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J Kovanen

Publications and source records attributed to J Kovanen.

At least 19 recordsLinked to original sources
PubMed2003

Economic burden and quality of life impairment increase with severity of PD.

PURPOSE: To define the interrelationship between cost-of-illness, quality of life (QoL) and Parkinson's disease (PD) severity in a common patient management setting in Finland.Scope. Two hundred and sixty consecutive outpatients with idiopathic PD participated. UPDRS, motor fluctuations, QoL, and the use of health care resources were measured. Direct and indirect costs were calculated. CONCLUSIONS: There is a strong relationship between QoL or cost-of-illness on the one hand, and severity of PD on the other. Treatment policies capable of reducing or delaying motor fluctuations would be expected to increase QoL and reduce some of the economic burden of PD.

Aged↗
PubMed1993

Clinical characteristics of familial and sporadic Creutzfeldt-Jakob disease in Finland.

The clinical features of 44 Finnish patients with Creutzfeldt-Jakob disease (CJD) were analyzed with special emphasis on the differences between the sporadic and familial forms. The 32 sporadic patients comprised all neuropathologically verified cases of CJD in 1974-89 in Finland. The 12 familial patients were members of the same pedigree where CJD has been linked with a mutation at codon 178 of the PRNP gene. The median age at the onset of the disease was 62.5 years and median duration 4.5 months in sporadic patients, and 49 years and 20.5 months in familial CJD, respectively. 90 percent of both sporadic and familial patients had myoclonus. Typical periodic EEG change was seen in 72% of sporadic patients, whereas the familial patients showed only a progressive slowing of EEG.

Adult↗
PubMed1992

Creutzfeldt-Jakob disease cosegregates with the codon 178Asn PRNP mutation in families of European origin.

We recently discovered an amino acid-altering heterozygous mutation in codon 178 of the PRNP amyloid precursor gene in patients with familial Creutzfeldt-Jakob disease. This mutation is now shown to be associated with the occurrence of disease in 7 unrelated families of Western European origin, among which a total of 65 members are known to have died from Creutzfeldt-Jakob disease. The mutation was detected in each of 17 tested patients, including at least 1 affected member of each family, and in 16 of 36 of their first-degree relatives, but not in affected families with other mutations, patients with the nonfamilial form of the disease, or 83 healthy control individuals. Linkage analysis in two informative families yielded a lod score of 5.30, which, because no recombinants were found, strongly suggests that codon 178Asn is the actual disease mutation.

Adult↗
PubMed1992

Phenotypic characteristics of familial Creutzfeldt-Jakob disease associated with the codon 178Asn PRNP mutation.

A group of 43 patients from seven families affected by Creutzfeldt-Jakob disease (CJD) with the codon 178Asn mutation of the PRNP amyloid precursor gene is compared to a group of 211 patients with the sporadic form of the disease. As a group, the patients with the codon 178Asn mutation had an earlier age at onset of illness (almost always presenting as an insidious loss of memory), a longer duration of illness, and an absence of periodic electroencephalographic activity. Transmission of disease to primates was accomplished using brain tissue homogenates from 6 of 10 patients, resulting in significantly shorter incubation periods than those due to sporadic CJD inocula. These findings are interpreted and discussed in terms of possible differences in the temporospatial evolution of damage to the brain, and of accelerated induction of polymerized amyloid protein by its mutationally altered template precursor.

Age Factors↗
PubMed1991

Familial Creutzfeldt-Jakob disease in Finland: epidemiological, clinical, pathological and molecular genetic studies.

In 1974-1984 30 patients died with a diagnosis of Creutzfeldt-Jakob disease (CJD) in Finland (annual mortality rate of CJD 0.9 per million population for the years 1979-1984). Six of these patients (20%) were familial, all belonging to the same kindred. The pedigree now includes 15 affected members in four generations, and the occurrence of disease is consistent with an autosomal dominant mode of inheritance. The clinical features of CJD in this family are in most respects typical of the familial disease described elsewhere. However, the mean age at onset is 47, periodic EEG activity has not been observed, and the mean duration of illness of 27.5 months is longer than usual for either familial or sporadic CJD. Neuropathological examination of brain biopsy and autopsy specimens revealed spongiform change without amyloid plaques, and brain tissue from one patient transmitted disease to a capuchin monkey. In an analysis of the histocompatibility antigens of the family, CJD was not linked with a single haplotype, but at least 12 out of 13 CJD patients shared the HLA antigen A28. Molecular genetic studies disclosed a new G-to-A mutation in codon 178 of the PRNP gene (resulting in a substitution of asparagine for aspartic acid) in the DNA of eight family members with CJD but not in any of ten currently healthy first degree relatives of the patients, or 86 controls. The codon 178 mutation thus seems to co-segregate with CJD in this family. Linkage analysis gave a LOD score value of 3.6.

Adult↗
PubMed1989

Lyme borreliosis in Finland in 1986-1988.

In 1986-1988 there were 123 patients with positive serology for Lyme borreliosis out of 4000 sera referred to the Department of Bacteriology and Immunology, University of Helsinki. Of the 63 patients with positive serology in 1986-1987 20 showed a predominant involvement of the nervous system, 18 complained of joint symptoms and 11 patients merely showed a skin involvement including 8 patients with erythema chronicum migrans (ECM) and 3 patients with acrodermatitis chronica atrophicans (ACA). Two of the patients had unspecific general symptoms and in 5 patients the type of involvement remained unknown. The serology was considered to be falsely positive in 2 patients with tuberculous meningitis, in one with syphilis and in another with recurrent fever.

