Non-convulsive status epilepticus associated with Whipple's disease.

A 69-year-old woman developed non-convulsive status epilepticus during inpatient investigation for abdominal pain. Initial detailed investigations did not identify the cause of seizures, but a jejunal biopsy and PCR testing in various fluids led to the diagnosis of Whipple's disease with neurological involvement. The seizures were controlled but she subsequently had moderate cognitive impairment. Whipple's disease is an important diagnosis, being treatable with antibiotics. Testing for Whipple's disease is not part of the recommended workup in for status epilepticus, but this case highlights the importance of considering this condition.

A pain like no other: From cluster to headache to Eagle's syndrome, a case report.

BACKGROUND: Eagle's syndrome, also called stylohyoid complex syndrome, is a rare syndrome pathology characterised by latero-cervical pain radiating to the face, linked to an abnormal enlargement of the styloid or calcification of the stylo-hyoid ligament. CASE: We report here the case of a young man of 25 suffering from cluster headache resistant to treatments, revealing Eagle's syndrome. CONCLUSION: Only surgery led to a real improvement of his condition.

Familial Mediterranean fever: What associations to screen for?

Familial Mediterranean fever (FMF) is the most common and best known of hereditary recurrent fever or periodic fever syndromes. It was described in 1945 and genetically characterized in 1992. It is caused by a point mutation in the MEFV gene located on the short arm of chromosome 16. It is particularly frequent among Sephardic Jews, Armenians, Turks and Middle Eastern Arabs, where the prevalence can reach 1/2000 to 1/1000. Recent publications described its frequent association with other diseases and/or syndromes, particularly those of autoimmune, genetic, and autoinflammatory origin. The objective of this review is to familiarize healthcare professionals with the main associations to look for in patients followed for FMF. The early detection of these associations makes it possible to improve the management and the prognosis of patients with FMF.

Ventricular arrhythmias in the context of chronic kidney disease and electrolyte imbalance.

Patients with chronic kidney disease face a high risk of sudden cardiac death, particularly in more advanced stages of renal dysfunction. Ventricular arrhythmias are prevalent and contribute to the heightened cardiovascular mortality. This review aims to explore the intricate interplay of disease-specific risk factors, arrhythmic triggers, and electrolyte disorders that amplify susceptibility to ventricular arrhythmias and sudden cardiac death in this population and influence the efficacy of available treatments.

Neuroimaging determinants of cognitive impairment in the memory clinic: how important is the vascular burden?

While neurodegenerative and vascular neurocognitive disorder (NCD) often co-occur, the contribution of vascular lesions, especially stroke lesions identified on MRI, to global cognition in a real-life memory clinic population remains unclear. The main objective of this retrospective study was to determine NCD neuroimaging correlates: the GM atrophy pattern and vascular lesions (especially stroke lesion localization by voxel-based lesion-symptom mapping, VLSM) in a memory clinic. We included 336 patients with mild or major NCD who underwent cerebral MRI and a neuropsychological assessment. The GM atrophy pattern (obtained by voxel-based morphometry, VBM) and the stroke lesion localization (obtained by VLSM) associated with G5 z-score (a global cognitive score), were included as independent variables with other neuroimaging and clinical indices in a stepwise linear regression model. The mean age was 70.3 years and the mean MMSE score 21.3. On MRI, 75 patients had at least one stroke lesion. The G 5 z-score was associated with GM density in the pattern selected by the VBM analysis (R2 variation = 0.166, p < 0.001) and the presence of a stroke lesion in the region selected by the VSLM analysis (mainly in the right frontal region; R2 variation = 0.018, p = 0.008). The interaction between the two factors was insignificant (p = 0.374). In conclusion, in this first study combining VBM and VLSM analysis in a memory clinic, global cognition was associated with a specific GM atrophy pattern and the presence of a stroke lesion mainly in the right frontal region.

Secreted recombinant P53 protein from Pichia pastoris is a useful antigen for detection of serum p53: autoantibody in patients with advanced colorectal adenocarcinoma.

The detection of P53 alteration by serological method is easier to perform, does not require tumor tissues and is of interest for patients monitoring. In this study, we described the development of a home made ELISA test based on recombinant human P53 protein produced in Pichia pastoris and used as antigen for the detection of serum p53-Abs in colorectal carcinoma patients. The human P53 was secreted as a His-tagged protein by recombinant KM71 strain (Kα21) via the peptide signal α of the Saccharomyces cerevisiae mating type gene. The recombinant P53-His was able to detect p53-Abs in 23.4% of patients. Serum p53-Abs correlated significantly with surgical treatment (P = 0.007), relapse during follow-up (P = 0.036), depth of invasion (P = 0.036) and the level of CA19-9 (P = 0.034). Survival analysis showed that patients negative for serum p53-Abs exhibited a prolonged disease free survival period (P log rank = 0.012). In conclusion, the secreted recombinant human P53-His produced in P. pastoris seems to be a useful antigen for detection of serum p53 Abs in patients with colorectal carcinoma.

Retinal Vascular Density Using Optical Coherence Tomography-Angiography in Optic Neuritis.

The aim of this study is to access the perifoveolar and peripapillary vascular density (VD) using optical coherence tomography-angiography (OCT-A) in eyes with optic neuritis (ON) and in fellow eyes, then compare that to healthy controls. METHOD: This is a cross-sectional study including 22 patients with unilateral ON and 20 control eyes of healthy subjects. A complete clinical examination and OCT-A were performed at least 6 months after the acute episode of optic neuritis. Vascular plexuses of the peripapillary and perifoveolar images obtained from OCT-A were used to calculate the VD in each plexus: superficial, deep, and peripapillary capillaries for each group (ON eyes, fellow eyes, healthy eyes). RESULTS: Compared to healthy control eyes, in the peripapillary area, we found a significant decrease in VD not only in ON eyes but also in fellow eyes in average (p ≤ 0.05) and in the temporal sector (p < 0.001). In the perifoveolar area, the VD of the superficial capillary plexus is decreased in all sectors (p < 0.001) in ON eyes and only in the upper sector (p = 0.037) of fellow eyes compared to control eyes. VD correlates with ganglion cell layer (GCL) thickness in ON and in fellow eyes. CONCLUSION: Peripapillary vascular density is decreased in both affected eyes and fellow eyes after a unilateral episode of optic neuritis, suggesting a subclinical involvement of the disease. Further studies are needed to clarify the mechanism and clinical implications of these data.