Background:
Cervical dystonia is the most common form of focal dystonia. The frequency and pattern of degenerative changes of the cervical spine in patients with cervical dystonia and their relation ...to clinical symptoms remain unclear as no direct comparison to healthy controls has been performed yet. Here, we used magnetic resonance imaging (MRI) to investigate (1) whether structural abnormalities of the cervical spine are more common in patients with cervical dystonia compared to age-matched healthy controls, (2) if there are clinical predictors for abnormalities on MRI, and (3) to calculate the inter-rater reliability of the respective radiological scales.
Methods:
Twenty-five consecutive patients with cervical dystonia and 20 age-matched healthy controls were included in the study. MRI scans of the cervical spine were analyzed separately by three experienced raters blinded to clinical information, applying different MRI rating scales. Structural abnormalities were compared between groups for upper, middle, and lower cervical spine segments. The associations between scores differentiating both groups and clinical parameters were assessed in dystonia patients. Additionally, inter-rater reliability of the MRI scales was calculated.
Results:
Comparing structural abnormalities, we found minor differences in the middle cervical spine, indicated by a higher MRI total score in patients but no significant correlation between clinical parameters and MRI changes. Inter-rater reliability was satisfying for most of the MRI rating scales.
Conclusion:
Our results do not provide evidence for a role of MRI of the cervical spine in the routine work-up of patients with cervical dystonia in the absence of specific clinical signs or symptoms.
Cerebral white matter lesions (WML) have been found in normal aging, vascular disease and several neuropsychiatric conditions. Correlations of WML with clinical parameters in BD have been described, ...but not with the number of affective episodes, illness duration, age of onset and Body Mass Index in a well characterized group of euthymic bipolar adults. Herein, we aimed to evaluate the associations between bipolar course of illness parameters and WML measured with volumetric analysis.
In a cross-sectional study 100 euthymic individuals with BD as well as 54 healthy controls (HC) were enrolled to undergo brain magnetic resonance imaging using 3T including a FLAIR sequence for volumetric assessment of WML-load using FSL-software. Additionally, clinical characteristics and psychometric measures including Structured Clinical Interview according to DSM-IV, Hamilton-Depression, Young Mania Rating Scale and Beck's Depression Inventory were evaluated.
Individuals with BD had significantly more (F = 3.968, p < .05) WML (Mdn = 3710 mm3; IQR = 2961 mm3) than HC (Mdn = 2185 mm3; IQR = 1665 mm3). BD men (Mdn = 4095 mm3; IQR = 3295 mm3) and BD women (Mdn = 3032 mm3; IQR = 2816 mm3) did not significantly differ as to the WML-load or the number and type of risk factors for WML. However, in men only, the number of manic/hypomanic episodes (r = 0.72; p < .001) as well as depressive episodes (r = 0.51; p < .001) correlated positively with WML-load.
WML-load strongly correlated with the number of manic episodes in male BD patients, suggesting that men might be more vulnerable to mania in the context of cerebral white matter changes.
Celotno besedilo
Dostopno za:
DOBA, IZUM, KILJ, NUK, PILJ, PNG, SAZU, SIK, UILJ, UKNU, UL, UM, UPUK
Abstract Švehlík M, Zwick EB, Steinwender G, Linhart WE, Schwingenschuh P, Katschnig P, Ott E, Enzinger C. Gait analysis in patients with Parkinson's disease off dopaminergic therapy. Objective To ...compare time-distance, kinematic, and kinetic gait parameters in patients with idiopathic Parkinson's disease (PD) off dopaminergic therapy with a group of healthy control subjects. Design A group-comparison study. Setting Gait analysis laboratory. Participants Patients with PD (n=20) and healthy age-matched controls (n=20). Interventions Not applicable. Main Outcome Measures Time-distance, kinematic, and kinetic gait variables. Results PD patients walked slower with shorter stride-length, comparable cadence, and longer double support times. Kinematics showed a reduction of the range of motion in the hip, knee, and ankle joints. Maximum hip extension and the ankle plantar flexion were significantly reduced. Kinetic gait parameters showed reduced push-off ankle power and lift-off hip power generation. Strong correlations between these important body advancement mechanisms and the walking velocity were observed. Conclusions In addition to previously described dysfunctional kinematics, abnormal kinetic parameters play an important role in the characterization of gait in PD patients off therapy. Hence, these parameters could be used to document treatment effects of parkinsonian gait disorders.
