Aim. The article is related to the epidemiological and clinical studies on the Creutzfeldt-Jakob disease (CJD) in Poland that are carried out in the 1st Neurological Department of the Institute of Psychiatry and Neurology. In this study an attempt was made to explain the reasons of discrepancies in the process of diagnostic reasoning between physicians referring patients with suspected CJD to the hospital and the clinical diagnoses made there.
Subjects and method. 62 patients with suspected CJD were hospitalized in the years 1996-2000. Symptoms indicated by the referring physicians in justification of suspected CJD were analyzed, and the patient group with suspected CJD was divided into sub-groups according to their, final clinical diagnosis made at the Department.
Results. In 18 patients from (his group the diagnosis of CJD was confirmed, while in 44 cases same other condition was diagnosed after clinical examinations. A majority of the latter (27/44) were diagnosed with Alzheimer s disease, Huntington's disease, post-inflammatory encephalopathy, or a range of degenerative illnesses.
Conclusions. Incorrect diagnoses made by the referring physicians were probably due to their not taking into account the diagnostic criteria for CJD, particularly the duration of illness and dynamics of its course. Moreover, they seemed to have insufficient knowledge of the type of cognitive impairments in various diseases of the nervous system.
Aim. The aim of the study was to assess 24-hour EEG recording in chronic alcohol abusers with epileptic seizures.
Subjects. Participants in the study were 21 patients aged 31-64 years who had been abusing alcohol in the past 5 to 10 years. The reason for their hospitalisation was: status epilepticus in 2 cases, cluster seizures in 6, and single epileptic seizures in 13 cases.
Method. In all the patients neurological examination, biochemical and electrolytic tests, EEG with photo- and phonic stimulation, EEG after sleep deprivation, 24-hour EEG monitoring, and head CT scan were performed.
Results. Neurological status of 11 patients was normal, 6 patients had pyramidal hemiparesis, one patient had symptoms of disseminated CNS lesions, there was a single case of the cerebellar syndrome and 2 cases of polyneuropathy. No abnormalities were found in head CT scans of 12 patients, while in other cases various structural intracranial lesions were found. Routine EEG examination as well as that following sleep deprivation was normal in 16 patients, and in 5 cases abnormalities were demonstrated. 24-hour EEG recording revealed generalised paroxysmal changes in 2 cases and sleep pattern disturbances in another 13 patients, i.e. absence of the slow-wave sleep phases.
Conclusions. An analysis of EEG recordings obtained from the group of alcohol abusers with epileptic seizures indicated no significant superiority of 24-hour EEG monitoring in detection of paroxysmal abnormalities. However; the technique allowed to reveal (in 62% of cases) sleep pattern disturbances associated with duration of alcohol abuse.
Objective. Chronic obstructive pulmonary disease (COPD) is associated with the risk of temporary or continuous hypoxemia. A low oxygen concentration may result in brain dysfunction and neuropsychological deficits leading to dementia. Sometimes law oxygen concentration and hypercapnia may be the cause of delusions and hallucinations. The aim of this study was to find out whether an impairment of cognitive functions and dementia may develop in the course of COPD and hypoxemia.
Subjects and method. Participants in the study were 36 patients with COPD, 32 patients with COPD and respiratory insufficiency, and 44 healthy volunteers. All patients were enrolled in the study on the basis of their medical history and physical examination; they were submitted to the Mini-Mental State Examination and spirometry.
Results - In 6% of the patients from the first group and in 19% of those in the second group dementia was diagnosed. There were no cases of dementia in the control group. A mild cognitive impairment was found in 22% of patients in both COPD groups, and in 2% of the controls. The mean Mini Mental State Examination scores were 27.13 ±2.54, 24.5 ± 1.07, and 28.5 ± 1.07, respectively. The cognitive impairment severity was correlated with FEV, in the first group (r = .79, p < .05), and with pO2 in the group with hypoxemia (r = .32, p = .0004).
Conclusion. COPD is probably a risk factor for cognitive impairment and dementia.
Aim. Typical changes in results of systemic sclerosis (SSc) patients' clinical and neuroimaging examinations and their role in predicting the nervous system involvement are reviewed in the paper:
Review. SSc is a chronic inflammatory connective tissue disorder of unknown origin, characterised by an overproduction and accumulation of collagen and other extracellular matrix proteins. In the course of the disease not only massive fibrosis of internal organs, but also features of vasculitis can be seen. Neurological manifestations in SSc either result directly from the disease or are secondary to the involvement of other organs. In some patients SSc is associated with other disorders of the connective tissue, in the course of which implication of the nervous system is a part of the typical pattern of the disease.
