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.

Reduced jaw opening from paradoxical activity of mandibular elevator muscles treated with botulinum toxin.

The aim of the study was the effect of injections with botulinum toxin A (BTX-A) on reduced jaw opening, caused by paradoxical, antagonistic activity of jaw elevator muscles after brain stem lesions. The study included a male (51 years) and a female (69 years) patient. Subjective assessment, clinical recordings, muscle blocks and electromyography (EMG) were used to diagnose paradoxical activity, and to plan, guide and evaluate the treatment. The paradoxical innervation pattern was unilateral in the male and bilateral in the female. The paradoxical activity during jaw opening amounted to 24-109% of the level during maximum biting, and bursts of paradoxical activity were also present during chewing. EMG-guided blocks and later BTX-A injections of the affected muscles increased the opening by 9-23 mm from pre-treatment values of 15-18 mm, and normalized chewing. The study proved BTX-A to be an effective treatment for reduced jaw opening caused by paradoxical activity. Treatment was optimized by EMG evaluation of the current activity of the jaw elevator muscles, permitting individual treatment plans with longer intervals between BTX-A injections and lower doses than with conventional treatment for oromandibular dystonia. Thus the treatment only had to be repeated one to two times per year to maintain acceptable jaw mobility.

A novel mutation in the epsilon-sarcoglycan gene causing myoclonus-dystonia syndrome.

Two families were referred with different clinical diagnoses of dystonia. Twenty-four family members were examined clinically, and mutation analyses were performed. Most of the affected individuals had laryngeal myoclonus and more severe dystonia of the legs than usually reported in myoclonus-dystonia syndrome. Sequence analyses revealed a previously unreported deletion (974delC or R325X) in exon 7 in the epsilon-sarcoglycan gene in members of both families. The two families were found to be related.

[Neurogenic bladder disturbances]. / Neurogene blaereforstyrrelser.

Knowledge of the neurophysiology of bladder control is growing, as better diagnostic methods are developed and because clinical interest in the field is greater and treatments are better. It is problematic that the symptoms are very few and do not provide much information about the lesions in the nervous system. We describe the bladder symptoms in most neurological diseases, and the neurophysiology of normal voiding is described. A strategy for handling neurological bladder symptoms is outlined.

[Hereditary dystonias]. / Hereditaere dystonier.

Dystonia is a heterogeneous, neurological disease characterized by involuntary, sustained muscle contractions, frequently causing twisting and repetitive movements or abnormal postures. The patients are often difficult to diagnose, and the treatment is almost always only symptomatic. It is believed that about 75% of all patients with dystonia have primary dystonia, and 25-85% of these are hereditary. Seven gene loci for autosomal, dominant inherited dystonia and two for X-linked, recessive inherited dystonia are known at present, but the underlying genes are known only for DYT1 and DYT5. Testing is possible for these two in Denmark. Growing molecular genetic knowledge will lead to earlier and correct diagnosing, including prognosis, and may elucidate the pathogenesis, making better treatment possible.

[Botulinum toxin treatment of patients with oromandibular dystonia]. / Botulinumtoksin-behandling af patienter med oromandibulaer dystoni.

INTRODUCTION: Oromandibular dystonia (OMD) is a frequently disabling focal dystonia, which may be treated with injections of botulinum toxin in the affected muscles. The aim of the present study was to evaluate the population, effect and side-effects of patients treated in Denmark during a nine year period. METHODS: We evaluated all 45 consecutive patients treated with quantitative EMG guided injections of botulinum toxin for OMD. RESULTS: The OMD symptoms varied but were most often mixed symptoms (n = 13), jaw closing (n = 11) and jaw opening (n = 7). Thirty-two patients (71%) had other focal or generalised dystonia, and in 24 the additional dystonia were also treated with botulinum toxin. The 45 patients had a total of 277 treatments (mean 6.2 treatments pr. patient), each including one to six muscles. Marked effect was observed or experienced after 193 (70%) treatments, and 33 patients (73%) experienced at least one effective treatment. Side-effects occurred after 35 treatments (13%) experienced by a total of 16 patients (35.6%), most frequently as transient mild dysphagia. DISCUSSION: The study shows that botulinum toxin treatment of OMD, guided by quantitative EMG, is safe and effective.

[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.

[Treatment of dyskinetic syndromes]. / Behandling af dyskinetiske syndromer.

Dyskinetic syndromes are conditions with involuntary movements. They can have different causes, but are often due to dysfunction of the basal ganglias. The clinical picture varies but all show spontaneous alterations in intensity as well as deterioration with stress. This often leads to misjudgment of cases of dyskinesia. It is however important to be aware of these syndromes as medical treatment is effective in many cases. The treatment of tremor, tics, chorea, myoclonus, dystonia and medically induced dyskinesia is reviewed and the clinical pictures are briefly described.

[Treatment of dyskinetic syndromes]. / Behandling af dyskinetiske syndromer.

Dyskinetic syndromes are conditions with involuntary movements. They can have different causes, but are often due to dysfunction of the basal ganglias. The clinical picture varies, but all show spontaneous alterations in intensity as well as deterioration with stress. This often leads to misjudgement of cases of dyskinesia. It is however important to be aware of these syndromes as medical treatment is effective in many cases. The treatment of tremor, tics, chorea, myoclonus, dystonia and medically induced dyskinesia is reviewed and the clinical pictures are briefly described.

[Amyotrophic lateral sclerosis: an autoimmune disease?]. / Amyotrofisk lateralsklerose: en autoimmun sigdom?

Amyotrophic lateral sclerosis is characterized by degeneration of the motor neurones in the central nervous system and, as a rule, the condition results in rapid incapacity and death. The etiology is unknown but experimental results from recent years suggest that immunological mechanisms are of pathophysiological significance. Gangliosides constitute an important membrane component in nerve tissue and the majority of patients probably have high titres of circulation polyclonal IgM-antibodies to these compounds, particularly gangliosides GM1 and GD1a. The antibody titre appears to be correlated with the clinical condition and selective immune suppression (e.g. with cyclophosphamide) may possibly be of therapeutic value.