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A malfunctioning heater caused a severe carbon monoxide (CO) intoxication leading to unconsciousness and predominantly right-sided extrapyramidal syndrome in a 29-year-old man. Follow-up included thorough clinical monitoring, and brain MRI and PET studies. Nine days after the poisoning, brain MRI showed symmetrical necrosis in the globus pallidi, but no abnormality was found in the substantia nigra. In addition, white matter periventricular lesions were seen. In a control scan 14 months later the white matter changes had subsided but small necrotic lesions were still noted bilaterally in the globus pallidi. A 6-[(18)F]fluoro-L-dopa PET examination performed 5 weeks after the intoxication revealed impaired presynaptic dopaminergic function in the left putamen whereas in the right putamen the dopaminergic activity was within normal limits. [(11)C] raclopride PET imaging 4 months after the poisoning showed no abnormality in postsynaptic D2 binding in the striatum. Clinically, the parkinsonian symptoms resolved 1.5 years after the poisoning. The final outcome of the recovery was excellent, and the patient returned to work. This is the first case reported where unilateral presynaptic, dopaminergic hypofunction in putamen could be confirmed with fluoro-l-dopa PET imaging on a patient with extrapyramidal syndrome caused by CO poisoning. Our results emphasize that CO intoxication can lead to striatal dopaminergic hypofunction, and that PET is a sensitive tool in evaluating extrapyramidal system after sudden neurotoxic insult., ((c) 2010 Elsevier Inc. All rights reserved.)

The objective of this questionnaire study was to assess the effect of cervical dystonia on patients' working capacity. Of the 303 working-aged members of the Finnish Dystonia Association (N = 433) who participated in the study 247 (82%) had cervical dystonia. Their median age was 50 years, the median duration of CD symptoms was 12.3 years. Most (78%) subjects were on botulinum toxin treatment. Ninety-seven (39%) had retired because of CD at a median age of 48 years; 96 (39%) of the subjects were working: 87 full-time and 9 part-time. The remaining participants were on sick leave, unemployed, studying or retired of other reasons. Retirement occurred more than ten years earlier compared with the general Finnish population. All possibilities to help CD patients to continue longer in work should be considered early., (Copyright (c) 2009 Elsevier Ltd. All rights reserved.)

We have prospectively followed 16 Finnish xeroderma pigmentosum (XP) patients for up to 23 years. Seven patients were assigned by complementation analysis to the group XP-A, two patients to the XP-C group and one patient to the XP-G group. Six of the seven XP-A patients had the identical mutation (Arg228Ter) and the seventh patient had a different mutation (G283A). Further patients were assigned to complementation groups on the basis of their consanguinity to an XP patient with a known complementation group. The first sign of the disease in all the cases was severe sunburn with minimal sun exposure in early infancy. However, at the time the diagnosis was made in only two cases. The XP-A patients developed neurological and cognitive dysfunction in childhood. The neurological disease advanced in an orderly fashion through its successive stages, finally affecting the whole nervous system and leading to death before the age of 40 years. Dermatological and ocular damage of the XP-A patients tended to be limited. The two XP-C patients were neurologically and cognitively intact despite mild brain atrophy as seen by neuroimaging. The XP-G patients had sensorineural hearing loss, laryngeal dystonia and peripheral neuropathy. The XP-C patients had severe skin and ocular malignancies that first presented at pre-school age. They also showed immunosuppression in cell-mediated immunity. Neurological disease appears to be associated with the complementation group and the failure of fibroblasts to recover RNA synthesis following UV irradiation, but not necessarily to the severity of the dermatological symptoms, the hypersensitivity of fibroblasts to UVB killing or the susceptibility of keratinocytes to UVB-induced apoptosis.

Ingestion of windshield washer liquid resulted with an acute severe methanol intoxication in a 49-year old man. He developed optic atrophy with blindness, and an extrapyramidal syndrome. Putaminal injury and hyperintensity in the subcortical white matter was seen in a brain MRI. PET scanning with 6-[18F]fluoro-L-dopa confirmed symmetrical impaired presynaptic dopaminergic activity in the striatum, indicative of functional impairment of dopaminergic nigrostriatal neurons. The striatal uptake was more markedly impaired in the putamina (40% of controls) than in the caudate nuclei (60% of controls). To our knowledge, this is the first report of an 18F-dopa PET scanning result in a case of an acute methanol poisoning.

We performed a nation-wide study on geographical variation in the incidence and prevalence of medicated parkinsonism among the Finns aged > or =30 years using Bayesian spatial conditional autoregressive models. Registry of reimbursed medication for parkinsonism and a prescription database of purchase of these drugs were used to identify the study subjects. They were located by the map coordinates of the place of residence and aggregated into regular 100 km(2) grid cells. A total of 7,190 incident and 10,616 prevalent cases were found. The age-adjusted annual incidence was 32.6/100,000 (95% HDR 31.8-33.4) during the years 1995 to 2000 and prevalence was 268/100,000 (95% HDR 263-274) in 2000. The male to female ratio was 1.45 (95% HDR 1.39-1.51) in incidence and 1.54 (95% HDR 1.47-1.61) in prevalence. There was strong evidence for geographic variation in incidence and prevalence. A zone with high incidence and prevalence was identified in the eastern and central part of Finland. There was no evidence for difference in incidence and prevalence between urban and rural areas. The marked (more than two-fold) geographic variation can hardly be caused solely by practices of the registration and collection of data on diagnosis or by methodological issues, but rather suggests to geographic variation in protective and predisposing factors of Parkinsonism in Finland.

