Manifestations neurologiques de l’infection par SARS-Cov-2

L'apparition en décembre 2019 du coronavirus SARS-Cov-2 (severe acute respiratory syndrome coronavirus 2) responsable de l'infection COVID-19, a totalement bouleversé le système de santé et a entrainé une situation inédite tant sur le plan scientifique que social. Depuis sa propagation rapide à travers le monde et sa qualification de pandémie par l'Organisation mondiale de la santé (OMS), l'explosion du nombre de cas de COVID-19 a permis de décrire plusieurs formes cliniques concomitantes avec les mutations de ce virus. Bien que le COVID19 soit surtout connu pour son tropisme respiratoire (syndrome respiratoire aigu impliquant les voies aériennes supérieures ou basses), il peut également provoquer plusieurs atteintes extrapulmonaires telles que les manifestations neurologiquesL'apparition en décembre 2019 du coronavirus SARS-Cov-2 (severe acute respiratory syndrome coronavirus 2) responsable de l'infection COVID-19, a totalement bouleversé le système de santé et a entrainé une situation inédite tant sur le plan scientifique que social. Depuis sa propagation rapide à travers le monde et sa qualification de pandémie par l'Organisation mondiale de la santé (OMS), l'explosion du nombre de cas de COVID-19 a permis de décrire plusieurs formes cliniques concomitantes avec les mutations de ce virus. Bien que le COVID19 soit surtout connu pour son tropisme respiratoire (syndrome respiratoire aigu impliquant les voies aériennes supérieures ou basses), il peut également provoquer plusieurs atteintes extrapulmonaires telles que les manifestations neurologique

The encephalitic syndrome revealing cerebral gliomatosis in an adolescent

Cerebral gliomatosis is a rare glial tumour which is defined by a diffuse and not very destructive infiltration of the encephalon by the glial neoplastic cells in the absence of individualizable tumour mass (Sanson et al., 2005). The clinical and radiological presentation is often misleading and not very specific, and the diagnosis is rarely mentioned. Histological diagnosis remains difficult. Finally, gliomatosis poses a specific therapeutic problem compared to other glial tumours due to the toxicity of panencephalic radiotherapy and the impossibility of achieving surgical reduction of the tumour (Sanson et al., 2005). Data from the literature show a median overall survival of 14.5 months, a higher frequency of oligodendroglial forms. The prognosis is linked to age, functional status, histological grade, oligodendroglial differentiation (Sanson et al., 2005). We report the observation of a gliomatosis occurring in a 14-year-old boy, having presented focal subintral epileptic attacks accompanied by hemiparesis. Flair sequence brain MRI showed a left fronto-temporo-insular hyper signal. The brain biopsy revealed gliomatosis. The evolution was favourable after radiotherapy. Gliomatosis is a diagnosis to be systematically evoked in the presence of a diffuse cerebral affection. Its etiopathogenic mechanism is unknown, and evolution is unpredictable (Millan. BS et al., 2010)

West syndrome and multiple sclerosis association

Introduction. West syndrome is a rare and severe infantile epileptic encephalopathy, beginning around the age of six months, characterized by a classic electro-clinical triad. This is a pathology totally different from multiple sclerosis (MS) which is a demyelinating disease of the central nervous system caused, affecting young adults, especially females. The association of these two pathologies has never been described. Observation. We report here an exceptional presentation of MS in a 14-year-old girl with a history of West syndrome. She had normal development until the age of six months, when she began to have flexion spasms. The diagnosis of West syndrome was made with a normal MRI. The infantile spasms disappeared after treatment with vigabatrin and adrenocorticotropic hormone (ACTH). It had generally progressed to Lennox Gastaut encephalopathy, with delayed psychomotor development and epileptic sequelae. At 14, she presented with left hemiparesis within a few days. A cerebral MRI showed multiple nodular hyperintensities of the supra and infratentorial white matter, with the presence of an active lesion, fulfilling the diagnostic criteria for multiple sclerosis. CSF analysis was normal. Anti-AQP4, anti-MOG, anti-NMDA and anti-GABA (AB) antibodies were absent in the blood. Antibodies against HIV and viral hepatitis were. Biotinidase activity and autoimmunity tests were correct. The patient received high doses of methylprednisolone IV (1g/day) for three days with remarkable clinical improvement after 15 days. Discussion. MS is a complex and heterogeneous central nervous system (CNS) demyelinating disease. It is not uncommon for epilepsy to be the first symptom of multiple sclerosis. Seizures, on the other hand, are more common after disease progression. Although the disease is characterized by inflammatory lesions of the white matter, various neuropathological and radiological studies have shown that the disease also affects the grey matter. Several studies have shown that seizures are three to six times more common in MS patients than in the general population. Even though MS can start with epilepsy and a seizure may be the only symptom of a relapse of MS, it is still not known whether the two diseases coexist or whether MS predisposes to seizures. Conclusion. The association of these two totally different pathologies can lead us to say that the mechanism of multiple sclerosis may begin in childhood and that the clinical signs appear in adulthood

