Subdivision of essential tremor patients according to physiologic characteristics

OBJECTIVE: To test the effect of different forearm postures on tremor characteristics. We hypothesized that changes in tremor characteristics in relation to posture can subclassify essential tremor (ET) patients. METHODS: Fourteen ET patients were tested while seated and holding a full cup in three well-defined arm postures: 15 cm above the armrest, elbow at 90 degrees flexion and when the cup was near the mouth. Hand movements were recorded using a triaxial accelerometer. Concomitantly, we recorded surface electromyogram (EMG) signals from the wrist extensor muscles. Hand acceleration and the primary tremor frequency from the power spectrum were calculated for each posture in two independent trials. The coherence at the primary tremor frequency between the EMG and the accelerometry signals was calculated. RESULTS: ET patients could be classified into two groups: ET-1 (nine patients) had position-dependent peak frequencies while ET-2 (five patients) had position-independent peak frequencies. The latter group had significantly higher coherencies between EMG and accelerometry of tremor and insignificantly higher tremor amplitudes compared with ET-1. In both groups tremor amplitude increased when the hand was near the mouth. CONCLUSION: The results suggest that ET patients can be classified based on the position dependence of frequency and other physiologic properties.     

Presence of platelet antibodies in idiopathic thrombocytopenic purpura may discriminate acute from chronic disease

This study estimated the prevalence of serum antibodies against thrombocyte glycoproteins, at disease onset (54 patients) and later on during the course of the disease (71 patients), in sera from children with idiopathic thrombocytopenic purpura (ITP). Only a minority had serum antibodies at disease onset, with a significantly higher frequency in those who developed the acute form of the disease than in those who developed the chronic form. Serum antibodies may persist after spontaneous cure of acute disease. There was no switch from immunoglobulin M (IgM) to IgG antibodies over time. CONCLUSION: The pathogenesis of the acute and chronic forms of ITP may be different.     

Evaluating the risk of tuberous sclerosis in cases with prenatal diagnosis of cardiac rhabdomyoma

OBJECTIVE: To evaluate the prenatal parameters that increase the risk of tuberous sclerosis in prenatal management of fetal cardiac tumors suspected as rhabdomyoma. METHODS: The study was a retrospective survey of 18 documented cases in which cardiac rhabdomyoma was suspected during pregnancy. The following parameters were evaluated as possible risk factors associated with tuberous sclerosis: tumor size, isolated or multiple, and family history of tuberous sclerosis. RESULTS: Eighteen documented cases in which cardiac rhabdomyoma was found during pregnancy were evaluated. Of these cases, seven (39%) had proven tuberous sclerosis and 11 were found to be non-associated tuberous sclerosis tumors. When combining the present data with previous series, cases with diagnosis of tuberous sclerosis had equal mean tumor size to those with normal outcome. Family history of tuberous sclerosis in the presence of cardiac rhabdomyoma almost invariably ended with tuberous sclerosis (86%). All other cases with diagnosis of tuberous sclerosis and no family history had all multiple cardiac tumors. CONCLUSION: The present data suggest that 39% of in utero suspected cardiac rhabdomyoma would have tuberous sclerosis. Family history and multifocality remain the strongest predictors of tuberous sclerosis, whereas size of the cardiac tumor can not reliably be used to predict tuberous sclerosis in prenatal counseling.     

Rhabdomyoma in the Fetus: Illustration of Tumor Growth During the Second Half of Gestation

Rhabdomyomata are the most common cardiac tumors in childhood and are associated with tuberous sclerosis. These tumors tend to regress in the first years of life. Little is known about their intrauterine growth pattern. We describe three fetuses with cardiac rhabdomyomata and illustrate the tumor growth by serial echocardiographic views during the second half of gestation. Tumor growth is proportional to cardiac growth and tends to be somewhat slower toward the end of pregnancy.     

Laparoscopic Repositioning of Heliogast® Gastric Band after Anterior Slippage

Background: Gastric slippage is a well-described complication of gastric banding. The Heliogast? band is equipped with a locking mechanism that enables its straightforward reopening and repositioning. Our experience with Heliogast? band salvage after anterior slippage is reported. Methods: The study sample comprised 418 consecutive patients who underwent 2-step laparoscopic gastric banding with the Lap-Band? first (n=235) followed with the Heliogast? band (n=183). Gastric slippage was diagnosed by symptoms of dysphagia and vomiting and was confirmed with Gastrografin? swallow. Patients who did not respond to conservative treatment were laparoscopically reoperated. In the Heliogast? group, the band was dissected free, unlocked, and repositioned. In the Lap-Band? group, when reopening proved impossible, the band was removed with or without replacement. Results: 10 patients (2.4%) underwent reoperation for anterior slippage, 5 with a Lap-Band? (2.1%) and 5 with a Heliogast? band (2.7%). Band repositioning was feasible in all 5 patients in the Heliogast? group, but in only one of the patients in the Lap-Band? group; in the others, the band was removed. Band repositioning was confirmed radiologically. No postoperative complications were recorded, and all patients were discharged on the first day after surgery. At a median 10 months' follow-up after Heliogast? repositioning, all patients had satisfactory weight loss. Conclusion: Laparoscopic Heliogast? band salvage after anterior slippage is comparatively simple and safe, with excellent postoperative results and no interference with continued weight loss. This constitutes an important means of management for the bariatric surgeon.     

