Belgian Fabry study: prevalence of Fabry disease in a cohort of 1000 young patients with cerebrovascular disease.

Background And Purpose: Data on the prevalence of Fabry disease in patients with central nervous system pathology are limited and controversial. In this study, we assessed the prevalence of Fabry disease in young patients presenting with cerebrovascular disease in Belgium.

Methods: In this national, prospective, multicenter study, we screened for Fabry disease in 1000 patients presenting with ischemic stroke, transient ischemic attack, or intracranial hemorrhage; unexplained white matter lesions; or vertebrobasilar dolichoectasia.

Cerebellar cognitive affective syndrome associated with topiramate.

The cerebellar cognitive affective syndrome (CCAS) represents a spectrum of cerebellar-induced neurocognitive and affective disturbances. In this report a patient is described who developed CCAS under a treatment with standard daily dose of the anti-epileptic drug topiramate (TPM). Cognitive disturbances consisted of impaired visuo-spatial memory, concentration deficits and executive dysfunctions.

Paroxysmal neuralgic upper cervical pain attacks: the lower syndrome of cluster headache.

We present a group of seven patients with a lower syndrome (LS) of cluster headache (CH). Seventy-three newly diagnosed patients with CH were subjected to a 9 years follow-up study; 66 patients were classified as upper syndrome (US) and only seven patients (9.5%) as LS.

Some controversies about type and nature of aphasic symptomatology in Landau-Kleffner's syndrome: a case study.

The syndrome of acquired aphasia and convulsive disorders in childhood has received ample discussion since the initial description by Landau and Kleffner in 1957. Despite numerous investigations considering the linguistic and epileptic phenomena of the syndrome, the symptomatology still remains of a puzzling nature. Two aspects of the syndrome, the acquired nature of the aphasia and the type of linguistic symptomatology, have not been discussed thoroughly.

Single-dose and steady-state pharmacokinetics of sabeluzole in senile dementia of Alzheimer type patients.

The single- and repeated-dose pharmacokinetics of sabeluzole have been determined in six elderly patients with [senile] dementia of the Alzheimer type. After a single oral dose of 10 mg sabeluzole, the peak plasma concentration was attained at 1 to 4 h; it averaged 42 ng.ml-1.

Evolution of technetium-99m-HMPAO SPECT and brain mapping in a patient presenting with echolalia and palilalia.

A 78-yr-old woman presented with transient echolalia and palilalia. She had suffered from Parkinson's disease for 2 yr. Routine laboratory examination showed hypotonic hyponatremia, but was otherwise unremarkable.

Slow progressive bilateral posterior artery infarction presenting as agitated delirium, complicated with Anton's syndrome.

Three patients presented with an acute agitated delirium as the earliest sign of bilateral posterior cerebral artery infarction. All patients showed a unique slow progressive deterioration with a remarkably long interval between the first neuropsychological and subsequent visual and neurological symptoms, ranging from 3 to 30 days. Repeated CT scans demonstrated hypodensities in the posterior artery territory only after a long interval of 9-12 days, in case 3, and between 33 and 48 days in case 2.

Methanol intoxication. Parkinsonism and decreased Met-enkephalin levels due to putaminal necrosis.

Due to methanol intoxication, a 38-year-old male developed permanent parkinsonism with visual impairment. Brain computerized tomography (CT) and magnetic resonance imaging (MRI) revealed cystic resorption of the putamen. An alteration of the central noradrenergic activity and the opioid system can be assumed because of increased dopamine beta-hydroxylase (D beta H) activity and decreased methionine-enkephalin (Met-Enk) levels in the cerebrospinal fluid (CSF).

Parkinsonian syndrome after cardiac arrest: radiological and neurochemical changes.

Following a cardiac arrest, a 21-year-old man developed a Parkinson syndrome. This was due to, as shown by brain computerized tomography (CT) and magnetic resonance imaging (MRI), symmetrical infarctions of the basal ganglia, especially the globi pallidi. The levels of homovanillic acid (HVA) in the CSF were lower than normal, pointing to a possible alteration of the central dopaminergic activity.