Background and Purpose: In this present study, we tried to find out if there is a subgroup of patients that should not undergo transoesophageal echocardiography (TEE) after an ischaemic stroke or transient ischaemic attack (TIA)., Methods: A total of 441 consecutive unselected patients with ischaemic stroke or TIA suitable for anticoagulation were examined with TEE in the acute phase. The patients were divided into five subcategories according to their rhythm, age and the findings in carotid sonography, and into two groups according to the presence of clinical risk factors for ischaemic stroke or TIA., Results: From the 441 studied patients, 60 (14%) had chronic or paroxysmal atrial fibrillation (AF) and 381 (86%) were in sinus rhythm (SR). Of the patients in SR, 46 (12%) were below 50 years old. The carotid sonography was conducted in 240 patients above 50 years old and in SR, and <50% internal carotid artery (ICA) stenosis was found in 194 (81%) patients and > or =50% ICA in 46 (19%) patients. Potential cardiac sources of embolism were found in patients both with AF or in SR (70% versus 46%), both below and above 50-year-old patients in SR (37% versus 47%), both in over 50-year-old patients in SR with <50% ICA stenosis and > or =50% ICA stenosis (41% versus 61%) and in patients in SR either without or with clinical risk factors for ischaemic stroke or TIA (43% versus 51%). On the basis of the TEE study, oral anticoagulation was started in 36 (9%) patients in SR., Conclusion: These results support TEE in patients with ischaemic stroke or TIA who are candidates for receiving oral anticoagulation.

Purpose: Unverricht-Lundborg disease (ULD) is currently classified as progressive myoclonus epilepsy. Myoclonus, the characteristic symptom in ULD, suggests that dopamine neurotransmission may be involved in the pathophysiology of ULD. Our purpose was to examine brain dopaminergic function in ULD patients., Methods: Four genetically and clinically diagnosed ULD patients and eight healthy controls were scanned with [(11)C]raclopride-PET. PET images were coregistered to individual 1.5 T MR images and region-of-interest analysis was performed for the striatum and thalamus. Standardized uptake values and individual voxel-wise binding potential maps of the patients and controls were also analyzed., Results: ULD patients had markedly higher (31-54%) dopamine D2-like receptor availabilities than healthy controls in both the striatum and the thalamus. The proportionally highest binding potentials were detected in the thalamus. There were no significant differences in the cerebellar uptake of [(11)C]raclopride in ULD patients versus healthy controls. Voxel-based results were in accordance with the region-of-interest analysis., Conclusions: These results suggest that dopaminergic modulation at the level of the striatum and thalamus could be a crucial factor contributing to the symptoms of ULD. In the light of our data, we propose that ULD with dopamine dysfunction and dyskinetic symptoms shares certain pathophysiological mechanisms with classical movement disorders. Future studies are therefore warranted to study the effect of dopaminergic pharmacotherapy in ULD.

The purpose of the present study was to establish how Parkinson's disease (PD) affects working capacity. Altogether, 937 (70%) of 1,343 working-aged members of the Finnish Parkinson Association participated in a questionnaire study. The ages of the subjects were between 29 and 65 years, median 59.0 years; 508 (54%) were men. The median (quartile range) duration of PD symptoms was 7.3 (4.3-11.3) years, and the median self-estimated severity of the disease on the Hoehn & Yahr scale was 2. Only 150 of 937 subjects (16%) were still working: 112 (12%) full-time and 38 (4%) part-time. Among those 343 (37%) who had retired purely because of PD at a median age of 53.4 years, the median working time was 1.7 years after established diagnosis. In part-time work, PD patients had been able to continue for a median of 4.3 years. PD often leads to early retirement; compared with the general Finnish population of similar age, PD patients had retired on the average 6 years earlier. Young age, mild symptoms, and short duration of the disease as well as adjustments enabling easier employment were associated with the possibility to continue longer in the work force., (Copyright 2006 Movement Disorder Society.)

Purpose: To assess the benefit of combining carotid sonography and transesophageal echocardiography (TEE) for the evaluation of patients with stroke or transient ischemic attack (TIA) in the territory of the carotid artery., Methods: During a 2-year period in Turku University Hospital, consecutive in patients with stroke or TIA who were candidates for carotid endarterectomy and for oral anticoagulation were evaluated with carotid sonography for symptomatic moderate (50-69%) or severe (>or=70%) internal carotid artery (ICA) stenosis, and with TEE for potential cardiac sources of embolism., Results: In 20% (40/197) of patients, a severe symptomatic ICA stenosis and/or a major risk factor for a cardiac source of embolism were found. In 56% (110/197) of patients, a moderate or severe symptomatic ICA stenosis and/or a potential cardiac source of embolism were found, whereas 11% (21/197) of patients had both a moderate or severe symptomatic ICA stenosis and a potential cardiac source of embolism., Conclusions: This study suggests that the presence of a moderate or severe symptomatic ICA stenosis does not exclude the presence of a potential cardiac source of embolism and vice versa. Carotid sonography and TEE complement each other and are valuable diagnostic tools that should be recommended in patients with ischemic stroke or TIA in the territory of the carotid artery when they are candidates for carotid endarterectomy and for oral anticoagulation.

