Tag: ataxia

Truncal ataxia or disequilibrium is an unrecognised cause of orthostatic intolerance in patients with myalgic encephalomyelitis

Introduction:

Chronic fatigue syndrome (CFS) causes a marked reduction in the activities of daily living and impairs the quality of life. Recently, dysfunction of the central nervous system associated with myalgic encephalomyelitis (ME) has been postulated as the main cause of CFS.1 Most patients with ME/CFS have orthostatic intolerance (OI) which is the primary factor restricting the daily functional capacity and in turn quality of life.2-4 OI is characterised by the inability to remain upright without severe signs and symptoms, such as hypotension, tachycardia, light-headedness, pallor, fatigue, weakness, dizziness, diminished concentration, tremulousness and nausea. Most symptoms of OI have been surmised to be related to reduced cerebral blood flow with or without impaired cerebral circulatory autoregulation, and the compensatory activation of the sympathetic nervous system.5, 6 Indeed, many patients have postural orthostatic tachycardia, delayed orthostatic hypotension and neurally mediated hypotension.4, 5, 7-9 Also many patients have low cardiac output in association with a small left ventricle.10-12 With further progression of the disease, patients may have even sitting intolerance and finally become bedridden.

Although static balance is an essential element for the performance of daily activities as well as postural stability, the possible relation between disequilibrium and OI has never been investigated. The possible role of static or truncal ataxia in the genesis of both orthostatic and sitting intolerance was examined in patients with ME.

Source: Miwa K, Inoue Y. Truncal ataxia or disequilibrium is an unrecognised cause of orthostatic intolerance in patients with myalgic encephalomyelitis. Int J Clin Pract. 2017 Jun;71(6). doi: 10.1111/ijcp.12967. PMID: 28613452. https://onlinelibrary.wiley.com/doi/10.1111/ijcp.12967 (Full text)

Pediatric de novo movement disorders and ataxia in the context of SARS-CoV-2

Abstract:

Objective: In the fourth year of the COVID-19 pandemic, mortality rates decreased, but the risk of neuropsychiatric disorders remained the same, with a prevalence of 3.8% of pediatric cases, including movement disorders (MD) and ataxia.

Methods: In this study, we report on a 10-year-old girl with hemichorea after SARS-CoV-2 infection and immunostained murine brain with patient CSF to identify intrathecal antibodies. Additionally, we conducted a scoping review of children with MD and ataxia after SARS-CoV-2 infection.

Results: We detected antibodies in the patient’s CSF binding unknown antigens in murine basal ganglia. The child received immunosuppression and recovered completely. In a scoping review, we identified further 32 children with de novo MD or ataxia after COVID-19. While in a minority of cases, MD or ataxia were a symptom of known clinical entities (e.g. ADEM, Sydenham’s chorea), in most children, the etiology was suspected to be of autoimmune origin without further assigned diagnosis. (i) Children either presented with ataxia (79%), but different from the well-known postinfectious acute cerebellar ataxia (older age, less favorable outcome, or (ii) had hypo-/hyperkinetic MD (21%), which were choreatic in most cases. Besides 14% of spontaneous recovery, immunosuppression was necessary in 79%. Approximately one third of children only partially recovered.

Conclusions: Infection with SARS-CoV-2 can trigger de novo MD in children. Most patients showed COVID-19-associated-ataxia and fewer-chorea. Our data suggest that patients benefit from immunosuppression, especially steroids. Despite treatment, one third of patients recovered only partially, which makes up an increasing cohort with neurological sequelae.

Source: Wilpert NM, de Almeida Marcelino AL, Knierim E, Incoronato P, Sanchez-Sendin E, Staudacher O, Drenckhahn A, Bittigau P, Kreye J, Prüss H, Schuelke M, Kühn AA, Kaindl AM, Nikolaus M. Pediatric de novo movement disorders and ataxia in the context of SARS-CoV-2. J Neurol. 2023 Jul 29. doi: 10.1007/s00415-023-11853-5. Epub ahead of print. PMID: 37515734. https://link.springer.com/article/10.1007/s00415-023-11853-5 (Full text)