[Cerebral vasculitis secondary to Varicella-Zoster virus infection]. / Vasculite cérébrale secondaire à une infection à VZV.

INTRODUCTION: Central nervous system infection by the varicella-zoster virus (VZV) can be responsible for myelitis, meningitis, ventriculitis and large and small-vessels encephalitis. CASE REPORT: We report the case of a 57-year-old-man hospitalized for deteriorating general health. Physical examination revealed likely encephalitis associated with headache without meningeal syndrome. Successive cerebral MRIs showed bilateral necrosis of the amygdaloid bodies and multiple deep and sub-cortical infarcts suggestive of vasculitis. Cerebral arteriography was normal. Three cerebral fluid examinations disclosed mononuclear pleiocytosis with few red blood cells. PCR analysis for VZV was only positive at the third time. DISCUSSION: The diagnosis of VZV encephalitis is difficult without the rash typical of zoster and because of the low sensitivity of PCR VZV in comparison with PCR HSV. CONCLUSION: In active viral disease, where the prognosis depends on early treatment, we highlight the usefulness of repeated PCR analysis and the search for antibodies in blood and cerebrospinal fluid.

[Diagnostic importance of middle latency auditory evoked potentials (MLAEP) in multiple sclerosis]. / Intérêt diagnostique des potentiels évoqués auditifs de latence moyenne (PEALM) dans la sclérose en plaques.

This study was aimed at assessing the interest of middle latency auditory evoked potentials (MLAEP) recordings in multiple sclerosis (MS). Brainstem auditory evoked potentials (BAEP) and MLAEP were recorded in 40 control subjects and 65 patients who had MS according to Poser's criteria. Na and Pa latencies were significantly delayed in the MS group. These abnormalities were detected in 60% of the patients. In 26% of the cases with normal BAEP, abnormalities of MLAEP were present. In 71% of the patients abnormalities of both BAEP and MLAEP were observed. These results suggest the ability of MLAEP recordings to detect lesions of the auditory pathways rostral to the pons and show the value of their combined recordings in MS patients.

[Sensorimotor polyneuropathy in a flare-up of Crohn disease]. / Polyneuropathie sensitivomotrice au cours d'une poussée de maladie de Crohn.

A 22-year-old man developed an axonal sensorimotor polyneuropathy and an episode of pyloroduodenal stenosis at the same time. Rectal biopsy established the diagnosis of Crohn's disease. The etiologic investigations were negative. The neurological and gastrointestinal troubles improved and followed a parallel course. Without vitamin deficiency or metronidazole treatment, peripheral polyneuropathy is a rare event in Crohn's disease. An autoimmune cause is suspected.

Do visual-evoked potentials and spatiotemporal contrast sensitivity help to distinguish idiopathic Parkinson's disease and multiple system atrophy?

A large number of patients with Parkinson's disease were reported to have abnormal visual-evoked potentials (VEPs) and spatiotemporal contrast sensitivity (STCS) suggesting dopaminergic deficiency in the visual pathway, probably the retina. Until now, VEPs and STCS have not been studied in multiple system atrophy (MSA). We investigated 12 patients with idiopathic Parkinson's disease (IPD) and 12 patients with MSA. The age medians were 64.5 years for IPD and 63.5 years for MSA. None of the patients showed any ocular disease that could interfere with the results. Checkboard VEPs and STCS measurements to horizontal sinusoidal gratings were evaluated. Statistical analysis was performed, including Student's t test and two- or three-way analysis of variance. A significant interocular difference in spatial contrast sensitivity was observed in IPD, which was not present in MSA. VEPs were not delayed in MSA, whereas latency of the major component and the second negative deflection were increased in IPD. VEPs and STCS measurements might provide useful help for distinguishing IPD from MSA.

The first identified French family with dentatorubral-pallidoluysian atrophy.

We report the first French family with dentatorubral-pallidoluysian atrophy (DRPLA) in which three members, a 36-year-old woman (proband), her 34-year-old sister, and 14-year-old brother were affected. There was no family history of DRPLA and their father presented at age 66 with pes cavus but without any other neurologic symptoms. Molecular analysis of the DRPLA gene from blood leukocytes showed CAG repeat sizes to be 68/16 in the proband, 62/15 in her father, and 16/16 in her mother. This study provides support for the variable clinical presentation of this disease with incomplete penetrance in the father and demonstrates that DRPLA can be observed in the French Caucasian population.