Clinical Neuroscience

[PRAMIPEXOLE THERAPY OF RESTLESS LEGS SYNDROME]

VIDA Zsuzsanna, SZAKÁCS ZOLTÁN

JANUARY 20, 2007

Clinical Neuroscience - 2007;60(01-02)

[The restless legs syndrome is a disorder belonging to the family of movement disorders during sleep, often remains unrecognized, although it is the second most common cause of chronic sleep deficiency and daytime sleepiness. In accordance with international guidelines, pharmacotherapy of this disorder should begin with a dopamine agonist. Owing to their efficacy and favorable safety profile, newly introduced, selective dopamine agonists have become extensively used for this purpose. This study evaluated the efficacy of one of the products in this group, pramipexole. Fifty-one patients suffering from idiopathic restless legs syndrome underwent monotherapy with pramipexole in daily doses of 0.25 to 1.0 mg. Therapeutic efficacy was evaluated using three tools, i.e. follow-up questionnaires, actigraphy, and Forced Immobilisation Test. An excellent therapeutic effect was seen in more than 80 per cent of the study population. As shown by findings of the follow-up questionnaires, pramipexole resulted in substantial improvements of both daytime and nighttime symptoms of RLS. Actigraphy monitoring demonstrated a statistically significant increase in the ratio of time spent without limb movement to the time spent in bed; furthermore, the result of the Forced Immobilisation Test also improved. It seems fair to conclude from the findings of this study that pramipexole monotherapy is an effective treatment in restless legs syndrome.]

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Clinical Neuroscience

[APPLICATION OF FUNCTIONAL MR-IMAGES ACQUIRED AT LOW FIELD IN PLANNING OF NEUROSURGICAL OPERATION CLOSE TO AN ELOQUENT BRAIN AREA]

AUER Tibor, SCHWARCZ Attila, JANSZKY József, HORVÁTH Zsolt, KOSZTOLÁNYI Péter, DÓCZI Tamás

[Aim of the study - Presentation of functional MRI performed at low magnetic field (1 Tesla) for planning microsurgical operation in a patient suffering from tumor close to an eloquent brain area. Methods - Microsurgical removal navigated by frameless stereotaxy of an intrinsic tumor located in eloquent area is indicated if speech function is not damaged, i.e. exact localisation and relationship of the tumor and speech area can be defined. Before operation an optimized EPI based 2D sequence was applied to yield functional MR images. At the planning of the operation the paradigm used for the localization of the sensory language cortex contained passive listening to a text. Control investigations were performed one month postoperatively. A specific psychological test, as an additional investigation to estimate the accurate level of the sensory language function, was also conducted. Results - Low resolution (matrix of 64×64) functional MR images visualized sensory speech center and auditory cortex satisfactorily. The scans showed clearly that the Wernicke's region was situated just above the tumor (WHO grade II glioma), and this finding increased the safety of intraoperative localization and reduced the risk of morbidity. Control examinations revealed minimal decrease in sensory language function, however, it was not noticeable for either the patient or her surroundings. Conclusion - Optimized functional MR imaging performed at low magnetic field can support planning of neurosurgical operations and reduce the morbidity of microsurgical interventions.]

Clinical Neuroscience

[CLINICAL EXPERIENCE WITH LEVETIRACETAM FOR ADULTS WITH EPILEPSY]

BARCS Gábor, SZŰCS Anna

[Objective - A retrospective study to evaluate the efficacy of levetiracetam in the treatment of adult pharmacoresistant epilepsy. Method - Retrospective work up of our treatmentexperiences with 55 pharmacoresistant patients treated with levetiracetam (11 of them on monotherapy) for 6-39 months. Three treatment groups were analysed: idiopathic generalised epilepsy (9 patients); partial epilepsy (30 patients); malignant or malignated epileptic syndromes (16 patients). Result - Seven idiopathic generalised patients (77%) and 5 partial epilepsy patients (16%) became seizure free. One idiopathic generalised epileptic patient, 10 partial epilepsy patients (33%) significantly improved. Six patients (37%) from the group of malignant or malignated epileptic syndromes also significantly improved. Five of the improved idiopathic generalised epilepsy patients and 6 of the improved partial epilepsy patients received levetiracetam monotherapy. Altogether seven patients (12% of the whole population) relapsed after a 4-15 months improved period. Fifteen patients (27%) suffered side effects (mainly somnolence, headache, dizziness and irritability) improving after dose reduction of levetiracetam (generally below 2000 mg pro day). Conclusion - Levetiracetam is an effective, well tolerable, broad-spectrum drug as adjunctive treatment or monotherapy in adult patients unsuccessfully treated with other antiepileptic drugs.]

