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Tremor
Kinds of Tremor Terms modified by Tremor Selected AbstractsComputerized Tremor Analysis of Valproate-induced Tremor: A Comparative Study of Controlled-release versus Conventional ValproateEPILEPSIA, Issue 2 2005Martina Rinnerthaler Summary:,Purpose: Valproate (VPA) induces postural tremor in 6,45% of patients. The characteristics of VPA-induced tremor have not yet been quantitatively assessed, and it is not known whether tremor prevalence or severity is affected by VPA formulation (controlled-release CR-VPA vs. conventional VPA). The aim of this study was quantitatively to assess tremor in epilepsy patients receiving VPA and to compare the effects of two VPA formulations (CR-VPA vs. VPA) on tremor severity. Methods: In a prospective study, 18 consecutive patients with newly diagnosed focal or generalized epilepsy were assigned to receive alternately either VPA (n = 10) or CR-VPA (n = 8) monotherapy. Computerized tremor analysis was performed at baseline 1 day before initiating VPA treatment and repeated after a seizure-free period of ,8 weeks, during which VPA doses had remained stable. Rest and postural tremor were recorded by accelerometry, and surface electromyograms (EMGs) were recorded from the wrist flexors and extensors. Results: At baseline, the two groups had similar postural tremor amplitudes. At follow-up, the CR-VPA group had remained at the same level, whereas VPA subjects exhibited a significant increase in tremor amplitudes (p < 0.05) despite comparable VPA doses and comparable plasma VPA concentrations at the time of tremor testing. Conclusions: This is the first study to assess quantitatively VPA-induced tremor by standardized tremor analysis. These results suggest that CR-VPA may cause less tremorigenic activity as compared with standard VPA. The mechanisms underlying this difference are unclear but may include greater peak,trough variation with VPA than with CR-VPA. [source] A preliminary look at the percentage of patients with Restless Legs Syndrome who also have Parkinson Disease, Essential Tremor or Tourette Syndrome in a single practiceJOURNAL OF SLEEP RESEARCH, Issue 4 2003Arthur S. Walters No abstract is available for this article. [source] Midbrain SERT in degenerative parkinsonisms: A 123I-FP-CIT SPECT study,MOVEMENT DISORDERS, Issue 12 2010Francesco Roselli MD Abstract SPECT imaging is widely used for the differential diagnosis of degenerative parkinsonisms by exploiting the high affinitiy of the radiotracer 123I-FP-CIT for the dopamine transporter. Reduced levels of DAT are found in Parkinson Disease (PD), Dementia with Lewy Bodies (DLB), and Progressive Supranuclear Palsy (PSP) compared to in Essential Tremor (ET) and Healthy Controls (HC). However, the extent of the neurodegenerative process may extend beyond nigrostriatal system. We have exploited the affinity of the same radiotracer 123I-FP-CIT for the serotonin transporter to investigate SERT levels in the midbrain of patients with PD, DLB, PSP, and ET compared to HC. Using MRI images as anatomical templates for midbrain uptake quantification, we found a mild decrease in SERT levels in PD compared to ET and HC, with marked inter-individual variability; on the other side, PSP and DLB patients displayed markedly reduced to undetectable levels of SERT, respectively. These findings show that the neurodegenerative process affects serotoninergic neurons in parkinsonisms, with much more severe involvement in DLB than in PD patients, despite the comparable loss of striatal DAT. SERT-dependent 123I-FP-CIT uptake may allow a more comprehensive assessment of neurochemical disturbances in degenerative parkinsonisms and may have a value for differential diagnosis. © 2010 Movement Disorder Society [source] Differentiating vascular parkinsonism from idiopathic Parkinson's disease: A systematic review,,MOVEMENT DISORDERS, Issue 2 2010Seema Kalra MRCP Abstract Vascular parkinsonism (VP) remains a loose constellation of various clinical features. We systematically reviewed studies comparing clinical, neuroimaging and other investigations that might distinguish VP from idiopathic Parkinson's disease (PD). Medline, Embase, Cinahl (R), and PsycINFO were searched by querying appropriate key words. Reports were included if the study population contained comparative findings between patients with VP and PD. Twenty-five articles fulfilled the selection criteria. Patients with VP were older, with a shorter duration of illness, presented with symmetrical gait difficulties, were less responsive to levodopa, and were more prone to postural instability, falls, and dementia. Pyramidal signs, pseudobulbar palsy, and incontinence were more common in VP. Tremor was not a main feature of VP. Structural neuroimaging was more likely to be abnormal in VP (90,100% of cases) than in PD (12,43% of cases), but there was no specific abnormal structural imaging pattern for