Epileptological emergencies in accident and emergency: a survey at St James's university hospital, Leeds.

Many patients attending an Accident and Emergency (A&E) department with seizures never come into contact with a neurological service. This survey was designed to find out how many patients with epileptological emergencies come to A&E and how they are managed. Cases were identified using the computerized A&E database. The A&E records of all adult patients attending the casualty department at St James's University Hospital with emergencies related to epilepsy between 1 April and 30 September 1998 were reviewed retrospectively. Out of a total of 36 024 adults attending A&E, 190 were related to epileptological emergencies. A problem relating to a previously recognized seizure disorder was the commonest reason for attendance. Patient management was highly variable and often suboptimal. Descriptions of seizure semiology and examination findings were frequently deficient. Up to 37.5 mg of diazepam, in up to five boluses, was given. Twenty per cent of patients with a diagnosis of status epilepticus were discharged home after diazepam treatment. Neurologists only became involved in 24.2% of cases. Epileptological emergencies only make up a small proportion of cases seen in adult A&E departments. Treatment and referral guidelines should be agreed between A&E staff and neurologists. The communication between general, specialist and acute services needs to be improved.

Postoperative pseudostatus: not everything that shakes is epilepsy.

Postoperative epileptic seizures are recognised but rare. Psychogenic seizures and pseudostatus epilepticus are relatively common, particularly in the peri-operative period. Our series of five cases of postoperative pseudostatus epilepticus demonstrates that the failure to recognise the psychogenic nature of this condition may cause anaesthetists to give inappropriate and potentially harmful treatment. Psychogenic 'status' is easy to diagnose once it has been considered. Convulsive episodes lasting longer than 90 s, closed eyes during a 'tonic-clonic' attack, retained pupillary response and resistance to eye opening are useful signs. Often there is a history of multiple admissions with 'status epilepticus' and of previous postoperative 'status'.

Massive nerve root enlargement in chronic inflammatory demyelinating polyneuropathy.

OBJECTIVE: To report three patients with chronic inflammatory demyelinating polyneuropathy (CIDP) presenting with symptoms suggestive of cervical (one patient) and lumbar root disease. METHODS: Nerve conduction studies, EMG, and nerve biopsy were carried out, having found the nerve roots to be very enlarged on MRI, CT myelography, and at surgery. RESULTS: Clinically, peripheral nerve thickening was slight or absent. Subsequently one patient developed facial nerve hypertrophy. This was mistaken for an inner ear tumour and biopsied, with consequent facial palsy. Neurophysiological tests suggested a demyelinating polyneuropathy. Sural nerve biopsy showed in all cases some loss of myelinated fibres, inflammatory cell infiltration, and a few onion bulbs. Hypertrophic changes were much more prominent on posterior nerve root biopsy in one patient: many fibres were surrounded by several layers of Schwann cell cytoplasm. There was an excellent response to steroids in two patients but not in the third (most advanced) patient, who has benefited only marginally from intravenous immunoglobulin therapy. CONCLUSIONS: MRI of the cauda equina may be a useful adjunct in the diagnosis of CIDP.

Inter-relation between "classic" motor neuron disease and frontotemporal dementia: neuropsychological and single photon emission computed tomography study.

The purpose of this study was to examine the possible association between "classic" motor neuron disease (cMND) and frontotemporal dementia (FTD), using neuropsychological evaluation and single photon emission computed tomography (SPECT). Psychological tests assessing language, perceptuospatial, memory, and "frontal lobe" functions were given to patients with cMND and test scores were compared with those of normal control subjects. 99mTc-HMPAO SPECT was performed on patients with cMND, FTD and motor neuron disease (FTD/MND), FTD alone, and normal control subjects. Regional cerebral blood flow indices (rCBFi) were determined in 36 cortical regions, and differences between grouped rCBFi data were investigated by canonical discriminant analysis. There were significant group differences in the scores of picture sequencing and token tests in patients with cMND compared with normal controls. Regional CBFi data showed frontal and anterior temporal reductions in patients with cMND compared with normal controls. A similar pattern of SPECT abnormality was seen in patients with FTD/MND and FTD alone, but to a more pronounced degree than in patients with cMND. Neuropsychological and SPECT findings in cMND, FTD/MND, and FTD showed a common pattern of cerebral involvement, most pronounced in the second two conditions. It is suggested that cMND, FTD/MND, and FTD represent a clinical range of a pathological continuum.

Favourable outcome in non-traumatic anterior interosseous nerve lesions.

Six patients with non-traumatic anterior interosseous nerve palsy are described. In five patients the onset was acute with upper arm pain, which peaked within 4 weeks and thereafter declined. Virtually complete spontaneous recovery occurred in all patients between 9 and 24 months. It is concluded that non-traumatic anterior interosseous nerve lesions are most likely to reflect a circumscribed form of brachial neuritis and that surgical decompression should be deferred for at least a year or indefinitely if recovery is proceeding.

Progressive language disorder due to lobar atrophy.

Sixteen patients with progressive language disorder have been studied longitudinally. Anomia was a prominent presenting characteristic and mutism ultimately occurred. Patients, however, were clinically heterogeneous. Some exhibited nonfluent, agrammatic features, whereas others demonstrated a fluent aphasia, with profound loss of word meaning. Although language disorder remained the sole symptom in a minority of patients, in others an associative agnosia or personality and behavioral changes, or both, emerged. Findings on computed tomography and single photon emission tomography mirrored the areas of dysfunction suggested by the neuropsychological profiles and demonstrated abnormalities restricted to the left hemisphere or involving bilateral frontotemporal cortices. Brains of 3 patients, with distinctive clinical pictures, have been examined at autopsy. Each revealed a focal distribution of atrophy, gliosis and spongiform change, and an absence of senile plaques and neurofibrillary tangles. There was clinical and pathological overlap with frontal lobe dementia. We argue that progressive language disorder is clinically heterogeneous and forms part of a spectrum of clinical presentations of non-Alzheimer lobar atrophy.

Frontal lobe dementia and motor neuron disease.

Four patients are described, in whom a profound and rapidly progressive dementia occurred in association with clinical features of motor neuron disease. The pattern of dementia indicated impaired frontal lobe function, confirmed by reduced tracer uptake in the frontal lobes on single photon emission computed tomography (SPECT). Pathological examination of the brains of two patients revealed frontal-lobe atrophy, with mild gliosis and spongiform change. The spinal cord changes were consistent with motor neuron disease. The clinical picture and pathological findings resembled those of dementia of frontal-lobe type and were distinct from those of Alzheimer's disease. The findings have implications for the understanding of the spectrum of non-Alzheimer forms of primary degenerative dementia.