Neurosarcoïdoses : description d’une cohorte de 35 patients et rentabilité des examens complémentaires à visée diagnostique

Abstract : Neurosarcoidosis (NS) is a rare and polymorphic entity, often inaugural and isolated. To date, diagnostic assessment is still a matter of debate. At our knowledge, it is the first time that 2018 diagnosis consensus criteria were applied. We sought to describe a cohort of patients diagnosed with NS. A retrospective study of all patients diagnosed with NS was conducted in Rouen University Hospital between 1992 and 2018. We included patients diagnosed with definite, probable and possible NS, as described by the Neurosarcoidosis Consortium Group 2018 and collected clinical, biological and imaging data. Subgroup analysis were conducted according to gender, age, inaugural or secondary NS. A total of 35 patients were included, 24 (69%) of whom with inaugural NS. Sex ratio male/female was 0.84. The mean age of onset was 48.0 years. NS diagnosis seemed to be more frequent since 2015. NS was considered as definite in four patients (11%), probable in 17 (49%) and possible in 14 (40%). Possible NS were up-represented about 6% compared to previous Marangoni et al criteria. The median time to diagnostic was 3.0 months. In cases of already known sarcoidosis, neurological symptoms mainly occurred within 6 months after the onset of sarcoidosis (54%). All patient had almost one systemic manifestation with clinical and paraclinical dissociation. All patient had extra neural involvement. Pulmonary involvement was associated in 26 patients (74%). Headache (49%), cranial neuropathies (57%), such as facial nerve palsy, parenchymal involvement (29%) were the most common signs. Only six patients (17%) presented longitudinal extensive traverse myelitis and four patients (11%) peripheral nervous impairment. Biological tests showed: lymphopenia (71%), increased ACE levels (42.4%). CSF analysis revealed abnormalities in 86% whose lymphocytic pleocytosis > 5/mm3 (48%), high protein content (71%). Diffuse T2 white matter hyperintensities (87%) and focal meningeal enhancement (73%) were the most common brain MRI finding. No difference was found in terms of demographic features, biological tests and imaging in subgroup between: inaugural NS or not, age, sex. Women younger than 40 years seemed to have more cranial nerve palsy and a better prognosis. Here we highlight application of 2018 diagnosis consensus criteria of NS. Probability having at least one of the following (CSF, MRI, EMG) abnormal was 94.3% showing the interest of these three investigations in suspected cases of NS
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Master Thesis
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Clément Richard. Neurosarcoïdoses : description d’une cohorte de 35 patients et rentabilité des examens complémentaires à visée diagnostique. Médecine humaine et pathologie. 2019. ⟨dumas-02183592⟩

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