Disability profile of MS did not change over 10 years in a population-based prevalence cohort.

Objective: To assess whether the level of multiple sclerosis (MS) -related disability in the Olmsted County population has changed over a decade, and to evaluate how the rate of initial progression to moderate disability impacts further disability.

Methods: The Minimal Record of Disability (MRD) measured impairment, disability, and handicap for the 2000 (n = 201) prevalence cohort. The authors compared these results with the 1991 (n = 162) cohort; 115 patients were in both cohorts.

Change in MS-related disability in a population-based cohort: a 10-year follow-up study.

Objective: S: To study the change in disability over 10 years in individual patients constituting the 1991 Olmsted County, MN, multiple sclerosis (MS) prevalence cohort.

Methods: The authors reassessed this 1991 cohort in 2001. The authors determined the Expanded Disability Status Scale scores (EDSS) for each patient still alive, and within the year prior to death for those who died.

Incidence and prevalence of multiple sclerosis in Olmsted County, Minnesota, 1985-2000.

Background: Epidemiologic data for multiple sclerosis (MS) in Olmsted County, MN, have been recorded for almost 100 years and have indicated that the increasing prevalence rate was likely due in part to an increasing incidence rate.

Methods: All cases of MS diagnosed from 1985 to 2000 were identified using the centralized diagnostic index at the Mayo Clinic and the Rochester Epidemiology Program Project, a shared database of all medical practitioners in the county. Patients were required to have established residency at least 1 year prior to diagnosis of MS.

Patient and hospital delays in acute ischaemic stroke in a Dublin teaching hospital.

A limiting factor for thrombolysis in ischaemic stroke is delayed presentation to hospital. Prolonged A&E stay and delayed rehabilitation affects care. We evaluated the delay in presentation, A&E stay and rehabilitation delivery in 117 consecutive stroke patients.

Pseudosubarachnoid haemorrhage in subdural haematoma.

Two patients with large bilateral subdural haematomas with patterns of non-enhanced brain computed tomography (CT) falsely suggesting coexistent subarachnoid haemorrhage are presented. The CT images showed marked effacement of the basal cisterns with hyperdense signal along the tentorium, sylvian fissure, and the perimesencephalic cisterns. In both cases, the suspicion of subarachnoid haemorrhage led to the performance of angiographic studies to rule out vascular lesions.

Anti-neuronal nuclear autoantibody type 2: paraneoplastic accompaniments.

We identified the IgG autoantibody ANNA-2 ("anti-Ri") in 34 patients in a 12-year period by immunofluorescence screening of sera from approximately 75000 patients with subacute neurological disorders that were suspected to be paraneoplastic. Detailed clinical information was available for 28 patients (10 men, 18 women). Cancer was diagnosed in 24 patients (86%); 21 had histologically proven carcinoma (10 lung, 9 breast, 1 cervical, 1 bladder), and 3 had an intrathoracic imaging abnormality.

OKT3 neurotoxicity presenting as akinetic mutism.

Background: Muromonab-CD3 (OKT3), a mouse monoclonal antibody directed against human T lymphocytes, is a potent immunosuppressive agent used to reverse and more recently to prevent allograft rejection, mostly in cardiac transplant recipients. Neurotoxicity from OKT3 usually manifests itself as a transient aseptic meningitis and remains uncommon.

Methods: The authors describe a dramatic neurologic syndrome after orthotopic heart transplant characterized by akinetic mutism, blepharospasm, anomic aphasia, and delirium.

A double-blind randomised placebo-controlled evaluation of three doses of botulinum toxin type A (Dysport) in the treatment of spastic equinovarus deformity after stroke.

Background/objectives: Calf muscle hypertonicity following stroke may impair walking rehabilitation. The aim of this study was to assess botulinum toxin (Dysport) in post-stroke calf spasticity.

Methods: A prospective, multicentre, double-blind, placebo-controlled, dose-ranging study was performed to evaluate dysport at 500, 1,000 or 1,500 units in 234 stroke patients.

The "spray can" sign: validation of a clinical observation in chronic inflammatory demyelinating polyneuropathy.

Background: The presentation of chronic inflammatory neuropathies is variable. The decision regarding when to intervene with treatment is ideally determined by identifying early markers of loss of function.

