Multiple sclerosis and related disorders, Jan 2, 2018
Though the Expanded Disability Status Scale (EDSS) is commonly used to assess disability level in... more Though the Expanded Disability Status Scale (EDSS) is commonly used to assess disability level in relapsing-remitting multiple sclerosis (RRMS), the criteria defining disability progression are used for patients with a wide range of baseline levels of disability in relatively short-term trials. As a result, not all EDSS changes carry the same weight in terms of future disability, and treatment benefits such as decreased risk of reaching particular disability milestones may not be reliably captured. The objectives of this analysis are to assess the probability of confirmed disability worsening to specific EDSS milestones (i.e., EDSS scores ≥3.0, ≥4.0, or ≥6.0) at 288 weeks in the Tysabri Observational Program (TOP) and to examine the impact of relapses occurring during natalizumab therapy in TOP patients who had received natalizumab for ≥24 months. TOP is an ongoing, open-label, observational, prospective study of patients with RRMS in clinical practice. Enrolled patients were naive ...
Skip to Main Content. ... Fulminant thrombotic thrombocytopenic purpura in two patients with syst... more Skip to Main Content. ... Fulminant thrombotic thrombocytopenic purpura in two patients with systemiclupus erythematosus and phospholipid autoantibodies. M. Osman Musa 1 ,; RandaNounou 2 ,; Entezam Sahovic 1 ,; Priti Seth 1 ,; Abdulelah Qadi 1 ,; Mahmoud Aljurf 1. ...
Résumé/Abstract Nous rapportons un cas de purpura thrombotique thrombocytopénique (PTT) fulminant... more Résumé/Abstract Nous rapportons un cas de purpura thrombotique thrombocytopénique (PTT) fulminant, survenu au décours d'un traitement par ciprofloxacine chez une patiente âgée de 43 ans. Une méningo-encéphalite bactérienne aiguë avec coagulation intravasculaire disséminée fut d'abord suspectée. La patiente présentait une anémie hémolytique et une thrombopénie sévère empêchant la réalisation immédiate d'une ponction lombaire. Celle-ci ne fut réalisée qu'après transfusion de plaquettes sanguines ...
Paraneoplastic choreo-athetoses are rare. We report a case of anti-Hu syndrome with choreo-atheto... more Paraneoplastic choreo-athetoses are rare. We report a case of anti-Hu syndrome with choreo-athetosis. A 48-year-old woman developed a small-cell lung carcinoma revealed by an anti-Hu syndrome. The neurological features included choreo-athetosis predominating in the upper limbs, chronic sensorimotor axonal polyneuropathy, and opsoclonus. The cerebrospinal fluid was acellular and contained several oligoclonal IgG bands, not found in the corresponding serum. Magnetic resonance imaging revealed bilateral high-intensity lesions on T2/FLAIR sequence in the corona radiata. Moderate transitory improvement of the paraneoplastic neurological syndrome was observed after several carboplatin-etoposid cycles. A paraneoplastic origin must be considered in all cases of unexplained choreo-athetosis. Paraneoplastic choreo-athetosis is most often associated with other neurological symptoms. The most frequent associated tumor is a small-cell lung carcinoma with anti-CRMP5 and/or anti-Hu antibodies. Our...
Natalizumab (Tysabri(®)) is highly efficacious in controlling disease activity in relapsing multi... more Natalizumab (Tysabri(®)) is highly efficacious in controlling disease activity in relapsing multiple sclerosis (MS) patients. As it is one of the more recent therapies for MS, there remains a need for long-term safety and efficacy data of natalizumab in a clinical practice setting. The Tysabri observational program (TOP) is an open-label, multicenter, multinational, prospective observational study, aiming to recruit up to 6,000 patients with relapsing-remitting MS from Europe, Canada and Australia. The objectives of this study are to collect long-term safety and efficacy data on disease activity and disability progression. We report here the interim results of the 563 patients included in TOP between December 2007 and 2012 from Belgium. This patient cohort was older at baseline, had longer disease duration, higher neurological impairment, and a higher baseline annualized relapse rate, when compared to patients included in the pivotal phase III AFFIRM trial. Nevertheless, the efficac...