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[NONSENSE MUTATION 193C>T OF NEUROFIBROMATOSIS TYPE 2 - A NEUROSURGICAL CHALLENGE]

BOBEST Mátyás, TÓTH Csaba, GYURCSÓ Mária, MOLNÁR Mária Judit, GARZULY Ferenc

[A 15 years old male was operated because of incidentally found intercostal schwannoma. Two years later severe cerebellar ataxy and left sided anacusis developed. MRI revealed bilateral vestibularis tumors and multiple cervical intradural extramedullar myelon compressing lesions. After partial resection of the huge left sided cerebello-pontin tumor, histologically schwannoma, and the exstirpation of the multiple cervical meningiomas the patient died three months later due to septic complications. The 24 years old mother had been operated on similar lesions 12 years earlier, after two weeks postoperative period she died. Her 14 years old twins are living, a boy also with bilateral acustic tumours and a girl who is intact. Genetic investigation revealed C>T nonsense mutation at position 193 in the exon 2 of the NF2 gene. This mutation cause premature truncation of the gene protein and is probably in connection with the clinically severe phenotype. Early diagnosis of this type of neurofibromatosis is mandatory concerning the therapy.]

Clinical Neuroscience

[UPDATES IN PRACTICAL NEUROLOGY - I. THE PRINCIPLES OF MODERN LEVODOPA THERAPY IN PARKINSON’S DISEASE]

KLIVÉNYI Péter, VÉCSEI László

[Despite the levodopa is used for the treatment of Parkinson’s disease for a long time, recently many questions raised about its clinical use. New issues emerged based on the clinical trials, on latest neuroimaging data and on better understanding the pathomechanism of motor complications. These observations have changed the routine clinical use of levodopa. In this review we summarize the evidences and practical implications of levodopa therapy.]

Clinical Neuroscience

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[Congress calendar 2007;60(01-02)]

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Restless leg syndrome in adult patients with β-thalassemia major

ERDEM Ramazan , ERDEM Şimşek Nazan, KURTOĞLU Erdal

To investigate the quality of sleep and the presence of Restless Legs Syndrome (RLS) in the Turkish population with β-thalassemia major (TM). The second aim was to assess the risk factors of RLS in TM adults. The study sample comprised of 121 patients at least 18 years old with TM. The patients’ socio-demographic information, body mass indexes (BMI), current medications, laboratory data were recorded. The patients were asked if they had a history of chronic kidney disease, diabetes mellitus (DM), and polyneuropathy. Restless legs syndrome was diagnosed according to the International Restless Legs Syndrome Study Group criteria. The sleep quality of the patients was assessed using the Pittsburgh Sleep Quality Index (PSQI) scale. The Epworth Sleepiness Scale (ESS) was used to assess excessive daytime sleepiness in the patients. The median age of the patients was 25 years (range 18-52). The mean BMI was 21.49±2.5 (R 14-26.5) for all patients. The prevalence of RLS was 5% in TM adult patients. The TM patients with RLS had no major complications of TM. The median PSQI global score of all patients was 3. Twenty-two (18.1%) patients had poor sleep quality. The reason for poor sleep quality was RLS symptoms in four patients (18%). There was no significant association between PSQI total score and blood parameters of the patients. Twelve (9.9%) patients had ESS scores greater than 10, which indicates excessive daytime sleepiness. The prevalence of RLS in TM patients was similar to that of the general Turkish adult population. These results indicate that RLS may occur in patients with TM, although they had a high level of serum ferritin.

