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Manual for the Extrapyramidal Symptom Rating Scale (ESRS).

The Extrapyramidal Symptom Rating Scale (ESRS) was developed to assess four types of drug-induced movement disorders (DIMD): Parkinsonism, akathisia, dystonia, and tardive dyskinesia (TD). Comprehensive ESRS definitions and basic instructions are given. Factor analysis provided six ESRS factors: 1) hypokinetic Parkinsonism; 2) orofacial dyskinesia; 3) trunk/limb dyskinesia; 4) akathisia; 5) tremor; and 6) tardive dystonia. Two pivotal studies found high inter-rater reliability correlations in both antipsychotic-induced movement disorders and idiopathic Parkinson disease. For inter-rater reliability and certification of raters, >or=80% of item ratings of the complete scale should be +/-1 point of expert ratings and >or=70% of ratings on individual items of each ESRS subscale should be +/-1 point of expert ratings. During a cross-scale comparison, AIMS and ESRS were found to have a 96% (359/374) agreement between TD-defined cases by DSM-IV TD criteria. Two recent international studies using the ESRS included over 3000 patients worldwide and showed an incidence of TD ranging from 10.2% (2000) to 12% (1998). ESRS specificity was investigated through two different approaches, path analyses and ANCOVA PANSS factors changes, which found that ESRS measurement of drug-induced EPS is valid and discriminative from psychiatric symptoms.

Basal Ganglia Diseases↗

An overview of parkinsonian syndromes: data from the literature and from an Italian data-base.

Recent molecular biology research on neurodegenerative diseases, including parkinsonisms, has identified mutations in the genes that code for the proteins alpha-synuclein and tau, which have been used to classify them into synucleinopathies and tauopathies. The synucleinopathies include, besides the most common and well studied Parkinson's disease (PD), dementia with Lewy bodies, which accounts for approximately 20% of all cases of dementia in the elderly, and multiple system atrophy, whereas the tauopathies include rare and rapidly progressive syndromes, such as progressive supranuclear palsy and corticobasal degeneration. Data we collected at our center in over 2900 parkinsonian patients show that PD accounts for no more than 70% of parkinsonisms. The various syndromes have many features in common that make the differential diagnosis difficult in the early stages of disease. Our data are consistent with the findings reported in the international literature and provide additional information useful for differential diagnosis.

Aged↗

Investigation of axial symptoms after cervical laminoplasty, using questionnaire survey.

BACKGROUND CONTEXT: Cervical laminoplasty is a good strategy for cervical myelopathy, but some postoperative patients complain of obstinate axial symptoms after surgery, that is, nuchal pain, neck stiffness, and shoulder pain. It was reported that these symptoms proved to be more serious than has been believed and should be considered in the evaluation of the outcome of cervical spinal surgery. However, axial symptoms are sometimes recognized before surgery, or also after corpectomy. Addressing this issue becomes complicated. PURPOSE: We investigate the difference in axial symptoms before and after laminoplasty and discuss the characteristics of these symptoms as a surgical complication. STUDY DESIGN/SETTING: We conducted a questionnaire survey and reviewed the medical records of respondents. PATIENT SAMPLE: All of the 180 patients who underwent a spinous process-splitting laminoplasty for cervical myelopathy caused by degenerative disease in our institution from 1993 until 2002 and were followed for 2 years or longer after surgery. OUTCOME MEASURES: Self-report measures and functional measures. The questionnaire elicited information as follows: the location and characteristics of pre- and postoperative symptoms, frequency and duration of postoperative symptoms, and impairment in activities of everyday living, analgesic use, and the duration of use of cervical orthosis after surgery. METHODS: We divided axial symptoms into four characteristics based on previous reports: "pain," "heaviness," "stiffness," and "other." An illustration of the upper back on which respondents could mark each characteristic was utilized to acquire information about the location of axial symptoms. The following information was gathered from medical records and statistically analyzed: whether postoperative axial symptoms were related or not, age, sex, neurological findings, the period of cervical orthosis, surgery time, blood loss, with or without reconstruction surgery of the semispinalis cervicis muscle, and preoperative axial symptoms. RESULTS: For all of the 51 respondents, the average time since surgery was 4.1 years at the time of investigation; 42 patients complained of postoperative axial symptoms; 26 patients stated the duration of symptoms after surgery to be "more than 2 years." The surgical outcome of this group, however, did not differ from that of the 2-year-or-less group. Axial symptoms, which accounted for 13.3% of all answers about postoperative impairment of everyday living, were similar to hand numbness. Of respondents with postoperative axial symptoms, 52.2% stated the frequency of affliction to be "all day long," but 34.8% replied "rarely" to frequency of use of analgesics. Axial symptoms in the nuchal region increased from 45.2% to 48.6% after surgery. "Stiffness" was the most common characteristic before and after surgery, but "pain" significantly increased from 24.6% before surgery to 38.4% after surgery. We speculate that the principal manifestation of axial symptoms might be pain and that the nuchal region might be the predominant region for axial symptoms. There was no significant difference in age, blood loss, operative time, sex, duration of use of cervical orthosis, reconstructive surgery, and preoperative symptoms between two groups--those who complained of axial symptoms after surgery, and those who did not. CONCLUSIONS: In this survey, axial symptoms were not usually so severe as to require analgesic use and did not worsen the Japanese Orthopaedic Association score after surgery; symptoms were, however, considered to continuously affect everyday life as much as hand numbness. Regarding their features, we speculate the main characteristics of axial symptoms might be pain and that the nuchal region might be the predominant region for axial symptoms. Our data are consistent with the hypothesis that laminoplasty is not, as such, an effective treatment for axial neck pain and that axial symptoms may in fact be worsened by the procedure.