ABSTRACT IntroductionParaneoplastic choreo-athetoses are rare. We report a case of anti-Hu syndro... more ABSTRACT IntroductionParaneoplastic choreo-athetoses are rare. We report a case of anti-Hu syndrome with choreo-athetosis.Case reportA 48-year-old woman developed a small-cell lung carcinoma revealed by an anti-Hu syndrome. The neurological features included choreo-athetosis predominating in the upper limbs, chronic sensorimotor axonal polyneuropathy, and opsoclonus. The cerebrospinal fluid was acellular and contained several oligoclonal IgG bands, not found in the corresponding serum. Magnetic resonance imaging revealed bilateral high-intensity lesions on T2/FLAIR sequence in the corona radiata. Moderate transitory improvement of the paraneoplastic neurological syndrome was observed after several carboplatin-etoposid cycles.ConclusionA paraneoplastic origin must be considered in all cases of unexplained choreo-athetosis. Paraneoplastic choreo-athetosis is most often associated with other neurological symptoms. The most frequent associated tumor is a small-cell lung carcinoma with anti-CRMP5 and/or anti-Hu antibodies. Our patient developed paraneoplastic choreo-athetosis related to an anti-Hu syndrome in the absence of anti-CRMP5/CV2 antibodies. Paraneoplastic choreo-athetosis might result from a central lesion, and/or from proprioceptive deafferentation subsequent to peripheral neuropathy.
Sirs: Cerebrospinal fluid (CSF) oligoclonal immunoglobulin (Ig) G bands are a sensitive marker of... more Sirs: Cerebrospinal fluid (CSF) oligoclonal immunoglobulin (Ig) G bands are a sensitive marker of multiple sclerosis (MS) and other inflammatory diseases of the central nervous system (CNS). However, they may also be detected in some non-inflammatory disorders, with an unknown frequency [1, 4]. We report a patient with intramedullary cavernous angiomas (ICAs) in the thoracolumbar region and CSF oligoclonal IgG bands. A 54-year-old woman was admitted to our department with a 5-year history of gait ataxia and a 3-month history of right cruralgia. On clinical examination she was seen to have a mild proximal paresis of the right leg, with brisk tendon reflexes in the lower limbs, and bilateral Babinski’s sign. There was no sensory or sphincter disturbance. Cranial nerves were normal. Serum laboratory findings were normal, including normal protein electrophoresis and negative autoimmune, antiviral and anti-Borrelia serologies. Magnetic resonance imaging (MRI) of the brain was unremarkable, and the CSF was acellular, with several oligoclonal IgG bands on immunoblotting. The multimodality evoked potentials were normal, except for a symmetrical increase in the somatosensory central conduction time after stimulation of the posterior tibial nerves. A spinal form of MS was first suspected. However, spinal cord MRI, performed 2 months later, showed two ICAs at the thoracic T10 and T11 levels (Figs. 1, 2). They each had with a reticulated centre of mixed signal intensity surrounded by a rim of hypointensity on T2weighted images. A total resection of the ICAs was performed after thoracic T9–T11 laminectomy and was followed by a transient spastic paraparesis, related to an acute spinal oedema. The patient had a chronic myelopathy, due to two thoracic ICAs. ICAs are rare and comprise 1–3% of all intramedullary tumours [2]. They may cause acute, progressive or episodic spinal cord dysfunctions [3]. Acute presentation usually results from haemorrhage. In patients presenting with a progressive myelopathy, several pathophysiological mechanisms may be proposed, such as enlargement of the malformation, neurotoxic effect of haemosiderin and compromised surrounding microcirculation [3]. MRI is the procedure of choice to detect ICAs, showing the characteristic appearance of a reticulated centre of mixed signal intensity surrounded by a rim of signal hypointensity on T2weighted images [2, 3]. ICAs must be included in the differential diagnosis of spinal dural arteriovenous malformations and both relapsing/remitting and secondary progressive types of multiple sclerosis (MS). Radical resection of the ICAs is