Publications by authors named "K Viala"
Article Synopsis
- Hereditary transthyretin amyloidosis (ATTRv) is a genetic disease that typically starts in adults and leads to progressive nervous system issues, but there are effective treatments available.
- A study conducted in France over five years analyzed 553 patients over 50 with progressive nerve problems, finding that 2.7% had pathogenic gene variants linked to ATTRv, primarily the Val30Met variant.
- Patients with ATTRv were more likely to experience symptoms like severe weight loss and orthostatic hypotension, and the diagnosis allowed for specific treatments and identified additional cases in family members, emphasizing the importance of early detection.
Investigation and Management of Immunoglobulin M- and Waldenström-Associated Peripheral Neuropathies.
Hematol Oncol Clin North Am
August 2023
Article Synopsis
- IgM-associated peripheral neuropathies (PN) are a diverse group of disorders linked to conditions like IgM monoclonal gammopathy of undetermined significance (MGUS) or Waldenström macroglobulinemia.
- Identifying the relationship between paraproteins and neuropathies is crucial for determining effective treatment strategies.
- The most common IgM-PN is Antimyelin-Associated-Glycoprotein neuropathy, but many cases have different underlying causes; treatment options include rituximab or chemotherapy, especially when functional impairment worsens.
Article Synopsis
- * A study analyzed nine patients without a prior history of blood cancer, confirming neurolymphomatosis through detailed clinical evaluations including nerve biopsies, imaging, and lab tests over a 15-year period.
- * The findings showed common symptoms of neuropathy including pain and weakness, with many patients experiencing rapid deterioration, highlighting the need for awareness of this condition when diagnosing peripheral neuropathy.
Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a rare autoimmune disorder of the peripheral nervous system, primarily affecting the myelin sheath. The pathophysiology of CIDP is complex, involving both humoral and cellular immunity. The diagnosis of CIDP should be suspected in patients with symmetrical proximal and distal motor weakness and distal sensory symptoms of progressive onset, associated with decreased/abolished tendon reflexes.
View Article and Find Full Text PDFBackground And Purpose: In this retrospective study involving 14 university hospitals from France and Switzerland, the aim was to define the clinicopathological features of chronic neuropathies with anti-disialosyl ganglioside immunoglobulin M (IgM) antibodies (CNDA).
Results: Fifty-five patients with a polyneuropathy evolving for more than 2 months and with at least one anti-disialosyl ganglioside IgM antibody, that is, anti-GD1b, -GT1b, -GQ1b, -GT1a, -GD2 and -GD3, were identified. Seventy-eight percent of patients were male, mean age at disease onset was 55 years (30-76) and disease onset was progressive (82%) or acute (18%).