Tremor in inflammatory neuropathies Saifee, Tabish Aziz; Schwingenschuh, Petra; Reilly, Mary M ...
Journal of neurology, neurosurgery and psychiatry,
11/2013, Letnik:
84, Številka:
11
Journal Article
Recenzirano
Tremor is known to occur in patients with neuropathies although its reported prevalence varies widely. Tremor has been shown to cause disability in children with Charcot-Marie-Tooth disease but no ...data exit about the disability caused by tremor in inflammatory neuropathies. Little is known about the response of neuropathic tremor to treatment and why it selectively occurs in some people and not others.
This case control study investigates the presence and severity of tremor in 43 consecutively recruited patients with inflammatory neuropathies at the National Hospital for Neurology and Neurosurgery, London. Clinical assessment, including Fahn-Tolosa-Marin Scale for tremor, sensory scores, power scores and Overall Neuropathy Limitations Scale, were recorded. Results of nerve conduction studies were retrieved and assessed. Nine patients' tremors were recorded with accelerometry.
Tremor was most common in IgM paraproteinaemic neuropathies, as previously reported, but also occurred in 58% of those with chronic inflammatory demyelinating polyradiculoneuropathy and 56% of those with multifocal motor neuropathy with conduction block. We describe, for the first time, tremor in the majority of patients with multifocal motor neuropathy with conduction block. Tremor in all of these patients seems generally refractory to treatment except in a small number of cases where tremor improves with treatment of the underlying neuropathy. We provide evidence that tremor may add to disability in patients with inflammatory neuropathy. Mean tremor frequency was 6 Hz and did not vary with weight loading. We demonstrate for the first time that although tremor severity correlates with F wave latency, it is not sufficient to distinguish those with, from those without, tremor.
Tremor in inflammatory neuropathies is common, adds to disability and yet does not often respond to treatment of the underlying neuropathy. When present, tremor severity is associated with F wave latency.
Abstract Background The diagnosis of psychogenic paroxysmal movement disorders (PPMD) can be challenging, in particular their distinction from the primary paroxysmal dyskinesias (PxD) remains ...difficult. Methods Here we present a large series of 26 PPMD cases, describe their characteristics, contrast them with primary PxD and focus on their distinguishing diagnostic features. Results Mean age at onset was 38.6 years, i.e. much later than primary PxD. Women were predominantly affected (73%). Most subjects (88.4%) had long attacks, and unlike primary PxD there was a very high within-subject variability for attack phenomenology, duration and frequency. Dystonia was the most common single movement disorder presentation, but 69.2% of the patients had mixed or complex PxD. In 50% of PPMD cases attack triggers could be identified but these were unusual for primary PxD. 42.3% of patients employed unusual strategies to alleviate or stop the attacks. Response to typical medication used for primary PxD was poor. Precipitation of the disorder due to physical or emotional life events and stressors were documented in 57.6% and 65.3% of the cases respectively. Additional interictal psychogenic signs were documented in 34.6% and further medically unexplained somatic symptoms were present in 50% of the cases. 19.2% of patients had a comorbid organic movement disorder and 26.9% had pre-existing psychiatric comorbidities. Conclusion Although the phenotypic presentation of PPMD can be highly diverse, certain clinical characteristics help in distinguishing this condition from the primary forms of PxD. Recognition is important as multidisciplinary treatment approaches led to significant improvement in most cases.
The knowledge about personality traits in Parkinson's disease (PD) is still limited. In particular, disgust proneness has not been investigated as well as its neuronal correlates. Although several ...morphometric studies demonstrated that PD is associated with gray matter volume (GMV) reduction in olfactory and gustatory regions involved in disgust processing, a possible correlation with disgust proneness has not been investigated. We conducted a voxel-based morphometry analysis to compare GMV between 16 cognitively normal male PD patients with mild to moderate symptoms and 24 matched control subjects. All participants had answered questionnaires for the assessment of disgust proneness, trait anger and trait anxiety. We correlated questionnaire scores with GMV in both groups. The clinical group reported selectively reduced disgust proneness toward olfactory stimuli associated with spoilage. Moreover, they showed GMV reduction in the central olfactory system orbitofrontal cortex (OFC) and piriform cortex. Disgust items referring to olfactory processing were positively correlated with OFC volume in PD patients. Our data suggest an association between PD-associated neurodegeneration and olfactory related facets of the personality trait disgust proneness.
Attention to self in psychogenic tremor van Poppelen, Daniel; Saifee, Tabish A.; Schwingenschuh, Petra ...
Movement disorders,
December 2011, Letnik:
26, Številka:
14
Journal Article
Positron emission tomography and single photon emission computed tomography scanning have 87-94% sensitivity and 80-100% specificity to differentiate patients with Parkinson's disease (PD) from ...control subjects and patients with essential (ET) or atypical tremor. More than 10% of patients diagnosed as early PD can have scans without evidence of dopaminergic deficiency (SWEDDs). This study investigated whether smell tests can help identify possible cases with SWEDDs.
The 40 item University of Pennsylvania Smell Test (UPSIT) was used to evaluate the sense of smell in 21 SWEDDs patients. Twenty-six ET patients, 16 patients with a diagnosis of idiopathic adult onset dystonia (D), 191 non-demented PD patients and 136 control subjects were also tested. Multiple regression analyses were used to compare the mean UPSIT score in the SWEDDs group with the other four groups (ET, D, PD and controls) after adjusting for the effects of relevant covariates.
