Huntington’s disease (HD) belongs to a large group of neurodegenerative diseases caused by a genetic defect consisting in an increased number of repetitive sequences of DNA within the altered gene in the chromosome 4 encoding huntingtin. The consequence of this mutation is selective destruction of neurons mainly within the caudate nucleus, putamen, globus pallidus and also other structures, including thalamus and white matter. Apart of involuntary movements, symptoms include other neurological signs (e.g. parkinsonism), disturbed cognitive functions (dementia) and various psychiatric disorders. In spite of a significant progress in understanding HD pathogenesis, no effective cause-oriented treatment has been developed to date. Currently available therapies include attempts at correction of individual symptoms, which, as in the case of Parkinson’s disease, is handicapped by mutual interdependence of neurological and mental state, as well as by effects of administered drugs, both psychotropic and those targeted on particular neurological signs. In the paper we present an evidence based review of available modalities for symptomatic treatment of motor disorders (involuntary movements, parkinsonism, dystonias), other neurological symptoms, mental disorders (depression, psychoses) and disturbances of cognitive functions associated with HD. Separately discussed are attempts at administration of neuroprotective drugs (including creatine, unsaturated fatty acids and minocycline), which are recommended expecting to correct the natural course of the disease.
Electroconvulsive therapy was established in 1939 and it still has been developing. The idea of therapeutic effect of electric shocks came from the observations, that psychotic symptoms disappeared in patients, who underwent seizures. The electric stimulus was used to produce grand mal seizures in psychiatric patients. Nowadays the modifications in technical aspects of electrotherapy results in good tolerance of electroshocks. In the contrary to common opinions this method is more safe and efficacious than pharmacotherapy in many psychiatric conditions. Fast antidepressant effects is beneficial in lowering the risk of suicide in major depression. Electroconvulsive therapy is first line treatment in acute fatal catatonia and in schizophrenia resistant to pharmacotherapy.
Post-ECT delirium is a serious complication that may lead to stop treatment. There are two types of delirium: postictal delirium that occurs within an hour after the course of ECT and interictal delirium that happens between the ECT courses (usually after a period of clear consciousness). The paper reviews the current hypotheses and scientific data about post-ECT delirium. It also presents results of the study performed in Lodz Psychogeriatric Medical Centre which objective was to examine relations between sex, age, diagnosis, seizure duration, parameters of electric stimuli and severity of post-ECT delirium. The study group consisted of 42 patients who underwent ECT with bitemporal electrode placement and brief-pulse stimulation in general anesthesia with the frequency 2-3 sessions a week – 498 sessions in total. Results: the frequency of postictal delirium was 28,6% – 52,6%, interictal delirium – 0 – 10%. The severity of postictal delirium was positively statistically correlated (p<0,05) to length of seizures, electric dose and current width. The severity of interictal delirium was positively statistically correlated to electric dose and current width. We observed more impaired consciousness in terms of both postictal delirium and (more often) interictal delirium in the group of schizophrenia than in the group of depression disorder. 5 patients (11,9%) developed delirium that stopped treatment. The interactions between sex and age and post-ECT delirium were inconsistent.
Transcranial magnetic stimulation (TMS) is rapidly developing as a powerful, non-invasive tool for studying the human brain. TMS is currently a target of interest of both basic science investigators and clinicians. In this procedure a pulsed magnetic field generated extracranially induces focal intracranial electrical discharges that can temporarily excite or inhibit specific brain areas. In this way there is possible to alter the functioning of the brain beyond the time of stimulation, offering potential for therapy. TMS has been used in the investigation of neurological and neuropsychiatric disorders, and more recently has emerged as a tool in the study and treatment of depressed mood. TMS is being evaluated as a possible alternative to electroconvulsive therapy for the treatment of refractory depression. This review examines the basic principles underlying transcranial magnetic stimulation. Published studies in this field are summarized and paper reviews TMS as an investigational and treatment tool in schizophrenia, affective disorders and anxiety disorders. It concludes by discussing safety issues, limitations, and directions for future research.
