: Guillain-Barré Syndrome (GBS) is generally related to peripheral nervous system involvement, but certain variants with central nervous system manifestations have been described. In the present study we report 2 patients with GBS associated with hallucinations and onirism. Two men (age 64 and 49 years) presented GBS without proven infectious origin who required intensive care because of respiratory problems. The disease progressed and manifestations of encephalitis (hallucinations and onirism) appeared. The sensorimotor signs and encephalitis manifestations evolved in parallel with full recovery in the first patient and death after 11 months of intensive care in the second. GBS may be associated with stereotypic central nervous system symptoms, mimicking delirium tremens. The manifestations would be related to the severity of the initial period, but not to long-term prognosis.
  Revue Neurologique 01/2005; 160(12):1207-10. · 0.49 Impact Factor
• Article: [Ataxic neuropathy associated with disialosylated antibodies: description of new clinical and biochemical forms].

  A Delval, T Stojkovic, J de Sèze, J-F Hurtevent, F Glowacki, A Beaume, A Destée, P Vermersch
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  ABSTRACT: Polyneuropathies associated with IgM monoclonal gammopathy were recently recognized. Antibodies can react with glycoproteins such as myelin associated glycoprotein (MAG), or gangliosides containing one sialosyl epitope such as GM1 or several sialosyl epitopes (polysialyted gangliosides) including GD2, GD3, GT1b, GT1a, GQ1b. We report on three patients presenting oculomotor dysfunction, chronic sensitive ataxic polyneuropathy, high sedimentation rate, IgM monoclonal paraprotein of unknown signification and antidisialosyl IgM antibodies and for two of them cold agglutinins. Such features have been previously described under the acronym "CANOMAD" (chronic ataxic neuropathy with ophthalmoplegia, M protein, agglutination and disialosyl antibodies). One of the patients presents extramembranous glomerulopathy and severe motor disability associated with this syndrome. The pathophysiology of the glomerulopathy seems to be linked with the polyneuropathy. Patients were treated either by intravenous immunoglobulin, corticosteroids or cyclophosphamid. Response to treatment differs in the three cases and there is currently no consensus. Our study demonstrates that spectrum of polyneuropathy associated with monoclonal polyneuropathy may be larger than originally described.
  Revue Neurologique 11/2004; 160(10):910-6. · 0.49 Impact Factor
• Article: [Pure subacute pandysautonomia: an assessment of treatment with intravenous polyvalent immunoglobulins].

  C Ramirez, J de Sèze, T Stojkovic, D Ferriby, S Delalande, S Defoort-Dhellemmes, P Vermersch
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  ABSTRACT: Acute or sub-acute pure dysautonomia is uncommon. We report a case of sub-acute pure pandysautonomia with favorable outcome after intravenous immunoglobulin therapy. A 29-year-old right-handed student, with an uneventful medical history presented, for one month, bilateral loss of visual acuity and digestive disorders, associating diarrhea, vomiting and anorexia. Physical examination revealed bilateral intrinsec oculomotor nerve palsy, a dryness syndrome and severe orthostatic hypotension. Ophthalmologic examination showed bilateral diffuse parasympathic impairment associating an Argyll Robertson pupil and full pupil light reflex abolition. Elevated protein level (0.93g/l) was the only cerebrospinal fluid anomaly. Serum tests were negative for anti-gangliosides antibodies. The patient improved slowly after two series of intravenous immunoglobulin infusions. Clinical course and laboratory findings suggest that acute or sub-acute pure pandysautonomia events are likely to be related to acute polyradiculoneuritis. Therefore intravenous polyvalent immunoglobulin infusions should be attempted, even if their efficacy needs to be confirmed.
  Revue Neurologique 11/2004; 160(10):939-41. · 0.49 Impact Factor
• Article: [BCcogSEP: a French test battery evaluating cognitive functions in multiple sclerosis].