Balo's concentric sclerosis is a severe demyelinating disease with poor prognosis. To date, nothing is known about the effects of pregnancy on the course of Balo's disease. We describe here the first successful pregnancy of a patient with Balo's concentric sclerosis. Upon neurological worsenings during pregnancy, our patient was treated with high-dose methylprednisolone and plasma-exchange. Intravenous immunoglobulin and interferon-beta-1a were given postpartum. At the last follow-up visit 2.5 years after the delivery the patient had only minor residual symptoms.

Background: Because enteroviruses can be detected in various clinical samples during enteroviral meningitis, we analyzed the combined diagnostic utility of polymerase chain reaction (PCR) of cerebrospinal fluid (CSF), feces, and serum for detection of enterovirus in specimens obtained from adults with aseptic meningitis or encephalitis., Methods: PCR results were analyzed for 34 adults for whom enteroviral meningitis was diagnosed on the basis of virus isolation and antibody detection in our hospital during 1999-2003. PCR results were also analyzed for 77 adults with meningitis or encephalitis of another defined cause for whom this assay was used for diagnostic evaluation during that period., Results: Twenty-six (76%) of 34 CSF samples and 24 (96%) of 25 fecal samples collected from patients with enteroviral meningitis had positive PCR results. The diagnostic yield of the test was lower for CSF specimens obtained >2 days after clinical onset, compared with CSF collected < or =2 days after onset. Instead, PCR of feces was highly useful also later, because 12 of the 13 fecal specimens obtained 5-16 days after clinical onset had positive test results. None of 75 CSF samples and 2 of 48 fecal samples obtained from patients with nonenteroviral infection had positive PCR results. All serum samples were PCR negative., Conclusions: PCR of fecal specimens obtained throughout the course of enteroviral meningitis had the highest clinical sensitivity for detecting enterovirus. It is recommended that, in addition to performance of CSF PCR, fecal samples collected from patients with suspected enteroviral meningitis should be tested by PCR, especially when the duration of symptoms is >2 days.

Background: Herpes simplex virus 2 (HSV-2) and HSV-1 have been recognized as causes of recurrent aseptic lymphocytic meningitis (RALM). However, the role of other herpesviruses has not been systematically assessed., Objectives: To evaluate the cause of RALM by using polymerase chain reaction (PCR) tests detecting varicella-zoster virus (VZV), cytomegalovirus (CMV), or human herpesvirus 6 (HHV-6), in addition to HSV, on cerebrospinal fluid (CSF) samples; and to assess the utility of PCR and antibody analyses in consecutive episodes of RALM., Design: The PCR and antibody results for herpesviruses were analyzed from 14 patients having 48 episodes of RALM., Results: The CSF PCR results for VZV, CMV, and HHV-6 were negative in 12, 10, and 11 patients investigated, respectively, and antibodies against VZV, CMV, and HHV-6 showed only old immunity. Herpes simplex virus 2 was detected from the CSF in 10 patients, and HSV-1 in 1 patient. In 6 of these 11 patients, the HSV PCR result was positive in more than one disease episode. A significant increase of serum antibodies for HSV was seen in only 1 of 15 episodes examined. An intrathecal antibody response to HSV was not recognized in 9 episodes investigated in these 11 patients., Conclusions: We could not find evidence of VZV, CMV, or HHV-6 in the pathogenesis of RALM, although most patients were previously infected by those viruses. Herpes simplex virus 2 was detected from the CSF in most patients, and often repeatedly, which further confirms the role of this virus in RALM. The causative diagnosis was obtained only by PCR, whereas antibody analysis was not clinically useful.

Objective: To evaluate the usefulness of the broad range bacterial rDNA polymerase chain reaction (PCR) method combined with DNA sequencing in the aetiological diagnosis of intracranial or spinal infections in neurosurgical patients., Methods: In addition to conventional methods, the broad range bacterial PCR approach was applied to examine pus or tissue specimens from cerebral or spinal lesions in patients treated in a neurosurgical unit for a clinical or neuroradiological suspicion of bacterial brain abscess or spondylitis., Results: Among the 44 patients with intracranial or spinal lesions, the final diagnosis suggested bacterial disease in 25 patients, among whom the aetiological agent was identified in 17. A causative bacterial species was identified only by the rDNA PCR method in six cases, by both the PCR methodology and bacterial culture in six cases, and by bacterial culture alone in five. All samples in which a bacterial aetiology was identified only by the PCR approach were taken during antimicrobial treatment, and in three patients the method yielded the diagnosis even after >/= 12 days of parenteral treatment. One case also identified by the PCR approach alone involved a brain abscess caused by Mycoplasma hominis, which is not readily cultured by routine methods., Conclusions: In patients with brain abscesses and spinal infections, the broad range bacterial rDNA PCR approach may be the only method to provide an aetiological diagnosis when the patient is receiving antimicrobial treatment, or when the causative agent is fastidious.

Objective: To study the incidence and risk factors of stroke after coronary artery bypass grafting (CABG) or percutaneous transluminal coronary angioplasty (PTCA)., Design: During 1983-1992, coronary revascularization procedures (n = 2160) were recorded in patients aged 35-64 years as part of the population-based FINMONICA Myocardial Infarction Register. The FINMONICA Stroke Register and National Hospital Discharge Register were used to ascertain subsequent stroke events in such patients., Results: During the average follow-up of 5.83 years, 155 patients (7.2%) had a stroke. The cumulative incidence of stroke was 1.55% in the first year after revascularization and varied between 0.8 and 1.4% during subsequent years. In Cox proportional hazard models the relative risk of stroke was 3.01 (p = 0.0007) for a previous stroke, 2.61 (p = 0.0001) for diabetes mellitus, 2.15 (p = 0.007) for low income (compared with high income), 2.06 (p = 0.03) for male sex, and 1.43 (p = 0.02) for a 10-year increment in age., Conclusion: The incidence of stroke during the first year after revascularization was five times higher than among the age- and sex-matched general population. Patients with a previous stroke, diabetes mellitus, advanced age, male sex and low socioeconomic status need special attention because of increased risk of stroke after CABG or PTCA.

