The utility of EMG interference pattern analysis in botulinum toxin treatment of torticollis: a randomised, controlled and blinded study.

OBJECTIVE: The significance of electromyography (EMG) guidance in botulinum toxin (BT) treatment has been much debated. The aim of this study was to evaluate if EMG guidance in the treatment of torticollis in BT-naive patients had a better outcome than treatment after clinical evaluation alone. METHODS: Twenty-six patients with torticollis were included and treated for 1 year in this prospective, blinded study. Quantitative EMG was performed simultaneously in the four most frequently affected muscles: the sternocleidomastoid muscles and the posterior neck muscles on both sides. EMGs were analysed for turns per second. Clinical ratings were performed by an experienced neurologist (A). Injections were given by another neurologist (B), who was blinded to the ratings. In group 1, the results of the EMG were available to the treating neurologist B, whereas in group 2, neurologist B was blinded. In group 1, treatment with BT was given when turns per second were higher than 100. RESULTS: In patients treated guided by EMG, clinical outcome, evaluated by objective ratings, was better than in patients treated based on clinical judgement alone (p = 0.05). In group 2, 105 muscles were treated with BT. Of these, 37 did not show dystonic EMG activity. CONCLUSIONS: Treatment with BT guided by EMG results in better clinical outcome than treatment without EMG and reduces the amount of BT used. SIGNIFICANCE: EMG guidance by interference pattern analysis may optimise BT treatment in torticollis by a more precise injection and may reduce side effects and the risk of development of antibodies to BT.

Dopamine transporter imaging and the effects of deep brain stimulation in patients with Parkinson's disease.

PURPOSE: Single-photon emission computed tomography (SPECT) with [123I]FP-CIT is a marker for loss of presynaptic dopamine transporters in the striatum in Parkinson's disease (PD). We used [123I]FP-CIT SPECT in order to evaluate binding to the dopamine transporter before and after neurosurgical treatment with bilateral stimulation in the subthalamic nucleus (STN). METHODS: Thirty-five patients with levodopa-responsive PD were examined with [123I]FP-CIT SPECT pre-operatively (baseline scan: mean 3 months before surgery), and 3 and 12 months after surgery. RESULTS: Pre-operatively, all patients already had substantial signs of severe nigrostriatal neuronal loss as determined from the [123I]FP-CIT SPECT scans. One year after surgery the specific [123I]FP-CIT binding to the striatum was significantly reduced by 10.3% compared with the pre-operative baseline scan. The mean time span from the baseline scan before surgery to the follow-up scan 1 year after surgery was 16.2 months. Hence, the rate of reduction equals a mean annual reduction of 7.7%. A comparable control group of patients with PD who did not undergo surgery was also examined longitudinally. In this group the specific binding of [123I]FP-CIT was reduced by 6.7% per year. CONCLUSION: The specific binding of [123I]FP-CIT was reduced equally in the STN-stimulated patients and a group of non-operated PD patients with advanced disease. Our study does not support the notion that electrode implantation and STN stimulation exert a neuroprotective effect by themselves.

Imaging of dopamine transporters and D2 receptors in patients with Parkinson's disease and multiple system atrophy.

PURPOSE: The aim of this study was to ascertain whether combined presynaptic and postsynaptic dopaminergic single-photon emission computed tomography (SPECT) scanning is useful for differentiation between patients with idiopathic Parkinson's disease (IPD), patients with multiple system atrophy of the striatonigral type (MSA) and healthy subjects. METHODS: SPECT measurements of the dopamine transporter (DAT) were done with 123I-beta-CIT, while for determination of the dopamine D2-like receptors (D2), 123I-epidepride was used. Clinical evaluation and SPECT scans were carried out in 14 patients with IPD, eight patients with MSA and 11 healthy age-matched control subjects. RESULTS: Putaminal DAT binding was reduced to 32% of control values in IPD and to 19% of control values in MSA . Significantly higher striatal asymmetry in DAT binding was found in MSA than in controls, but IPD patients had significantly higher asymmetry than MSA patients. Striatal D2 binding did not differ significantly between patients and healthy controls but the ratio between caudate DAT and D2 binding was significantly higher in patients with IPD than in those with MSA, even when disease severity was taken into account. CONCLUSION: Patients with reduced striatal 123I-beta-CIT binding and a side-to-side difference greater than 15% are likely to suffer from IPD. Patients with reduced striatal 123I-beta-CIT binding and a side-to-side difference of between 5% and 15% are more likely to have MSA. 123I-epidepride SPECT measurements may add further diagnostic information, since the ratio between DAT and D2 receptor binding is significantly higher in IPD than in MSA.

Sympathetic dysfunction of central origin in patients with ALS.