VP. Two studies of presynaptic striatal dopamine transporters (using single photon emission computed tomography) showed a significant reduction in striatal uptake ratios in PD but not in VP, whereas another study found that only the mean asymmetry index was significantly lower in VP. Various other investigations, including alternative imaging techniques, electrophysiological, and neuropsychological studies, are reported, but the diverse diagnostic criteria used makes it difficult to reach any firm conclusions. The development of accepted international diagnostic criteria for VP is urgently needed to facilitate further studies. © 2010 Movement Disorder Society [source] Program: Twenty Third Annual Symposium on Etiology, Pathogenesis, and Treatment of Parkinson's Disease and Other Movement DisordersMOVEMENT DISORDERS, Issue 12 2009Article first published online: 11 SEP 200 The symposium will consist of two keynote speakers and peer-reviewed platform and poster presentations designed to communicate recent research advances, including new pharmacological and non-pharmacological treatment options, in the field of Parkinson's disease, Huntington disease, ataxia, dystonia, myoclonus, Tourette's syndrome, Essential Tremor and other movement disorders thereby enhancing patient care. Professionals in neurology and related disciplines as well as practitioners, psychologists, educators, and researchers are invited to attend. The gaps in clinical practice we wish to address are the unmet needs pertaining to the translational and clinical evaluation, along with the care and treatment of patients and families affected by Parkinson's disease and other movement disorders. At the conclusion of this session, participants should be able to: 1) Identify and describe by scholarly review, oral presentation and group discussion the current research into the diagnosis, prevention and treatment of Parkinson's disease (PD) and Essential Tremor (ET) which may be relevant to current treatment or which may lead to the development of further research protocols; 2) Distinguish and assess the important advances in research and clinical treatments relating to Parkinson's disease and Essential Tremor in terms of available treatment options or new methodologies for clinical research; 3) Explain new pharmacological and non-pharmacological treatment options available for Parkinson's disease and other movement disorders in connection with their clinical practice or with regard to further clinical research methods; 4) Interpret the mechanisms (genetic, environmental, pathophysiology, neurobiology) linked to Parkinson's disease and other movement disorders when assessing Parkinson's disease or other movement disorder patients or when developing new research protocols; and 5) Employ diagnostic approaches and tools available for assessing Parkinson's disease and Essential Tremor when diagnosing new patients or when conducting clinical research. [source] The clinical spectrum of freezing of gait in atypical parkinsonism,MOVEMENT DISORDERS, Issue S2 2008Stewart A. Factor DO Abstract Freezing of gait (FOG), commonly seen in advanced Parkinson's disease (PD), has been classified as its fifth cardinal feature. However, its presence frequently leads to a misdiagnosis of PD. FOG is actually more common in atypical parkinsonism (AP): including vascular Parkinsonism (VP), progressive supranuclear palsy (PSP), multiple system atrophy (MSA), corticobasal degeneration (CBD), dementia with Lewy bodies (DLB), and higher level gait disorders (HLGDs). VP is the result of multiple small vessel infarcts (lacunar state or Binswanger's disease), particularly involving the frontal, parietal, and basal ganglia regions. Approximately 50% have FOG (often referred to as lower body parkinsonism). FOG is also common in neurodegenerative forms of AP, present in 45,57%. Of these, FOG is present in 53% of PSP, 54% MSA, 54% DLB, 25% CBD, and 40% HLGD. It is generally seen in the late stages. There are two syndromes closely associated with AP that are dominated by FOG; pure akinesia (PA) and primary progressive freezing gait (PPFG). PA is characterized by akinesia of gait (including FOG), writing, and speech. Tremor, rigidity, dementia, and response to levodopa are notably absent. PPFG is defined by early FOG (often the initial feature) that progresses to include postural instability. It is accompanied by bradykinesia, rigidity, postural tremor, dementia, and levodopa unresponsiveness. Both syndromes are heterogeneous but PSP seems to be the most common cause. CBD and DLB can also present as PPFG. FOG is a common feature of AP and although typically occurring late in disease may also be an early symptom. © 2008 Movement Disorder Society [source] Silas Weir Mitchell's essential tremorMOVEMENT DISORDERS, Issue 9 2007Elan D. Louis MD Abstract Silas Weir Mitchell (1829,1914) is recognized as an important American neurologist. Biographers refer in brief to