Objectives: To investigate prospectively the role of transoesophageal echocardiography (TEE) in selecting patients for anticoagulation in an unselected stroke population., Methods: Transthoracic echocardiography (TTE) and TEE were done in all clinically suitable hospitalised patients (n = 457) with transient ischaemic attack or ischaemic stroke in the acute phase during a two year period in Turku University Hospital. 441 patients were successfully evaluated for cardiac sources of embolism using TEE within 31 days of the event., Results: A major risk factor for a cardiac source of embolism excluding atrial fibrillation, acute myocardial infarction, and prosthetic valve was detected in 10% of patients and a minor risk factor for a cardiac source of embolism in 46%. When a major risk factor of a cardiac source of embolism was detected using TTE or TEE and no contraindications were present, the patient was given anticoagulation drugs. If a minor risk factor for a cardiac source of embolism was detected, anticoagulation treatment was started after clinical assessment, if no contraindications were present. In 62 (14%) cases, the patient was given oral anticoagulation drugs or the necessity of ongoing anticoagulation treatment was confirmed on the basis of TEE. When these anticoagulation treated patients were evaluated using logistic regression analysis, they were found to have significantly more atrial fibrillation and histories of myocardial infarctions. Moreover, the patients were mainly men. When patients in sinus rhythm and without any history of cardiac disease were analysed, 8% of patients were found to have been given anticoagulation drugs on the basis of TEE data., Conclusion: This study suggests that TEE should be used in patients with stroke even without any clinical evidence of cardiac disease when the patients are candidates for anticoagulation.

The objective was to assess uncertainty in a cost-utility analysis of adjunct entacapone treatment with levodopa among Parkinson's disease patients by probabilistic sensitivity analysis using second-order simulation methods. The cost-effectiveness of two treatment alternatives of Parkinson's disease - levodopa with or without entacapone - was compared in a cost-utility analysis employing a Markov model. Monte Carlo simulation was used to quantify the uncertainty due to sampling variation. The results strengthened confidence in the conclusions that entacapone as an adjunctive treatment to levodopa is both cost-saving and increases the quality of life of Parkinson's disease patients.

Purpose: To study the occurrence of Parkinson's disease (PD) in the relatives of parkinsonian patients (n=119), and of their matched controls (n=238).Scope: More patients reported a positive family history of PD in their first degree relatives, compared to their controls (OR 2.7, 95% CI 1.3-5.9), and the incidence of PD among those relatives was also significantly higher (OR 1.4, 95% CI 1.1-1.8).Conclusions: Familial occurrence of PD is not necessarily a sign of genetic mechanisms in the etiology of PD. Shared environment with common risk factors might be even more important.

Dysphagia is a common symptom in various neurological disorders affecting pharyngeal functions. Cricopharyngeal dysfunction is one of the major findings in these patients. The most effective treatment for restoring normal swallowing function in persistent cricopharyngeal dysfunction is cricopharyngeal myotomy, especially when mechanical obstruction or a well-localized neuromuscular dysfunction, such as a cricopharyngeal muscle spasm, is present. However, when there is a more diffuse neurological disorder present the results of surgery are more disappointing. In unclear cases, or in patients with temporary problems, no good method other than swallowing training, bougienage, and tube feeding are available. During the past decade, botulinum toxin has been found to be of therapeutic value in the treatment of a variety of neurological disorders associated with inappropriate muscular contractions such as torticollis and spasmodic dysphonia. Recently, injections of botulinum toxin in patients with cricopharyngeal muscle dysfunction have been reported to result in marked relief of dysphagia. In this article we describe our experiences with botulinum toxin injections to treat four patients suffering from deglutition problems and cricopharyngeal dysphagia of different origins. Botulinum toxin was injected into the cricopharyngeus muscle that was identified by endoscopy under general anesthesia. In this study, no major side effects were observed. Three patients obtained a significant improvement of esophageal symptoms after the first injection. The treatment had limited effect in one patient who had reflux disease and only slight cricopharyngeus dysfunction.

We have assessed the ability of xeroderma pigmentosum and normal keratinocytes grown out from skin biopsies to undergo apoptosis after irradiation with ultraviolet B. Keratinocytes have been studied from xeroderma pigmentosum complementation groups A (three biopsies), C (three biopsies), D (one biopsy), xeroderma pigmentosum variant (two biopsies), and Cockayne syndrome (one biopsy). The three xeroderma pigmentosum group A and the xeroderma pigmentosum group D samples were at least six times more sensitive than normal cells to ultraviolet B-induced apoptosis. The xeroderma pigmentosum variant samples showed intermediate susceptibility. Xeroderma pigmentosum group C samples proved heterogeneous: one showed high sensitivity to apoptosis, whereas two showed near normal susceptibility. The Cockayne syndrome sample showed the high susceptibility of xeroderma pigmentosum groups A and D only at a higher fluence. These results suggest that the relationships between repair deficiency, apoptosis, and susceptibility to skin cancer are not straightforward. Ultraviolet B-induced skin cancer is also thought to be due in part to ultraviolet B-induced impairment of immune responses. The release of the inflammatory cytokines interleukin-6 and tumor necrosis factor-alpha from cultured xeroderma pigmentosum keratinocytes tended to occur at lower fluences than in normals, but was less extensive, and was more readily inhibited at higher fluences of ultraviolet B.