West syndrome and multiple sclerosis association: About a case

Introduction. West syndrome is a rare and severe infantile epileptic encephalopathy, beginning around the age of six months, characterized by a classic electro-clinical triad. This is a pathology totally different from multiple sclerosis (MS) which is a demyelinating disease of the central nervous system caused, affecting young adults, especially females. The association of these two pathologies has never been described. Observation. We report here an exceptional presentation of MS in a 14-year-old girl with a history of West syndrome. She had normal development until the age of six months, when she began to have flexion spasms. The diagnosis of West syndrome was made with a normal MRI. The infantile spasms disappeared after treatment with vigabatrin and adrenocorticotropic hormone (ACTH). It had generally progressed to Lennox Gastaut encephalopathy, with delayed psychomotor development and epileptic sequelae. At 14, she presented with left hemiparesis within a few days. A cerebral MRI showed multiple nodular hyperintensities of the supra and infratentorial white matter, with the presence of an active lesion, fulfilling the diagnostic criteria for multiple sclerosis. CSF analysis was normal. Anti-AQP4, anti-MOG, anti-NMDA and anti-GABA (AB) antibodies were absent in the blood. Antibodies against HIV and viral hepatitis were. Biotinidase activity and autoimmunity tests were correct. The patient received high doses of methylprednisolone IV (1g/day) for three days with remarkable clinical improvement after 15 days. Discussion. MS is a complex and heterogeneous central nervous system (CNS) demyelinating disease. It is not uncommon for epilepsy to be the first symptom of multiple sclerosis. Seizures, on the other hand, are more common after disease progression. Although the disease is characterized by inflammatory lesions of the white matter, various neuropathological and radiological studies have shown that the disease also affects the grey matter. Several studies have shown that seizures are three to six times more common in MS patients than in the general population. Even though MS can start with epilepsy and a seizure may be the only symptom of a relapse of MS, it is still not known whether the two diseases coexist or whether MS predisposes to seizures. Conclusion. The association of these two totally different pathologies can lead us to say that the mechanism of multiple sclerosis may begin in childhood and that the clinical signs appear in adulthood

Epilepsie : s’agit-il d’une crise ?

Les crises psychogènes non épileptiques (CPNE) sont des manifestations paroxystiques (motrices, cognitives, émotionnelles..), qui évoquent en premier lieu des crises épileptiques, mais qui sont en rapport avec une origine psychogènes. Elles se voient surtout chez la femme et considérées comme une pathologie fréquente, sous-estimée et de diagnostique tardif car elles sont prises à tort pour une épilepsie. La CPNE est classée au niveau des troubles somatoformes dans la catégorie des troubles de conversion. L’enregistrement vidéo et électro-encéphalographique en monitoring reste l’examen de choix pour les différencier des crises d’épilepsie. La difficulté du diagnostic des CNEP nécessite une approche conjointe entre neurologues et psychiatres, et l’annonce du diagnostic est capitale et conditionne la prise en charge de cette pathologie complexe.Les crises psychogènes non épileptiques (CPNE) sont des manifestations paroxystiques (motrices, cognitives, émotionnelles..), qui évoquent en premier lieu des crises épileptiques, mais qui sont en rapport avec une origine psychogènes. Elles se voient surtout chez la femme et considérées comme une pathologie fréquente, sous-estimée et de diagnostique tardif car elles sont prises à tort pour une épilepsie. La CPNE est classée au niveau des troubles somatoformes dans la catégorie des troubles de conversion. L’enregistrement vidéo et électro-encéphalographique en monitoring reste l’examen de choix pour les différencier des crises d’épilepsie. La difficulté du diagnostic des CNEP nécessite une approche conjointe entre neurologues et psychiatres, et l’annonce du diagnostic est capitale et conditionne la prise en charge de cette pathologie complexe

Diagnosis and classification of optic neuritis

There is no consensus regarding the classification of optic neuritis, and precise diagnostic criteria are not available. This reality means that the diagnosis of disorders that have optic neuritis as the first manifestation can be challenging. Accurate diagnosis of optic neuritis at presentation can facilitate the timely treatment of individuals with multiple sclerosis, neuromyelitis optica spectrum disorder, or myelin oligodendrocyte glycoprotein antibody-associated disease. Epidemiological data show that, cumulatively, optic neuritis is most frequently caused by many conditions other than multiple sclerosis. Worldwide, the cause and management of optic neuritis varies with geographical location, treatment availability, and ethnic background. We have developed diagnostic criteria for optic neuritis and a classification of optic neuritis subgroups. Our diagnostic criteria are based on clinical features that permit a diagnosis of possible optic neuritis; further paraclinical tests, utilising brain, orbital, and retinal imaging, together with antibody and other protein biomarker data, can lead to a diagnosis of definite optic neuritis. Paraclinical tests can also be applied retrospectively on stored samples and historical brain or retinal scans, which will be useful for future validation studies. Our criteria have the potential to reduce the risk of misdiagnosis, provide information on optic neuritis disease course that can guide future treatment trial design, and enable physicians to judge the likelihood of a need for long-term pharmacological management, which might differ according to optic neuritis subgroups

Diagnosis and classification of optic neuritis.

There is no consensus regarding the classification of optic neuritis, and precise diagnostic criteria are not available. This reality means that the diagnosis of disorders that have optic neuritis as the first manifestation can be challenging. Accurate diagnosis of optic neuritis at presentation can facilitate the timely treatment of individuals with multiple sclerosis, neuromyelitis optica spectrum disorder, or myelin oligodendrocyte glycoprotein antibody-associated disease. Epidemiological data show that, cumulatively, optic neuritis is most frequently caused by many conditions other than multiple sclerosis. Worldwide, the cause and management of optic neuritis varies with geographical location, treatment availability, and ethnic background. We have developed diagnostic criteria for optic neuritis and a classification of optic neuritis subgroups. Our diagnostic criteria are based on clinical features that permit a diagnosis of possible optic neuritis; further paraclinical tests, utilising brain, orbital, and retinal imaging, together with antibody and other protein biomarker data, can lead to a diagnosis of definite optic neuritis. Paraclinical tests can also be applied retrospectively on stored samples and historical brain or retinal scans, which will be useful for future validation studies. Our criteria have the potential to reduce the risk of misdiagnosis, provide information on optic neuritis disease course that can guide future treatment trial design, and enable physicians to judge the likelihood of a need for long-term pharmacological management, which might differ according to optic neuritis subgroups