In our prospective study, age, disease severity and CSF beta-amyloid levels taken together were a risk factor suggesting shorter survival times. Dementia is relatively frequent in ALS and may be a consequence of either FTLD or result from co-existing Alzheimer disease.

Autoimmune humoral response to neurocytoskeletal proteins is associated with ALS.

A Czech family with three individuals carrying a novel mutation, 290 A-->T (Glu97Val), in the myelin protein zero gene (P0) is reported. The two eldest carriers developed progressive sensorineural hearing loss and abnormal pupillary reaction at age 18. These preceded the onset of the classic signs of Charcot-Marie-Tooth disease (CMT) by more than a decade. Sural nerve biopsy and nerve conduction studies were compatible with the axonal type of CMT. The authors show that progressive hearing loss can be the first symptom in P0 mutation carriers.

This paper describes an IgM monoclonal antibody (49H.8) which was produced following immunization of BALB/c mice with human neuraminidase-treated erythrocytes (NE-RBC). 49H.8 reacts with NE-RBC, neuraminidase-treated T lymphocytes (NE-T) and NE B lymphocytes of both human and murine origin. Little or no reactivity with untreated T or B cells could be detected. Thus the 49H.8 antigen is "cryptic" in most normal lymphocytes of both humans and mice. In contrast, the 49H.8 antigen was detected in non-cryptic or unsubstituted form on many non-treated murine lymphomas of both B- and T-cell origin, on the spontaneous murine mammary carcinoma, TA3-HA and on several human adenocarcinomas. The 49H.8 antigen appears to be related to the previously described 49H.24 antigen as shown by sugar inhibition experiments. 49H.24 reacts most strongly with the synthetic disaccharide (betaGa1 (I leads to 3)alpha Ga1NAc) but not at all with beta Ga1(I leads to 3)beta Ga1Nac. 49H.24 does not react with any of the murine or human tumors tested. 49H.8 reacts with both the alpha and beta forms of the disaccharide but reacts most strongly with phenyl-beta-galactoside-containing compounds. In contrast, phenyl-alpha-galactoside-containing compounds produced no reaction. The natural determinant detected by this antibody was not determined but various possibilities are considered. 49H.8 was used to detect antigen apparently shed from growing TA3-Ha cells into the serum and ascites of tumor-bearing mice. These observations suggest that the 49H.8 monoclonal antibody will be valuable as a specific reagent for a common tumor-associated antigen shared by certain murine and human tumors, and as a means of assaying shed tumor antigen in circulation as in the TA3-Ha mammary adenocarcinoma model.

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ObjectTo evaluate the potential of quantitative MR techniques [voxel-based morphometry (VBM), T2-relaxometry, mean diffusivity (MD), fractional anisotropy (FA)] in the diagnostics of amyotrophic lateral sclerosis (ALS). Materials and methods Thirty-three ALS patients and thirty age-and sex-matched healthy volunteers were included in the cross-sectional study. T1WI, T2WI and T2 relaxometry sequences were performed at 1.5T. DWI was performed in a subgroup of 12 patients. Disease severity was estimated with the ALS Functional Rating Scale (ALS-FRS). Results We detected decreased T2 relaxation rate (R2) in the frontal white matter (FWM) (left and right P < 0.005) and caudate nucleus (left P < 0.005) in ALS patients. R2 in the FWM correlated with age in patients and controls. A correlation (P < 0.01, cluster-level corrected) between Conclusions Decreased R2 in the left caudate and bilateral FWM may help in the diagnostic process and disqualifies these regions as internal controls in ALS studies. The PLIC is not a reliable diagnostic marker of ALS.

Impairment of social function was measured by questionnaire in 270 patients with Crohn's disease. In about half of them, the disease had had its onset below the age of 20 years. In 10%, schooling or job training was delayed or even had to be abandoned. Ten percent of the patients were forced to change their occupation because of the disease. 23.5% of the patients stated that their illness had prevented them from having whole-day jobs. Ten percent had temporary or permanent pensions; 3.8% were unemployed. 43.7% of patients stated that the relation to their family and (or) partner was impaired by the disease; in 36.4% the disease had interfered with leisure time activities. The results show that Crohn's disease has social effects which in the individual case can be very serious; they correlate with the duration and severity of the disease. Nonetheless, the majority of patients succeeds in mastering their chosen training and occupation and leading an active life.

Background SARS-Coronavirus-2 (COVID-19) infection is a pandemic with various clinical presentations including pericarditis which seems to be rare. Purpose The aim of this observational cohort study was to describe characteristics and management of the patients hospitalized for pericarditis secondary to COVID 19 in the Hospital of Haguenau, France. Methods We retrospectively enrolled patients admitted for pericarditis secondary to COVID 19 affection, either confirmed by a typical chest CT scan, or a positive Covid 19 PCR. Data were collected by a careful review of their medical record. Results 7 patients (4 men, 3 women) were included, with a median age of 60 and a median body mass index of 27.8 kg/m 2 . One of them took angiotensin II receptor antagonists, and none angiotensin converting enzyme inhibitors. Chest pain and dyspnea were the most common initial symptoms. Pericarditis were diagnosed with a median delay of 21 days after the onset of symptoms. The biology showed an inflammatory syndrome (median CRP at 104 mg/L). The troponin peak value was increased in two cases. Chest computed tomography revealed a typical lung COVID-19 affection in 4 cases and a pericardial effusion in every cases. Left ventricle ejection fraction assessed by echocardiography was normal. Two cases evolved into cardiac tamponade, which needed pericardiocentesis. One of the two cases of tamponade had a negative COVID 19 nasopharyngeal PCR and no pulmonary sign of the affection on the chest CT, but the Covid-19 PCR on pericardial fluid was positive. One patient required oxygen supply. Treatment with Colchicine was systematically initiated. Non-steroidal anti-inflammatory drugs were not introduced considering the risk of respiratory worsening. The 1-month follow-up revealed no death and a decrease of the pericardial effusion. Conclusion This study suggest a higher prevalence of COVID-19-associated pericarditis than initially presumed, with heterogeneous clinical presentations.