Inaugural tumor-like multiple sclerosis: clinical presentation and medium-term outcome in 87 patients.

BACKGROUND: Tumefactive demyelinating lesions of the central nervous system can be the initial presentation in various pathological entities [multiple sclerosis (the most common), Balo's concentric sclerosis, Schilder's disease and acute disseminated encephalomyelitis] with overlapping clinical presentation. The aim of our study was to better characterize these patients. METHODS: Eighty-seven patients (62 women and 25 men) from different MS centers in France were studied retrospectively. Inclusion criteria were (1) a first clinical event (2) MRI showing one or more large demyelinating lesions (20 mm or more in diameter) with mass-like features. Patients with a previous demyelinating event (i.e. confirmed multiple sclerosis) were excluded. RESULTS: Mean age at onset was 26 years. The most common initial symptoms (67% of the patients) were hemiparesis or hemiplegia. Aphasia, headache and cognitive disturbances (i.e. atypical symptoms for demyelinating diseases) were observed in 15, 18 and 15% of patients, respectively. The mean largest diameter of the tumefactive lesions was 26.9 mm, with gadolinium enhancement in 66 patients (81%). Twenty-one patients (24%) had a single tumefactive lesion. During follow-up (median time 5.7 years) 4 patients died, 70 patients improved or remained stable and 12 worsened. 86% of patients received initial corticosteroid treatment, and 73% received disease-modifying therapy subsequently. EDSS at the end of the follow-up was 2.4 ± 2.6 (mean ± SD). CONCLUSION: This study provides further evidence that the clinical course of MS presenting with large focal tumor-like lesions does not differ from that of classical relapsing-remitting MS, once the noisy first relapsing occurred.

[Unexplicated neuropsychiatric disorders: Do not ignore dysimmune encephalitis. A case report of a dysimmune encephalitis with anti-leucine rich glioma inactivated 1 (LGI-1) antibodies]. / Troubles neuropsychiatriques inexpliqués : penser aux encéphalites dysimmunitaires. À propos d'une observation d'encéphalite à anticorps anti-leucine rich glioma inactivated 1 (LGI-1).

INTRODUCTION: Anti-leucine rich glioma inactivated 1 encephalitis is a common and a treatable etiology of autoimmune encephalitis. Its diagnosis is a challenge because the initial diagnostic work-up is often normal. CASE REPORT: A 48-year-old man experienced cognitive and behavioral troubles, facio-brachial dystonic seizures and a syndrome of inappropriate antidiuretic hormone secretion. First line tests excluded infectious, neoplastic, systemic inflammatory, endrocrine or toxic etiologies. Cerebral (18)Fluoro-desoxy-glucose (FDG) position emission tomography and research of specific antibodies in cerebro-spinal fluid and serum led to diagnose an anti-leucine rich glioma inactivated 1 encephalitis. Intravenous immunoglobulins and corticosteroids were partially effective. Cyclophosphamid permitted a good recovery. CONCLUSION: In the presence of acute neuropsychiatric disorders with a negative etiologic research, physician should think about dysimmune encephalitis. Facio-brachial dystonic seizures and syndrome of inappropriate antidiuretic hormone secretion are highly evocative of anti-leucine rich glioma inactivated 1 encephalitis. The diagnosis needs specific diagnostic tests (cerebral (18)FDG position emission tomography and antibodies research in cerebro-spinal fluid and in serum), after the exclusion of alternative diagnoses. Extensive and repeated diagnostic work-up for neoplasia is required. Immunosupressive therapies are effective in most cases.

Is critical illness polyneuropathy associated with decreased heart rate variability?

INTRODUCTION: This pilot study assessed the association between critical illness polyneuropathy (CIP) and decreased heart rate variability (HRV) in intensive care patients. METHODS: All patients admitted to the intensive care unit and expected to be ventilated for at least 72 hours were included and underwent weekly electromyograms and HRV analyses for three weeks. HRV was assessed by time domain analysis of 24h recording electrocardiograms, and alterations in HRV were assessed as the square root of the mean squared differences of successive RR intervals (RMSSD) ≤ 15. RESULTS: We evaluated 26 patients, 12 men and 14 women, median age 64 years. During follow-up, 12 patients died and 9 developed CIP. CIP was not associated with age, sex, simplified acute physiology score II and treatment agents. Altered RMSSD tended to be associated with onset of CIP (P=0.06). Altered RMSSD occurred earlier or at the same time as electromyogram abnormalities in all CIP patients, but the difference was not significant. CONCLUSION: Altered HRV, may be associated with the onset of CIP in ICU patients. Although not statistically significant (P=0.06), altered RMSSD may be a surrogate marker of CIP in ICU patients undergoing mechanical ventilation. The physiological pathway linking HRV and CIP remains uncertain.

[Wernicke encephalopathy after subtotal gastrectomy for morbid obesity]. / Encéphalopathie de Gayet-Wernicke après gastrectomie partielle pour obésité morbide.

Wernicke's encephalopathy (WE) is one of the potential complications of obesity surgery. It is an acute neuropsychiatric syndrome resulting from thiamine deficiency often associated with repeated vomiting. The classic triad is frequently reported in these patients (optic neuropathy, ataxia and confusion), associated with uncommon features. Cerebral impairment affects the dorsal medial nucleus of the thalamus and the periaqueductal grey area, appearing on MRI, as hyperintense signals on T2, Flair and Diffusion weighted imaging. Early diagnosis and parenteral thiamine are required to decrease morbidity and mortality. We report a case of WE and Korsakoff's syndrome in a young obese patient after subtotal gastrectomy, who still has substantial sequelae. The contribution of MRI with diffusion-weighted imaging is illustrated. The interest of nutritional supervision in the first weeks and preventive thiamine supplementation in case of repeated vomiting are of particular importance in these risky situations.