a tremor. The objective of this review was to characterize Mitchell's tremor using handwriting samples, to examine handwriting samples of family members to determine whether this tremor was familial, and study Mitchell's allusions to tremor in personal, scientific, and fictional writings. Primary sources were the Papers of S. Weir Mitchell, College of Physicians of Philadelphia, and Mitchell's scientific and fictional writings. Mitchell's early handwriting was tremor-free yet, by 1873, the writing was tremulous. Handwriting in the 1880s and 1890s shows clear oscillations of moderate-amplitude. By the first decade of the 20th century, his handwriting was virtually illegible. Letters written by two siblings, his mother, and maternal grandfather also reveal tremor. Tremor was not prominent in Mitchell's personal or scientific writings and Mitchell referred to tremor in only 4 of 27 fictional writings In conclusion, Mitchell had a familial action tremor that began when he was in his early 40's and worsened considerably with age. The likely diagnosis was essential tremor. Curiously, Mitchell rarely alluded to tremor in personal writings and tremor was not prominently featured in his scientific or fictional works. © 2007 Movement Disorder Society [source] Tremor induced by thalamic deep brain stimulation in patients with complex regional facial pain,MOVEMENT DISORDERS, Issue 8 2004Constantine Constantoyannis MD Abstract We report on two patients who developed a new postural and action tremor after chronic stimulation of the contralateral thalamus (VPM nucleus) during treatment of a complex regional facial pain syndrome. The tremor was only present during deep brain stimulation (DBS) and was suppressed with adjustment of the stimulation parameters. Tremor was seen only with low frequency stimulation (50 Hz or lower) and disappeared with higher stimulation frequencies. In addition to being an unusual side effect of thalamic DBS, we believe that this phenomenon affords insight into one possible mechanism underlying essential tremor (ET). A central oscillatory mechanism involving the olivocerebellar complex and the thalamus, which is a part of the cerebro,cerebello,cerebral circuit, is thought to play an important role in the genesis of ET. Induction of a tremor resembling ET in our patients indicates an active role for low frequency stimulation. A plausible explanation for this is that low frequency stimulation in the thalamic area enhances the output of the tremor-producing network. This leads credence to the concept of central oscillations in a "tremor circuit," of which the thalamus is a part, as being important in ET. © 2004 Movement Disorder Society [source] Cerebellar metabolic symmetry in essential tremor studied with 1H magnetic resonance spectroscopic imaging: Implications for disease pathologyMOVEMENT DISORDERS, Issue 6 2004Elan D. Louis MD Abstract The pathological basis for essential tremor (ET) is not known; however, metabolic changes in the cerebellum can be observed in positron emission tomography (PET) and 1H magnetic resonance spectroscopic imaging (MRSI) studies. Tremor is relatively symmetric in ET, suggesting that underlying metabolic changes could be also symmetric. The degree of metabolic asymmetry in the cerebellum, however, has not yet been studied in ET, and knowledge about distribution and laterality of metabolic changes might shed some light on basic disease mechanisms. We measured brain metabolism (N -acetylaspartate[NAA]/creatine [tCR]) to obtain an asymmetry index for cerebellar cortical metabolism ET patients compared with that in controls. This index, a percentage, was calculated as |(value right , value left)|/(value right + value left) × 100. Multislice 1H MRSI data were acquired for 20 patients and 11 controls. In ET patients, mean right and left cerebellar cortical NAA/tCR values were 1.61 ± 0.42 and 1.55 ± 0.38, respectively, compared with 1.81 ± 0.62 and 1.87 ± 0.49 in controls. The difference between right and left cerebellar cortical NAA/tCR was also calculated for each subject. In ET patients, the mean right-left difference was 0.14 ± 0.11, compared with 0.32 ± 0.27 in controls (P = 0.016). The mean cerebellar cortical asymmetry index was low in ET (8.8 ± 6.1%), one-half of that in controls (17.0 ± 13.7%, P = 0.027). These data suggest that pathological lesions in ET patients, which remain elusive, might be distributed similarly in each cerebellar cortex. Postmortem studies are needed to confirm these preliminary imaging results. © 2004 Movement Disorder Society [source] The pathophysiology of tremorMUSCLE AND NERVE, Issue 6 2001Günther Deuschl MD Abstract Tremor is defined as rhythmic oscillatory activity of body parts. Four physiological basic mechanisms for such oscillatory activity have been described: mechanical oscillations; oscillations based on reflexes; oscillations due to central neuronal pacemakers; and oscillations because of