Imaging astrocytosis with PET in Creutzfeldt-Jakob disease: case report with histopathological findings.

In a previous study, patients with suspect Creutzfeldt-Jakob's disease (CJD) have been examined with Positron Emission Tomography (PET) combining N-[11C-methyl]-L-deuterodeprenyl (DED) and [(18)F] 2- fluorodeoxyglucose (FDG) in an attempt to detect astrocytosis and neuronal dysfunction, two of the hallmarks in CJD. Increased DED uptake with pronounced hypometabolism matching the areas with high DED retention was found in the fronto-parieto-occipital areas and cerebellum of patients with confirmed CJD. However, the temporal lobes did not present such a pattern.

Seizure risk associated with neuroactive drugs: data from the WHO adverse drug reactions database.

Purpose: To explore the association between the use of neuroactive drugs and reports of epileptic seizures.

Material: Using the WHO adverse drug reactions (ADR) database, VigiBase, we surveyed reports of suspected seizures from 1968 until February 2006. Case reports of ADRs, that were classified as convulsions were collected and compared to the total number of ADRs reported.

Multitracer study with positron emission tomography in Creutzfeldt-Jakob disease.

During the period February 1997 to April 2000, 15 patients with clinical symptoms of Creutzfeldt-Jakob disease (CJD) were referred to Uppsala University PET Centre. Positron emission tomography (PET) was performed to detect characteristic signs of the disease, e.g.

The prevalence of migraine in women aged 40-74 years: a population-based study.

The objective of this study was to investigate the age-dependence of the prevalence and characteristics of migraine headache and migraine visual aura. A neurologist interviewed 728 women attending a mammography screening programme. International Headache Society (IHS) criteria were used.

Cytochrome P450 2D6 and glutathione S-transferase M1 genotypes and migraine.

Background: Migraine is thought to be a disease of the brain and trigeminovascular system. Migraine patients often claim that stress, food, and beverages trigger their attacks. Chemical substances in these foodstuffs with the property of triggering migraine attacks have not yet been characterised.

Neuroimaging study in autosomal dominant cerebellar ataxia, deafness, and narcolepsy.

Four patients affected with autosomal dominant cerebellar ataxia, deafness, and narcolepsy underwent brain CT and MRI. Radiologic findings were supratentorial atrophy (more pronounced than infratentorial atrophy), pronounced dilatation of the third ventricle, low T2 signal intensity in the basal ganglia, loss of cerebral cortex-white matter differentiation, and periventricular high-signal rims. 2-[18F]Fluoro-2-deoxy-D-glucose PET was done with one patient, without specific findings.

Characteristics and prevalence of transient visual disturbances indicative of migraine visual aura.

The objective of this study was to estimate the prevalence of and to compare the characteristics of transient visual disturbances (TVDs) of possible migraine origin in a clinical and a general population. Data were obtained in interviews from 100 consecutive female migraine patients (17-69 years) and 245 women (40-75 years) from the general population. The lifetime prevalences were 37% and 13%, respectively.

[A study of Creutzfeldt-Jakob disease during 1985-96. No indication of cases of the "mad cow disease" in Sweden].

A retrospective study of Creutzfeldt-Jakob disease (CJD) in Sweden during the period 1985-96 yielded an annual incidence of 1.18 per million. Data for incidence, age distribution (at onset and at death), and duration of illness were similar to those of other countries, with the exception of new variant CJD (nvCJD) cases in the UK, and as far as can be judged the symptomatology was also similar.

Creutzfeldt-Jakob disease in Sweden.

Objectives: To find and investigate, retrospectively, as many cases as possible of Creutzfeldt-Jakob disease (CJD) in Sweden dying during the period 1 January 1985 to 31 December 1996 and to detect any possible case(s) of new variant CJD.

Methods: The patients were found through computer search of all death certificates in Sweden on which CJD was mentioned, through information from the Swedish neuropathologists, and spontaneous reports from Swedish doctors and hospitals. Data concerning the patients were then collected from patients' case records and from brain histopathology reports.

Rhabdomyolysis in autosomal dominant progressive external ophthalmoplegia.

Multiple mtDNA deletions have been reported to be a cause of inherited recurrent myoglobinuria. We report a 57-year-old man with autosomal dominant progressive external ophthalmoplegia and multiple mtDNA deletions who developed acute rhabdomyolysis provoked by alcohol. A repeated alcohol intake resulted in a 57-fold increase in serum myoglobin.

Regional cerebral blood flow and oxygen metabolism during migraine with and without aura.

Eleven cases of migraine with and without aura were investigated with positron emission tomography (PET). Regional cerebral blood flow (rCBF), oxygen metabolism (rCMRO2) and oxygen extraction (rOER) were measured during baseline (n = 11), aura (n = 6), headache (n = 10) and after treatment with sumatriptan (n = 4). Data were analysed using an ROI-based approach from 26 different anatomically defined regions, and also an exploratory approach whereby all subjects were normalized to a stereotactic brain atlas; t-maps were constructed by depicting significant changes between states.

Anticipation of autosomal dominant progressive external ophthalmoplegia with hypogonadism.

A large Swedish family with members affected by progressive external ophthalmoplegia with hypogonadism were followed-up and reviewed. Hypogonadism included delayed sexual maturation, primary amenorrhea, early menopause, and testicular atrophy. Cataracts, cerebellar ataxia, neuropathy, hypoacusia, pes cavus, tremor, parkinsonism, depression, and mental retardation were other features observed in this family.

Monozygotic twins with MELAS-like syndrome lacking ragged red fibers and lactacidaemia.

Typical cases of MELAS present a combination of clinical and neuroradiological features, lactacidaemia, and ragged red fibers (RRFs) in striated muscle. We have observed a MELAS-like syndrome in monozygotic twins. They developed seizures typically in conjunction with physical exertion, sleep deprivation or febrile episodes.

Muscle-nerve involvement in autosomal dominant progressive external ophthalmoplegia with hypogonadism.

Sixteen members of a family with a history of autosomal dominant progressive external ophthalmoplegia (adPEO) with hypogonadism were examined. The muscular involvement commenced cranially and descended in relation to increasing disease duration. The neuromuscular signs were PEO, dysarthria, dysphonia, limb muscle weakness with wasting, absence of Achilles tendon reflexes, and distal vibration sensory loss.