[From stiff man syndrome to stiff person spectrum disorders]. / Vom Stiff-man-Syndrom zu den Stiff-person-Spektrum-Erkrankungen.

The identification of new variants of the stiff man syndrome (SMS) and of new, probably pathogenic neuronal autoantibodies has led to the concept of stiff man (or person) spectrum disorders (SPSD). This is an expanding group of rare chronic autoimmune inflammatory diseases of the central nervous system (CNS) that have in common the main symptoms of fluctuating rigidity and spasms with pronounced stimulus sensitivity. These core symptoms are mandatory and can be accompanied by a wide variety of other neurological signs. The SPSDs are associated with autoantibodies directed against neuronal proteins that attenuate excitability. Neither clinical phenotypes nor the course of SPSD correlate closely with the antibody status. The treatment of these diseases aims at maintaining mobility and is pragmatically oriented to the degree of impediment and comprises antispastic, anticonvulsant and immunomodulating or immunosuppressive medication strategies.

Effects of use of anabolic steroids on the masticatory system: a pilot study.

The use of androgenic anabolic steroids (AAS) has increased significantly among athletes in Brazil and other countries. These drugs alter the physiological behavior of bone and muscles, also affecting these structures in masticatory system. This paper aims to evaluate bone and dental changes in users of AAS, as well as the incidence of temporomandibular dysfunction (TMD), compared to athletes not using AAS. Eight athletes were equally divided in two groups, AAS users and non-users. The groups were evaluated using Helkimo index, McNamara cephalometric tracing and cast analysis. The AAS users presented more intense TMD signs and symptoms (Di total value, P = 0.096, Mann-Whitney test), increased cephalometric measures (Co-A, P = 0.020, Mann-Whitney test) and Angle Class II malocclusion, compared to the non-users. These results suggested that the use of AAS alters masticatory structures and increases the incidence of TMD.

Mokken scale analysis of the UPDRS: dimensionality of the Motor Section revisited.

The dimensionality and reliability of the Motor Section of the Unified Parkinson Disease Rating Scale (UPDRS III) was studied with non-parametric Mokken scale analysis. UPDRS measures were obtained on 147 patients with PD (96 men, 51 women, mean age 61, range 35-80 yrs). Mokken scale analysis revealed a four-dimensional structure of the UPDRS III. Left-sided bradykinesia and rigidity appeared to co-occur with axial signs, gait disturbance, and speech/hypomimia, whereas right-sided bradykinesia and rigidity formed a second scale. Two further small scales were found consisting of right- and left-sided tremor. Results from the scale analysis reveal that all four subscales are strong. The reliability of the two tremor scales is low because they only contain three and four items, respectively.

Biasing effect of the electromyogram on BIS: a controlled study during high-dose fentanyl induction.

OBJECTIVE: A biasing effect of the electromyogram (EMG) on the Bispectral Index (BIS) may explain discrepancies in previous studies assessing BIS in the presence of neuromuscular activity. Our aims were: to evaluate variations of BIS in the presence of high EMG activity associated with muscular rigidity after administration of high-dose fentanyl; to compare muscular rigidity, as measured by the EMG variable of the BIS monitor, in patients who were administered two different dosages of fentanyl at induction of cardiac anaesthesia. METHODS: 26 patients undergoing CABG surgery, after premedication with morphine 0.15 mg/kg, were randomized to receive either fentanyl 50 mcg/kg (group F) or fentanyl 10 mcg/kg plus etomidate 0.2 mg/kg (group EF). The induction dose was administered over 2 minutes. Patients were manually ventilated with O2 via face mask. Five minutes after induction was complete, patients were clinically assessed using the Responsiveness portion of the Observer's Assessment of Alertness/Sedation scale (OAAS). Haemodynamic data were recorded and arterial blood samples obtained at the time of OAAS observation. Patients were administered a neuromuscular blocking agent only after the OAAS assessment. BIS (3.4) was recorded from an A-2000 EEG monitor (Aspect Medical Systems) using disposable sensors (BIS Sensor, Aspect Medical Systems) applied per manufacturer's instructions. Data were recorded on a PC for off-line analysis. RESULTS: At the time of OAAS observation, mean (95% CI) BIS in group F was 85 (77-92) compared to 67 (56-79) in group EF (p = 0.01). Similarly, mean (95% CI) EMG was 50 dB (45-56) in F and 41 dB (35-47) in EF (p = 0.01). Correlation between BIS and EMG was very high (r2 = 0.88). OAAS scores were significantly higher in group F (p = 0.03). Non significant correlation was observed between BIS and OAAS scores (r2 = 0.32, p = 0.1). Backward stepwise multiple regression analysis including EMG, pH, CO2, O2 and OASS scores showed EMG as strong predictor of BIS (p < 0.0001, r2 = 0.7). Regression of EMG against BIS yielded the equation: BIS = 3.7 + (1.6 x EMG). CONCLUSION: During fentanyl-induced muscular rigidity BIS recordings reflect EMG variations. When assessing BIS in the absence of neuromuscular blockade, it is necessary to evaluate the effect of EMG on BIS before making conclusions about depth of sedation. Fentanyl-induced rigidity appears to be a dose-related phenomenon which the EMG variable of BIS 3.4 is able to quantify.

