[Intracranial localization revealing Hodgkin's disease]. / Lésion intracrânienne révélatrice d'une maladie de Hodgkin.

The central nervous system's (CNS) involvement is uncommon in Hodgkin's disease (HD) and usually occurs in patients with relapsing disease many years after the initial diagnosis. An isolated involvement of the CNS is extremely rare and therefore, difficult to diagnosis. We report the case of a 27-year-old woman with seizure and a left cerebrodural mass on the cerebral imaging; secondarily, she developed cervical lymph node swelling; histological examination of the node revealed HD of the nodular sclerosis type. The dural lesion resolved after chemotherapy for HD.

[Listeria monocytogenes encephalitis revealed by left progressive hemiparesis]. / Hémiparésie d'installation progressive révélant une encéphalite à listéria monocytogènes.

INTRODUCTION: Central nervous system listeriosis is a diagnostic and therapeutic challenge for the clinician. CASE REPORT: We report the case of a 66-year-old woman who was admitted for a left progressive hemiparesis associated with headaches. She was treated for one year by immunosuppressive drugs for vulgaris pemphigus. At the time of admission, examination revealed left hemiparesis without fever, and a computed tomography brain scan demonstrated a focal lesion in the right frontal lobe. Blood analyses were normal. Two days after, she suddenly developed fever (40 degrees C), and aggravation of her motor deficit followed by partial motor seizures. The cerebrospinal fluid was normal. Treatment with amoxicillin (3g IV q6h), cefotaxim, gentamycin (120mg IV q12h) and aciclovir was started empirically. The brain MRI without gadolinium displayed infiltrative lesions in the right hemisphere and in the mildbrain. The blood culture grew Listeria monocytogenes. The antimicrobial regimen was changed to amoxicillin for seven weeks and gentamicin for the first ten days. Four days after beginning the antimicrobial regimen, the brain MRI with gadolinium displayed several abscesses measuring less than one cm diameter. The clinical and imaging outcome excellent. CONCLUSION: Meningitis is by far the most central nervous system listeriosis. In our patient, the diagnosis of listeria monocytogenes encephalitis was established on the basis of positive blood cultures, as such patients do not have sterile cerebrospinal fluid.

[Neurological aspects of Fabry's disease]. / Manifestations neurologiques de la maladie de Fabry.

Fabry disease is a rare X-linked disorder caused by deficient activity of the lysosomal enzyme alpha-galactosidase A. Progressive accumulation in lysosomes of the undegraded glycosphingolipids leads to a multi-system disease with dermatological, ocular, renal, cardiac, and neurological manifestations. Peripheral nerve involvement, neuropathic pain and chronic acroparesthesiae, are frequent and early-onset signs revealing the disease. They are due to the involvement of small nerve fiber, thus explaining the normality of electroneuromyography. Cochleo-vestibular and autonomic nervous system involvement is frequent. Besides rare aseptic meningitis, central nervous system involvement is essentially represented by cerebrovascular events (stroke, transient ischemic attack). Affecting essentially the posterior circulation, their etiologies have to be clarified: progressive stenosis of small vessels with globotriasocylceramide deposits, arterial remodeling, endothelial dysfunction, pro-thrombotic state, cerebral hypoperfusion consecutive to dysautonaumy, cardiac embolism. MRI shows numerous silent lesions, increasing with age, mainly in small perforant arteries (periventricular white matter, brainstem, cerebellum, basal ganglia). Pulvinar calcifications, due to an increase in cerebral hyperperfusion, could be specific of Fabry disease. Positon tomography analysis shows a reduced cerebral flow velocity and impaired cerebral autoregulation, secondary to the glycosphingolipid storage in vascular endothelial cells. Enzyme replacement therapy has to be carefully monitored.