Background and objective: Pharmacotherapy of Parkinson’s disease not always enables controlling the symptoms. As the results there is growing interest in stereotactic surgery, which is permanent (after ablation) or temporary (after stimulator implantation) inhibition of too exhibited structures of extrapyramidal system due to development of the disease. The aim of this paper was the analysis of the causes of disqualification from surgical treatment of patients with Parkinson’s disease, who were directed to Neurosurgical Department of Military Clinical Hospital in Bydgoszcz. Recommendations for an operation were verified during the diagnostic stay of the patients in the Department of Neurology. Material and methods: From January 2003 to December 2004, 86 patients (59 male and 27 female), from the age of 29 to 81, were admitted to Department of Neurology in order to be qualified for surgical treatment. The qualification criteria included: 1. Diagnosis of idiopathic Parkinson’s disease. 2. At least 5 years since the onset of symptoms. 3. Good responsiveness to levodopa. 4. Exclusion of severe dementia and depression (MMSE and MADRS). 5. Good results of neuroimaging (MRI/CT). 6. Optimal pharmacological therapy before surgery. Results: 45 patients were disqualified from surgical treatment (52%). The most common reason for disqualification was deficient pharmacological therapy before surgery and significantly increased depression – the same number of 19 patients (42%) in both groups. In 16 patients (35.5%), the diagnosis of idiopathic Parkinson’s disease was not confirmed. In 28% (13 patients) the reason for disqualification was dementia. Nine patients (20%) did not qualify since they been ill for less than 5 years. Conclusions: The decisions to send patients to hospital for surgery were often made even though the pharmacological therapy was incomplete. A doctor should never get pressurized by patients, but always clearly present acceptable recommendations for surgical treatment, its advantages and disadvantages.
BNP, brain natriuretic peptide, is a member of a family of hormones with natriuretic, diuretic and vasorelaxant activity. It is secreted mostly from the cardiac ventricles, but it is synthetized in the human brain too. Till now measurement of BNP has been valuable in the rapid diagnosis of heart failure. Reports of changes in BNP content in cerebrovascular diseases suggest that BNP plays a role in pathogenesis and pathophysiology of ischemic stroke. BNP is elevated in nearly two thirds of acute stroke patients. This rise is caused inter alia 1) by the sympathetic system activation, which may have toxic effect on the myocardium, leading to myocardial dysfunction and increased secretion of BNP from the damaged heart, and 2) by increased production of BNP by the damaged part of brain. The attempts to find correlation between circulating NT-proBNP concentration and stroke topography, infarct size or severity of neurological deficit do not give unequivocal results. The same applies to prognostic value of BNP concentration during ischemic stroke. Despite the contradictory findings, it is worth continuing this research, because there is an association between cardiac failure and poor prognosis in acute cerebrovascular diseases. Early prognosis of heart failure in ischemic stroke may be useful in the selection of an adequate therapy and may improve prognosis of acute stroke patients.
Tuberous sclerosis (TS) is a progressive, dominantly inherited disorder affecting multiple organs, mainly: skin, central nervous system, kidneys, lungs, heart, eyes. This diseases is associated with mutation in two tumor suppressor genes: TSC1 and TSC2, that function as a complex to suppress signaling in the mTOR pathway. TSC1 is located on chromosome 9q34, the gene contains 23 exons and encodes hamatin. TSC2 is located on chromosome 16p13, the gene contains 41 exons and encodes tuberin. The diagnostic criteria of TS are based on the premise that there are probably no truly pathognomonic clinical sign. They were lately revised in 1998. In this paper we present a case of 32-year-old woman suffering from TS. The right diagnosis was made after 26-year course of disease, because of phenotypic variability in this disease. To our knowledge, it is the first so long observation of patient with TS after neurosurgical removal of brain tumor (histologically SEGA) and radiotherapy by cobalt.
Dystonic and athetoid movements are usually caused by lesion of the basal ganglia, however the etiopathogenesis of involuntary movements could be also different. The 27-year-old female was admitted to the Neurological Department of the Kopernik Hospital in ŁódŸ, because of developing in two days writhing dystonic and athetoid movements of the hands and weakness with paresthesias of upper and lower limbs. The neurological examination also revealed the loss of abdominal reflexes, which could suggest demyelinating process. The laboratory tests of blood and composition of CSF were normal. No demyelinating lesions were disclosed in the brain MRI. The only one lesion was noticed in posterior cervical spine in the cervical MRI. The results of EVP and BEARs were normal. The involuntary movements diminished after treatment with intravenous infusions of amantadine, but the patient decided to discharge from the hospital before the end of therapy. In one week the patient was admitted to the hospital again because of the same complaints and appearance of pathological symptoms and Lhermitte’s sign. Repeated MRI of the brain and cervical spine showed only the lesion found earlier. The amplitude of F wave was extremely high in EMG examination. The involuntary movements as a clinical manifestation of the single cervical demyelinating lesion are described in literature rarely.