This paper aims at studying the development and the risk factors for stroke prospectively during a 6-year follow-up in the Turku Elderly Study, Turku, Finland. The study cohort consisted of 1032 people aged 70 years at baseline. The stroke events (ICD-9 codes 430-434) were identified by computer linkage from the hospital discharge and death registers, and from a follow-up questionnaire. During the 6 years of follow-up, 71 patients (6.9%) suffered a stroke. Previous stroke (RR 5.82), history of transient ischemic attack (RR 4.14), diabetes mellitus (RR 2.50), poorly controlled hypertension (RR 2.42), smoking (RR 1.94) and male sex (RR 1.65) were independent risk factors for stroke. Atrial fibrillation, cardiac failure and previous myocardial infarction did not appear to be significant independent predictors of stroke in the elderly. The risk of stroke in the elderly population appears to be strongly related to the concomitant clinical disease, and this should be remembered when identifying persons at increased risk of stroke. Poorly controlled hypertension was associated with an increased risk of stroke. Thus, achieving a good control of blood pressure in elderly hypertensives receiving treatment has the potential to prevent strokes.

The objective of this study was to examine the quality of life in patients with Parkinson's disease (PD) in a community-based sample (n = 228 patients) using a Medical Outcomes Study 36-Item Short Form Health Survey (SF-36) as a measure. Associations to the variables age, age at onset, duration, clinical stage (Hoehn and Yahr), depression (Zung), and dementia (MMSE) were studied. Women scored significantly lower on five of the eight dimensions of SF-36. Depression, as measured in this study, was more common among parkinsonian women than men. Depression was the factor that was associated most significantly with the experienced quality of life, according to SF-36. With physical functioning, only the clinical stage had a more significant association than depression. To improve the quality of life in patients with PD, it is necessary to make every effort to recognize and relieve the depression of patients with PD.

We studied the environmental risk factors of Parkinson's disease (PD) in Finland, particularly those related to rural environment, in a prevalence material in 1992. The population numbered 196,864 people, including urban and rural areas. In this community-based study, we used a case-control method with personal investigation of the case subjects (n = 123) and matched control subjects (n = 246). Analyses were carried out by conditional logistic regression model. Case subjects had far fewer domestic animals at home during their lifetime, including cows, sheep, pigs, and chickens. The difference was even more obvious in those under the age of 20 years, including also cats and horses, but diminished after 20 years. The number of different animal species was smaller with case subjects as was the duration of animal contacts. Case subjects found their work physically heavier and exercised more. The mean age at onset in ever-smoking men was significantly higher than in never-smoking men. No special reason for non-smoking increased, and a physical reason decreased the risk of PD. Area of birth or living, farming and other occupations, types of drinking water, pesticide and herbicide use, head injuries, use of alcohol, education, and carbon monoxide poisonings were similar among case subjects and control subjects. In conclusion, domestic animals, or something that is connected with the animals, may have a protecting effect against PD. Alternatively, the observed negative associations of domestic animals at home and subsequent PD may only be a marker of other environmental conditions or lifestyles.

Objective: Investigation of the epidemiology of PD in southwestern Finland in 1992 (population 196,864), including urban and rural areas, with a comparison with a similar study, done in the same area in 1971, to evaluate the temporal pattern., Methods: Community-based method of patient ascertainment with personal investigation of cases., Results: The age-adjusted prevalence (to the Finnish general population in 1991) was 139 per 100,000 population in 1971 and 166 in 1992. Prevalence ratio for PD in men versus women was 1.2 (NS) in 1971 and 1.7 in 1992 (p < 0.001); in the rural versus urban populations the prevalence ratio was 0.8 (NS) in 1971 and 1.3 in 1992 (p = 0.013). The age-specific prevalence rates showed a male preponderance in all age groups in 1992 and a rural preponderance in the age groups over 60 years. In 1992, compared with 1971, the male and rural preponderance occurred in the age groups over 70 years. The age-adjusted incidence was 15.7 per 100,000 population in 1971 and 14.9 in 1992. Relative risk for PD in men versus women was 0.9 (NS) in 1971 and 1.9 (p < 0.001) in 1992, and in rural versus urban populations 1.4 (p = 0.093) in 1992., Conclusions: A very significant male and a significant rural predominance, not seen in 1971, suggests a possible environmental causative factor, perhaps more frequent in the rural environment, associated with PD. Men may be either more exposed to it or more susceptible to its effects than women.

Methods: Three patients with Borrelia burgdorferi infection and intracranial aneurysms are described., Results: All three patients had neurological symptoms. Perivascular and vasculitic lymphocytic inflammation were detected in the brain biopsy specimen of one patient. The aneurysm was located in the internal carotid arteries in two patients and in the basilar artery in one patient. The aneurysm ruptured in two patients., Conclusions: Cerebral lymphocytic vasculitis and intracranial aneurysms may be associated with B burgdorferi infection. It is suggested that inflammatory changes caused by B burgdorferi in vessel walls may be a pathogenetic mechanism for the formation of aneurysms.

