Accessory nerve palsies.

Accessory nerve palsies may cause considerable functional disability and they unfortunately continue to occur as a complication of surgery in and, around the posterior triangle of the neck. Here the causes of accessory nerve palsies are reviewed and the symptoms and signs arising as a consequence are summarised. In addition, the various treatments and their indications are highlighted and discussed.

Spinocerebellar ataxia type 17: extension of phenotype with putaminal rim hyperintensity on magnetic resonance imaging.

We report on a 50-year-old woman who presented with an 8-year history of involuntary movements, unsteadiness, and cognitive decline. Examination revealed multidomain cognitive deficits, jerky ocular pursuit movements, hypometric saccades, gaze impersistence, dysarthria, upper limb dystonia, and widespread chorea. TATA-binding protein gene test revealed trinucleotide expansion allele sizes of 47 and 39 repeats, confirming the diagnosis of spinocerebellar ataxia type 17 (SCA-17).

Abnormal cortical sensory activation in dystonia: an fMRI study.

Despite the obvious motor manifestations of focal dystonia, it is recognised that the sensory system plays an important role in this condition. This functional magnetic resonance imaging study examines the sensory representations of individual digits both within the subregions of the primary sensory cortex (SI) and in other nonprimary sensory areas. Patients with focal dystonia and controls were scanned during vibrotactile stimulation of both the index (digit 2) and little (digit 5) fingers of their dominant hand (which was the affected hand in all the dystonic subjects).

Guidelines for the management of Parkinson's disease. The Parkinson's Disease Consensus Working Group.

Parkinson's disease is a chronic and disabling illness and there is currently wide variation in its management. This article presents the first UK-specific guidelines for the management of Parkinson's disease and it contains a treatment decision free to aid the physician in deciding when and how to treat patients. We hope this document will prove useful to all those involved in the planning and delivery of care to patients with Parkinson's disease.

Measuring the rate of progression and estimating the preclinical period of Parkinson's disease with [18F]dopa PET.

Objectives: To measure the rate of progression in striatal [18F]dopa metabolism in a large group (n=32) of patients with Parkinson's disease, to estimate the average duration of preclinical period, and to examine the influence of the PET method on the assessment of rate of progression and preclinical period.

Methods: Thirty two patients with Parkinson's disease (mean age 58 (SD 13) years, mean duration 39 (SD 33) months) were assessed with [18F]dopa PET and UPDRS scoring on two occasions a mean of 18 (SD 6) months apart. PET data were sampled with separate caudate and putamen and total striatal regions of interest, and both graphical (Ki) and ratio methods of analysis.

Functional magnetic resonance imaging of single motor events reveals human presupplementary motor area.

Conventional functional imaging paradigms use periods of repetitive task performance to generate sustained functional signal changes. We have developed a technique of imaging the small, transient signal changes that occur after single cognitive events. The technique uses echo-planar imaging at 3 T to generate functional images of the whole brain with a temporal resolution of 3 seconds.

Short- and long-term survival and function of unilateral intrastriatal dopaminergic grafts in Parkinson's disease.

Six patients with Parkinson's disease were followed for 10 to 72 months after human embryonic mesencephalic tissue from four to seven donors was grafted unilaterally into the putamen (4 patients) or putamen plus caudate (2 patients). After 8 to 12 months, positron emission tomography showed a 68% increase of 6-L-[18F]-fluorodopa uptake in the grafted putamen, no change in the grafted caudate, and minor decreases in nongrafted striatal regions. There was therapeutically valuable improvement in 4 patients, but only modest changes in the other 2, both of whom developed atypical features.

Dopaminergic function in familial Parkinson's disease: a clinical and 18F-dopa positron emission tomography study.

There is increasing evidence for familial aggregation in Parkinson's disease (PD). It is possible that some asymptomatic relatives of PD patients have subclinical nigral Lewy body pathology and their identification could help determine the true prevalence of the disease. We used 18F-dopa positron emission tomography to investigate nigrostriatal dopaminergic terminal function in asymptomatic members of 7 unrelated kindreds in which at least 2 members had parkinsonism.

Regional changes in [18F]dopa metabolism in the striatum in Parkinson's disease.

We have investigated regional changes in dopamine metabolism within the basal ganglia with clinical progression of idiopathic Parkinson's disease, using coregistration of [18F]dopa-PET and MRI images and comparing six normal subjects with 15 Parkinson's disease patients in a cross-sectional study. We have demonstrated that [18F]dopa metabolism in the dorsal putamen is reduced to almost 50% of normal both caudally and rostrally early in the disease whilst the ventral putamen is not significantly affected. With progression of symptoms there is loss of dopa metabolism from the ventral putamen, the ventrocaudal putamen in advance of the ventrorostral putamen.