disturbed feedforward or feedback loops. New methodological approaches with animal models, positron emission tomography, and mathematical analysis of electromyographic and electroencephalographic signals have provided new insights into the mechanisms underlying specific forms of tremor. Physiological tremor is due to mechanical and central components. Psychogenic tremor is considered to depend on a clonus mechanism and is thus believed to be mediated by reflex mechanisms. Symptomatic palatal tremor is most likely due to rhythmic activity of the inferior olive, and there is much evidence that essential tremor is also generated within the olivocerebellar circuits. Orthostatic tremor is likely to originate in hitherto unidentified brainstem nuclei. Rest tremor of Parkinson's disease is probably generated in the basal ganglia loop, and dystonic tremor may also originate within the basal ganglia. Cerebellar tremor is at least in part caused by a disturbance of the cerebellar feedforward control of voluntary movements, and Holmes' tremor is due to the combination of the mechanisms producing parkinsonian and cerebellar tremor. Neuropathic tremor is believed to be caused by abnormally functioning reflex pathways and a wide variety of causes underlies toxic and drug-induced tremors. The understanding of the pathophysiology of tremor has made significant progress but many hypotheses are not yet based on sufficient data. Modern neurology needs to develop and test such hypotheses, because this is the only way to develop rational medical and surgical therapies. © 2001 John Wiley & Sons, Inc. Muscle Nerve 24: 716,735, 2001 [source] Mania as the first manifestation of Wilson's diseaseBIPOLAR DISORDERS, Issue 3 2008Alexandre Costa Machado Background:, Although mental changes are frequent in Wilson's disease, severe psychiatric disorders occur uncommonly and usually accompany the neurological picture. There are few reports in the literature of Wilson's disease patients with typical bipolar affective disorder (BPAD). Case report:, The authors report the case of a patient with Wilson's disease whose initial manifestation was a manic episode followed by depression. Tremor in the upper limbs appeared one year after the onset of symptoms. The diagnosis of Wilson's disease was established three years after the first symptoms appeared, based on the neuropsychiatric picture, the detection of Kayser,Fleischer rings and the results of diagnostic tests indicating chronic liver disease and copper excess. ATP7B genotyping and magnetic resonance imaging of the brain with proton spectroscopy study were also performed. The patient became asymptomatic two years after starting treatment with penicillamine and remained non-symptomatic controlled during the eight-year follow-up period, without any specific treatment for the BPAD. Conclusions:, To our knowledge, this is a singular report of a case of Wilson's disease in which a manic episode preceded the onset of neurological symptoms. The association between Wilson's disease and bipolar disorder is discussed. [source] Quality of life in a random sample of community dwelling older patients with essential tremorACTA NEUROLOGICA SCANDINAVICA, Issue 5 2007H. V. Nguyen Study Objective,,, Nested case-control study aimed to assess the quality of life of community dwelling participants aged 65 years or over with newly diagnosed Essential Tremor (ET). Methods and Results,,, Thirty-two participants with newly diagnosed ET and 32 age and gender matched controls were administered the Rand-SF36 quality of life questionnaire. Medical co-morbidities were also assessed in the two groups. Results,,, Participants with ET had significantly lower scores in the physical function, role limitation because of physical function, role limitation as a result of emotional problem, pain, and energy/vitality subscales of the Rand-SF36 when compared with controls. Conclusions,,, Older patients with newly diagnosed ET have poorer quality of life than their community dwelling counterparts without ET. [source] Fragile X-associated Tremor/Ataxia Syndrome (FXTAS)DEVELOPMENTAL DISABILITIES RESEARCH REVIEW, Issue 1 2004Paul J. Hagerman Abstract Carriers of fragile X mental retardation 1 (FMR1) premutation alleles (55 to 200 CGG repeats) are generally spared the more serious neurodevelopmental problems associated with the full-mutation carriers (>200 repeats) of fragile X syndrome. However, some adult male premutation carriers (55,200 repeats) develop a neurological syndrome involving intention tremor, ataxia, dementia, parkinsonism, and autonomic dysfunction. In excess of one-third of male premutation carriers over 50 years of age develop the fragile X- associated tremor/ataxia syndrome (FXTAS). FXTAS also represents a new form of inclusion disease, with eosinophilic intranuclear inclusions found throughout the brain in both neurons and astrocytes. Because FXTAS appears to be relatively specific to male