Objective: This study was designed to establish whether a ratio of three units of Dysport is equivalent to one unit of Botox for the treatment of cervical dystonia., Methods: Patients with predominantly rotational cervical dystonia, and a minimum of four previous Botox treatments, were randomised to receive either the clinically indicated dose of Botox or three times that dose in Dysport units. Study botulinum toxin was administered in a double blind fashion, to one or more clinically indicated muscles, at one or more sites per muscle. Patients returned for assessment two, four, eight, and 12 weeks after treatment., Results: A total of 73 patients (Dysport, 38; Botox, 35) were entered. The Dysport group received a mean (SD) dose of 477 (131) (range 240-720) Dysport units, and the Botox group received a mean (SD) dose of 152 (45) (range 70-240) Botox units. The mean (SEM) post-treatment Tsui scores for the Dysport group (4.8 (0.3)) and the Botox group (5.0 (0.3)) were not statistically different (p=0.66). The study had 91% power to detect a clinically significant difference of two points. Both groups showed substantial improvement in Tsui score by week 2 (mean (SD); Dysport, 46 (28)%; Botox, 37 (28)%), with a peak effect at week 4 (mean (SD); Dysport, 49 (29)%; Botox, 44 (28)%). A similar response profile was seen for other assessments of efficacy. The duration of effect, assessed by time to retreatment, was also similar (mean (SD); Dysport, 83.9 (13.6) days; Botox, 80.7 (14.4) days; p=0.85). During the study 22 of 38 (58%) Dysport patients reported 39 adverse events, and 24 of 35 (69%) Botox patients reported 56 adverse events (p=0.35). A global assessment of efficacy and safety considered that 29 of 38 (76%) Dysport patients and 23 of 35 (66%) Botox patients were treatment successes (p=0.32)., Conclusion: Patients with predominantly rotational cervical dystonia treated with the clinically indicated dose of Botox or three times that dose in Dysport units show similar improvements and do not have significantly different safety profiles.

Objectives: The objective of this study was to determine the association of atrial fibrillation (AF) with stroke recurrence and mortality and with the causes of death in ischemic stroke patients aged 75 years and older., Design: A population-based study., Setting: The cities of Turku and Kuopio in Finland., Participants: The study cohort consisted of 2635 consecutive patients aged 75 years and older, with a first ischemic stroke, registered in the FINMONICA Stroke Register., Measurements: 28-day and 1-year stroke mortality, causes of death, and recurrence of stroke., Results: There were 767 stroke patients with AF (mean age 82.2) and 1868 patients without AF (mean age 81.4). Mortality was higher in the AF group both 28 days (33.9% vs 28.1%, P = .003) and 1 year after the attack (52.7% vs 43.0%, P < .001). The age- and sex-adjusted relative risk of death at 28 days was 1.25 in the AF group (95% confidence interval (CI) 1.04-1.50, P = .018), and at 1 year it was 1.41 (95% CI 1.18-1.67, P < .001). In a Cox proportional hazards model, 1-year mortality risk comparing the AF-group with non-AF group was 1.24 (95% CI 1.10-1.39, P < .001). The strongest risk factor predicting 1-year mortality was recent myocardial infarction (MI) (RR 1.90, 95% CI 1.49-2.42). Myocardial infarction was more often the underlying cause of death in the AF group during the period of 28 days, but not from 28 days up to 1 year. The 1-year recurrence rate among those alive at day 28 was 11.5% in the AF group and 9.4% in the non-AF group (P = .240)., Conclusion: Recent MI and AF are independent negative prognostic factors in older patients with stroke. Although the relative risk estimates attributable to AF are of the same magnitude in older as in middle-aged stroke patients, the much higher prevalence of AF in the older patients emphasizes its absolute impact on the mortality and recurrence after the first ischemic stroke in the age group 75 years and older. The treatment of coexisting cardiac disease also has the potential to prevent deaths and recurrent stroke events in older persons.

We describe a 64-year-old man who suffered from rapidly progressive paraparesis. At operation the cervical cord of the patient was found to be displaced anteriorly due to compression caused by an epidural synovial cyst. The cyst was located bilaterally on the dorsolateral aspect of both CVII facet joints. The rapid development of paraparesis in this patient can, thus, be explained by the enlargement of the cyst on both sides of the spinal cord. After microsurgical removal of the cystic tumor, the recovery of the patient was good. Cervical epidural cysts are extremely rare, and only anecdotal cases have been reported in the literature. Among all previously described patients the present case is unique due to the bilateral location of the cyst.

Background and Purpose: Atrial fibrillation (AF) is a risk factor for stroke. This study was undertaken to determine the influence of AF on the mortality of stroke patients and on the causes of death after a stroke event., Methods: Patients with first ischemic stroke who were .35 to 74 years old and registered in the FINMONICA stroke register during 1982 through 1992 were analyzed (n = 6912). There were 642 patients with AF (9.3%) (mean age, 67 years) and 6270 patients without AF (90.3%) (mean age, 63 years). The association between AF and stroke mortality was investigated by use of logistic regression and Cox proportional hazards models., Results: Mortality was higher in the AF group both at 28 days (19.5% versus 14.4%, P < .001) and 1 year after the attack (30.5% versus 21.8%, P < .001). After adjustment for age and sex, the odds ratio for 28-day case fatality (AF versus non-AF) was 1.27 (95% CI, 1.03 to 1.56; P = .003), and that for 1-year mortality was 1.36 (95% CI, 1.14 to 1.63; P < .001). In the proportional hazards model, AF was a significant independent risk factor for 1-year mortality (hazard ratio, 1.26; 95% CI, 1.09 to 1.46; P = .002). Cardiac causes of death were more common in the AF group at the acute stage. In the course of 1 year, there were no differences in the distributions of causes of death., Conclusions: Stroke patients with AF are at high risk of death both at the acute phase of stroke and during the subsequent year after the first acute stroke event. Mortality from cardiac diseases prevailed in the AF group during the acute phase of stroke. Careful cardiac evaluation and treatment are essential in patients with AF and stroke.