Aged↗

Left ventricular compliance: mechanisms and clinical implications.

Left ventricular diastolic compliance is determined by the level of operating pressure and the diastolic pressure-volume relation. This relation is curvillinear and the slope of a tangent (operative chamber stiffness) to the pressure-volume curve increases as the chamber progressively fills. Such preload-dependent changes in compliance occur during any acute alteration in ventricular volume. At a given diastolic pressure, operative chamber stiffness (or its reciprocal, operative chamber compliance) is determined by the relative values for ventricular volume and muscle mass and by the stiffness of a unit of myocardium. Thus, there may be a leftward shift of the diastolic pressure-volume curve (increase in the modulus of chamber stiffness) as a consequence of ventricular hypertrophy or an increase in the stiffness of heart muscle itself (increase in modulus of muscle stiffness). To distinguish between hypertrophy and stiff muscle, it is useful to examine the modulus of chamber stiffness, derived from pressure-volume data, together with the volume/mass ratio of the ventricle. In this fashion, changes in the modulus of chamber stiffness that are inappropriate for a given volume/mass ratio may be attributed to changes in the material properties of the heart muscle. Examples of clinical and experimental pressure-volume studies are presented to illustrate the variety of mechanisms by which acute and chronic changes in ventricular chamber compliance evolve during the course of clinical heart disease. The pathophysiology of pulmonary congestion is best understood by considering the factors responsible for producing changes in chamber stiffness of the ventricle, whereas an examination of muscle stiffness is likely to provide more insight into the extent of irreversible functional and structural defects of the myocardium.

Angina Pectoris↗

Sport stretching: effect on passive muscle stiffness of short hamstrings.

OBJECTIVE: To evaluate the effects of one 10-minute stretch on muscle stiffness in subjects with short hamstrings. DESIGN: Randomized control trial. SETTING: Laboratory for human movement sciences in the department of rehabilitation of a university hospital. SUBJECTS: Sixteen students from the Department of Human Movement Sciences participated with informed consent in the experiment. Subjects were limited to men and women without a history of neurological and orthopedic disorders. To select subjects with short hamstrings, the finger-ground distance had to be greater than 0cm (unable to touch the floor when bending forward) and the manual leg lifting was not to exceed 80 degrees. One group of 10 subjects performed static stretching exercises during 10 minutes interspersed with relaxing, whereas the untreated group of 6 subjects was used as a control. MAIN OUTCOME MEASURES: The instrumental straight-leg-raising set-up enables the measurement of the force needed to lift the leg, range of motion (ROM), pelvic-femoral angle, and the electromyogram of the hamstrings. These variables provide information about the stiffness, elongation, and state of activity of the hamstring muscles. RESULTS. One 10-minute sport stretch resulted in a significant increase in passive muscle moment, ROM, and elongation of the hamstrings. There was no significant change in the course of the passive muscle stiffness curve with respect to the prestretch stiffness curve. CONCLUSIONS: One session of static stretching does not influence the course of the passive muscle stiffness curve. The increased ROM, i.e., the extensibility of the hamstrings, results from an increase in the stretch tolerance.

Adult↗

Waking cervical pain and stiffness, headache, scapular or arm pain: gender and age effects.

A random population-based study using a structured telephone questionnaire was undertaken to determine the frequency, duration and prevalence of waking cervical spine pain and stiffness, headache, and aching between the scapulae or in the arm and their relationship to age and gender. Seventy-six per cent of households contacted completed the questionnaire, yielding a total of 812 questionnaires. Subjects most commonly reported waking with scapular or arm pain (27%) followed by headache (19%), cervical pain (18%) and cervical stiffness (17%). The majority of subjects reported that symptoms lasted for more than an hour on one or two occasions during the week. Subjects who reported the presence of one type of waking symptom were significantly more likely to report other waking symptoms. Females were significantly more likely to wake with a headache than males (OR 2.5, 95% CI 1.6 to 4.0), while all subjects aged over 60 years were significantly less likely to wake with a headache than subjects in other age groups (OR 0.6, 95% CI 0.4 to 1.0). Females exhibited a decline in waking cervical pain, stiffness and headache with increasing age. In contrast, males exhibited a peak prevalence of waking cervical pain, stiffness and headache in the 40 to 59 year age group. The prevalence of waking with aching between the scapulae or in the arm behaved differently from the other symptom groups in both genders.