Long-term effect of electrostimulation of the subthalamic nucleus in bradykinetic-rigid Parkinson's disease.

The motor effects of unilateral stimulation of the subthalamic nucleus on hypokinesia were studied in two patients 58 and 52 years old, both modified Hoehn and Yahr 2.5, at 16 and 15 months after the implantation of a quadripolar electrode (Medtronic). Motor UPDRS, time in the pegboard test, walking time, tapping, and serial reaction times were recorded. Chronic unilateral stimulation was associated with reversible improvement of measures of reaction time and hypokinesia > 1 year after the stereotactic electrode implantation. The beneficial effect was mainly contralateral to the stimulation. However, improvement of axial functions was also observed (phonation, walking).

Catatonia and other motor syndromes in a chronically hospitalized psychiatric population.

BACKGROUND: To determine the motor characteristics of chronic catatonia, catatonia and other motor disorders were systematically rated in a long-term hospitalized sample. METHOD: Chronically hospitalized psychiatric inpatients (N = 42) with a clinical diagnosis of catatonic schizophrenia (295.2X) were rated for catatonia with a novel 23-item catatonia rating scale, and for parkinsonism, dyskinesia and akathisia using standard rating scales with scale-based criteria for case definition. RESULTS: Catatonia was the sole motor syndrome in nine cases (21%), co-existed with parkinsonism in five (12%), tardive dyskinesia in four (10%), and both parkinsonism and tardive dyskinesia in 10 (24%). There was no correlation between total scores across the four rating scales. 'Rigidity' was the sole catatonic sign which overlapped with other scales. The symptom profile of catatonia in this chronic sample was similar to previous reports based on acutely ill patients. CONCLUSION: Catatonia is distinguishable from other motor disorders in chronic psychiatric patients using the 23-item catatonia rating scale. The features of chronic catatonia are described, and the distribution of catatonic signs is similar for chronic and acute catatonia.

Hypokinesia in Parkinson's disease: influence of age, disease severity, and disease duration.

The aim of this cross-sectional study was to compare the role of aging in measures reflecting diurnal activity and immobility in 60 parkinsonian patients with predominant features of hypokinesia and rigidity and 100 healthy subjects aged 50 to 98 years. In the patients, we also studied the relation between disease duration and subjective and objective measures of disease severity. Motor activity was recorded during 5 successive days at home with a wrist-worn activity monitor. For each subject, two mean measures reflecting the diurnal activity level and the relative proportion of activity and immobility were calculated. Diurnal measures of activity revealed in both groups a prominent absolute reduction of activity and an increase of the time spent without movement ("immobility") with advancing age. Parkinsonian patients showed significantly lower values for both motor-activity measures than did the healthy subjects. The rate of the age-related decline of both diurnal activity measures in both groups, however, is comparable. Disease duration showed no relation with subjective and objective measures reflecting disease severity. This study shows that if care is taken to control for disease severity, the rate of the age-related decline of measures reflecting diurnal activity and immobility is similar in both groups. The lack of relation between disease duration and subjective and objective measures of disease severity suggests that the rate of progression of Parkinson's disease can be reliably studied only by means of longitudinal studies.

[Rigid spine syndrome and its nosological borders. 2 cases]. / Le syndrome de la colonne vertébrale rigide et ses frontières nosologiques. Deux observations.

The rigid spine syndrome is characterized clinically by rigidity due to deficient extensor muscles of the spine (chiefly cervical) and radiologically, by a normal spinal image. Electromyography demonstrates abnormalities suggestive of a myogenic process, and histology shows alterations of muscular fibres. From the case-records of two male patients aged 14 and 15 years respectively, a relationship could be established between various diseases having in common a rigid spine syndrome. These include congenital muscular dystrophias , sequelae of arthrogryposis, Emery - Dreifuss myopathy and myosclerosis .