premutation carriers, who are known to possess elevated levels of FMR1 mRNA, the neuropathology may arise as a consequence of a toxic gain-of-function of the mRNA itself, although this proposal requires additional direct testing. One of the critical needs at present is a better estimate for the prevalence of this disorder, because FXTAS is likely to be underdiagnosed in the adult movement disorders clinics. MRDD Research Reviews 2004;10:25,30. © 2004 Wiley-Liss, Inc. [source] Palatal tremor in childhood: clinical and therapeutic considerationsDEVELOPMENTAL MEDICINE & CHILD NEUROLOGY, Issue 12 2006J Campistol-Plana PhD Palatal tremor (PT) is a rhythmic movement of the soft palate that often causes an ear click. PT can be symptomatic (SPT) or essential (EPT). The symptomatic form usually occurs in adults and the essential form mainly occurs in children. Several different treatments for EPT in children appear in the literature with variable reported efficacy. This report details four paediatric patients with EPT (three males, one female; mean age 6y 4mo [SD 6mo]; age at onset 6,7y) treated with piracetam (2-oxo-1-pyrrolidine acetamide). Piracetam was used to treat EPT because of its antimyoclonic properties. All children showed a good response to doses of 100 to 300mg/kg/day. EPT relapsed on withdrawal of piracetam and remitted on reintroduction. Piracetam's effect on EPT was sustained. It is concluded that piracetam is an effective drug for the treatment of EPT in children. [source] Stimulant medication in 47,XYY syndrome: a report of two casesDEVELOPMENTAL MEDICINE & CHILD NEUROLOGY, Issue 8 2005Anne Ruud MD In two males, 11 and 12 years of age, referred for attention-deficit-hyperactivity disorder (ADHD), 47,XYY syndrome was diagnosed. A team that included a neuropsychologist, a physiotherapist, and a physician examined them. Stature (patients were above 97.5% height for age), muscle consistency, and tremor indicated chromosome analysis. Psychological tests results did not fully fit the ADHD diagnosis. On the basis of our clinical observation we felt that stimulant medication was indicated. Administration of methylphenidate led to improved motor and cognitive functions as well as social adaptation in both patients. We suggest that this treatment might well be considered in clinically similar patients with XYY sex chromosomes; we further suggest that learning problems in such individuals may be related to ADHD. [source] Gliomatosis cerebri in a 10,year-old girl masquerading as diffuse encephalomyelitis and spinal cord tumourDEVELOPMENTAL MEDICINE & CHILD NEUROLOGY, Issue 2 2001Sandeep Jayawant Gliomatosis cerebri is the unifying term used when diffuse glial infiltration occurs throughout the cerebral hemispheres. The very few cases reported in children have presented with intractable epilepsy, corticospinal tract deficits, unilateral tremor, headaches, and developmental delay. Antemortem diagnosis is difficult because of the vagueness of the physical, radiological and pathological findings. Adult cases may simulate an acute diffuse encephalomyelitis and show postmortem evidence of a marked swelling of the spinal cord. Apparently benign intracranial hypertension with papilloedema has also been recorded. We report a 10,year-old girl who presented with a history and physical signs suggestive of benign intracranial hypertension. A diffuse encephalomyelopathy occurred, which was complicated by spinal cord swelling, followed by deterioration and death. Gliomatosis cerebri affecting the brain and spinal cord was found at postmortem examination. [source] A review of studies describing the use of acetyl cholinesterase inhibitors in Parkinson's disease dementiaACTA PSYCHIATRICA SCANDINAVICA, Issue 6 2005I. D. Maidment Objective:, To review the literature relating to the use of acetyl cholinesterase inhibitors in Parkinson's disease dementia (PDD). Method:, MEDLINE (1966 , December 2004), PsychINFO (1972 , December 2004), EMBASE (1980 , December 2004), CINHAL (1982 , December 2004), and the Cochrane Collaboration were searched in December 2004. Results:, Three controlled trials and seven open studies were identified. Efficacy was assessed in three key domains: cognitive, neuropsychiatric and parkinsonian symptoms. Conclusion:, Cholinesterase inhibitors have a moderate effect against cognitive symptoms. There is no clear evidence of a noticeable clinical effect against neuropsychiatric symptoms. Tolerability including exacerbation of motor symptoms , in particular tremor , may limit the utility of cholinesterase inhibitors. [source] Clinical, neuropsychological, neurophysiologic, and genetic features of a new Italian pedigree with familial cortical myoclonic tremor with epilepsyEPILEPSIA, Issue 5 2009Antonio Suppa Summary We studied the clinical, neuropsychological, neurophysiologic, and genetic features of an Italian family with familial cortical myoclonic tremor with epilepsy (FCMTE). Clinically affected members of the family had limb and voice tremor, seizures, and myoclonus involving the eyelids during blinking. Neuropsychological testing disclosed visuospatial impairment, possibly due to temporal lobe dysfunction. Neurophysiologic findings suggested increased primary motor cortex excitability with normal sensorimotor integration. Linkage analysis excluded the 8q24 locus, where patients shared a common haplotype spanning 14.5 Mb in the pericentromeric region of chromosome 2. [source] Clinical picture of EPM1-Unverricht-Lundborg diseaseEPILEPSIA, Issue 4 2008Reetta Kälviäinen Summary Unverricht-Lundborg disease (ULD), progressive myoclonic epilepsy type 1 (EPM1, OMIM254800), is an autosomal recessively inherited neurodegenerative disorder characterized by age of onset from 6 to 16 years, stimulus-sensitive myoclonus, and tonic,clonic epileptic seizures. Some years after the onset ataxia, incoordination, intentional tremor, and dysarthria develop. Individuals with EPM1 are mentally alert but show emotional lability, depression, and mild decline in intellectual performance over time. The diagnosis of EPM1 can be confirmed by identifying disease-causing mutations in a cysteine protease inhibitor cystatin B (CSTB) gene. Symptomatic pharmacologic and rehabilitative management, including psychosocial support, are the mainstay of EPM1 patients' care. Valproic acid, the first drug of choice, diminishes myoclonus and the frequency of generalized seizures. Clonazepam and high-dose piracetam are used to treat myoclonus, whereas levetiracetam seems to be effective for both myoclonus and generalized seizures. There are a number of agents that aggravate clinical course of EPM1 such as phenytoin aggravating the associated neurologic symptoms or even accelerating cerebellar degeneration. Sodium channel blockers (carbamazepine, oxcarbazepine) and GABAergic drugs (tiagabine, vigabatrin) as well as gabapentin and pregabalin may aggravate myoclonus and myoclonic seizures. EPM1 patients need lifelong clinical follow-up, including evaluation of the drug-treatment and comprehensive rehabilitation. [source] Autosomal Dominant Early-onset Cortical Myoclonus, Photic-induced Myoclonus, and Epilepsy in a Large PedigreeEPILEPSIA, Issue 10 2006Elena Gardella Summary:,Purpose: Cortical tremor, a form of rhythmic cortical myoclonus (rhythmic CM), and epilepsy have been described in families with autosomal dominant inheritance. Linkage analyses revealed two putative loci on chromosome 2p and 8q. Clinical photosensitivity was not a prominent feature in such families. We describe a large Italian family with rhythmic CM, photosensitivity, and epilepsy. Methods: Twenty-three individuals of a five-generation family were studied. Linkage analyses for the loci on chromosome 2p11.1 and 8q23.3 were performed. Results: Of the 23 studied family members, 16 were affected. Rhythmic CM of childhood onset was present in all 16 individuals (onset ranging from 3 to 12 years), was associated with photic-induced myoclonic jerks in seven, and with epileptic seizures in six (onset ranging from 23 to 34 years). Five children of the V generation manifested also episodes of arousal with generalized tremor in early infancy ("tremulous arousals"). Jerk-locked back-averaging of rhythmic CM of six affected individuals, documented a premyoclonic EEG correlate. C-reflex at rest was present in two affected adults. Linkage analyses excluded mapping to the 2p11.1 and 8q23.3 loci. Conclusions: Clinical variability and severity of the phenotypes in this family are in line with those of previously described pedigrees with autosomal dominant cortical myoclonus and epilepsy. In this family, a progression of symptoms was found: rhythmic CM and tremulous arousals occurred in childhood, whereas visually induced manifestations and epileptic seizures occurred during adolescence,adulthood. Exclusion of linkage to the two known loci is consistent with genetic heterogeneity of such familial clustering of symptoms. [source] Adding Video Recording Increases the Diagnostic Yield of Routine Electroencephalograms in Children with Frequent Paroxysmal EventsEPILEPSIA, Issue 5 2005Nathan Watemberg Summary:,Purpose: To report on the usefulness of adding video recording to routine EEG studies of infants and children with frequent paroxysmal events. Methods: We analyzed the efficacy of this diagnostic means during a 4-year period. The decision whether to add video recording was made by the pediatric EEG interpreter at the time of the study. Studies were planned to last between 20 and 30 min, and, if needed, were extended by the EEG interpreter. For most studies, video recording was added from the beginning of EEG recording. In a minority of cases, the addition of video was implemented during the first part of the EEG test, as clinical events became obvious. In these cases, a new study (file) was begun. The success rate was analyzed according