F. Blanc

RELAPSING INFLAMMATORY OPTICNEURITIS: IS IT NEUROMYELITIS OPTICA?

Subacute loss of vision accompanied by pain is most commonly due to some form of inflammatory optic neuropathy (ON), and may be the first symptom of multiple sclerosis (MS). ON may also be due to viral or bacterial infection or systemic diseases. Although ON is frequently limited to a single episode, some patients experience recurrent episodes. The name recently given to these recurrent episodes of inflammatory ON with a negative workup for MS or other causes of ON is relapsing inflammatory ON (RION).1 MRI scans are normal and oligoclonal bands are rarely found in the CSF. Some patients with an initial diagnosis of RION are, after several years of follow-up, diagnosed with neuromyelitis optica (NMO), due to the occurrence of one or more episodes of myelitis.2 Recently, a specific marker of NMO, named NMO antibody, was found.3 There is now evidence to suggest that patients who experience recurrent episodes of myelitis and are positive for NMO antibodies are at a high risk of developing NMO.4 A similar hypothesis could be applied to RION. In the first study on NMO antibodies, a positive result was found in 2 of 8 patients with RION.3 However, the …

Myélite aiguë et neuroborréliose

Resume Introduction Les myelites aigues de Lyme representent 4 a 5 p. 100 des cas de neuroborreliose. Seuls 8 cas ayant fait l’objet a la fois d’une etude du liquide cephalo-rachidien et d’une IRM medullaire ont ete decrits dans la litterature. Nous rapportons ici 3 nouveaux cas. Methode Dans une serie de 45 patients atteints de neuroborreliose diagnostiques en 8 ans entre le 1er janvier 1997 et le 31 decembre 2004, 3 avaient un tableau de myelite aigue. Nous en avons analyse les caracteristiques cliniques, biologiques et radiologiques. Observations Ces 3 patients avaient tous une atteinte motrice, sensitive et sphincterienne a des degres divers. Chaque patient avait aussi une atteinte extra-medullaire : fievre et cephalees pour l’un, paralysie faciale peripherique unilaterale pour le second et hemorragie meningee pour le dernier. Il existait une pleiocytose d’intensite variable de 10 a 520 globules blancs par mm3. La serologie de Lyme etait positive dans le liquide cephalo-rachidien dans les 3 cas. L’index de synthese intrathecale anti-Borrelia burgdorferi etait positif ou intermediaire. L’IRM medullaire retrouvait un hypersignal de plus de 3 metameres de haut pouvant etre transverse, posterieur ou centro-medullaire. L’evolution sous antibiotherapie specifique pendant au moins 3 semaines etait favorable pour les 3 cas. Conclusion Ces 3 cas ainsi que les autres decrits dans la litterature montrent la diversite des tableaux clinico-radiologiques de myelite aigue de Lyme : myelite transverse, posterieure ou centrale. Ainsi, une serologie de Lyme dans le liquide cephalo-rachidien doit etre systematiquement pratiquee dans les myelites aigues etendues, en particulier en zone d’endemie.

Neuropathies optiques inflammatoires récidivantes

PURPOSE: To analyze clinical and paraclinical characteristics of recurrent isolated optic neuropathy. PATIENTS: and method: In three university hospitals (Montpellier, Nimes, and Strasbourg), between October 2005 and September 2006, the charts of patients with corticosensitive recurrent isolated optic neuropathy and normal cerebral magnetic resonance imaging included prospectively were reviewed. The following parameters were analyzed: date of the first relapse, age at onset, duration at the time of inclusion, recurrence after steroid withdrawal, unilateral or bilateral involvement, number of relapses, visual acuity, retinal nerve fiber layer thickness, diagnostic workup, and long-term treatment with immunosuppressive or immunomodulatory drugs. RESULTS: During the predefined period, 13 patients (11 women, 2 men; age, 17-54 years at onset) matched the inclusion criteria. Between two and six relapses of optic neuropathy were observed. The median duration was 4 years. In untreated patients (n=7), a significant (Spearman p=0.0156) inverse correlation was observed between visual acuity and duration of the disease; this correlation was not found in the group of patients (n=6) with long-term treatment (Spearman p=0.1032). CONCLUSION: The progressive loss of vision over time in this retrospective study of recurrent isolated optic neuropathy could be related to axonal loss. A prospective cohort study is necessary to confirm this hypothesis and to evaluate the benefit of long-term treatment on this progression.