Previous studies suggest that Leber's hereditary optic neuropathy (LHON) may be a systemic disorder with manifestations in organs other than the optic nerves. To evaluate nervous system involvement 38 men and eight women with LHON were re-examined. The patients were divided into three groups according to mtDNA analysis--namely, patients with the 11778 or with the 3460 mutation and patients without these primary mutations. Fifty nine per cent of patients had neurological abnormalities but there was no significant difference between the three groups. Movement disorders were the most common finding; nine patients had constant postural tremor, one chronic motor tic disorder, and one parkinsonism with dystonia. Four patients had peripheral neuropathy with no other evident cause. Two patients had a multiple sclerosis-like syndrome; in both patients MRI showed changes in the periventricular white matter. Thoracic kyphosis occurred in seven patients, five of whom had the 3460 mutation. In one patient the 3460 mutation was associated with involvement of the brain stem. It is suggested that various movement disorders, multiple sclerosis-like illness, and deformities of the vertebral column may associate pathogenetically with LHON.

Long-term survival was examined for 218 patients with Alzheimer's disease (AD) and 115 patients with multi-infarct dementia (MID). The 14-year survival rate for AD was 2.4% versus an expected rate of 16.6%, and for MID 1.7% versus 13.3% expected. MID showed a more malignant natural course than AD. Men carried a less favourable survival prognosis than women, both in AD and MID: the relative risk of dying for women was half that for men in both diseases. In MID, advanced disability indicated a relative risk of dying over twice as high. In both diseases the risk of death was substantially higher in the event of occurrence of primitive reflexes.

Rabbits with herpes simplex virus (HSV) encephalitis induced by corneal virus challenge exhibit rotational behaviour linked with altered brain dopamine functions. The neuropathology and the distribution of the HSV-specific nucleic acids were studied, using probes for the viral trans-inducing factor alpha TIF and for the latency-associated transcript LAT-1 RNA to detect productive and latent infections, respectively. The rotational behaviour began 4 days after inoculation, and at that time the inflammatory process was observed only in the brain stem and the productive infection, revealed by in situ hybridisation, was seen in the trigeminal entry and nuclei. No HSV-specific nucleic acids or neural destruction were observed in the regions of the serotoninergic raphe or dopaminergic substantia nigra. At 8 days after inoculation, when the rotational behaviour was beginning to attenuate, the inflammatory lesions spread into the hemispheres, involving particularly the ventral parts of the limbic system including the olfactory system. In no cases were HSV-specific nucleic acids detected in the olfactory system. The inflammation in the limbic system was also detectable in animals without inflammatory lesions in the olfactory bulbs or tracts, suggesting that the infection had spread from the brain stem. The present study shows that in this model the altered neurotransmitter functions observed previously, appearing as rotational behaviour, occur without productive infection or necrosis, suggesting specific interaction of HSV with monoaminergic neurons. Additionally, the results suggest that HSV could reach the limbic system via ascending serotoninergic projections from the raphe neurons.

Increased monoamine metabolism in experimental herpes simplex virus (HSV) encephalitis is well established. Both serotonin (5-HT) and dopamine (DA) systems are affected. HSV invades the raphe nuclei after its entry into the brain stem. However, no studies have been published concerning influences of HSV on the neurotransmitters in the raphe. In the present study, concentrations of 5-HT and DA and their metabolites in the raphe nuclei and related brain regions in rabbits with fulminant HSV encephalitis have been analyzed using high-pressure liquid chromatography. Encephalitis was induced by corneal inoculation with HSV. Homovanillic acid (HVA) and dihydroxyphenyl acetic acid (DOPAC) concentrations and HVA/DA ratios were increased in the raphe nuclei suggesting increased DA turnover. The most substantial changes were bilaterally decreased 5-HT and 5-hydroxyindoleacetic acid (5-HIAA) concentrations in the raphe nuclei. The decrease in the raphe 5-HT was reflected also to the projection areas in the hemispheres, where 5-HT concentrations were lower in HSV-inoculated rabbits than in controls. The changes strongly suggest a direct influence of HSV on serotoninergic neurons. Since the ventral parts of the limbic system have rich serotoninergic afferents from the raphe, this also suggests that HSV may reach hemispheres in HSV encephalitis from the brain stem via the ascending serotoninergic system.

In this study we have examined brain concentrations of monoamine neurotransmitters and striatal and mesencephalic D-2 receptors in a chronic model of herpes simplex virus (HSV) encephalitis. The HSV-inoculated rabbits were killed two months after inoculation. Dopamine (DA), noradrenaline, serotonin and their metabolites were determined in the substantia nigra, caudate nucleus, putamen, nucleus accumbens, and olfactory tubercles using HPLC with electrochemical detection. The Bmax and Kd values of D-2 receptors were studied in the striatum and in the mesencephalon using 3H-spiroperidol as ligand. The animals showed rotational behaviour, consisting of posture tilting to the inoculated side and circling in the same direction during the first week, then slowly subsiding. Compared with controls, the concentration of homovanillic acid (HVA) was reduced in the ascending DA system on both sides. Neither in the number nor affinity of D-2 receptors were there any differences between the HSV-inoculated and control rabbits. The decreased HVA concentrations suggest that dopaminergic hypofunction can develop as a consequence of previously experienced acute HSV brain infection.