  K Dujardin, P Sockeel, M Cabaret, J De Sèze, P Vermersch
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  ABSTRACT: In French language, there is no standardized procedure to assess cognitive function in patients with multiple sclerosis (MS). Such an assessment is however very useful to determine the consequences of the disease on cognitive function, to evaluate the disease progression and the consequences of usual treatments on cognition. This study aimed to develop and validate a French language battery based on "the Brief Repeatable Battery of Neuropsychological tests for Multiple Sclerosis" (BRB-N) often used in other countries. In an initial phase, the battery was composed of the French version of the 5 BRB-N tests to which were added 4 tests assessing immediate and working memory as well as executive function. 52 healthy control subjects (20-50 years) participated in the study. A principal component analysis (PCA) of their data examined the contribution of each test into the battery. 93 other healthy subjects participated in a second phase where analyses of variance were carried out to investigate the effect of the main demographical variables. Finally, the performance of two patient groups (20 with a EDSS score<or=3 et 31 with a EDSS score>3) was compared to the healthy controls in order to investigate the ability of our battery to detect cognitive impairment in MS patients. The PCA showed that each test, except one, had a specific contribution to the battery. The final battery (BCcogSEP) was thus comprised of 8 tests. MANOVA and ANOVA showed significant effects of age, sex and educational level on performance. In consequence, a procedure allowing to take into account these factors was developed. The battery was able to detect cognitive impairment in MS patients, even when the disease is not severe. In this case, deficits were observed in tests assessing executive function, information processing speed, immediate and working memory. This short battery with reliable psychometric qualities allows the interpretation of a MS patient's performance considering his/her demographical characteristics and is able to detect cognitive abnormalities even in case of mild physical handicap.
  Revue Neurologique 01/2004; 160(1):51-62. · 0.49 Impact Factor
• Article: Hemiparkinsonism revealing an infiltrating low-grade oligodendroglial tumor.

  J de Sèze, L Defebvre, M M Ruchoux, S Blond, J De Reuck, A Destée
  European Neurology 12/1998; 40(4):234-6. · 1.81 Impact Factor
• Article: [Brain stem metastasis revealing bronchopulmonary cancer: 2 cases].

  J de Sèze, G Touzet, C Lucas, O Godefroy, S Blond
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  ABSTRACT: Brainstem metastasis is a very rare revealing feature of lung carcinoma. We report two cases of brainstem metastasis revealing lung carcinoma. In the first case, diagnosis was made on stereotaxic biopsy. In the second, the biopsy was not necessary as lung lesion was observed on thoracic CT-scan. Diagnosis of metastasis is difficult when a brainstem tumor occurs. Stereotaxic biopsy is an interesting tool to distinguish between primary lesion and metastasis, but only when diagnosis work-up of primary cancer is negative.
  La Revue de Médecine Interne 05/1998; 19(4):271-4. · 0.61 Impact Factor
• Article: [MRI aspect and course of supra-tentorial sarcoidosic lesions].

  D Caparros-lefebvre, B Wallaert, I Girard-Buttaz, J De Sèze, S Blond, M M Ruchoux, J P Pruvo, H Petit
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  ABSTRACT: Central nervous system lesions resulting from sarcoidosis occur in 5% of cases, but supratentorial mass lesions are uncommon. We report 3 cases of intracranial pseudo-tumoral lesions, due to sarcoidosis: 1 woman and 2 men. Clinical features included left facial myoclonus, headache and vertigo, right hemiparesis and unique general seizure. Sarcoidosis was diagnosed upon conjonctival biopsy in the first case, infiltrative lesions of the lungs and mediastinal lymph nodes in the second case, and intracerebral lesion plus lymph nodes biopsies in the last case. In the first case, CT scan and MRI images showed diffuse subcortical high signal, suggesting pachymeningitis with vasogenic oedema. In the second case, GT scan and MRI revealed numerous small granuloma in the left rolandic area, and one in the striatum. In the third case, CT scan showed an enlargement of the left temporal horn, due to an enhanced left periventricular lesion. MRI with gadolinium showed that the lesion encircled the ventricle horn. In all cases, clinical and radiological improvement was obtained after corticoid therapy.
  Revue Neurologique 04/1996; 152(3):196-201. · 0.49 Impact Factor