Moindre réactivité émotionnelle aux stimuli négatifs dans la sclérose en plaques, résultats préliminaires

BACKGROUND Charcot first described emotional deficits in multiple sclerosis (MS) in the XIXth century. Despite this early description, there are very few studies about emotions and MS. OBJECTIVES This study aimed at better understanding the emotional process in MS and more specifically recognition of facial emotions and emotional experience. METHODS Thirteen women with remittent MS (R-MS), with a mean EDSS score of 2, were compared with thirteen healthy control subjects, matched for age (mean age of 42±2), sex and educational level. The Beck Depression Inventory (BDI), the Hamilton Anxiety Scale and the brief repeatable battery of neuropsychological tests for MS (BCcogSEP) were administered. Recognition of faces and facial expression of emotion were assessed by the Benton facial recognition test and recognition of facial emotions was assessed by Ekmans facial expression test. We have also presented 12 different sounds and pictures from the International Affective Digitized Sounds and Picture System (IADS and IAPS) in order to study the emotional experience by using criteria of valence and arousal. RESULTS No deficit of facial emotion recognition was found in MS in this small population. Nevertheless, patients who had difficulty recognizing faces were the least able to recognize facial expressions. No significant difference was observed between the patient and control group for the experience of emotional valence. However, independently of their mood and cognitive status, the self-assessment of the MS patient population suggested that the patients were less reactive to negative sounds (P=0.005) and negative pictures (P=0.002) as compared with the control group, pointing to lesser sensitivity towards aversive stimuli. CONCLUSION These data suggest disorders in emotional processes in R-MS, mainly a poor reactivity to negative stimuli which may have an impact on everyday life. A larger population should be studied to confirm these modifications of emotion.

Confirmation de l’intérêt de la biopsie cutanée dans la neuropathie des petites fibres. Résultats préliminaires

INTRODUCTION In small-fiber neuropathy, skin biopsy reveals a reduction of intraepidermal nerve fiber density (IENFD), a feature often necessary for diagnosis. In France, this technique has not been widely used for this purpose. PATIENT AND METHOD To validate this method, we studied 13 patients with suspected small-fiber neuropathy, analyzed their nervous intra- and subepidermal network with a punch skin biopsy and compared our data with those of literature. RESULTS Ten patients had pure small-fiber neuropathy and three an axonal polyneuropathy involving large-caliber nerve fibers. In the group of patients with pure small-fiber neuropathy, we found medium IENFD (11.6 +/- 4.46 fibers per millimeter in the proximal thigh and 7.15 +/- 3.59 fibers per millimeter in distal leg), well correlated with the electron microscopy quantitative and qualitative analysis of the unmyelinated subepidermal fibers. CONCLUSION This work demonstrated the good reproducibility of skin biopsy for analyzing the small-fibers in our cohort. These results require further confirmation in a larger cohort and validation in comparison with controls analyzed on a local level. Nevertheless, these techniques seem to be useful to assess the difficult diagnosis of small-fiber neuropathy.

Isolated anti-β2-glycoprotein I antibodies in neurology: a frontier syndrome between multiple sclerosis and antiphospholipid syndrome?

Anti‐β2‐glycoprotein I (anti‐β2‐GPI) antibodies are part of the heterogeneous family of antiphospholipid antibodies and seem to be present in various neurological manifestations in addition to antiphospholipid syndrome (APS). Our objective was to analyse the clinical, radiological and therapeutic characteristics of neurological patients with positive anti‐β2‐GPI antibodies and without the Sapporo criteria for APS.

MémoireMoindre réactivité émotionnelle aux stimuli négatifs dans la sclérose en plaques, résultats préliminairesReduced emotional reactivity to negative stimuli in multiple sclerosis, preliminary results

BACKGROUND Charcot first described emotional deficits in multiple sclerosis (MS) in the XIXth century. Despite this early description, there are very few studies about emotions and MS. OBJECTIVES This study aimed at better understanding the emotional process in MS and more specifically recognition of facial emotions and emotional experience. METHODS Thirteen women with remittent MS (R-MS), with a mean EDSS score of 2, were compared with thirteen healthy control subjects, matched for age (mean age of 42±2), sex and educational level. The Beck Depression Inventory (BDI), the Hamilton Anxiety Scale and the brief repeatable battery of neuropsychological tests for MS (BCcogSEP) were administered. Recognition of faces and facial expression of emotion were assessed by the Benton facial recognition test and recognition of facial emotions was assessed by Ekmans facial expression test. We have also presented 12 different sounds and pictures from the International Affective Digitized Sounds and Picture System (IADS and IAPS) in order to study the emotional experience by using criteria of valence and arousal. RESULTS No deficit of facial emotion recognition was found in MS in this small population. Nevertheless, patients who had difficulty recognizing faces were the least able to recognize facial expressions. No significant difference was observed between the patient and control group for the experience of emotional valence. However, independently of their mood and cognitive status, the self-assessment of the MS patient population suggested that the patients were less reactive to negative sounds (P=0.005) and negative pictures (P=0.002) as compared with the control group, pointing to lesser sensitivity towards aversive stimuli. CONCLUSION These data suggest disorders in emotional processes in R-MS, mainly a poor reactivity to negative stimuli which may have an impact on everyday life. A larger population should be studied to confirm these modifications of emotion.