Amyotrophic lateral sclerosis (ALS) is a severe, progressive disease affecting both the central and peripheral parts of the motor nervous system. Some studies have shown unequivocal indications of a more disseminated disease also affecting the autonomic nervous system. We therefore evaluated the centrally and peripherally mediated autonomic vascular reflexes by (i) the local 133-Xenon washout technique, and (ii) the head-up tilt table test. The results correlated to clinical scores. We examined nine ALS patients and 15 age-matched controls. The 133-Xenon washout test showed a significant reduction in the centrally mediated sympathetic vasoconstrictor response, but a preserved locally mediated response in the patients. In the head-up tilt table test, the patients had a significantly higher mean arterial blood pressure (MAP) compared with controls, probably due to a general increase in vascular resistance. There were no correlations between the ALS Severity Scores and blood flow changes, diastolic blood pressure or MAP. Our study supports previous results, but indicates abnormalities consistent with a solely centrally located sympathetic dysfunction in ALS, independent of the stage of the disease.

Unchanged total number of neurons in motor cortex and neocortex in amyotrophic lateral sclerosis: a stereological study.

Modern stereological methods provide precise and reliable estimates of the number of neurons in specific regions of the brain. The total number of neurons in the neocortex and motor cortex from eight patients suffering from amyotrophic lateral sclerosis (ALS) and nine controls was estimated. No attempt was made to estimate subpopulations of neurons such as the number of giant pyramidal cells of Betz. No difference was found in the average number of neurons in neocortex in ALS and controls, 21.7 and 22.3 x 10(9), respectively, and 1.33 and 1.29 x 10(9) in motor cortex, respectively. In the light of our stereological measurements, results obtained from in-vivo proton magnetic resonance spectroscopy (1H-MRS), suggesting neuronal loss in ALS, may instead be due to neuronal metabolic dysfunction and/or alteration in the size or the volume fraction of the neurons.

[Whiplash injury syndrome. A prospective study of 39 patients with whiplash injury]. / Piskesmaeldsyndromet. En prospektiv undersøgelse af 39 patienter med piskesmaeldlaesioner.

The acute symptoms after whiplash trauma can be explained by the neck sprain, but the pathogenesis of the "late whiplash syndrome" and the reasons why only some people have persistent symptoms more than six months are still unknown. Thirty-four consecutive cases of piskesmaeld injury were examined clinically three times; respectively within 14 days, after one month and finally seven months post-injury. In addition, MRI of the brain and the cervical spine, neuropsychological tests and motor evoked potentials (MEP) were done one month post-injury and repeated after six months, if abnormalities were found. We found the total recovery rate (asymptomatic patients) was 29% after seven months. All MEP examinations were normal. The correlation between MRI and the clinical findings was poor. Cognitive dysfunction as a symptom of brain injury was not found. Stress at the same time as the accident predicted more symptoms at follow-up. We conclude that long-lasting distress and poor outcome were more related to the occurrence of stressful life events than to clinical and paraclinical findings.

Phenotypic heterogeneity in motor neuron disease patients with CuZn-superoxide dismutase mutations in Scandinavia.

Four-hundred and fifty-one blood samples from Scandinavian patients with motor neuron disease were analysed for mutations in the CuZn-superoxide dismutase gene. Forty-one (9.6%) of the 427 patients with the amyotrophic lateral sclerosis (ALS) form of the disease were found to have a disease-associated mutation, and 14 of these patients were apparently sporadic cases. A mutation was found in 12 of the 51 families with recognized familial ALS. The five different mutations found (Ala4Val, Val14Gly, Asp76Tyr, Asp90Ala, Gly127insTGGG) have different genetic characteristics and are associated with very variable phenotypes spanning from rapidly progressing disease with only lower motor neuron signs to very slowly progressing disease with both the upper and lower motor neuron systems affected. The patients showed different sites of onset, though the progressive bulbar palsy form of the disease appears to be rare among patients with a CuZn-superoxide dismutase mutation. The progression of motor signs and symptoms followed the same basic pattern in patients with different mutations. Extra-motor system symptoms were frequent among patients with a CuZn-superoxide dismutase mutation. The results suggest that patients with mutations in the CuZn-superoxide dismutase gene constitute one disease entity. The Val14Gly and Asp76Tyr mutations have not been reported before, and the latter is the first mutation to be found in exon 3 of the CuZn-superoxide dismutase gene.

Pancreatic enzyme secretion and pancreatic pseudocysts in patients with chronic pancreatitis.

CONCLUSIONS: Pseudocyst contents are not indicative of pancreatic function. We presume that the nonresolving pseudocysts did not resolve because they were noncommunicating. The question of the kinetics of secretion into pancreatic pseudocysts is still unresolved. BACKGROUND: Pseudocysts are one of the complications of chronic pancreatitis. We wanted to investigate whether it is of any value to measure enzyme activities (amylase and lipase) in pseudocyst contents, in order to answer the following questions: Is the enzymatic activity: 1. Reproducible in consecutive punctures of the same cyst; 2. Related to cyst age; 3. Indicative for the risk of relapse; and 4. Representative of exocrine pancreatic function. METHODS: In a retrospective study we examined 28 patients with chronic pancreatitis complicated with pancreatic pseudocysts. RESULTS: Reproducibility of enzyme activity in cyst juice in consecutive punctures of the same cyst was poor. We did not observe any correlation between enzyme activity and cyst age defined as the time between two cyst punctures. Enzyme activity in cyst juice was without indication for the risk of relapse. No correlation between enzyme activity and exocrine pancreatic function was found. The pseudocyst contents are not pancreatic juice in the common sense of the word

Quantification of brain metabolites in amyotrophic lateral sclerosis by localized proton magnetic resonance spectroscopy.