Brain dopamine receptors were determined in experimental herpes encephalitis using an animal model, where herpes simplex virus type 1 was inoculated onto the cornea of rabbits. The animals exhibit an asymmetric posture and circling to the side of inoculation, which appears to be connected to the altered dopamine transmission in the mesostriatal system. In this study striatal and mesencephalic D-1 and D-2 dopamine receptors were measured by radioligand techniques using 3H-SCH 23390 and 3H-spiroperidol as ligands. In the striatal D-1 and D-2 receptors there were no significant differences between HSV-inoculated and control rabbits. In the substantia nigra-ventral tegmental area there was a significant decrease in the D-2 receptors (Bmax) on the side contralateral to the primary virus inoculation and the direction of the rotational behaviour, without any changes in the D-1 receptors. Thus experimental herpes simplex virus infection seems to affect the mesencephalic dopamine autoreceptors, leading to unilateral activation of the mesostriatal dopamine system and rotational behaviour.

Variations in the activities of xenobiotic metabolizing liver enzymes may be involved in the pathophysiology of diseases, including Parkinson's disease. We therefore studied the activity of the debrisoquine metabolizing enzyme in 97 patients with newly diagnosed Parkinson's disease. The urine debrisoquine metabolic ratios (MR) of the patients were compared with a group of 176 healthy subjects. There were 4 poor metabolizers (4.1%) among the parkinsonians. This proportion did not differ from that found in the group of healthy subjects (51%). In contrast to earlier finding, the parkinsonian poor metabolizers (PM) had the onset of the disease later than the parkinsonian extensive metabolizers (EM). In the parkinsonian patients, it was observed that the excretion of debrisoquine and 4-OH-debrisoquine into urine correlated inversely with the actual age and age at disease onset. Our results indicate that in patients with Parkinson's disease, debrisoquine hydroxylation is comparable with healthy subjects.

The motor behaviour and brain concentrations of dopamine, noradrenaline, serotonin and their metabolites have been examined in rabbits with experimental herpes simplex virus (HSV) brain infection achieved by unilateral corneal inoculation. The animals showed altered motor behaviour, consisting of a posture tilting to the side of inoculation and circling in the same direction, that began on day 4-5 post inoculation, and was most vigorous on day 7 post inoculation. Compared with controls, the concentration of 5-hydroxyindoleacetic acid was increased in the caudate nucleus on both sides and that of serotonin was decreased in the nucleus accumbens on the side of inoculation. The circling of the animals correlated positively with the ratio of homovanillic acid concentrations between the left and right caudate nucleus, although the actual concentrations did not differ from the controls. The posture asymmetry correlated with the ratio of the left and right nucleus accumbens homovanillic acid concentrations. The results demonstrate that experimental HSV infection in the brain alters motor behaviour, in association with changes in brain dopamine and serotonin metabolism in the major ascending monoamine systems.

Parkinson's disease is a progressive disorder and no permanent cure has ever been documented. The clinical onset, which usually occurs at an age of 55-65 years, is probably preceded by a preclinical period of two or more decades. The progression rate of the disease is extremely variable in different patients; in individual patients on the other hand, the progression of motor symptoms is fairly constant. The tremor-dominant type of the disease usually has a more favourable prognosis than the hypokinetic type. Onset at an older age may be associated with a faster progression rate and the development of cognitive failure. In natural conditions, the average duration of Parkinson's disease is 10 years, although with a considerable range. The disease shortens life expectancy, which can be restored at least partially by treating patients with levodopa and other modern drugs. The highest benefit in increasing life expectancy is obtained when the treatment is initiated at a relatively early stage of the disease.

We prospectively identified patients with transient global amnesia in an urban population of 163,000. In 1982, 16 patients experienced their first transient global amnesia. The annual incidence rate was 10 per 100,000 population, and 32 per 100,000 among those aged 50 years or older. There was a substantial female predominance in the incidence of transient global amnesia.

The Finnish Twin Cohort includes all Finnish same-sexed twins born before 1958 and alive in 1967; the number of individuals alive in 1975 was 33,247. We performed a search for cases with Parkinson's disease among this cohort by linking the Twin Cohort Register with the Finnish Hospital Discharge Register and the Finnish Sickness Insurance Register. We ascertained altogether 42 cases of Parkinson's disease occurring in 41 twin including 18 monozygotic pairs, 14 dizygotic pairs, and nine pairs of undetermined zygosity. Only one dizygotic pair was concordant for Parkinson's disease; all other pairs were discordant. In 1981, the expected number of cases among the Twin Cohort, calculated according to the age- and sex-specific prevalence rates of Parkinson's disease in Finland, was 33. At the same time, the observed number of patients alive was 35. This study, further substantiating the low concordance for Parkinson's disease in monozygotic as well as in dizygotic twins and indicating that the prevalence of Parkinson's disease in twins compares with the prevalence in the general population, suggests that Parkinson's disease is an acquired disease not caused by a hereditary process.