The mean UPSIT score for the SWEDDs group was greater than in the PD group (p<0.001) and not different from the mean UPSIT in the control (p = 0.7), ET (p = 0.4) or D (p = 0.9) groups. Smell tests indicated a high probability of PD in only 23.8% of SWEDDs as opposed to 85.3% of PD patients.
In a patient with suspected PD, a high PD probability on smell testing favours the diagnosis of PD, and a low PD probability strengthens the indication for dopamine transporter imaging.
Despite extensive research over the last decades the clinical significance of white matter lesions (WMLs) is still a matter of debate. Here, we review current knowledge of the correlation between ...WMLs and cognitive functioning as well as their predictive value for future stroke, dementia, and functional decline in activities of daily living. There is clear evidence that age-related WMLs relate to all of these outcomes on a group level, but the inter-individual variability is high. The association between WMLs and clinical phenotypes exists particularly for early confluent to confluent changes, which are ischaemic in aetiology and progress quickly over time. One reason for the variability of the relationship between WMLs and clinic on an individual level is probably the complexity of the association. Numerous factors such as cognitive reserve, concomitant loss of brain volume, and ultrastructural changes have been identified as mediators between white matter damage and clinical findings, and need to be incorporated in the consideration of WMLs as visible markers of these detrimental processes.
Abstract Background Previous case series suggested a link between Klinefelter syndrome (KS) and essential tremor (ET) or an ET-like syndrome. Methods We investigated three KS-patients with tremor ...including tremor-analyzes and discuss our data in context to findings from a literature review. The clinical outcome after deep brain stimulation (DBS) is also reviewed. Results Tremor in KS is predominantly a postural and kinetic tremor that resembles ET. Our patients were further characterized by absent family history for tremor in first degree relatives, lack of subjective alcohol responsiveness inquired by history, and tremor onset in childhood. One of our patients and two cases from literature improved after DBS of the ventral intermediate nucleus (VIM) of the thalamus. Conclusions Tremor in KS shares several features with ET. If other characteristics such as family history, alcohol responsiveness, and age at tremor onset may serve as discriminating factors from ET, needs to be further investigated. First observations suggest that VIM-DBS may be efficacious.
Background
Parkinson’s disease (PD) is heterogeneous, both phenotypically and in terms of temporal progression. The Hoehn and Yahr (HY) scale is a well‐established PD staging approach, and identifies ...5 stages of the disease. Morphometric effects in deep gray matter regions of the brain associated with HY stages are complex; a recent large‐scale ENIGMA‐PD study showed higher local subcortical volumes in early HY stages relative to controls, followed by a precipitous decrease after stage 2 1. This finding motivates a closer look at fine‐level morphometry beyond gross volume measures. Here, we developed and applied a novel machine learning algorithm to reveal the subcortical shape signatures of HY staging.
Method
We computed shape features in 7 bilateral subcortical regions 2 based on T1‐weighted MRI data from 2,322 PD subjects and 1,207 controls from 20 ENIGMA‐PD cohorts (HY stages in Table 1). We developed a sparse, spatially coherent (total variation/TV‐L1) ordinal linear logistic classifier 3 to predict HY stages with a single linear model. We applied the model to vertex‐wise medial thickness features. We optimized regularization parameters for balanced recall (sensitivity) and precision using a 4‐fold cross‐validation grid search. Very low numbers of HY4 and HY5 samples necessitated merging stages 3‐5 into one category. For comparison, we also trained 4 binary TV‐L1 logit models on the same features 4, discriminating (1) PD‐Control; (2) HY1‐HY2; (3) HY1‐HY345; (4) HY2‐HY345, using ROC area‐under‐the‐curve (AUC) evaluation.
Result
Across‐stage mean out‐of‐sample precision and recall were 0.43, and 0.393, respectively (chance=0.33). Table 2 shows the confusion matrix and precision/recall for each HY stage. All models’ linear coefficient maps are displayed in Figures 1,2. Binary classification ROC‐AUC was 0.66 for PD‐Control, and ranged from 0.62 to 0.73 for HY prediction (Figure 2).
Conclusion
We developed an ordit machine learning model for morphometric shape‐based ordinal classification of disease stages, training it for Parkinson’s Disease Hoehn and Yahr stage prediction on a large MRI collection. Performance was substantially above chance. Model weight maps indicate early increased thalamic thickness, followed by a complex thinning pattern associated with later HY stages.
OBJECTIVES:This study aims to investigate if patients with inflammatory neuropathies and tremor have evidence of dysfunction in the cerebellum and interactions in sensorimotor cortex compared to ...nontremulous patients and healthy controls.
METHODS:A prospective data collection study investigating patients with inflammatory neuropathy and tremor, patients with inflammatory neuropathy without tremor, and healthy controls on a test of cerebellar associative learning (eyeblink classical conditioning), a test of sensorimotor integration (short afferent inhibition), and a test of associative plasticity (paired associative stimulation). We also recorded tremor in the arms using accelerometry and surface EMG.
RESULTS:We found impaired responses to eyeblink classical conditioning and paired associative stimulation in patients with neuropathy and tremor compared with neuropathy patients without tremor and healthy controls. Short afferent inhibition was normal in all groups.
CONCLUSIONS:Our data strongly suggest impairment of cerebellar function is linked to the production of tremor in patients with inflammatory neuropathy.
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