associated with a relatively low morbidity and is advocated whenever the malformations are symptomatic [2]. In our patient the thoracic ICAs were associated with CSF oligoclonal IgG bands. The coexistence of ICAs and MS was first considered. However, MRI revealed no other spinal or cerebral lesion, suggestive of MS. Vretem et al. [4] have reported a patient with cavernous angioma of the brainstem and CSF oligoclonal IgG bands, mimicking MS. As in our case, their patient did not have any other lesion suggestive of MS on brain MRI. Recently Cohen et al. [1] have also found CSF oligoclonal IgG bands in three patients with spinal arteriovenous malformations, located in the low thoracic level. The presence of CSF oligoclonal IgG bands in some cases of CNS cavernous angiomas or spinal arteriovenous malformations does not have any known pathogenetic significance. Attention LETTER TO THE EDITORS
Background Shift work is commonly associated with health problems resulting from circadian misali... more Background Shift work is commonly associated with health problems resulting from circadian misalignment and sleep restriction. About one in three shift workers is affected by insomnia and up to 90% report regular fatigue and/or sleepiness at the workplace. Epidemiological data shows that shift workers are at increased risk of cardiovascular disease, diabetes, obesity, breast cancer, mental-health problems, and shift-work disorder, which conditions typically lead to reduced work performance, processing errors, accidents at work, absenteeism, and reduced quality of life. Given these widespread and debilitating consequences, there is an urgent need for treatments that help improve the sleep, health, and functional performance of the shift-working population. The most common non-pharmacological recommendations are improved scheduling, bright-light exposure, napping, psychoeducation promoting sleep hygiene, and cognitive-behavioral techniques. The objectives of the present study are to i...
Multiple sclerosis and related disorders, Jan 2, 2018
Though the Expanded Disability Status Scale (EDSS) is commonly used to assess disability level in... more Though the Expanded Disability Status Scale (EDSS) is commonly used to assess disability level in relapsing-remitting multiple sclerosis (RRMS), the criteria defining disability progression are used for patients with a wide range of baseline levels of disability in relatively short-term trials. As a result, not all EDSS changes carry the same weight in terms of future disability, and treatment benefits such as decreased risk of reaching particular disability milestones may not be reliably captured. The objectives of this analysis are to assess the probability of confirmed disability worsening to specific EDSS milestones (i.e., EDSS scores ≥3.0, ≥4.0, or ≥6.0) at 288 weeks in the Tysabri Observational Program (TOP) and to examine the impact of relapses occurring during natalizumab therapy in TOP patients who had received natalizumab for ≥24 months. TOP is an ongoing, open-label, observational, prospective study of patients with RRMS in clinical practice. Enrolled patients were naive ...
Skip to Main Content. ... Fulminant thrombotic thrombocytopenic purpura in two patients with syst... more Skip to Main Content. ... Fulminant thrombotic thrombocytopenic purpura in two patients with systemiclupus erythematosus and phospholipid autoantibodies. M. Osman Musa 1 ,; RandaNounou 2 ,; Entezam Sahovic 1 ,; Priti Seth 1 ,; Abdulelah Qadi 1 ,; Mahmoud Aljurf 1. ...
Résumé/Abstract Nous rapportons un cas de purpura thrombotique thrombocytopénique (PTT) fulminant... more Résumé/Abstract Nous rapportons un cas de purpura thrombotique thrombocytopénique (PTT) fulminant, survenu au décours d'un traitement par ciprofloxacine chez une patiente âgée de 43 ans. Une méningo-encéphalite bactérienne aiguë avec coagulation intravasculaire disséminée fut d'abord suspectée. La patiente présentait une anémie hémolytique et une thrombopénie sévère empêchant la réalisation immédiate d'une ponction lombaire. Celle-ci ne fut réalisée qu'après transfusion de plaquettes sanguines ...
