The varied clinical and radiological manifestations of contrast-induced encephalopathy following coronary angiography.

Contrast-induced encephalopathy (CIE) is a rare complication of imaging using ionidated contrast media. Its pathogenesis remains unknown, and its clinical presentation is variable. We present two cases of CIE following coronary angiography (CAG) that underscore the multitude of clinical manifestations and imaging findings associated with the disorder.

Case-Fatality Rate in Parkinson's Disease: A Nationwide Registry Study.

Background: Patients with Parkinson's disease (PD) may have an increased risk of mortality, but robust estimates are lacking.

Objective: To compare mortality rates nationally between patients with PD and controls.

Methods: The case-fatality rates of Finnish PD patients diagnosed in 2004-2018 (n = 23,688; 57% male, mean age at diagnosis = 71 years) and randomly selected sex- and age-matched control subjects (n = 94,752) were compared using data from national registries.

Amyotrophic Lateral Sclerosis in Southwestern and Eastern Finland.

Introduction: The incidence of amyotrophic lateral sclerosis (ALS) worldwide is approximately 1-2.6/1,000,000 and prevalence is 5-6/100,000. ALS has been suggested to be relatively common in Finland, but epidemiological information on the subject is scarce and outdated.

A severe neurodegenerative disease with Lewy bodies and a mutation in the glucocerebrosidase gene.

Several heterozygous variants of the glucocerebrosidase gene (GBA1) have been reported to increase the risk of Parkinson's disease (PD) and dementia with Lewy bodies (DLB). GBA1-associated PD has been reported to be more severe than idiopathic PD, and more deleterious variants are associated with more severe clinical phenotypes. We report a family with a heterozygous p.

Clinical spectrum and genotype-phenotype associations in Finnish patients with Wilson's disease.

Genotype-phenotype correlation data covering all ages of Wilson's disease onset in Caucasian patients are limited. We therefore analyzed genotype-phenotype correlations in a retrospective cohort of Finnish patients. Six homozygous (HoZ) and 11 compound heterozygous (CoHZ) patients were included.

Association of biallelic RFC1 expansion with early-onset Parkinson's disease.

Background And Purpose: The biallelic repeat expansion (AAGGG) in the replication factor C subunit 1 gene (RFC1) is a frequent cause of cerebellar ataxia, neuropathy and vestibular areflexia syndrome (CANVAS) as well as late-onset ataxia. The clinical spectrum of RFC1 disease has expanded since the first identification of biallelic (AAGGG) and includes now various nonclassical phenotypes. Biallelic (AAGGG) in RFC1 in patients with clinically confirmed Parkinson's disease (PD) has recently been found.

Comorbidities in patients with Unverricht-Lundborg disease (EPM1).

Background: Unverricht-Lundborg disease (EPM1) typically leads to accumulating disability. Disability may also be caused by comorbidities but there are no data available on these.

Aims Of The Study: To investigate the frequency of comorbidities in EPM1.

Biallelic expansion in RFC1 as a rare cause of Parkinson's disease.

An intronic expansion (AAGGG) in the RFC1 gene has recently been shown to cause recessively inherited cerebellar ataxia, neuropathy, and vestibular areflexia syndrome and, furthermore, a few patients with ataxia and parkinsonism have been reported. We investigated 569 Finnish patients with medicated parkinsonism for RFC1 and found biallelic (AAGGG) in three non-consanguineous patients with clinically confirmed Parkinson's disease without ataxia suggesting that RFC1-related disorders include Parkinson's disease as well.

Validation of the Finnish Version of the Unified Dyskinesia Rating Scale.

Introduction: The Unified Dyskinesia Rating Scale (UDysRS) was developed to provide a comprehensive rating tool of dyskinesia in Parkinson's disease (PD). Because dyskinesia therapy trials involve multicenter studies, having a scale that is validated in multiple non-English languages is pivotal to international efforts to treat dyskinesia. The aim of the present study was to organize and perform an independent validation of the UDysRS Finnish version.

Unverricht-Lundborg disease (EPM1) in Finland: A nationwide population-based study.

Objective: To investigate the epidemiology and prognosis of Unverricht-Lundborg disease (EPM1) in a nationwide, population-based setting.

Methods: Data from multiple registries were combined and analyzed. Clinical data were obtained from medical records.

Wilson's Disease in Finland: A Nationwide Population-Based Study.

Background: Data on the epidemiology and prognosis of Wilson's disease are scarce, and no clinical data are available from Finland.

Methods: All persons diagnosed and treated for Wilson's disease in Finnish hospitals in 1998 to 2017 were identified. Data were collected from national registries and patient charts.

Parkinsonism with a Hint of Huntington's from 29 CAG Repeats in HTT.

Huntington's disease is caused by at least 36 cytosine-adenine-guanine (CAG) repeats in an HTT gene allele, but repeat tracts in the intermediate range (27-35 repeats) also display a subtle phenotype. This patient had a slightly elongated CAG repeat tract (29 repeats), a prominent family history of Parkinson's disease (PD), and a clinical phenotype mostly consistent with PD, but early dystonia and poor levodopa response. Neurophysiological test results were more consistent with Huntington's disease (HD) than PD.

Progressive multifocal leukoencephalopathy in Finland: a cross-sectional registry study.

Objective: To investigate if progressive multifocal leucoencephalopathy (PML) incidence has increased in Finland like in the neighbouring Sweden.

Methods: National administrative registries were searched for all PML admissions aged 16 years or more in 2004-2014 on all neurological and internal medicine wards in Finland. The mortality data of the patients was extracted from the national causes of death registry.

Steroid-responsive encephalopathy with a peculiar CSF biomarker profile in an 89-year-old man.

Being treatable, steroid-responsive encephalopathy associated with autoimmune thyroiditis (SREAT), or Hashimoto's encephalopathy, should be distinguished from untreatable conditions. Our patient was a previously healthy 89-year-old man, who presented with cognitive and balance deterioration over several months. His cerebrospinal fluid (CSF) examination was positive for protein 14-3-3 but no other test suggested Creutzfeldt-Jacob disease.

The effect of hypoglycaemia on neurocognitive outcome in children and adolescents with transient or persistent congenital hyperinsulinism.

Aim: To examine the hypoglycaemic effect on neurodevelopmental outcome in patients with transient and persistent congenital hyperinsulinism (CHI) born in the 21 century.

Method: A cohort of 117 patients (66 males, 51 females) with CHI aged 5 to 16 years (mean age 8y 11mo, SD 2y 7mo) were selected from a Finnish nationwide registry to examine all the patients with similar methods. Neurodevelopment was first evaluated retrospectively.

No association of moon phase with stroke occurrence.

Stroke occurrence shows strong correlations with sleep disorders and even subtle sleep disturbances have been shown to affect ischemic stroke (IS) occurrence. Chronobiology also exerts effects, like the morning surge in IS occurrence. Lunar cycles have also been shown to affect sleep and other physiological processes, but studies on moon phases and its possible association with occurrence of stroke are rare and nonconclusive.