Freezing of gait in multiple system atrophy (MSA)

BACKGROUND: Freezing of gait (FOG) is a mysterious symptom, observed in different parkinsonian syndromes, but considered to be rare in multiple system atrophy (MSA). OBJECTIVE: To assess the frequency of FOG in patients with MSA. METHOD: We studied the presence of FOG in 28 patients with clinical diagnosis of MSA. 21 patients had probable MSA and 7 had possible MSA. The clinical diagnosis was based on neurological examination performed by at least two experienced movement disorders specialists as well as on the results of ancillary examinations and the course of the disease. In 22 patients MSA was predominated by parkinsonism (MSA-P) and in 6 by cerebellar signs (MSA-C). The patients' mean age was 66.8+/-10.3 years, mean disease duration 6.4+/-4.0 years and mean worst Hoehn and Yahr (H&Y) stage was 3.6+/-0.6. Presence and severity of FOG was assessed during the last office visit, by the recently validated FOG questionnaire (FOG-Q), with a maximal score of 24, while patients that received at least one point in the last four questions were classified as having FOG. Severity of FOG was determined by the sum of these last four out of six questions (maximal score is 16). FOG-Q total score reflected general function and walking ability disturbances, caused by FOG.The comparison between groups of patients was performed by way of the Mann-Whitney two-sample test and chi-square or Fisher's exact tests. Correlations between various parameters were calculated using Spearman's correlation coefficient. RESULTS: Twenty-one patients were able to walk and 7 were bedridden at the time of the study. FOG appeared in a total of 75% of all MSA patients (in 82% of patients with MSA-P and in 50% patients with MSA-C). In the MSA-P group disease duration was about the same among 'freezers' and 'non-freezers', while among the MSA-C patients it was significantly shorter in the non-freezers. Mean score of the freezing subdivision of the FOG-Q was 8.2+/-5.1 for MSA-P group and 4.5+/-5.1 for MSA-C one. Mean FOG-Q total score was 9.1+/-4.0 and 6.2+/-4.6 (p>0.05) for MSA-P and MSA-C patients, respectively. CONCLUSION: Freezing of gait is a common symptom in MSA, both in MSA-P and MSA-C.     

Diagnostic assessment of haemorrhagic rash and fever

AIMS—To establish criteria for early distinction between meningococcal disease and other conditions with similar clinical features, and to identify other causes for haemorrhagic rashes accompanied by fever.
METHODS—In a prospective study, 264 infants and children hospitalised with fever and skin haemorrhages were studied.
RESULTS—We identified an aetiological agent in 28%: 15% had meningococcal disease, 2% another invasive bacterial infection, 7% enterovirus infection, and 4% adenovirus infection. Five clinical variables distinguished between meningococcal disease and other conditions on admission: (1) skin haemorrhages of characteristic appearance; (2) universal distribution of skin haemorrhages; (3) maximum diameter of one or more skin haemorrhages greater than 2 mm; (4) poor general condition (using a standardised observation scheme); and (5) nuchal rigidity. If any two or more of these clinical variables were present, the probability of identifying a patient with meningococcal disease was 97% and the false positive rate was only 12%. This diagnostic algorithm did not identify children in whom septicaemia was caused by other bacterial species.

     

Freezing of gait in patients with advanced Parkinson's disease

Summary. Background. Freezing of Gait (FOG) is one of the most disturbing and least understood symptom in advanced stage of Parkinson's disease (PD). The contribution of the underlying pathological process and the antiparkinsonian treatment to the development of FOG are controversial. Objective. To study the relationships between clinical features of PD and therapeutic modalities in patients with advanced PD and FOG. Methods. Consecutive patients with 5 years or more of PD symptoms (n = 172) (99 men) with mean age at symptoms onset of 58.3 ± 13.2 years and mean symptoms duration of 11.8 ± 5.6 years were studied. Clinical data were collected during the last office visit through physical examination, detailed history, review of patients' charts, and other documents. A patient was considered as "freezer" if he/she reported recent experience that the legs got stuck to the ground while trying to walk. The presence of dyskinesia, early morning dystonia or significant postural reflex abnormalities were assessed through history and neurological examination. Duration of treatment with antiparkinsonian drugs was calculated from history charts. Chi square and t test were used to compare the patients with and without FOG. Logistic regression was used for the comparison of association between the presence of FOG (dependent variable) disease duration and disease stage (explanatory variables) and duration of treatment with anti-parkinsonian drugs. Results. The study population consisted of 45 patients at Hoehn and Yahr (H&Y) stage 2.5 (26%), 104 patients at stage 3 (60.5%), and 23 patients at H&Y stages 4–5 (13.5%). Ninety one patients (53%) reported FOG at the time of the study. Severity of the disease expressed by H&Y stage at "off" was a significant contributing factor for FOG with a significant trend (z = 4.38, p < 0.0001), as was longer duration of levodopa treatment, and confirmed by FOG using the multivariate logistic regression (p = 0.01 and p = 0.004, respectively). Using a univariate model, longer duration of treatment with dopamine agonists contribute to the appearance of FOG (p = 0.07) while longer duration of amantadine treatment decreased the appearance of FOG (p = 0.09). There was a significant association between FOG and the presence of dyskinesia (p < 0.002), early morning foot dystonia (p < 0.003) and significant postural instability (p < 0.0005). Conclusion. FOG is a common symptom in advanced PD. It is mainly related to disease progression and levodopa treatment. Received April 19, 2000; accepted June 6, 2000