Adult↗

A clinical study of patients with genetically confirmed Huntington's disease from India.

BACKGROUND: Clinical data across the globe especially in genetic diseases like Huntington's disease (HD) is most helpful when collected using standardized formats. This helps in proper comparison of clinical and genetic data. METHODS: Herein, we report clinical data on 26 genetically confirmed HD patients from 19 Indian families predominantly from South India. Clinical data and evaluation was performed using standardized formats used by the Huntington Disease Study Group. RESULTS: Adult onset HD was commonest while Juvenile HD (onset <20 years) was observed in approximately 15% of patients. Chorea was the commonest presenting symptom (n=23, 88.5%) while remaining presented with psychiatric symptoms (n=3, 11.5%). Impairment of saccades was observed in approximately 75% of patients. Mean (SD) CAG repeats in the abnormal allele was 48.4 (8.7). Total motor score but not the total behavioral score worsens with duration of symptoms. The functional checklist score correlates with total motor score rather than with duration of symptoms. CONCLUSIONS: We detail clinical characteristics in genetically confirmed HD patients from a predominantly South Indian cohort. We observed a slightly higher occurrence of Juvenile HD. Functional disabilities in our patients correlate with worsening of motor rather than behavioral symptoms.

Adolescent↗

The effects of clonazepam and vigabatrin in hyperekplexia.

Hyperekplexia is an autosomal dominant disorder caused by a point mutation in the alpha1 subunit of the glycine receptor, characterized by excessive startle responses followed by temporary generalized stiffness. Clonazepam, effective in open case studies, potentiates, through unknown mechanisms, the neurotransmitter gamma-aminobutyric acid (GABA). Vigabatrin increases GABA by inhibition of the GABA catabolic enzyme GABA-transaminase. Effects of clonazepam (1 mg for 1 day) and vigabatrin (1000 mg per day for 5 days) were investigated in a double-blind placebo-controlled cross-over study in 4 patients with hyperekplexia. The pharmacodynamic parameters were startle reflexes, studied 3 times during the day. At each time, 2 trains of 10 auditive stimuli (113 dB) were given at intervals of 10 and 60 s. Startle movements were quantified with summed areas of EMG-bursts of the orbicularis oculi, sternocleidomastoid, biceps and thenar muscles. The degrees of stiffness and drowsiness were quantified with visual analogue scores (VAS) 10 times during the day, by both the patient and the observer. Clonazepam, but not vigabatrin, reduced startle activity significantly in both paradigms. The degree of stiffness and drowsiness was not significantly influenced by either drug.

Acoustic Stimulation↗

Clinical neurophysiologic studies in stiff-man syndrome: use of simultaneous video-electroencephalographic-surface electromyographic recording.

Three patients with a clinical diagnosis of stiff-man syndrome were studied with simultaneous video-electroencephalographic-surface electromyographic recordings in addition to routine electromyography in order to obtain objective data to confirm their diagnosis, to improve our understanding of the diagnosis of stiff-man syndrome, and to define reproducible clinical and neurophysiologic criteria for the stiff-man syndrome. These patients had the following features of this syndrome: (1) continuous muscle activity that varied with awake and sleep states, posture, passive and active movements, and medications; (2) superimposed intermittent generalized contractions while awake, which continued into drowsiness and interfered with onset of sleep; and (3) abnormal cocontractions of antagonistic muscles. The characteristic findings in the stiff-man syndrome can be recorded by using video-electroencephalographic-surface electromyographic techniques, a useful application of equipment already available in most electroencephalography laboratories. Neurophysiologic techniques can help in elucidating the clinical findings in the stiff-man syndrome. Further systematic study in patients before and during treatment is needed to identify common diagnostic criteria for this syndrome.

Adult↗

"On-off" effects in patients with Parkinson's disease on chronic levodopa therapy.

Fluctuations in performance in patients with Parkinson's disease on chronic levodopa therapy (the "on-off" effect) are due to several factors. The increasing severity during treatment of early morning akinesia, "freezing" episodes, and end-of-dose deterioration are probably due to progression of the underlying disease. Peak-dose dyskinesia and peak-dose akinesia are due to levodopa over dosage. "Yo-yo-ing", which is the severest form of such fluctuation in mobility and dyskinesias, may represent the sum of these disorders.

Dose-Response Relationship, Drug↗