Publications by authors named "Thierry Gendre"
Background: Neuropathies associated with IgA monoclonal gammopathy are poorly understood, and the interpretation of the presence of such gammopathy in a patient with neuropathy may be challenging.
Methods: The neurological and hematological features of all patients newly diagnosed with IgA gammopathy by immunofixation in our center from January 2016 to December 2020 were retrospectively analyzed. Patients with neuropathy were identified through the medical records.
Article Synopsis
- The study focuses on transthyretin cardiac amyloidosis (ATTR-CM) as a significant cause of heart failure in older patients, analyzing how age and amyloidosis subtype affect patient outcomes.
- It included 943 patients, revealing that geriatric patients (≥75 years) had worse health outcomes and lower 3-year survival rates (55%) compared to non-geriatric patients (<75 years) who had a survival rate of 76%.
- Key mortality predictors differed by age group, with geriatric patients relying on alkaline phosphatase and troponin T levels, while non-geriatric patients linked outcomes to NT-proBNP and glomerular filtration rates; a new 3-stage prognostic
Article Synopsis
- The study analyzed 166 patients with acute neurological symptoms linked to anti-GQ1b antibodies, revealing frequent symptoms like areflexia, sensory issues, and muscle weakness.
- The majority of patients were treated with intravenous immunoglobulins, leading to complete neurological recovery for 69% at the one-year mark, although 15% experienced relapses.
- Key predictors for incomplete recovery included age over 70, initial ICU admission, and absence of anti-GQ1b antibodies; no predictors for relapse were identified.
Background And Purpose: Peripheral neuropathy is a frequent complication of brentuximab vedotin (BV), used in CD30+ lymphoma treatment. Classic BV-induced neuropathy (BV-CN) is a mild distal sensory axonal polyneuropathy. Severe BV-induced inflammatory neuropathies (BV-IN) have been described.
View Article and Find Full Text PDFArticle Synopsis
- Recreational use of nitrous oxide (NO) has led to a rise in severe neurological disorders, particularly affecting young adults in economically disadvantaged areas.
- The study analyzed 181 patients, noting a significant incidence increase in 2020-2021, especially in those aged 20-25.
- Compared to other inflammatory neurological disorders, NI-NDs were found to be two to three times more common in these vulnerable populations.
Background And Objectives: Immune-mediated small fiber neuropathy (SFN) is increasingly recognized. Acute-onset SFN (AOSFN) remains poorly described. Herein, we report a series of AOSFN cases in which immune origins are debatable.
View Article and Find Full Text PDFArticle Synopsis
- Early diagnosis of cardiac amyloidosis (CA) is crucial for effective treatment, but it is often overlooked, misdiagnosed, and poorly managed.
- A retrospective study over 11 years analyzed 3,022 patients referred for suspected CA, showing a significant increase in referrals and a shift towards wild-type transthyretin amyloidosis (ATTRwt) being the most common diagnosis.
- The findings indicate that while awareness among cardiologists has grown, leading to more diagnoses of ATTRwt and less severe cases, patients with amyloid light chain (AL) amyloidosis still frequently present with severe cardiac symptoms.
Background: Neurological complications of systemic lupus erythematosus (SLE) are wide and may rarely involve the peripheral nervous system. However, no case of meningoradiculitis has been well-detailed.
Methods: We report a patient with lupus-associated meningoradiculitis.
Background: The three main cardiac amyloidosis (CA) types have different progression and prognosis. Little is known about the mode of death (MOD) which is commonly attributed to cardiovascular causes in CA. Improving MOD's knowledge could allow to adapt patient care.
View Article and Find Full Text PDFArticle Synopsis
- Charcot-Marie-Tooth (CMT) disease, a hereditary neuropathy, is often misdiagnosed as the treatable chronic inflammatory demyelinating polyradiculoneuropathy (CIDP).
- A study of 1104 patients found that 3.2% were misdiagnosed CMT cases, revealing key differences in age, symptoms, and treatment response compared to true CIDP patients.
- The financial analysis indicated that treating the misdiagnosed CMT patients cost significantly more (4.6 million euros) than conducting genetic tests for CMT (2.7 million euros), stressing the need for genetic analysis prior to CIDP diagnosis.
Aims: Hereditary (ATTRv) and wild-type (ATTRwt) transthyretin amyloidosis are severe and fatal systemic diseases, characterised by amyloid fibrillar accumulation principally in the heart or peripheral nerves (or both). Since 2012, tafamidis has been used in France to treat patients with ATTRv with neuropathy (alone or combined with cardiomyopathy). Recently, the Phase III ATTR-ACT trial showed that tafamidis decreased the relative risk of mortality in ATTR amyloidosis with cardiomyopathy.
View Article and Find Full Text PDFPurpose: Hereditary transthyretin amyloidosis (hATTR) is a severe adult-onset progressive disease mainly involving the peripheral nervous system and the heart, with a prominent impact on the autonomic nervous system. This review summarizes the clinical aspects of autonomic dysfunction in hATTR, and their impact on quality of life as well as potential therapeutic options.
Methods: Literature review.
Objective: Patients with functional movement disorders (FMD) often report a disability and psychiatric comorbidities. However, few studies have compared these aspects in FMD and in organic movement disorders (OMD). The objectives were to compare QoL and psychiatric comorbidities of FMD and OMD patients.
View Article and Find Full Text PDFBackground: Stiff-person syndrome (SPS) is a rare disorder characterized by progressive muscle stiffness, rigidity, and spasms involving the axial muscles. Acute respiratory distress has rarely been reported in this condition.
Methods: We report a case of a 49-year-old woman with autoimmune SPS diagnosed during an episode of acute respiratory failure secondary to repetitive episodes of apnea, requiring intensive care.