Objective: To test the hypothesis that an observation of functional impairment, defined by a patient with demyelinating neuropathy, can be used as a reproducible and reliable measure of improvement with intravenous immune globulin.

Ease of administration of the cognitive adaptive test/clinical linguistic and auditory milestone scale (CAT/CLAMS) during pediatric well-child visits.

Barriers to early identification of children with developmental delays include time constraints during well-child visits and lack of easily administered, quantitative measures that can be used by pediatricians. This study assesses the ease of administration of the Cognitive Adaptive Test/Clinical Linguistic and Auditory Milestone Scale (CAT/CLAMS) during well-child visits from 2 to 36 months of age. During a single visit 177 children were assessed by either a developmental pediatrician (n = 121) or a third-year pediatric resident (n = 56).

Evaluation of stroke management in an Irish university teaching hospital: the Royal College of Physicians stroke audit package.

Background: There are few data regarding the standard of stroke care in Ireland.

Aim: To investigate the level of documentation for 13 key areas of stroke management.

Methods: Using a validated stroke audit package, this study reviewed the medical records of 100 consecutive patients hospitalised with acute stroke.

Reversible myelopathy in a 34-year-old man with vitamin B12 deficiency.

Vitamin B12 deficiency is common, with most patients lacking classic features of advanced severe deficiency. Early diagnosis and treatment prevent severe anemia and irreversible damage to the nervous system. We describe a 34-year-old man with pernicious anemia who presented with clinical and radiologic features of early myelopathy and borderline low serum levels of vitamin B12.

A randomized, double-blind, placebo-controlled study of the efficacy and safety of botulinum toxin type A in upper limb spasticity in patients with stroke.

Objective: To study the efficacy and safety of botulinum toxin type A (BtxA) in the treatment of upper limb muscle spasticity, caused by stroke.

Methods: This was a randomized, controlled trial. Patients received either placebo injections or a total of 1000 IU of BtxA (Dysport) into five muscles of the affected arm.

Rapid weaning from mechanical ventilator in acute cervical cord multiple sclerosis lesion after steroids.

We report a patient with multiple sclerosis (MS), who developed neuromuscular respiratory failure requiring ventilation because of a cervical cord relapse. Serial pulmonary function tests documented improvement after steroid treatment. Cervical cord or brainstem relapses should be suspected in MS patients with respiratory failure.

The oral cavity in Crohn's disease.

We assessed the utility of expert oral examination as a part of the diagnostic evaluation of patients with suspected Crohn's disease. Of 45 patients with newly diagnosed CD, 25 had been examined by a dentist. Twelve (48%) of these had oral CD lesions.

Traumatic bilateral carotid dissection with concomitant cerebral infarction.

A 40-year-old man presented with a major nondominant hemisphere stroke syndrome after a road traffic accident. Cranial computed tomography scan revealed an extensive right hemisphere infarction involving the entire anterior and middle cerebral artery territories. Duplex Doppler ultrasound and cerebral angiography revealed bilateral internal carotid artery dissection with evidence of underlying fibromuscular dysplasia.

Rapid-onset dystonia-parkinsonism: a clinical and genetic analysis of a new kindred.

Background: Rapid-onset dystonia-parkinsonism (RDP) is an autosomal dominant disorder linked to chromosome 19q13 that is characterized by sudden onset of primarily bulbar and upper limb dystonia with parkinsonism.

Methods: The authors evaluated 12 individuals from three generations of an Irish family and obtained detailed medical records on a deceased member. The authors describe the clinical, psychiatric, and genetic features of the affected individuals.

Polysialylated neural cell adhesion molecule expression in the dentate gyrus of the human hippocampal formation from infancy to old age.

Modulation of neural cell adhesion molecule polysialylation (NCAM PSA) state has been proposed to underlie morphofunctional change associated with consolidation of memory in the rodent, and its age-dependent decline to be related to impaired cognitive function. To establish whether this may be a human correlate of cognitive decline, we determined the age-dependent expression of PSA in the human hippocampal dentate gyrus using postmortem tissue derived from individuals who exhibited no obvious neuropathology. As in the rodent, PSA immunoreactivity in the 5-month human infant was associated mainly with a population of granule-like cells and their mossy fibre axons.