Zusammenfassung
Patienten mit einer Parkinson-Erkrankung oder einem Tremor können zusätzliche funktionelle Symptome aufweisen. Diese können sowohl vor oder aber auch erst nach Manifestation der ...eigentlichen nichtfunktionellen Erkrankung auftreten. Dies erschwert die Differenzialdiagnose erheblich. Bei der Parkinson-Krankheit liegt die Prävalenz einer zusätzlichen funktionellen Bewegungsstörung bei 7 %. Bei Tremorsyndromen ist diese nicht systematisch untersucht. Beim funktionellen Parkinsonismus findet man am häufigsten motorische Beschwerden wie beispielsweise einen zusätzlichen Tremor gefolgt von Gangstörungen. Klassische klinische Zeichen sind eine auffallende, ablenkbare und variable Langsamkeit bei automatisierten, alltäglichen Aufgaben, eine flüsternde Sprache, eine Bradykinese im Finger-Tapping ohne Dekrement, ein variabler Tremor oder ein auffälliges Schnaufen, Grimassieren oder Atemanhalten beim Gehen. Eine Dopamintransporterszintigraphie (123)I-FP-CIT-SPECT; DaTSCAN™ kann in der Differenzialdiagnose eines funktionellen Parkinsonismus sehr hilfreich sein. Die Diagnose eines funktionellen Tremors bei einer bestehenden Tremorerkrankung erfolgt auf demselben Wege wie bei einem ausschließlich funktionellen Tremor. Dazu zählen die motorischen, kognitiven und suggestiven Ablenkungsmanöver ebenso wie etablierte neurophysiologische Messmethoden mithilfe der Akzelerometrie. Das Erkennen funktioneller Symptome im Rahmen anderer Bewegungsstörungen ist von großer Bedeutung, um frühzeitig entsprechende Therapiemöglichkeiten auszuschöpfen. Neben der Erläuterung der Diagnose ist eine anschließende multidisziplinäre Behandlung, die Physio‑, Ergo- und Sprachtherapie sowie psychotherapeutische Interventionen, insbesondere kognitive Verhaltenstherapie, umfasst, notwendig.
Functional tremor Schwingenschuh, Petra; Espay, Alberto J.
Journal of the neurological sciences,
04/2022, Letnik:
435
Journal Article
Recenzirano
Odprti dostop
Functional tremor is the most common functional movement disorder. It can be diagnosed with clinically definite certainty at the bedside by ascertaining its inconsistent (distractibility, frequency ...variability) and incongruent features (entrainment, ballistic suppression), requiring no additional neurological investigations except, in selected cases, those serving to elevate the diagnostic category to laboratory supported using accelerometry and surface electromyography. In the background of excessive attention to the affected body part and abnormal beliefs and expectations, functional correlates include the impairment of emotion processing, sense of agency, and abnormal connectivity between limbic and motor regions. While the treatment options remain under-studied, promising interventions in physiotherapy, cognitive behavioral therapy, and other psychotherapies are under evaluation to assessing their efficacy in attenuating this important source of neurological disability.
This article is part of the Special Issue “Tremor” edited by Daniel D. Truong, Mark Hallett, and Aasef Shaikh.
•Highly reliable clinical signs allow an inclusionary diagnosis of functional tremor.•Neurophysiology can provide a laboratory-supported diagnostic certainty in unclear cases.•Pathophysiological abnormalities include excessive attentional focus, discrepant beliefs / expectations, and abnormal sense of agency.•Psychological abnormalities are neither necessary nor sufficient for the diagnosis.•Early individually-tailored multidisciplinary treatment should be offered.
The Purdue Pegboard Test (PPT) is widely used as a measure of manual dexterity. Declining manual dexterity may predict cognitive decline among elderly people, but normative data for this population ...are scarce.
To identify demographic and clinical predictors of PPT results in normal middle-aged and elderly Austrian people and to provide norms stratified by significant determinants.
A prospective, community-based cohort study using baseline data of participants from two study panels (1991-1994 and 1999-2003).
Monocentric study Participants: 1,355 healthy, randomly selected, community-dwelling people ages 40 to 79 yr.
Extensive clinical examination, including completion of the PPT.
The number of pegs placed within a 30-s time limit on four subtests: using the right hand, left hand, both hands, and assembly (within 60 s), respectively. Demographic outcomes were the highest grade achieved.