An [18F]dopa-PET and clinical study of the rate of progression in Parkinson's disease.

We have studied disease progression in a group of 10 patients with recent onset idiopathic Parkinson's disease [mean age 53.7 +/- 12.6 years, mean duration 18.

Functional magnetic resonance imaging in clinical neurology.

Functional magnetic resonance imaging is a relatively new, noninvasive technique which has a maximum spatial resolution in the range of 1 mm and temporal resolution of 0.1-1 s. The technique is not quantitative but results have proved reliable and reproducible and studies are quick to perform.

Clinical and [18F] dopa PET findings in early Parkinson's disease.

Twenty seven patients with recent onset (mean symptom duration 22 (SD 14) months, Hoehn and Yahr score 1.8 (SD 0.7)) Parkinson's disease were studied with [18F]dopa PET.

The effect of entacapone (OR-611) on brain [18F]-6-L-fluorodopa metabolism: implications for levodopa therapy of Parkinson's disease.

We used PET and [18F]-6-L-fluorodopa ([18F]dopa) to measure the effect of a peripheral COMT inhibitor, entacapone, on the extracerebral metabolism and subsequent striatal uptake of [18F]dopa. Four parkinsonian patients and six age-matched normal controls were each scanned twice, once after carbidopa (150 mg) plus placebo and once after carbidopa (150 mg) plus entacapone (400 mg or 800 mg). Without entacapone premedication, by 90 minutes from injection, only 22% of the [18F] signal in plasma represented unmetabolized [18F]dopa (the balance being 3-O-methyl[18F]dopa).

PET studies of the presynaptic and postsynaptic dopaminergic system in Tourette's syndrome.

Dysfunction of the dopaminergic pathway has been postulated to underlie the symptomatology of Tourette's syndrome. Presynaptic functional integrity of dopaminergic terminals was assessed with 18F-dopa PET in 10 patients with Tourette's syndrome, three of whom were drug free and seven of whom were on neuroleptic treatment. Dopamine D2 receptor site density was measured with 11C-raclopride PET in a further group of five drug free patients with Tourette's syndrome.

Differential diagnosis of Parkinson's disease, multiple system atrophy, and Steele-Richardson-Olszewski syndrome: discriminant analysis of striatal 18F-dopa PET data.

Clinicopathological series indicate that the clinical diagnosis of Parkinson's disease is correct in only 80% of cases. Multiple system atrophy (MSA) and Steele-Richardson-Olszewski syndrome (SRO) comprise most of the misdiagnoses. By means of 18F-dopa PET the pattern of nigrostriatal dopaminergic dysfunction in 28 patients with clinically probable Parkinson's disease, 25 with MSA, and 10 patients with SRO, was assessed and compared with the pattern in 27 normal subjects.

Separating Parkinson's disease from normality. Discriminant function analysis of fluorodopa F 18 positron emission tomography data.

Objective: To explore the relationship between normal and parkinsonian fluorodopa F 18 (18F-6-L-fluorodopa [18F-dopa]) uptake data to identify clinically normal subjects who may have preclinical Parkinson's disease.

Design: A statistical comparison of striatal fluorodopa F 18 positron emission tomography scan data from patients with Parkinson's disease and normal controls.

Setting: Positron emission tomography unit within a postgraduate teaching hospital.

Total lymphoid irradiation in multiple sclerosis.

Following a report of the efficacy of total lymphoid irradiation (TLI) in the treatment of chronic progressive multiple sclerosis a further randomised double-blind placebo-controlled study was undertaken with the intention of entering 56 patients. In the event it was possible to recruit only 27 patients in a 2.5 year period.

Evidence for long-term survival and function of dopaminergic grafts in progressive Parkinson's disease.

Two patients with idiopathic Parkinson's disease (Patients 3 and 4 in our series) were followed up to 3 years after grafting of human embryonic dopamine-rich mesencephalic tissue unilaterally into the putamen. During the first postoperative year both patients showed significant amelioration of parkinsonian symptoms and increased 6-L-[18F]-fluorodopa uptake in the grafted putamen, as assessed with positron emission tomography. Three years after grafting the patients still exhibited increased fluorodopa uptake in the grafted putamen and significant clinical improvements, evidenced by a reduction of the severity of symptoms and of the time spent in the "off" phase, and by a prolongation of the effect of a single dose of L-dopa.