Autoantibody to nerve tissue in a patient with a peripheral neuropathy and an IgG paraprotein.

The antibody activity of a benign IgG lambda paraprotein to nerve tissue in a case of peripheral neuropathy has been investigated using immunohistochemical methods on tyrpsin-treated, formalin-fixed, paraffin-embedded tissue. IgG lambda was found in th sural nerve biopsy of the patient. Specific binding of the purified IgG lambda paraprotein and its isolated F(ab')2 fragment to homologous nerve and brain tissue was demonstrated. Similar activity was not demonstrable on fresh frozen cryostat sections. The results suggest that tests for autoantibodies to nerve tissue in neuropathological disorders should not be confined to fresh frozen tissue substrates.

Severe ocular side effects of perhexilene maleate: case report.

We report a case of perhexilene maleate (PEXID) toxicity in which the presenting feature was loss of vision secondary to chronic papilloedema. Vortex keratopathy similar to that seen in amiodarone keratopathy was present, and corneal and conjunctival biopsy findings are presented. To our knowledge this is the first case report of a keratopathy occurring in perhexilene toxicity. After withdrawal of the drug the papilloedema and keratopathy subsided, but some visual deficit remains. The properties of perhexilene maleate and other amphiphilic drugs are described, and the possible aetiology of vortex keratopathy is discussed.

Dietary modification of Parkinson's disease.

To investigate the efficacy of a low-protein diet suitable for long-term use, and to observe the effect in a double-blind manner of re-introducing large neutral amino acids or an inactive placebo amino acid, eight patients with 'on-off' phenomenon were given a low-protein diet suitable for long-term use, and then the diet was supplemented with large neutral amino acids (LNAA) or placebo in a double-blind crossover trial. Five patients had improved Parkinsonian disability on a low-protein diet compared to normal diet. On supplementing the diet with LNAA or placebo, three subjects' Parkinson's disease was significantly better on placebo and there was a trend towards improvement on placebo noticed subjectively by seven patients; however, there was no overall significant difference in disability measured by mobility diaries. Thus, we have been unable to show that the improvement on a low-protein diet is due to a reduction in LNAA, rather than a placebo effect, possibly due to the paucity of subjects; however, the patients' identification of the LNAA supplement suggests that it is a genuine effect.

Spinal cord compression due to metastatic spread from a primary carcinoid tumour of the thymus.

We reported a patient with spinal cord compression due to the metastatic spread of a primary carcinoid tumour of the thymus to bone. Prompt surgical decompression with local radiotherapy led to good functional recovery and survival at 2 years without further recurrence.

Status epilepticus: management and outcome of 107 episodes.

Morbidity and mortality from status epilepticus might be reduced by attention to recommended management protocols. We studied our experience of 107 episodes of status epilepticus in 43 patients over a 5-year period. Overall mortality was 2% and permanent sequelae developed in 11 cases (10%). Although hospital admission was rapid, treatment could be initiated more quickly in the community (P < 0.0001). However treatment given before admission did not significantly reduce the duration of status (median difference 38 minutes, 95% C.I., 24 to 55 minutes). Diazepam was the first line treatment in 98 episodes, chlormethiazole and phenytoin were used in 27 and 18 episodes, respectively. Paraldehyde was used in 12 episodes. Midazolam was used in the intensive care setting in two cases. Clonazepam, lorazepam, lignocaine and phenobarbitone were not used at all. There was a marked failure to adhere to the recommended management protocols.

Impact of lamotrigine on patients with refractory epilepsy: the Leicester experience.

We prospectively monitored our experience of lamotrigine as add-on therapy in 45 patients with refractory epilepsy. Nine patients (20%) withdrew from treatment due to adverse drug reactions and five patients (11%) withdrew because of a deterioration in seizure frequency. A further 17 patients (38%) showed little (< 25%) reduction in seizure number, eight patients (18%) showed a 25-50% reduction whilst 15 patients (33%) had a 50% or greater reduction in seizure number (P = 0.002). Lamotrigine was of greater benefit in patients with tonic-clonic seizures (seven of fourteen [50%] showed a > 50% seizure reduction; P = 0.01) than in patients with complex partial seizures (seven of 22 [32%] showed a > 50% seizure reduction; P = 0.02). Despite a high withdrawal rate due to ADRs, lamotrigine proved of significant benefit to one-third of our study group.