Conclusions. Contrary to the common opinion that both the central and peripheral nervous systems are spared in SSc patients, a majority of the affected individuals present subtle and often nonspecific neurological symptoms. More and more widely used neuroimaging techniques as well as electroencephalography and electromyography contribute to our gaining a new insight into the brain function and its disturbances in this group of patients.
Aim. Loss of neurons, most/y cholinergic, is a common feature of Alzheimer's and other neurodegenerative diseases. Excitotoxic reactions related to an excess glutamate release in the CNS probably contribute to cell death. There is a need for agents that prevent or inhibit the process of dementia, so drugs that slow the disease progression are urgently needed.
Review. NMDA receptors mediate fast excitatory synaptic transmission in the CNS, and are involved in learning and memory processes. These receptors comprise a ligand-gated ion channel that is permeable to Ca²+. In pathological conditions, an altered calcium homeostasis in neurons may result in cell death, and calcium-dependent neurotoxicity is thought to derive from an excessive activation of NMDA receptors. Thus, antagonists at NMDA receptors may prevent cell death and retard the progression of symptoms in patients with dementia. Many pre-clinical and clinical observations indicate the therapeutic utility of memantine. Memantine has been proved to be a non-competitive NMDA receptor antagonist, with sufficient affinity to block the CNS NMDA receptors at therapeutic doses.
Conclusions. In animal models memantine can protect against the excitotoxic destruction of neurons, and at clinically relevant concentrations memantine promotes synaptic plasticity and preserves or enhances memory functions. Consequently, the drug has received a considerable attention in recent years in the treatment of dementia.
Aim. To present contemporary views on the biological mechanisms underlying nicotine dependence, and treatment methods.
Review. Smoking is a chronic problem and at present about 40 percent of adults in Poland are cigarette smokers. The dopamine pathway seems to be one of the mechanisms leading to the development of nicotine addiction from tobacco smoking. This pathway originates from dopaminergic neurons of the midbrain and ascends to the nucleus accumbens and the prefrontal fields of the cerebral cortex. The dopamine system activation not only plays an important role in the onset of dependence, but also enhances motivation and increases efficacy of cognitive functions. Besides dopamine, many other mediators are released by nicotine, including noradrenaline, serotonin, endorphines, and acetylocholine. Bupropion being a dopamine re-uptake inhibitor is a useful drug in the tobacco smoking cessation treatment.
Conclusions. Tobacco dependence is the leading preventable cause or death; cigarette smoking is responsible for 1 in every 5 deaths. Half of the regular smokers die prematurely of tobacco-related diseases. Smoking cessation reduces the risk of tobacco-related diseases, slows the progression of already existing tobacco-related conditions, increases life expectancy, and improves quality of life in ex-smokers.
Aim. To summarize current knowledge about one of the drugs most frequently used in neurology and gerontology – the still highly controversial selegiline.
Review. Selegiline is a selective, irreversible inhibitor of monoaminooxygenase type B (MAO-B). Many papers have been recently published concerning the efficacy, tolerability and safety of selegiline in Parkinson s disease. The publications systematize our knowledge about the drug and should influence its rational use in clinical practice. Selegiline is a useful drug producing a modest benefit, mostly in an early stage of the disease. According to many authors it may either delay the time when levodopa is necessary, or when administered concomitantly, allows to decrease the levodopa dose. Great hopes have been raised by the alleged neuroprotective action of selegiline – unfortunately, no such effect of the drug has been univocally proved so far. This mode of action is supposed to result from inhibition of free radicals formation in the process of dopamine metabolism due to inhibition of MAO-B. According to recent research, this neuroprotection may be obtained in a way unrelated to MAO-B inhibition, but due to antiapoptotic activity of desmethyloselegiline, one of selegiline metabolites. Selegiline was also used in clinical trials in Alzheimer's disease, narcolepsy and attention-deficit hyperactivity disorder in children.
Conclusions. Selegiline is a useful, but rather weak drug in the symptomatic treatment of Parkinson s disease, with no proven neuroprotective action (although many authors believe in such a mechanism). Its use in Alzheimer's disease, narcolepsy and Tourettism requires further study. Despite its wide use, possible side effects of selegiline should be kept in mind, as well as its relatively numerous potential interactions with other drugs, especially in the elderly.
Aim. To present a case of unexpected behaviour with criminal consequences, due to treatment with lorazepam.
Case report. Lorazepam had been administered to the patient in the dose of 1-2.5 mg per day for several weeks, because of depressive symptoms and insomnia. In the critical time the patient at first took 30 mg of lorazepam in attempted suicide, and about a week later – again a large dose of lorazepam and tianeptine. At night he committed murder of his wife and attempted to kill his daughter.
Comment. The authors draw attention to this rarely occurring but clinically important source of risk associated with the use of benzodiazepines. (Eds.)