Paraneoplastic choreo-athetoses are rare. We report a case of anti-Hu syndrome with choreo-atheto... more Paraneoplastic choreo-athetoses are rare. We report a case of anti-Hu syndrome with choreo-athetosis. A 48-year-old woman developed a small-cell lung carcinoma revealed by an anti-Hu syndrome. The neurological features included choreo-athetosis predominating in the upper limbs, chronic sensorimotor axonal polyneuropathy, and opsoclonus. The cerebrospinal fluid was acellular and contained several oligoclonal IgG bands, not found in the corresponding serum. Magnetic resonance imaging revealed bilateral high-intensity lesions on T2/FLAIR sequence in the corona radiata. Moderate transitory improvement of the paraneoplastic neurological syndrome was observed after several carboplatin-etoposid cycles. A paraneoplastic origin must be considered in all cases of unexplained choreo-athetosis. Paraneoplastic choreo-athetosis is most often associated with other neurological symptoms. The most frequent associated tumor is a small-cell lung carcinoma with anti-CRMP5 and/or anti-Hu antibodies. Our...
Natalizumab (Tysabri(®)) is highly efficacious in controlling disease activity in relapsing multi... more Natalizumab (Tysabri(®)) is highly efficacious in controlling disease activity in relapsing multiple sclerosis (MS) patients. As it is one of the more recent therapies for MS, there remains a need for long-term safety and efficacy data of natalizumab in a clinical practice setting. The Tysabri observational program (TOP) is an open-label, multicenter, multinational, prospective observational study, aiming to recruit up to 6,000 patients with relapsing-remitting MS from Europe, Canada and Australia. The objectives of this study are to collect long-term safety and efficacy data on disease activity and disability progression. We report here the interim results of the 563 patients included in TOP between December 2007 and 2012 from Belgium. This patient cohort was older at baseline, had longer disease duration, higher neurological impairment, and a higher baseline annualized relapse rate, when compared to patients included in the pivotal phase III AFFIRM trial. Nevertheless, the efficac...
ABSTRACT IntroductionParaneoplastic choreo-athetoses are rare. We report a case of anti-Hu syndro... more ABSTRACT IntroductionParaneoplastic choreo-athetoses are rare. We report a case of anti-Hu syndrome with choreo-athetosis.Case reportA 48-year-old woman developed a small-cell lung carcinoma revealed by an anti-Hu syndrome. The neurological features included choreo-athetosis predominating in the upper limbs, chronic sensorimotor axonal polyneuropathy, and opsoclonus. The cerebrospinal fluid was acellular and contained several oligoclonal IgG bands, not found in the corresponding serum. Magnetic resonance imaging revealed bilateral high-intensity lesions on T2/FLAIR sequence in the corona radiata. Moderate transitory improvement of the paraneoplastic neurological syndrome was observed after several carboplatin-etoposid cycles.ConclusionA paraneoplastic origin must be considered in all cases of unexplained choreo-athetosis. Paraneoplastic choreo-athetosis is most often associated with other neurological symptoms. The most frequent associated tumor is a small-cell lung carcinoma with anti-CRMP5 and/or anti-Hu antibodies. Our patient developed paraneoplastic choreo-athetosis related to an anti-Hu syndrome in the absence of anti-CRMP5/CV2 antibodies. Paraneoplastic choreo-athetosis might result from a central lesion, and/or from proprioceptive deafferentation subsequent to peripheral neuropathy.
