[Facial diplegia and acute inflammatory demyelinating neuropathy secondary to varicella]. / Diplégie faciale au cours d'une neuropathie démyélinisante inflammatoire aiguë secondaire à une varicelle.

INTRODUCTION: Primo-infection by varicella-zoster virus (VZV) may be associated with several neurologic complications. Bilateral facial palsy is a rather uncommon manifestation. CASE REPORT: We report the case of a 38-year-old woman who developed bilateral facial diplegia and paresthesia affecting all four limbs with subacute onset several days after varicella virus primoinfection. Ancillary tests showed hyperproteinorachia and signs of demyelinating polyneuropathy in nerve conduction tests. The diagnosis of Guillain-Barré syndrome was retained and a treatment with intravenous immunoglobulines was started, leading to progressive improvement. CONCLUSION: Immunotherapy is a possible therapeutic approach in the context of neurologic postinfectious complications after VZV infection where an underlying mechanism is probable.

[Atypical CADASIL phenotypes and pathological findings in two new French families]. / CADASIL: aspects phénotypiques inhabituels et observations anatomo-pathologiques dans deux nouvelles familles françaises.

Atypical phenotypes of CADASIL and corresponding anatomical data in two cases are described in 6 members of 2 new French families. In the first family, 4 cases in the same kindred were probably affected, two of them with a predominant psychiatric presentation, two others with dementia and a pseudo-bulbar syndrome of progressive evolution. No history of migraine or ischemic event were documented in any. In the propositus, the diagnosis was documented by skin biopsy, Notch 3 gene mutation and autopsy after the patient had died when 67 years old, 8 years after onset. Brain examination showed a widespread leukoencephalopathy with subcortical infarcts. Characteristic granular lesions of the small arteries of the brain and other organs were observed. In the second family, two cases are reported. One patient died when 63 years old after a subacute evolution mimicking intracranial hypertension. The anatomical diagnosis was retrospectively proven typical of CADASIL with Notch 3 immunostaining of arterial smooth muscle cells. The other case had a progressive evolution over 20 years of limb paresthesia with a mild spasticity diagnosed as a progressive form of multiple sclerosis. It was followed by a pseudo-bulbar syndrome and a mild subcortical dementia without acute ischemic attack. The diagnosis was confirmed by skin biopsy and mutation of the Notch 3 gene. This report illustrates

[Tuberculous meningitis in an immunocompetent adult: contribution of cerebral imaging techniques to the diagnosis and follow-up]. / Méningite tuberculeuse de l'adulte sans immunodépression acquise: contribution de l'imagerie cérébrale au diagnostic et au suivi évolutif.

We have studied 5 men, mean age 47 years, affected by tuberculous meningitis (TM) without documented immunodepression. The diagnosis of TM was supported by clinical and biological investigations and confirmed by the cultures of CSF. All the patients received a four-drug combination therapy and steroids. No drug resistance of the bacilli was observed. Cerebral imaging by CT and MRI was rarely diagnostic but most useful during the follow-up. All the patients developed complications including tuberculomas (5), hydrocephalus (4), ischemic lesions (2), arachnoiditis (1) and abscess of spinal cord (1). Four patients recovered and one died. The mean duration of the follow-up was 16 months. MRI was more sensitive than CT scan to identify inflammatory lesions such as granulomas, angeitis or arachnoiditis and to follow their outcome. Tuberculomas and hydrocephalus were easily diagnosed by CT scan with contrast enhancement. Recommendations of sequential imaging are suggested to identify unexpected or asymptomatic complications of TM during therapy and evaluate the outcome.

[Malignant intravascular lymphoma (malignant angioendotheliomatosis), with adrenal localization. Report of a case]. / Lymphome malin intravasculaire (angioendotheliomatose maligne), avec localisation surrénale. A propos d'un cas.

A past history of epilepsy and terminal dementia in a fifty five year old patient lead to the post mortem diagnosis of malignant intravascular lymphoma (malignant angioendotheliomatosis). There was a scattered involvement of capillaries and venulae in leptomeninges, peripancreatic and perirenal adipose tissue, pericardium, myocardium. The only adrenal involvement was typically tumoral at macroscopic examination. Such a localization, often reported in B intravascular malignant lymphoma, is studied according to the recent publications of this entity.

[Total deafness after multiple pontine infarctions. Dolichoectasia of the basilar trunk]. / Surdité totale après infarctus protubérantiels multiples. Dolichoectasie du tronc basilaire.

A 54 year-old man was affected by three successive infarctions in the vertebro-basilar territory. These infarctions were related to a dolichoectatic basilar artery visualized by arteriography and NMR. Deafness occurred first on the left side and then, after the third infarction, on the right side. The authors underline that deafness can be observed after a pontine infarction in the territory of the anterior inferior cerebellar artery. A dolichoectatic basilar artery can be the source of thrombotic or embolic strokes. Their prevention by antiaggregant or anticoagulant therapy is suggested.