to the indications for the EEG study: paroxysmal eye movements, tremor, suspected seizures, myoclonus, staring episodes, suspected stereotypias and tics, absence epilepsy follow-up, cyanotic episodes, and suspected psychogenic nonepileptic events. Results: Video recording was added to 137 of 666 routine studies. Mean patient age was 4.8 years. The nature of the event was determined in 61 (45%) of the EEG studies. Twenty-eight percent were hospitalized patients. The average study duration was 26 min. This diagnostic means was particularly useful for paroxysmal eye movements, staring spells, myoclonic jerks, stereotypias, and psychogenic nonepileptic events. About 46% of 116 patients for whom cognitive data were available were mentally retarded. EEG with added video recording was successfully performed in all 116 cases and provided useful information in 29 (55%) of these 53 patients. Conclusions: Adding video recording to routine EEG was helpful in 45% of cases referred for frequent paroxysmal events. This technique proved useful for hospitalized children as well as for outpatients. Moreover, it was successfully applied in cognitively impaired patients. Infants and children with paroxysmal eye movements, staring spells, myoclonic jerks, stereotypias, and pseudoseizures especially benefited from this diagnostic means. Because of its low cost and the little discomfort imposed on the patient and his or her family, this technique should be considered as a first diagnostic step in children with frequent paroxysmal events. [source] Computerized Tremor Analysis of Valproate-induced Tremor: A Comparative Study of Controlled-release versus Conventional ValproateEPILEPSIA, Issue 2 2005Martina Rinnerthaler Summary:,Purpose: Valproate (VPA) induces postural tremor in 6,45% of patients. The characteristics of VPA-induced tremor have not yet been quantitatively assessed, and it is not known whether tremor prevalence or severity is affected by VPA formulation (controlled-release CR-VPA vs. conventional VPA). The aim of this study was quantitatively to assess tremor in epilepsy patients receiving VPA and to compare the effects of two VPA formulations (CR-VPA vs. VPA) on tremor severity. Methods: In a prospective study, 18 consecutive patients with newly diagnosed focal or generalized epilepsy were assigned to receive alternately either VPA (n = 10) or CR-VPA (n = 8) monotherapy. Computerized tremor analysis was performed at baseline 1 day before initiating VPA treatment and repeated after a seizure-free period of ,8 weeks, during which VPA doses had remained stable. Rest and postural tremor were recorded by accelerometry, and surface electromyograms (EMGs) were recorded from the wrist flexors and extensors. Results: At baseline, the two groups had similar postural tremor amplitudes. At follow-up, the CR-VPA group had remained at the same level, whereas VPA subjects exhibited a significant increase in tremor amplitudes (p < 0.05) despite comparable VPA doses and comparable plasma VPA concentrations at the time of tremor testing. Conclusions: This is the first study to assess quantitatively VPA-induced tremor by standardized tremor analysis. These results suggest that CR-VPA may cause less tremorigenic activity as compared with standard VPA. The mechanisms underlying this difference are unclear but may include greater peak,trough variation with VPA than with CR-VPA. [source] Cognition in essential tremor: should we worry about progressive cognitive decline?EUROPEAN JOURNAL OF NEUROLOGY, Issue 10 2010E. Tolosa No abstract is available for this article. [source] A clinical maneuver to increase tremor differentiation between Parkinson disease and essential tremorEUROPEAN JOURNAL OF NEUROLOGY, Issue 9 2010R. Mazzocchio No abstract is available for this article. [source] Alzheimer's disease, Parkinson's disease and essential tremor: three common degenerative diseases with shared mechanisms?EUROPEAN JOURNAL OF NEUROLOGY, Issue 6 2010E. D. Louis No abstract is available for this article. [source] Paraoxonase 1 (PON1) polymorphisms and risk for essential tremorEUROPEAN JOURNAL OF NEUROLOGY, Issue 6 2010E. García-Martín Background:, The polymorphic enzyme human serum paraoxonase 1 (PON1), encoded by the gene PON1 (chromosome 7q21.3), plays a major role in the metabolism of organophosphorus compounds. We investigated the possible association between the PON1 genotype and allelic variants of the polymorphisms Leu55Met and Glu192Arg, and the risk for essential tremor (ET). Methods:, We studied the frequency of the PON1 genotypes and allelic variants in 201 patients with ET and 220 healthy controls using a PCR-RLFP method. Results:, The frequencies of the PON1 genotypes and allelic variants of the polymorphisms Leu55Met and Gln192Arg did not differ significantly between patients with ET and controls. These polymorphisms were unrelated with the age of onset of ET. Conclusions:,PON1 polymorphisms are not related