Adult↗
PubMed1988

Microangiopathy with encephalopathy, hearing loss and retinal arteriolar occlusions: two new cases.

Two young women developed encephalopathy, hearing loss and retinal arteriolar occlusions. Their behaviour became immature and cognitive functions were severely impaired. One of the patients underwent brain biopsy, which showed several microinfarcts in both white and grey matter and microangiopathic changes, with thickened arteriolar segments staining intensely for laminin and fibronectin. These findings support the concept of a new type of microangiopathy involving the brain, inner ear and retina.

Adult↗
PubMed1988

Descriptive epidemiology of Creutzfeldt-Jakob disease in Finland.

In 1974-84 30 patients died with a diagnosis of Creutzfeldt-Jakob disease (CJD) in Finland. Sixteen of the patients were pathologically confirmed and 14 were probable cases; 6 were familial (20%). One further familial patient was alive at the end of 1984. The median age of the 30 patients at death was 59.5 years (range 46-73 years). The familial patients were significantly younger than sporadic cases (median and range 49.5 and 46-57 versus 61.5 and 51-73; P less than 0.01). Only 5 of the 24 sporadic patients were men (male to female ratio 1:3.8). The annual number of new cases as well as the death rate increased in the late 1970's (annual death rate 0.57 per million in 1974-84 and 0.91 in 1979-84). This probably reflects the growing awareness of CJD among neurologists, rather than a real increase of the incidence and death rate. Annual age-specific death rate per 1 million population in 1974-84 reached a peak value of 2.57 in the age group of 60-64 years. In sporadic CJD the age-specific death rate of women was higher than that of men in all age groups. A chronic medical condition, precedent or concomitant with CJD, was seen in 15 patients.

Aged↗
PubMed1987

Suspected dementia: evaluation of 323 consecutive referrals.

A neurological outpatient department studied 323 consecutive referrals for suspected dementia: 135 (41.8%) were not demented. Of the patients 12.1% had diffuse cognitive disorder; 10.2% circumscribed memory disorder; 0.9% other circumscribed cognitive disorder, 14.2% psychiatric disorder, and 4.3% were judged to be normal. Of the nondemented, 44.1% had a potentially treatable cause for their cognitive symptoms; in 27.4% it was depression. The total of demented patients was 188 (58.2%): 38.8% had primary degenerative dementia; 37.2% vascular dementia including combined degenerative and vascular dementia; and 23.4% had a specific cause. Patients with specific cause were significantly younger than those with other causes of dementia. A potentially treatable cause was found in 10.7% of all demented patients, the most common being metabolic disorders, meningioma, hydrocephalus, subdural haematoma, and depressive pseudodementia.

Adult↗
PubMed1986

Bannwarth's syndrome and Lyme disease in Finland.

Three patients with lymphocytic meningoradiculitis were admitted in 1983-84 to the Department of Neurology, University Central Hospital of Helsinki. Two patients had classical Bannwarth's meningoradiculitis with facial diplegia, and a third patient showed a migrating arthralgia and cardiac involvement compatible with a diagnosis of Lyme disease. Cerebrospinal fluid (CSF) showed initially in all patients a lymphocytic pleocytosis and increased protein with signs of intrathecal IgG synthesis.

Adult↗
PubMed1984

Status epilepticus and alcohol abuse: an analysis of 82 status epilepticus admissions.

In 1979-80, 82 cases of grand mal status epilepticus (71 patients, 39 male and 32 female) were admitted to the Casualty Department of Meilahti University Hospital in Helsinki, Finland. The cause of the underlying epilepsy was symptomatic in 43 cases (52.4%) and idiopathic in 19 cases (23.2%). In 6 cases (7.3%), there was a history of alcohol withdrawal seizures, and in 14 cases (17.1%) there was no earlier history of convulsions. Status epilepticus was associated with an acute or progressive cerebral disorder in 14 episodes. These comprised 6 bouts of status with brain tumour, 4 with acute stroke and 4 with brain injury. Alcohol abuse preceded the status in 29 episodes (35.4%), 23 of which occurred in men (53.5% of the male cases). Excessive use of alcohol was the only obvious precipitating factor for status in 16 cases, and in 6 cases the status presented as a prolonged alcohol withdrawal seizure. A change or irregularity of anticonvulsive drug therapy could be documented in 14 cases and an acute infection outside the central nervous system in 7 cases. Intravenous diazepam, used as the only therapy for status epilepticus, was effective in 58 of 78 episodes. In 7 cases of prolonged status, a thiopental sodium anaesthesia proved effective. The total mortality was 4.2%, including 2 deaths from concomitant extracerebral disorders and one late death from brain metastasis.

Adolescent↗
PubMed1983

Acute myopathy associated with gasoline sniffing.

Acute myopathy and myoglobinuria with markedly elevated creatine kinase (CK) activity developed in an 18-year-old boy on two occasions after gasoline sniffing. No signs of CNS involvement were seen, and the symptoms receded in a few days. The amount of lead in blood and urine exceeded the reference value for subjects with occupational exposure. The component of gasoline that was responsible for the myopathy remains unknown. Acute myoglobinuria appears to be a rare complication of gasoline sniffing.

Acute Disease↗