Vigabatrin: a three year clinical analysis.

The effect of introducing vigabatrin into the management of 57 patients with severe epilepsy is reported. Overall it reduced fit frequency by 50% or more in 36% of our patients. We found it to be more effective than previously reported for primary tonic-clonic seizures, but less so for complex partial seizures. Fewer of our patients withdrew from treatment due to adverse drug reactions than in earlier studies.

Pregnancy, epilepsy, management and outcome: a 10-year perspective.

We analysed the management and outcome of 348 pregnancies in 207 women with epilepsy from 1977 to 1981 and 1987 to 1991. Outcome was successful in 88%. There was a marked change in anticonvulsant prescribing away from phenobarbitone and phenytoin towards carbamazepine and sodium valproate but a large number of patients (24%) were treated with two or more anticonvulsants in the second cohort despite a move towards monotherapy. A significant reduction in congenital malformation (4.8%), spontaneous abortion (6.9%) or low birth weight babies (9.4%) was not seen. Until experience is gained with the newer agents, monotherapy remains of major importance in the management of the pregnant woman with epilepsy.

Audit of the management of patients with refractory epilepsy.

Achieving rational management for patients with refractory epilepsy can be difficult. We audited our management of 55 patients with refractory epilepsy by comparing two 3-month periods each 9 months apart. Despite attempts to rationalize antiepileptic drug therapy, a smaller proportion of patients were managed with monotherapy by the second analysis (16% vs 22%); however fewer patients were taking three or more AEDs (28% vs 43%). A substantial impact on seizure frequency was made in only six patients whilst 22 patients had a significant deterioration in seizure number (P = 0.005). Monitoring of AED levels was of little value with only six clinical decisions based on 305 AED levels performed at a cost of 2525 pounds. The newer AEDs accounted for a disproportionate expenditure (65% of the total AED cost in the second 3-month period) considering their lack of therapeutic impact. We confirm the need for audit in this patient group to ensure that pre-defined targets are met.

A comparison of flight of colours with visually evoked responses in patients with multiple sclerosis.

The flight of colours test was performed in a series of patients with multiple sclerosis. Visually evoked responses were then measured in the same patients. The two tests were found to be comparable in their ability to detect disturbance in the visual system.

Neurosyphilis in the Leicester area.

Twenty patients with neurosyphilis are reviewed. They were gathered over a 15-year period during which the overall incidence was 0.18 per 100,000, though fewer cases appeared in the latter half of the study. The commonest presentation was with mental symptoms and classical forms of neurosyphilis were rarely seen. No patient presented with epilepsy. There is a case for the more selective use of serological testing.

Late deterioration after decompression illness affecting the spinal cord.

A former amateur diver presented with a progressive paraparesis. Thirteen years previously he had developed acute spinal cord dysfunction immediately after dry hyperbaric exposure. He had completely recovered motor function in the intervening period. No alternative reason for the later decline emerged from detailed investigation.

The place of peripheral neurectomy in the management of trigeminal neuralgia.

One hundred and forty-six patients with trigeminal neuralgia were studied. Of 49 patients ultimately maintained pain-free by non-medical means, 26 underwent peripheral neurectomy. Twenty of these achieved excellent pain control in the longer term and 5 of the remaining 6 became more responsive to carbamazepine after operation. Seven patients required repeat neurectomies. Peripheral neurectomy is a useful and simple method of pain control in trigeminal neuralgia.

Treatment of paroxysmal disorders in multiple sclerosis with carbamazepine (Tegretol).

During an eight year period, 32 patients with definite or suspected multiple sclerosis (MS) were seen with paroxysmal neurological disturbances, which included tonic seizures, paroxysmal dysarthria, paraesthesiae and pain in the limbs, as well as trigeminal neuralgia. In 21 of these patients the paroxysmal disorders were treated with carbamazepine, and in six the effect was compared with placebo. In the majority carbamazepine was effective in controlling the paroxysmal symptoms. Side-effects were troublesome in a few patients, but they could usually tolerate small doses, which still gave relief. Although the patho-physiological basis for these paroxysmal disorders remains unexplained, their response to carbamazepine suggests a common mechanism.