[An acute psychotic episode revealing neuropsychiatric lupus with atypical brain MRI]. / Un accès psychotique aigu révélateur d'un neurolupus avec images atypiques à l'IRM encéphalique.

INTRODUCTION: The frequency of neuropsychiatric symptoms in systemic lupus varies between 14 and 75%. Their mechanisms are multiple and their non-specificity makes the etiological diagnosis more difficult. The MRI lesions are dominated by small white matter hyperintensities. CASE REPORT: We report a case of 17-year-old girl without medical, surgical or familial past, who presented an acute psychotic episode with fever. The neurological examination showed a pyramidal syndrome. The brain MRI objectified diffuse, bilateral and symmetrical white and gray matter hyperintensities. The diagnosis of neuropsychiatric lupus was retained with association of more than four criteria among the 11 ARA revised criteria: pleural effusion, neuropsychiatric manifestations, anemia, lymphopenia and positive ANA. The treatment was based on corticosteroids. The evolution was fatal. DISCUSSION: Psychiatric disorders are polymorphic and appear during the course of the disease or at diagnosis; they can be seen in 20% of cases and are a sign of a poor prognosis. Most often these disorders are a non-specific reaction, but severe clinical forms can be observed as suicidal risk or mood disorders (depression, mania...), or acute psychotic episode. CONCLUSION: The diagnosis of neuropsychiatric lupus must be evoked systematically in atypical neuropsychiatric disorders especially when confronted with minimal biological and brain imaging abnormalities.

[First case of presumed sporadic Creutzfeldt-Jakob Disease in Marrakech, Morocco]. / Premier cas présumé de la maladie de Creutzfeldt Jacob sporadique à Marrakech (Maroc).

INTRODUCTION: Sporadic Creutzfeldt-Jakob disease (CJD) is the most common form of subacute spongiform encephalopathy. The first case of sporadic CJD in Morocco was confirmed by postmortem histology in 2005. The absence of laboratory facilities for diagnosis of prion disease has resulting in underestimation of the incidence of CJD in Morocco. The purpose of this report is to describe another case of sporadic CJD. The patient was a 61-year-old farmer. Medical history included diabetic diet and poorly monitored but the patient had never received hormone therapy or undergone surgery or blood transfusion. There were no similar cases in the patient's family or entourage. Diagnosis of presumed sporadic CJD was based on absence of similar family cases and surgical history and on presence of rapidly progressive dementia, myoclonus, visual troubles, extrapyramidal syndrome, akinetic mutism and typical EEG. Brain magnetic resonance imaging (MRI) depicted supratentorial demyelination. The 14.3.3 protein was detected in cerebrospinal fluid (CSF). Other biological tests were normal. Supportive care included administration of a antihypertensive drug and nursing. The patient died four months after the onset of symptoms. CONCLUSION: Occurrence of CJD in Morocco is probably underestimated and the authors underline the need to set up specialized laboratory facilities for diagnosis of transmissible spongiform encephalopathy.