Sirs: Cerebrospinal fluid (CSF) oligoclonal immunoglobulin (Ig) G bands are a sensitive marker of... more Sirs: Cerebrospinal fluid (CSF) oligoclonal immunoglobulin (Ig) G bands are a sensitive marker of multiple sclerosis (MS) and other inflammatory diseases of the central nervous system (CNS). However, they may also be detected in some non-inflammatory disorders, with an unknown frequency [1, 4]. We report a patient with intramedullary cavernous angiomas (ICAs) in the thoracolumbar region and CSF oligoclonal IgG bands. A 54-year-old woman was admitted to our department with a 5-year history of gait ataxia and a 3-month history of right cruralgia. On clinical examination she was seen to have a mild proximal paresis of the right leg, with brisk tendon reflexes in the lower limbs, and bilateral Babinski’s sign. There was no sensory or sphincter disturbance. Cranial nerves were normal. Serum laboratory findings were normal, including normal protein electrophoresis and negative autoimmune, antiviral and anti-Borrelia serologies. Magnetic resonance imaging (MRI) of the brain was unremarkable, and the CSF was acellular, with several oligoclonal IgG bands on immunoblotting. The multimodality evoked potentials were normal, except for a symmetrical increase in the somatosensory central conduction time after stimulation of the posterior tibial nerves. A spinal form of MS was first suspected. However, spinal cord MRI, performed 2 months later, showed two ICAs at the thoracic T10 and T11 levels (Figs. 1, 2). They each had with a reticulated centre of mixed signal intensity surrounded by a rim of hypointensity on T2weighted images. A total resection of the ICAs was performed after thoracic T9–T11 laminectomy and was followed by a transient spastic paraparesis, related to an acute spinal oedema. The patient had a chronic myelopathy, due to two thoracic ICAs. ICAs are rare and comprise 1–3% of all intramedullary tumours [2]. They may cause acute, progressive or episodic spinal cord dysfunctions [3]. Acute presentation usually results from haemorrhage. In patients presenting with a progressive myelopathy, several pathophysiological mechanisms may be proposed, such as enlargement of the malformation, neurotoxic effect of haemosiderin and compromised surrounding microcirculation [3]. MRI is the procedure of choice to detect ICAs, showing the characteristic appearance of a reticulated centre of mixed signal intensity surrounded by a rim of signal hypointensity on T2weighted images [2, 3]. ICAs must be included in the differential diagnosis of spinal dural arteriovenous malformations and both relapsing/remitting and secondary progressive types of multiple sclerosis (MS). Radical resection of the ICAs is associated with a relatively low morbidity and is advocated whenever the malformations are symptomatic [2]. In our patient the thoracic ICAs were associated with CSF oligoclonal IgG bands. The coexistence of ICAs and MS was first considered. However, MRI revealed no other spinal or cerebral lesion, suggestive of MS. Vretem et al. [4] have reported a patient with cavernous angioma of the brainstem and CSF oligoclonal IgG bands, mimicking MS. As in our case, their patient did not have any other lesion suggestive of MS on brain MRI. Recently Cohen et al. [1] have also found CSF oligoclonal IgG bands in three patients with spinal arteriovenous malformations, located in the low thoracic level. The presence of CSF oligoclonal IgG bands in some cases of CNS cavernous angiomas or spinal arteriovenous malformations does not have any known pathogenetic significance. Attention LETTER TO THE EDITORS
Background Shift work is commonly associated with health problems resulting from circadian misali... more Background Shift work is commonly associated with health problems resulting from circadian misalignment and sleep restriction. About one in three shift workers is affected by insomnia and up to 90% report regular fatigue and/or sleepiness at the workplace. Epidemiological data shows that shift workers are at increased risk of cardiovascular disease, diabetes, obesity, breast cancer, mental-health problems, and shift-work disorder, which conditions typically lead to reduced work performance, processing errors, accidents at work, absenteeism, and reduced quality of life. Given these widespread and debilitating consequences, there is an urgent need for treatments that help improve the sleep, health, and functional performance of the shift-working population. The most common non-pharmacological recommendations are improved scheduling, bright-light exposure, napping, psychoeducation promoting sleep hygiene, and cognitive-behavioral techniques. The objectives of the present study are to i...
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