Do we know how to diagnose epilepsy early in Alzheimer's disease?

Epilepsy is an increasingly recognized comorbidity in Alzheimers disease (AD). First described as generalized in dementia patients, epileptic AD patients are nowadays fully described in earlier stages of the disease (with mild or subjective cognitive impairment). At such early stages, patients may present not only with generalized seizures, but also with focal seizures (commonly localized in the frontal or temporal lobe). Thus, partial or generalized epilepsy is part of the semiological spectrum of AD that should be borne in mind at all stages of disease to ensure early identification and prevent the risk of repeated seizures (such as accidents, injury, progression of cognitive impairment). This review of the available (and still growing) literature shows that there are already sufficient data to inform physicians on seizure semiology, and on the diagnostic value of electroencephalography and brain imaging. Taken together, these tools can help to rapidly identify epilepsy in AD patients. Nevertheless, epilepsy diagnosis can be challenging, and test medication is sometimes necessary. Some cerebrospinal fluid biomarkers (or their ratios) may also prove to be good predictors of seizures in AD, but further studies are needed. Epilepsy in AD patients is frequently pharmacosensitive, and a good response can be obtained with standard doses of antiepileptic drugs. For all these reasons and based on our review of the literature, it appears that, at present, the diagnosis of epilepsy in AD is not only possible at any stage of the disease, but also to be recommended to improve the patients prognosis.

Should a psychotic or manic episode be considered an early manifestation of Multiple Sclerosis? A multiple case study

BACKGROUND Early manifestations of Multiple Sclerosis (MS) can be atypical and misleading, and several case report studies have highlighted that MS onset sometimes takes the form of a psychotic or manic episode. METHODS All neurologists belonging to the French Multiple Sclerosis Observatory network were contacted by email and were asked to find patients with MS who presented with a history of psychiatric episode(s) near MS onset. RESULTS Seventeen patients were selected that met the criteria of presenting with psychotic or manic symptoms either before the diagnosis of MS (N=8), or at the time of the first neurological episode or shortly after (N=9). Patients with a history of a psychiatric episode occurring before the first neurological episode were diagnosed on average 7 years later than patients with either a first neurological or a mixed (both neurological and psychiatric) episode. However, psychiatric symptoms in the first group and the first neurological symptoms of MS in the second group occurred at a similar age. CONCLUSION Based on this multiple case study, we question whether past psychotic or manic episode should be considered equivalent to a first manifestation of MS.

Article originalMéningiome et neuropathie optique : un piège diagnostiqueOptic neuropathy and meningioma: A diagnostic trap☆

INTRODUCTION Meningiomas are benign primary meningeal tumors. Their diagnosis may be incidental or in response to a work-up for neurological or ophthalmological symptoms. PATIENTS AND METHODS The clinical course of five patients with ophthalmological symptoms leading to the diagnosis of meningioma is described. RESULTS The case reports consist of five women (48 to 54 years old - mean 52 years at the onset of symptoms), all suffering from a progressive unilateral decrease in visual acuity with a normal initial fundus examination and ipsilateral visual field changes. Ancillary testing, in particular MRI and CT-scans, had to be repeated to make the diagnosis of meningioma, which was delayed from 18 months to 4 years. DISCUSSION The clinical presentation of these five cases was that of a retrobulbar optic neuropathy, which biased the work-up towards an inflammatory disease of the central nervous system such as multiple sclerosis. However, the atypical character of the neuropathy, which did not respond to intravenous steroids, caused the diagnosis to be questioned and radiological examinations repeated. The iso-intense appearance of meningiomas on T1 MR imaging and only slightly hyperintense appearance on T2 may result in a diagnostic delay if the exam is not performed and interpreted by an experienced professional. Gadolinium contrast, fat suppression and centration on the anterior visual pathways are essential to a proper MRI examination. CONCLUSION When confronted with a progressive, painless optic neuropathy unresponsive to steroid treatment, the diagnosis of meningioma of the anterior visual pathways must be considered. This diagnosis is enabled by a targeted MRI of the anterior visual pathways.