with the risk for ET. [source] How are we doing with the treatment of essential tremor (ET)?EUROPEAN JOURNAL OF NEUROLOGY, Issue 6 2010Persistence of patients with ET on medication: data from 528 patients in three settings Background:, The pharmacological treatment of essential tremor (ET) is not optimal. There are only two first-line medications and troublesome side effects are common. It is not uncommon for patients to simply stop taking medication. Yet, no published data substantiate or quantify this anecdotal impression. Objectives:, To determine, amongst patients with ET who were prescribed medication for tremor, what proportion are still taking medication and what proportion have stopped? Methods:, Five hundred and twenty-eight patients with ET from three distinct study settings (clinical, brain donors, population) were interviewed. Results:, A clear pattern that emerged across settings was that the proportion of patients with ET who had stopped medication was sizable and consistently similar (nearly one-third): 31.4% (clinical), 24.3% (brain donors), 30.0% (population), 29.8% (overall). A similarly high proportion of cases with severe tremor had stopped their medication: 31.9% (clinical), 36.4% (brain donors). For the four most commonly used medications (propranolol, primidone, diazepam, topiramate), one-half or more of the treated patients had stopped the medication; amongst the less commonly used medications, the proportion who stopped was even higher. Conclusions:, Nearly one of every three patients with ET who had been prescribed medication for tremor had discontinued pharmacotherapy. Even more revealing was that a similar proportion of cases with severe tremor had stopped medication. These data make tangibly evident that there is a sizable population of patients with ET who are untreated and disabled, and underscore the inadequacy of current pharmacotherapeutic options for this common neurological disease. [source] MRI verified STN stimulation site , gait improvement and clinical outcomeEUROPEAN JOURNAL OF NEUROLOGY, Issue 5 2010E. L. Johnsen Background:, Deep brain stimulation (DBS) of the subthalamic nucleus (STN) is effective in alleviating Parkinson's disease (PD) symptoms (tremor, rigidity and bradykinesia) and may improve gait and postural impairment associated with the disease. However, improvement of gait is not always as predictable as the clinical outcome. This may relate to the type of gait impairment or localization of the active DBS contact. Methods:, The active contact was visualized on peri-operative magnetic resonance imaging in 22 patients with idiopathic PD, consecutively treated with bilateral STN DBS. Stimulation site was grouped as either in the dorsal/ventral STN or medial/lateral hereof and anterior/posterior STN or medial/lateral hereof. The localization was compared with relative improvement of clinical outcome (UPDRS-III). In 10 patients, quantitative gait analyses were performed, and the improvement in gait performance was compared with stimulation site in the STN. Results:, Of 44 active contacts, 77% were inside the nucleus, 23% were medial hereof. Stimulation of the dorsal half improved UPDRS-III significantly more than ventral STN DBS (P = 0.02). However, there were no differences between anterior and posterior stimulation in the dorsal STN. Step velocity and length improved significantly more with dorsal stimulation compared with ventral stimulation (P = 0.03 and P = 0.02). Balance during gait was also more improved with dorsal stimulation compared with ventral stimulation. Conclusions:, Deep brain stimulation of the dorsal STN is superior to stimulation of the ventral STN. Possible different effects of stimulation inside the nucleus underline the need for exact knowledge of the active stimulation site position to target the most effective area. [source] Another connection between essential tremor and Parkinson's disease?EUROPEAN JOURNAL OF NEUROLOGY, Issue 1 2010E. D. Louis No abstract is available for this article. [source] DRD3 Ser9Gly variant is not associated with essential tremor in a series of Italian patientsEUROPEAN JOURNAL OF NEUROLOGY, Issue 9 2008C. Vitale Background: Essential tremor (ET) is the most common movement disorder worldwide. Three susceptibility loci on chromosomes 3q13, 2p24.1, and 6p23 have been reported, but no causative genes were found. The Ser9Gly variant of dopamine D3 receptor (DRD3) receptor was found associated to ET in a French and US population. Methods: A case,control study to evaluate the association between the Ser9Gly variant and ET was performed in a cohort of 116 Italian patients with familial ET and in 158 normal controls. Results: No significant difference in allele and genotype frequencies was found between the two groups. Conclusions: These results do not support an association between DRD3 Ser9Gly and susceptibility to ET in Italian patients. [source] | |||||||||||||||||||||||||||||||