Objectives: (1)To describe the prevalence and severity of tremor in patients with multiple sclerosis (MS) registered within a large North American MS registry; (2) to provide detailed descriptions on the characteristics and severity of tremor in a subset of registrants and (3) to compare several measures of tremor severity for strength of agreement.
Setting: The North American Research Committee on MS (NARCOMS) registry.
Participants: Registrants of NARCOMS reporting mild or greater tremor severity.
Outcome Measures: We determined the cross-sectional prevalence of tremor in the NARCOMS registry over three semiannual updates between fall 2010 and fall 2011. A subset of registrants (n=552) completed a supplemental survey providing detailed descriptions of their tremor. Outcomes included descriptive characteristics of their tremors and correlations between outcome measures to determine the strength of agreement in assessing tremor severity.
Results: The estimated prevalence of tremor in NARCOMS ranged from 45% to 46.8%, with severe tremor affecting 5.5-5.9% of respondents. In the subset completing the supplemental survey, mild tremor severity was associated with younger age of MS diagnosis and tremor onset than those with moderate or severe tremor. However, tremor severity did not differ by duration of disease or tremor. Respondents provided descriptions of tremor symptoms on the Clinical Ataxia Rating Scale, which had a moderate to good (ρ=0.595) correlation with the Tremor Related Activities of Daily Living (TRADL) scale. Objectively scored Archimedes' spirals had a weaker (ρ=0.358) correlation with the TRADL. Rates of unemployment, disability and symptomatic medication use increased with tremor severity, but were high even among those with mild tremor.
Conclusions: Tremor is common among NARCOMS registrants and severely disabling for some. Both ADL-based and symptom-descriptive measures of tremor severity can be used to stratify patients.
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http://dx.doi.org/10.1136/bmjopen-2014-006714 | DOI Listing |
Cerebellum
January 2025
Department of Neurology, Division of Neuro-Visual & Vestibular Disorders, The Johns Hopkins University School of Medicine, Johns Hopkins Hospital, 600 N. Wolfe Street, Baltimore, MD, USA.
A 50-year-old woman with a 20-year history of gait instability presented with new-onset vertigo and oscillopsia. Examination revealed bilateral vestibular loss, cerebellar ataxia, sensory neuropathy, a "yes-yes" head tremor, nystagmus and a family history of a similar syndrome. Genetic testing for cerebellar ataxia with neuropathy and bilateral vestibular areflexia syndrome (RFC1) was negative, but whole exome sequencing identified a novel mutation in the DNA methyltransferase 1 (DNMT1) gene, broadening the differential diagnosis for this phenotype.
View Article and Find Full Text PDFBMJ Case Rep
January 2025
Internal Medicine, East Suffolk and North Essex NHS Foundation Trust Ipswich Hospital, Ipswich, UK.
This case report presents a complex medical scenario involving early 60s female patient with a history of chronic lymphocytic leukaemia (CLL) complicated by Evans syndrome, characterised by autoimmune haemolytic anaemia and immune thrombocytopenia. The patient had received various treatments, including steroids, rituximab, cyclosporine and acalabrutinib. The patient's neurological symptoms began around 3 years prior to presentation, with shaking of her right leg, followed by shaking of both hands, particularly the left hand.
View Article and Find Full Text PDFAlzheimers Dement
December 2024
Glenn Biggs Institute for Alzheimer's & Neurodegenerative Diseases, University of Texas Health Sciences Center at San Antonio, San Antonio, TX, USA.
Background: Lewy bodies (LBs), characterized by intraneuronal inclusions of misfolded alpha-synuclein (α-syn) protein, are the pathological hallmark of Parkinson's disease (PD) and dementia with Lewy bodies (DLB). Because this protein is phosphorylated at serine-129 in 90% of LBs, its phosphorylation is considered a crucial pathogenic event in LB formation and disease development. Here, we present a unique brain autopsy case of a DLB patient with widespread LBs that were negative for phosphorylated-α-syn, challenging traditional diagnostic criteria.
View Article and Find Full Text PDFHistorically, screening for incidence of AD-related MCI or conversion from MCI to AD dementia has relied on cognitive, activities of daily living, and brain imaging measures. Limitations of this diagnostic approach include dependency on education and language, time-consuming and costly measures, and long-term monitoring. Emerging studies suggest that non-tremor motor dysfunction in dementias is known to be highly associated with AD biomarkers, with signs of cognitive decline visible in gait and hand movement at various stages of the illness.
View Article and Find Full Text PDFAlzheimers Dement
December 2024
Department of Neurology, Division of Cognitive and Motor Aging, Albert Einstein College of Medicine, New York, NY, USA.
Background: Mild parkinsonian signs (MPS) are prevalent in older adults and linked to an increased risk of dementia. However, their association with Motoric Cognitive Risk syndrome (MCR), a pre-dementia syndrome characterized by slow gait speed and cognitive complaints, is unclear. This study aims to examine the association of MPS with incident MCR.
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