Lege Artis Medicinae

[Advances in sleep diagnostics]

HORVÁTH Róbert

[Despite the insufficient financial support, the number of the laboratories for sleep diagnostics and the examined patients are rising. The CPAP (Continuous Positive Airway Pressure) equipment playing the main role in the treatment of the obstructive sleep apnoe syndrome (OSAS) is recently available from three neurological centres (Budapest, Debrecen, Pécs). These centres can prescribe the machine as a ”model experiment” with 85% support given by the State Health Care. The increase of the number of the licensed hospitals is expected in the near future, the only condition is the polysomnography background. Differential diagnostics (narcolepsy, RLS, PLMS, sleepdependent laryngospasm, disorders of the circadian rhythm) and the follow-up of the CPAP using patients (indication, contraindication, compliance, CPAP pressure setting, CPAP therapy control) have been emphasized.]

Hypertension and nephrology

[Sleep disorders and quality of life in patients after kidney transplantation]

TURÁNYI Csilla Zita, ZALAI Dóra, MOLNÁR Miklós Zsolt, NOVÁK Márta, MUCSI István

[Kidney transplantation provides the best outcomes, concerning morbidity, mortality and health related quality of life for patients with end stage renal disease. Health related quality of life is increasingly recognized as an important outcome measure in patients with different chronic medical conditions, including chronic kidney disease. Sleep disorders, such as insomnia, sleep apnea syndrome and restless legs syndrome are common in patients with chronic kidney disease. The prevalence of insomnia and restless legs syndrome is similar in kidney transplanted patients to the prevalence observed in the general population. On the other hand, the prevalence of sleep apnea is considerably higher, about 30%. The association between sleep disorders and impaired health related quality of life has been relatively well documented in dialysis patients but only scarce information has been published about the kidney transplanted population. In this paper we summarize published data about sleep disorders and also about their association with health related quality of life in the kidney transplanted population.]

Clinical Neuroscience

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[Study objectives - In spite of the useful information provided by actigraphy in sleep medicine it is still not an independent tool either in the clinical diagnosis or in the follow-up. In the frame of a retrospective study, a simple new method of data reduction was applied with the aim of improving the clinical impact of actigraphy for the diagnosis of primary insomnia. Methods - Actigraphic records with a duration of 1 week produced on 47 subjects who met the inclusion-exclusion criteria. The daily activities during the investigational period were registered by means a self-completed questionnaire. Three parameters (sleep latency, sleep fragmentation and sleep efficiency) and only their three ’worse - as regards insomnia’ daily values were analyzed statistically. The study participants comprised 13 healthy controls, 17 healthy ‘bad sleepers’ and 17 subjects with primary insomnia. Results - The post-hoc tests did not reveal statistically significant difference in the three parameters between the healthy and ‘bad sleeper’ groups, but these two groups differed statistically from the primary insomnia group. Conclusion - The actigraphic analysis of sleep latency, sleep fragmentation and sleep efficacy allows a significant differentiation between subjects with primary insomnia and healthy controls, but not between healthy controls and healthy ‘bad sleepers’. Statistical algorithms indicated ‘models’ for clinically good and bad sleepers. Further studies on large populations are necessary before this method can be introduced in the routine medical care of individuals with primary insomnia.]

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[Restless legs syndrome in patients with chronic kidney disease]

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[The aging of the population, the high prevalence of chronic diseases and the consequent rapid increase of healthcare expenditures present a difficult challenge for the medical care system and for the society in the developed countries. Sleep disorders are increasingly recognized as very frequent chronic diseases with significant pathophysiological and psychosocial consequences. In the last 20 years an increasing number of studies reported high prevalence of sleep disorders, such as restless legs syndrome in patients with kidney disease. Chronic renal failure is the most common condition presenting with secondary restless legs syndrome. It is associated with insomnia, depressive symptoms and anxiety, impaired quality of life, as well as elevated cardiovascular risk. Compliance of the patients with restless legs syndrome is decreased, and it is more likely that they discontinue dialysis treatment. This may be related to higher mortality in kidney disease patients with restless legs syndrome.]