[Optic neuritis attributable to Mycoplasma pneumoniae]. / Névrite optique à Mycoplasma pneumoniae.

Optic neuritis (ON) is an inflammatory disease whose etiology remains obscure. We report a case of ON attributable to Mycoplasma pneumoniae (MP). A 26-year-old man presented a sudden onset bilateral loss of visual acuity, without any history of pulmonary or ear-nose-throat infection. Diagnosis of optic neuritis was made on the basis of visual field loss, though optic disks and visual evoked potentials were normal. Color vision could not be assessed due to a congenital dyschromatopsia. The neurological examination was normal. On magnetic resonance imaging, there was no enhancement or enlargement of optic nerves, but a demyelinating lesion of the cervical spinal cord. Lumbar puncture revealed lymphocytic meningitis with 60 white blood cells, all of them being lymphocytes. Oligoclonal bands were presents in the CSF. With no evidence of any other infection or auto-immune disease, the diagnosis of Mycoplasma pneumoniae infection was established due to the presence of Mycoplasma pneumoniae specific IgM antibodies. Outcome was quite favorable within three months without treatment. Neurological symptoms--encephalitis, meningitis, polyradiculitis, or more rarely ON or cerebella ataxia--are the main extra pulmonary manifestations of Mycoplasma pneumoniae infection. Search for anti-Mycoplasma pneumoniae IgM antibodies should be performed routinely when On is diagnosed.

Evolution of ELNES spectra as a function of experimental settings for any uniaxial specimen: a fully relativistic study.

We perform calculations of the fully relativistic, corrected geometrical weighting of the pi* and sigma* transitions measured from the 1s core loss electron energy loss spectroscopy (EELS) spectrum in any uniaxial specimen. We present a complete calculation of the differential scattering cross-section (DSCS), taking into account the collection angle, the illumination angle and the tilt of the sample over the optical axis. Owing to high electron velocity in an EELS experiment, the relativistic correction has to be considered. We thus, present a relativistic, corrected DSCS by using the theory recently developed by Jouffrey et al. [Ultramicroscopy 102 (2004) 61] and P. Schattschneider et al. [Phys. Rev. B 72 (2005) 045142]. The relativistic correction is first performed in the natural coordinate system of the scattering event. We then point out a straightforward method to introduce this correction in the microscopic coordinate system, where all calculations have to be done to be experimentally useful. Using the fully corrected DSCS, we present an expression predicting the evolution of the R=pi*/(pi*+sigma*) ratio (related to the ratio of sp2 and sp3 bondings) as a function of experimental settings. We show how the R-evolution can be predicted, for any experimental setting, by the knowledge of one unique reference value. We verify on graphite specimens, the validity of the R-calculation by comparing theoretical predictions presented in this work with experimental data published elsewhere [Daniels et al., Ultramicroscopy 96 (2003) 523 and Menon et al., Ultramicroscopy 74 (1998) 83].

[Cranial pachymeningitis or unknown origin]. / Pachyméningite crânienne de cause indéterminée.

Pachymeningitis of unknown origin is uncommon and is usually associated with headaches, cranial nerve lesions and cerebellar ataxia. Magnetic resonance imaging is particularly contributive to diagnosis. The default diagnosis must however be confirmed by brain biopsy. Treatment is not well defined. Basically, corticosteroid therapy, or immunosuppressive therapy in case of failure, is known to have little effect on the brain lesion. We report here a case characterized by headache associated with partial regression of the radiographic lesions.

Human African trypanosomiasis: MRI.

We report a case of human African trypanosomiasis caused by Trypanosoma brucei rhodesiense. After the febrile period of parasite dissemination, the patient had meningeal involvement but normal CT. MRI showed the appearances of meningitis. After two periods of arsenical treatment, a severe encephalopathy occurred suggesting post-therapeutic reactive encephalitis (PTRE). Nevertheless, T2-weighted MRI showed no oedema, but focal bilateral high signal areas in the white matter. PTRE was excluded and a third course of treatment was undertaken. The lesions progressively disappeared.

[Peripheral neuropathies caused by disulfiram]. / Les neuropathies périphériques dues au disulfirame.

Disulfirame is a rare cause of peripheral neuropathy in subjects with chronic alcoholism. We report here a case in a 42-year-old man and present the main clinical features and electrophysiological and pathology results. Disulfamine-induced peripheral neuropathy is dose-dependent and never occurs below 250 mg/d (1/2 tables of Espéral*). The pathogenesis is poorly understood.

[Athymhormic syndrome and progressive paraplegia disclosing adrenoleukomyeloneuropathy in an adult]. / Athymhormie et paraplégie progressive révélant une adrénoleucomyéloneuropathie chez un adulte.

A 40-year-old man presented with both paraparesia and an athymhormic syndrome. Bicapsular lesions together with abnormal evoked motor potentials suggested cortical and spinal involvement in the deficiency syndrome. The neuropsychologic symptoms appeared to be secondary to bilateral pallidium lesions. Associated signs were adrenoleukocystrophy and adrenomyeloneuropathy suggesting the term, adrenoleukocyeloneuropathy.