We performed proton magnetic resonance spectroscopy (1H-MRS) in patients with motor neuron disease (MND) to determine the absolute in vivo concentrations in the brain of the metabolites N-acetyl aspartate (NAA), choline (Cho), and creatine (Cr/PCr). We examined the spectra acquired from a 20 x 20 x 20-mm3 voxel placed in the motor cortex and in the cerebellum from seven patients with clinically probable or definite amyotrophic lateral sclerosis (ALS) according to the El Escorial criteria, from three patients with suspected ALS (progressive muscular atrophy), and from eight normal control subjects. We estimated the concentrations of the metabolites using the water signal as an internal standard. The concentrations of Cho and Cr/PCr in both brain regions, as well as the concentration of NAA in the cerebellum, were unaltered in the MND patients compared with the controls. Only MND patients with both upper and lower motor neuron signs had a significantly decreased concentration of NAA (9.13 +/- 0.28 mM, mean +/- SEM) in the primary motor cortex when compared with healthy controls (10.03 +/- 0.22 mM). In conclusion, the slightly decreased concentration of NAA in the primary motor cortex from ALS patients may represent a loss of neurons in this region.

A prospective study of 39 patients with whiplash injury.

INTRODUCTION: The acute symptoms after whiplash traumas can be explained by the neck sprain, but the pathogenesis of the "late whiplash syndrome" and the reason why only some people have persistent symptoms more than 6 months is still unknown. MATERIAL AND METHODS: Thirty-four consecutive cases of whiplash injury were examined clinically three times; within 14 days, after 1 month and finally 7 months postinjury. In addition, MRI of the brain and the cervical spine, neuropsychological tests and motor evoked potentials (MEP) were done one month postinjury and repeated after 6 months, if abnormalities were found. RESULTS: The total recovery rate (asymptomatic patients) was 29% after 7 months. MRI was repeated in 6 patients. The correlation between MRI and the clinical findings was poor. Cognitive dysfunction as a symptom of brain injury was not found. Stress at the same time predicted more symptoms at follow-up. All MEP examinations were normal. CONCLUSION: In this study, long-lasting distress and poor outcome were more related to the occurrence of stressful life events than to clinical and paraclinical findings.

[Lamotrigine treatment of 92 patients with intractable epilepsy]. / Lamotriginbehandling af 92 patienter med intraktabel epilepsi.

The efficacy of treatment with lamotrigine (LTG) was evaluated in 92 patients with refractory epileptic seizures (46 women and 46 men aged 14-80 years, median 32 years). Seventy-one patients had partial epilepsy and 21 had primary generalized epilepsy. Patients were treated from zero to four (most frequently two) other antiepileptic drugs (AEDs). Maintenance dose of LTG was 50-800 mg daily (median 300 mg). Fifteen percent of the patients became seizure-free (13% of patients with partial epilepsy, 24% with primary generalized epilepsy). Thirty-eight percent of patients experienced at least 50% reduction in seizure frequency. Twenty-six percent of patients had a significant increase in seizure frequency (the same percentage in the two groups). Adverse events were recorded in 61% of patients, but most symptoms disappeared after dose reduction in concomitant AEDs. LTG was discontinued in 22% of patients, either because of adverse events or lack of effect. We conclude, that LTG is effective in reducing seizure frequency in patients with therapyresistant primary generalized epilepsy or partial epilepsy. Toxicity appears to be limited.

Trends in a Danish urologic department during the years 1979 and 1984.

During recent years several international studies have shown increasing rates of prostatectomy over the past decade. In Denmark the number of prostatectomies increased by 43% in the period 1977-85. This development is still unexplained, but one of the answers is very intriguing, proposing a shift in indications for prostatectomy. Therefore we retrospectively examined the records of 207 patients in 1979 and 243 patients in 1984, admitted with a diagnosis of benign hyperplasia of the prostate (BPH). In these patients respectively 164 and 149 prostatectomies were performed in the same years, at the Department of Urology, Glostrup County hospital. We found no evidence of a change in indications towards a more liberal attitude as suspected, but in contrast, we discovered a more conservative attitude. Because the national rates since 1984 have been stable, we conclude, that the development in a specialized urologic Department can serve as a predictor for the development on a larger national scale.

[Pancreato-pleural fistula in a patient with chronic pancreatitis]. / Pankreatikopleural fisteldannelse hos en patient med kronisk pancreatitis.

A case with pancreaticopleural fistula formation in a 33-year-old man with alcohol-induced chronic pancreatitis with a pseudocyst in the head of the pancreas is reported. The patient was treated with repeated pleurocentesis, but the fistula first closed and was drained after treatment with the somatostatin-analogue, sandostatin, for 38 days and total parenteral nutrition for 26 days probably because a spontaneous pancreaticogastric fistula had then formed. At the most recent outpatient control re-examination of the pancreas with ultrasound no cyst was shown.