For all four subtests, increasing age (βs = -0.400 to -0.118, SEs = 0.006 to 0.019, p < .001) and male sex (βs = -1.440 to -0.807, SEs = 0.107 to 0.325, p < .001) was related to worse test results. Among vascular risk factors, diabetes (βs = -1.577 to -0.419, SEs = 0.165 to 0.503, p < .001) was related to worse test results but explained only a small portion (0.7%-1.1%) of the variability in PPT performance.
We provide age- and sex-specific norms of the PPT for a middle-aged and elderly population. The data represent useful reference values when assessing manual dexterity in older age groups. What This Article Adds: Advancing age and male sex relate to worse performance on the PPT in a community-dwelling cohort without signs and symptoms of neurological disease. Vascular risk factors explain only very little of the variance of test results in our population. Our study adds to the limited age- and sex-specific norms of the PPT among middle-aged and older people.
Patients with Parkinson's Disease or a tremor syndrome may present with additional functional movement disorders. The differential diagnosis is particularly difficult. In some cases, functional ...symptoms occur either before the manifestation of the organic disease or can emerge as an additional symptom after Parkinson's disease or tremor became apparent. In patients with Parkinson's disease the prevalence for additional functional symptoms is 7 %. In the case that patients with Parkinson's diseases have one side that is more severely affected, additional functional motor symptoms such as functional rest tremor also occur on that same, predominantly affected side. Functional gait disorders occur frequently. Clinically, patients appear notably slow in automatized, daily tasks. Their speech is more whispering than hypophonic, bradykinesia during finger tapping manifest without a decrement. The Dopamintransporterszintigraphy (123) I FP-CIT SPECT; DaTSCANTM) may be helpful to differentiate between functional Parkinsonism and Parkinson's disease. Functional tremor in patients with an organic tremor syndrome is diagnosed with the same distraction techniques as in solely functional tremor. This includes cognitive, motor, and suggestive distraction maneuvers. In some cases, additional neurophysiological investigations such as accelerometry are useful for the differential diagnosis. It is most important to identify patients with additional functional symptoms in non-functional movement disorders, because the therapeutic approach differs and a multi professional team is required to initiate effective treatment strategies.
BACKGROUNDHolmes' tremor is characterized by a combination of rest, postural, and kinetic tremor that is presumably caused by interruption of cerebello-thalamo-cortical and nigrostriatal pathways. ...Medical treatment remains unsatisfactory. CASE REPORTA 16-year-old girl presented with Holmes' tremor caused by a transient midbrain abnormality on magnetic resonance imaging (MRI). To explore the discrepancy between persistent tremor and resolved MRI changes, we performed dopamine transporter single-photon emission computed tomography (DaT-SPECT) with a 123I-ioflupane that revealed nearly absent DaT binding in the right striatum. Levodopa dramatically improved the tremor. DISCUSSIONThis is only the second report of a transient midbrain MRI abnormality disrupting nigrostriatal pathways. The case highlights the sometimes limited sensitivity of morphologic imaging for identifying the functional consequences of tissue damage and confirms that DaT imaging may serve as a predictor for levodopa responsiveness in Holmes' tremor.
Background and purpose
Quantification of neurofilament light chain protein in serum (sNfL) enables the neuro‐axonal damage in peripheral blood to be reliably assessed and monitored. There is a ...long‐standing debate whether essential tremor represents a ‘benign’ tremor syndrome or whether it is linked to neurodegeneration. This study aims to investigate sNfL concentrations in essential tremor compared to healthy controls (cross‐sectionally and longitudinally) and to assess whether sNfL is associated with motor and nonmotor markers of disease progression.
Methods
Data of patients with essential tremor from our prospective registry on movement disorders (PROMOVE) were retrospectively analysed. Age‐, sex‐ and body‐mass‐index‐matched healthy controls were recruited from an ongoing community‐dwelling aging cohort. sNfL was quantified by an ultra‐sensitive single molecule array (Simoa). All participants underwent detailed clinical examination at baseline and after approximately 5 years of follow‐up.
Results
Thirty‐seven patients with clinically diagnosed essential tremor were included and 37 controls. The essential tremor group showed significantly higher sNfL levels compared to healthy controls at baseline and follow‐up. sNfL levels increased over time in both groups, and the slope of sNfL increase was similar in the essential tremor and healthy control groups. Comparing patients with a disease duration under 5 years to those with a longer disease duration, the former group had a significantly greater increase of sNfL over time, which strongly correlated to worsening of tremor and cognition.
Conclusion
Our findings indicate that neurodegeneration, possibly happening at an early disease stage, might play a role in the pathophysiology of essential tremor.